Neuro Flashcards
(639 cards)
1
Q
Where in bones does Ewing Sarcoma arise and which bones are most often affected?
A
- Arise in the diaphysis of long tubular bones; in the medullary cavity and then invades cortex
- Especially the femur and flat bones of the pelvis
2
Q
What is the etiology of LGI1 encephalitis?
A
Failure to identify and treat these patients early may result in permanent brain injury with longterm cognitive deficits, especially short-term memory problems.
Up to 1/3 of patients may relapse after treatment
3
Q
What are the characteristic of normal pressure hydrocephalus and who is it seen in?
A
- Symmetric type usually occuring in adults >60 - Develops slowly over time; drainage of CSF is blocked gradually - Enlarged ventricles still exert pressure on brain and can become symptomatic w/ pt’s exhibiting dementia-like manifestatons - Dementia may be similar to Alzheimers and gait abnormalities may suggest Parkinson disease - NPH is often misdiagnoses as one of these diseases
4
Q
What effects due the AchE have?
A
bradycardia, bronchoconstriction, salivation, nausea, vomiting
5
Q
What is the MOA of the gout drug, Pegloticase?
How is it administered?
A
- Recombinant mammalian uricase, covalently attached to methoxy polyethylene glycol –> prolongs ciruclating half-life and diminished immunogenic response
- Converts uric acid to the far more soluble allantoin
- IV every two weeks
6
Q
general characteristics of myasthenia gravis:
A
fluctuating weakness (excessive fatiguability)
distribution of weakness (ocular muscles affected first usually; ptosis and diplopia usually)
clinical response to cholinergic drugs
7
Q
Primary OA classification in particular affects what?
A
- Primary OA most common, no identifiable cause is recognized
- Joints affected DIP (distal interphalangeal), PIP (proximal interphalangeal) of fingers
- 1st carpometacarpal joint (base of thumb)
- Hip and knee joints, cervical and lumbar spine
8
Q
What are the typical diagnostic features seen with an MRI of the head and CT of the spine in a patient with MS?
A
- Ovoid lesions of high signal on T2WI in periventricular white matter and spinal cord
- Acute lesions may enhance
9
Q
myotome vs. dermatome vs. sclerotome
A
myotome: muscle group supplied by single spinal root
dermatome: skin area supplied by single spinal root
sclerotome: area of bone supplied by single spinal root
10
Q
Which second gen. antipsychotic is more often assoc. with EPS/Tardive Dyskinesia, elevated prolactin, and weight gain/DM?
A
Risperidone
11
Q
What is the criteria for Bipolar II Disorder?
A
- Pt has at least one major depressive episode and one HYPOmanic episode in absence of any manic or mixed episodes
- Hypomanic is the key!
12
Q
What is commonly seen on radiographs in pt with Erosive OA and how can it be differentiated from RA?
A
Central erosions (vs. marginal erosions in RA) w/ “seagull” appearance in finger joints
13
Q
What is the characteristic morphology seen with with subchondral infarcts of osteonecrosis?
A
Triangular or wedge-shaped segment of tissue that has the subchondral bone plate as its base undergoes necrosis
14
Q
During a general medical exam of the skin of a patient with impaired consciousness what should be considered with acne?
A
Long-term antiepileptic use
15
Q
DTR loss in biceps seen in which nerve roots?
A
C5-C6
16
Q
What are the main characteristics of sensory causes of Disequilibrium?
A
- Proprioceptic deficit
- Visual impairment
- Compensated vestibular disorders
- Worse in dark
- Romberg sign
17
Q
In sarcoma botryoides (rhabdomysarcomas), where the tumor cells abut the mucosa of an organ, they form a submucosal zone of hypercellularity called what?
A
Cambium layer
18
Q
What is the progression and survival rate like for Primary Lateral Sclerosis?
A
- Slow progression, but can evolve into ALS
- Survival rate better than ALS
19
Q
what forms do the anti-hypertensives come in
A
tablets
20
Q
If the CT findings are normal in a patient believe to have suffered a stroke, what are some other causes you should consider?
A
Seizure, migraine, hypoglycemia
21
Q
What does the prefrontal cortex do, in which nicotine alters?
A
It’s in charge of executive function which include impulse control
differentiating between good and bad
Motivation to work toward a goal
22
Q
Before beginning antipsychotics the guidelines recommend determining in all patients which baseline items?
A
- Serum glucose + Lipids + Weight (BMI)
- Blood pressure
- Waist circumference and Personal/Family hx of metabolic and CV dz
23
Q
what form does ProCentra (dextroamphetamine sulfate) come in
A
liquid
IR Amphetamine
24
Q
What is the characteristic sign of Hepatic dysfunction causing stupor and coma?
A
- Asterixis!
- Pt extends arms and wrists, the wrists will twitch!
25
Which drugs can be used for methanol/ethylene glycol intoxication by blocking alcohol dehydrogenase and the conversion to formaldehyde?
Fomepizole and ethanol (give them a shot)
26
What is the criteria for Generalized Anxiety Disorder?
- Excessive anxiety and worry about different aspects of life for **most days** of ≥**6 months** assoc. w/ ≥**3** of the following:
- Restlessness
- Irratibility
- Sleep disturbance
- Fatigue
- Muscle tension
- Difficulty concentrating
27
What's the Kernohan's notch phenomenon?
Is a result of the compression of the cerebellar peduncle against the tentorium cerebelli due to transtentorial herniation. This produces ipsilateral hemiparesis or hemiplegia (on the side of the herniation) his produces a visible "notch" in the cerebellar peduncle. Because a Kernohan's notch is caused by an injury creating pressure on the opposite hemisphere of the brain, it is characterized as a false localizing sign..
28
A right hemisphere trans-tentorial herniation, causes a Kernohan's notch in the _____ cerebellar peduncle, which results in ________ motor impairment.
A right hemisphere trans-tentorial herniation, causes a Kernohan's notch in the left cerebellar peduncle, which results in right-sided motor impairment.
29
What is gliosis?
Proliferation of astrocytes in response to brain injury
30
Histo of osteopet
31
What are the requirements of diagnosis for a Vestibular Migraine (central)?
- At least **5 episodes of moderate/severe vestibular sx's** lasting **5 minutes to 72 hours**
- **Current or previous hx of migraine** w/ or w/o aura
- **One of more migraine features w/ at least 50% of episodes**: headache, photophobia, phonophobia, nausea, aura...
32
What are the characteristic of a complex partial seizure (i.e., signs/symptoms, time-line, consciousness post-ictal)?
- Nonresponsive staring, possible preceding aura
- Automatisms (action performed unconsciously or involuntarily)
- Loss of consciousness
- Last 1-3 mins
- Post-ictal state
33
What is the MOA of the second-gen antipsychotic agents?
## Footnote
- Block **D2** _post_-synaptic receptors **AND** the **5HT2A** receptors
- **Stronger** 5HT2A receptor blockers than D2 receptor blockers
34
lab findings of Guillan barre
increased protein in CSF; normal cell count and glucose
slow conduction velocity, focal conduction block, prolonged F-waves
35
compare adverse effects of memantine to the cholinergic medications
memantine has fewer AEs
36
What are the respiratory patterns of someone with a subtentorial mass lesion?
**Bizarre** respiratory patterns common
37
What are 7 common symptoms of patients with MS?
1) Paresthesias
2) Gait disturbances (i.e., transverse myelitis)
3) Weakness
4) Visual loss (i.e., optic neuritis)
5) Urinary difficulty
6) Dysarthria
7) Hemiparesis
38
Who does NMDA encephalitis most commonly affect?
Most commonly affects **young** or **middle-aged _women_**
39
Which translocation and fusion gene product is characteristic of Ewing Sarcoma?
**t(11;22)** --\> ***EWS-FL11*** gene
40
define radiculopathy
nerve root dysfxn may be caused by structural (discs, osteophytes, tumors, etc.) or non-structural (DM, infections, etc) conditions
41
Which type of anxiety disorder has a strong genetic component?
**Panic disorder**: 50% of all pt's have _at least one_ affected relative
42
Bisphosphonates are the drugs of first choice for what 5 conditions?
- **Postmenopausal** osteoporosis
- Osteoporosis **in men**
- **Glucocorticoid-induced** osteoporosis
- **Paget disease** of bone
- **Hypercalcemia of malignancy**
43
motor signs of PND?
atrophy, muscular weaknesss, decreased or absent deep tendon (muscle stretch) reflexes, fasciculations, cramps
44
Sudden onset of severe headache ("worst headache of my life"), often with rapid neurologic deterioration is consistent with a _________ hemorrhage
Subarachnoid
45
What is the most common parasitic nervous system disease in the world and can lead to hydrocephalus?
- Cysticercosis - Taenia Solium (pork tapeworm) CALCIFIED CYSTS
46
lower limb entrapment neuropathies common nerves involved
peroneal (mc by fibular head by lateral aspect of knee);note: inversion preserved unlike L5 issues
lateral femoral cutaneous n. (commonly from weight gain and tight clothing)
47
A patient presents after a direct blow to the head which initially knocked him unconscious, after a couple hour lucid interval he begins to exhibit neurological deterioration, what do you suspect?
Epidural hematoma
48
What are Cheynes-Stokes respirations?
Seen with what disorders?
- Hyperpnia regularly alternating w/ apnea (bilateral hemispheres or diencephalon)
- Many disorders ranging from **metabolic** to **structural**
49
Which 3 organisms are commonly cultured in osteomyelitis seen in pt's with UTI's are who are IV drug users?
***E. coli*** + ***Pseudomonas*** + ***Klebsiella***
50
What is the most common childhood form of muscular dystrophy?
Duchenne's muscular dystrophy (DMD)
51
Central: Vascular (ischemic) causes of equilibrium disoders are most commonly seen in?
The elderly
52
Uric acid indicator and what constitutes hyperuricemia?
uric acid \> **6.8 mg/dl**
53
With labyrinthine dysfunction (peripheral) leading to dysequilibrium what is the vertigo like, duration of nystagmus, direction of nystagmus, and neuro symptoms?
- Intense vertigo
- Brief nystagmus
- Horizontal/diagonal nystagmus that is fixed
- Never any neuro symptoms
54
CNS signs
UMN: spastic tone, normal bulk, no fasciculaitons, hyperactive DTRs, +babinski sign
55
Infarcts may occur in the supratentorial white matter of premature infants and are known as?
Characteristic finding?
- Periventricular leukomalacia
- Chalky yellow plaques = white matter necrosis and calcification
56
What are the most common forms of Spinocerebellar Ataxias?
Characteristic findings in these diseases?
- SCA 1 (olivopontocerebellar)
- SCA 3 (Machado-Joseph)
\*Slowly progressive cerebellar ataxia of limbs combined w/ brainstem signs (dysarthria, oculomotor disturbance, spasticity) and peripheral neuropathy
57
Which nerve is compressed in Kernohan's notch?
CN6
58
What is a synovial cyst and what is a common example of one?
- **Herniation** of the synovium thru a **joint capsule** or massive enlargement of a bursa
- **Baker cyst** seen in **popliteal space (behind the knee)** in setting of **RA**
59
what form does focalin come in
tablet
60
Diabetic Peripheral Neuropathy, Fibromyalgia, and Chronic musculoskeletal pain
**duloxetine**
61
What are Duret hemorrhages and are a result of what?
- Progression of transtentorial herniation producing secondary hemorrhagic lesions in the midbrain and pon - "Flame-shaped" lesions
62
Psoriatic arthritis can look like OA by presenting how?
Psoriatic arthritis can involve **DIP**; causes morning stiffness, joint **swelling dactylitis** and history of psoriasis
63
Diagnosis of osteomyelitis is strongly suggested by what characteristic radiographic findings?
**Lytic focus** of **bone destruction** surrounded by **zone of sclerosis**
64
Which bisphosphonate has been most commonly associated with osteonecrosis of the jaw and also dose-dependent kidney damage and rarely atrial fibrillation?
**Zolendronic acid**
65
Tell me about hyperuriciemia and gout
Causes **gouty arthritis**
**Monosodium urate** crystals in joint fluid or tophi
* **Crystals are needle-shaped**, **negative birefringent** by polarized light miscroscopy
* **Joints are hot**, **swollen**, tender, dusky, red; fever
* **Tophi** – **nodular deposits of monosodium urate crystals in skin**
* Alcohol promotes increased urate production and decreased excretion
66
What other symptoms can you see with LGI1 Encephalitis?
1) **Sleep disturbance** is seen in about 50% of patients.
2) May see **temporal lobe** (esp. hippocampal) **abnormality** in some patients acutely
67
what form does Mydayis come in
capsule
68
ESR is a good marker for monitoring disease activity, especially in what 2 conditions?
**Polymyalgia rheumatica** and **Giant Cell arteritis**
69
What are some of the complications which may arise in the setting of tuberculous spondylitis (Pott disease)?
- **Permanent compression fractures** --\> **scoliosis** or **kyphosis** & neurological deficits 2' to spinal cord and nerve compression
- **Other**: tuberculous arthritis, sinus tract formation, psoas abscess & amyloidosis
70
What is the criteria for persistent depressive disorder (dysthymia)?
- Often **milder**, ≥2 depressive sx's for **most of the day** ≥ **2 years** for **adults** and **_1 year_** in **children**
- During 2 years, **_cannot_ be w/o sx for \>2 months at a time** (is continuous)
71
What is the duration of disturbance and sx's for diagnosis of PTSD?
Lasts **\>1 month** with _significant distress_ or _impaired functioning_
72
What does the term sequestrum refer to in terms of osteomyelitis; what is seen with rupture of the periosteum?
- Dead bone following **subperiosteal abscess**
- **Rupture** of periosteum ---\> soft tissue **abscess** which can channel to become a **draining sinus**
73
What is Cerebral Palsy and what are the clinical manifestations of this disease?
Occurs due to insults during which period?
- **Non-progressive** neurologic motor deficits characterized by combination of spasticity, dystonia, ataxia/athetosis, and paresis
- Pre-natal and perinatal periods (are present from birth)
74
What is the response of microglia to neuronal injury?
Proliferate and accumulate during CNS injury
75
Pathway of ethylene glycol metabolism?
Ethylene glycol -\> Alcohol dehydrogenase -\> glycoaldehyde -\> Aldehyde dehydrogenase -\> Glycolic acid
76
Pain in elbow/forearm is seen in which nerve root
C7
77
Where does embryonal rhabdomyosarcoma most often arise, characteristic morphology, and age group?
- Arise in **genitourinary tract**
- Patients **1-5 y/o**
- **Primitive spindle cells, "strap cells"** or **Tadpole cell**
78
Which pharmacologic agent can cause a 50% reduction in suicidal thoughts in 24-hours and is great for tx of major depressive disorder?
**Ketamine**
79
What are the 6 second-gen. aka atypical/novel antipsychotics?
## Footnote
- Ari**piprazole**
- Cloz**apine**
- Olanz**apine**
- Queti**apine**
- Risperi**done**
- Ziprasi**done**
80
Pathophysioliogy of Myasthenia Gravis:
caused by a defect of neuromuscular transmission due to an antibody-mediated attack upon nicotinic acetylcholine receptors (AChR) on muscle membrane
81
Enuresis (bed wetting)/urinary incontinence anti depressant
imipramine
82
Anti CCP in OA is usually?
Negative
83
What is Rheumatoid factor and what is produced by?
It is an **IgM** (the most common autoantibody that does this) autoantibody that **targets the Fc portion of IgG immunoglobulin**
RF is produced by **B cells** in **synovial joints** of the **RA joints**
84
What are the hallmarks of subacute and chronic neuronal injury (i.e., degeneration)?
Cell loss ---\> Apoptosis - Reactive gliosis
85
What is the criteria for Bipolar I Disorder?
- ≥1 **manic** episode +/- a hypomanic or depressive episode
- Major depressive episode **not** required, but is often the episode that presents **first**!
86
Which 4 benzodiazepines have the longest half-lives all \>100 hr and all have active metabolites?
- Chlordiazepoxide
- Clorazepate
- Diazepam
- Flurazepam
87
How are lab studies usually for OA?
The ESR is generally normal in OA
88
Where is a common location for ganglion cysts to arise, how to they appear, and arise as a result of what?
- Around joints of **wrist**
- **Firm, fluctuant, pea-sized translucent** _(you can shine a light through it)_ nodule
- Arise as result of **cystic** or **myxoid degeneration** of CT; hence the cyst wall **lacks a cell lining**
89
What other random things do you see go up on labs for RA?
Thrombocytosis (acute phase reactant)
* ANA+ (30% of RA patients)
* Hyperglobulinemia
* Leukopenia / Granulocytopenia
* Low glucose in body fluids
* Synovial fluid – 2/3 PMN’s; WBC’s 5000 – 100,000/mm3
90
what form does adderall (dextroamphetamine/AMP) come in?
tablet
IR amphetamine
91
What are the sx's of withdrawl from barbiturates, benzo's and ethanol?
Anxiety + insomnia + delirium + tremors + seizures + death
92
What is the clinical presentation in DMD in kids?
1. **Proximal muscle weakness (symmetric)**
2. **Rarely have anti-gravity neck flexor strength**
3. Delayed walking, difficulty running, can’t “keep up” with peers
4. Develop a broad based, waddling, gait with exaggerated lordosis
5. Gower’s sign present
6. **Usually first noted around 1 ½ to 2 years old**, as developmental milestones in the gross motor domains fail to be met
7. Progresses rapidly, severe disease
8. Scoliosis develops
9. Calf and thigh muscle hypertrophy followed by pseudo-hypertrophy 10. **Cognitive dysfunction**
11. Toe walking and limited hip flexion (by 4-6 years-of-age)
`12. Life expectancy: late teens to mid-twenties
93
Other than D2-receptors, which other receptors may be blocked by first gen. antipsychotics and how does this correlate with AE's?
## Footnote
- **Muscarinic** receptors: dry mouth, constipation, blurred vision...
- **Histamine (H1)** receptors: **sedation**
- **α1-adrenergic** receptors: orthostatic hypotension, dizziness/syncope
94
Which rare type of brain tumor leads to increased CSF production, thus causing increased ICP and hydrocephalus?
Choroid Plexus Papilloma/Carcinoma
95
A CRP \>\_\_\_\_ mg/L is inflammatory
What other factors go up with inflammation?
\>**8 mg/L**
Leukocytosis, thrombocystosis, ferritin, fibrinogen and complement increase
96
Clinical px of botulism:
dry, sore mouth and throat, blurry vision, diplopia, n/v, hypohydrosis, etc
97
define classical conditioning
learning is the association of things that take place together in time
the repeated pairing of a neutral (unconditioned stimulus) with one (conditioned stimulus) that evokes a response (unconditioned response) so that the neutral stimulus eventually come to evoke the same response (conditioned response)
98
What is the morphology of a brain contusion?
What is seen early on and within 24 hours?
- **Wedge shaped** w/ a broad base lying along the surface at the point of impact
- Early stages: pericapillary edema and hemorrhage
- 24 hours: pyknosis of the nucleus, eosinophilia of cytoplasm and disintegration of the cell
99
What is seen with the trabeculae that remains in subchondral infarcts (osteonecrosis)?
- Acts as **scaffolding** for the deposition of new bone in process known as **"creeping substitution"**
- Pace is too slow to be effective, so there is **collapse** of the necrotic bone and distortion, fracture and even **sloughing** of the **articular cartilage**
100
When CIS patients do not have multiple demyelinating lesions on MRI, they have a \_\_\_\_\_\_% chance of developing MS within several years
20%
\*Low risk
101
What will an EMG of pt with ALS show?
Widespread **_denervation_** and **_reinnervation_**
102
What does **tuberculous spondylitis (pott's dz)** come from?
From **spine** involvment in mycobacteria infection cases
103
Do you get pain with subcondral infarcts? And can lead to what?
Yes and initially only associated with activity and then becomes constant
Often collapse and may lead to secondary osteoarthritis
104
What is the pathophysiology behind RA?
**Multigene Disease**
* 1/3 of patients susceptibility from genetic factors HLA-DRB 4 alleles/DRB1 0401 or 0404
* Pathologic changes in joints precedes synovitis in RA patients 5-10 years
* **Infiltration** of leukocytes, cytokines and macrophages act as antigen presenting cells to **activate T cells**
* “**B” lymphocytes produce autoantibodies,** cytokines (**TNF alpha, IL-1, IL6**), proinflammatory cytokines **synovial proliferation**, **increase synovial fluid**, leads to “**pannus**” that invades cartilage and bone
105
What are some conditions that can present w/ focal neuro deficits that may be confused with stroke?
1) **Migrane w/ aura** (primary headache disorder)
2) **Hyperglycemia**
3) **Hepatic abnormalities**
106
Explain four (4) functions that a Behavioral Health Consultant can provide to assist a physician in his or her day-to-day practice.
- Assessment, Education, Brief Intervention, Referral
- Warm hand-offs
- Chronic illness, Mental health disorders
- Prevention
- Quality improvement and quality assurance
107
DTR loss in finger flexors seen in
C8
108
Hypomanic episodes are more associated with which type of bipolar disorder?
Bipolar **type II**
109
What does dead bone look like microscopically?
**Empty lacunae** surrounded by necrotic _adipocytes_ which frequently rupture
110
What are some clinical manifestations for RA? So you can recognize them on the test?
* PIP, MCP/DIP almost never involved • Swan neck (hyperextension of PIP joints) • Boutonniere (button hole deformity) (hyperflexion of PIP joints)
* **Pain, swelling, warmth in multiple small joints ( one (1) year • \< 10% have abrupt onset of disease**
**Tenderness, swelling, warmth, erythema, fever, weight loss, anorexia, symmetrical joint involvement**
• **Skin – subcutaneous nodules; extensor surface of forearm**
111
What are the symptoms of a stroke occuring in the right hemisphere?
- Left hemineglect
- Left sided sensory and motor symptoms
- Left visual field cut
112
Most of the mutations underlying osteopetrosis interfere with what process?
Acidification of the osteoclast resorption pit, required for the dissoluation of the Ca2+ hydroxyapatite within the matrix
113
What are some systemic features of RA?
* Fatigue, **fever**, anemia (NC-NC)
* Elevated acute phase reactants (**ESR, CRP**)
* **Constitutional symptoms** – malaise, myalgia, depression
114
How is diagnosis of Nitrous Oxide Toxicity made and what is the treatment?
- Diagnosis: depletion of Vit B12 w/ similar symptoms
- Tx: B12 replacement
115
what form does dyanavel XR come in
**\*liquid\***
116
- **Dysarthria (speech difficulty)** + **dysphagia** (difficulty swallowing)+ **dysphonia** (poor voice quality) + **chewing difficulty** + **drooling**
- **Almost always** progresses to generalized disease i.e., **ALS**
117
Due to deficient osteo**clast** activity what is seen morphologically in the bones of osteopetrosis?
- Bones involved lack a medullary canal; instead contain **primary spongiosa** (which is normally removed during growth)
- Ends of long bones are bulbous (**Erlenmeyer flask** deformity)
- Neural foramina are small and **compress exiting nerves**
118
Which class and 2 agents are used for tx of Tardive Dyskinesia (TD) assoc. with first gen. antipsychotics?
Selective **VMAT2** inhibitor's: **Valbenazine** and **Deutetrabenazine**
119
Bobbing nystagmus (rapid down, slow up) indicates a lesion where?
Pons
120
define operant conditioning
learning is the association of things that take place sequentially
121
Which 2 benzodiazepines have the shortest half-lifes?
Triazolam and Midazolam
122
indications ketorolacwho cannot take it
short term (\<5 days) management of moderately severe acute pain that requires analgesia at the opioid level- usually post-operative stateNOT indicated in peds patients, or for minor or chronic conditions
123
A diagnosis of panic disorder requires what?
- **Panic attac**k followed by **≥1 month** of **≥1 of the following**:
- **Persistent concern** of _additional_ attack
- **Worrying** about **consequencs** of attack (i.e., losing control, MI, etc.)
- **Behavioral change** related to attack
124
Describe the defect caused by the mutations in **CA2** and ***CLCN7*** associated with osteopetrosis?
- ***CA2***: required to generate protons from CO2 and H2O --\> absence **prevents** osteo**clasts** from **acidifying the resporption pit** and **solubilizing hydroxyapatide**, and also blocks the acidification of urine by **_renal tubular cells_**
- ***CLCN7***, encodes a ***proton pump*** located on the surface of osteo**clasts**
125
What are the two genetic mutations asssociated with osteopetrosis?
***CA2*** and ***CLCN7***
126
Age of onset for Ataxia-Telangiectasia?
Common findings?
- Disease of **infancy** (\<4 yr. of age)
- **Progressive pancerebellar degeneration** involving nystagmus, dysarthria, and gait, limb and trunk ataxia
- Choreoathetosis, loss of vibration and position sense in legs, **areflexia**, and disorders of voluntary eye movements
127
Sensory for L4, L5, S1
L4: medial calf
L5: lat calf, dorsum foot
S1: postlat calf, lat foot
128
Pt's with C5, 6, 7, 8, and 9 deficiency have increased susceptibility to which organism causing osteomyelitis?
***Neisseria***
129
What are the two genetic abnormalities associated with BMD?
1. In-frame mutation in the dystrophin gene
2. Production of abnormal or semi-functional dystrophin
130
Subdural hematoma etiology and features?
Crescent shaped collection on CT, presents with progressive neurologic sign, due to tearing of bridging veins underneath the dura and above arachnoid, occurs in old people due to cerebral atrophy, herniation is a lethal complication
SLOW
131
The initial signs of a supratentorial mass lesion are usually (global or focal)?
How do the signs progress?
Motor signs are often?
- Initial signs usually **focal**
- Progression of signs is **rostral** to **caudal** (i.e., herniation pushing down)
- **Motor signs** are often **asymmetric**
132
Which benzodiazepine has a short half-life (5-14 hr), no active metabolite, and the slowest rate of onset?
Oxazepam
133
what antibodies other than **anti-AChR antibodies** can be seen with myasthenia gravis?
**MUSK and LRP-4 Antibodies**
134
Repeated episodes of isolated vertigo without other neurological symptoms should always suggest?
A non-neurologic cause
135
What are some of the drug induced causes of peripheral disequilibrium?
- Alcohol!
- Antibiotics - aminoglycosides, tetracycline, vancomycin
- Diuretics
- Chemotherapeutics: cisplatin, methotrexate, vincristine
136
Anatomy of the NMJ:
nerve AP--\> calcium entry--\> ACh release--\> muscle AP--\> muscle contraction
137
What tumor is NMDA encephalitis commonly associated with?
Commonly associated with presence of **_teratoma_**
138
What is central transtentorial herniation and the signs/symptoms?
- Herniation into **foramen magnum**
- Leads to early coma, **small pupils**, normal EOM's, posturing and later bilateral fixed pupils
- **Respiratory arrest** and **death**
139
Describe the morphological variants of osteomyelitis known as Brodie abscess and Sclerosis osteomyelitis of Garre?
- **Brodie abscess:** small **interosseous** abscess frequently involves cortex & is **walled off by reactive bone**
- **Sclerosing osteomyelitis of Garre**: in **jaw** and assoc. w/ extensive new bone formation that obscures much of the underlying osseous structure
140
Which syndromes is associated with fibrous dysplasia and what is seen in with it?
- **McCune-Albright disease**: unilateral bone lesions w/ **café-au-lait** skin pigmentations + **endocrine abnormalities**; esp. **_precocious puberty_**
141
What are the symptoms of NMDA encephalitis and over what period does it develop?
Typically presents with rapid onset (**less than 3 months**) of **at least four of the six** following major groups of symptoms:
– **Abnormal (psychiatric) behavior** or cognitive dysfunction
– **Speech dysfunction** (pressured speech, verbal reduction, mutism)
– **Seizures** – Movement disorder, dyskinesias, or rigidity/abnormal postures
– **Decreased level of consciousness**
– **Autonomic dysfunction** or central hypoventilation
142
Most important histopathologic indicator of CNS injury regardless of etiology? Characterized by what 2 things; what cell?
- Gliosis - BOTH hypertrophy and hyperplasia of astrocytes
143
giving magnesium salts in myasthenia gravis
should be avoided!; can replace calcium thus interfering with the NMJ
144
no DTR loss is seen in
L5
145
Transtentorial (uncinate, mesial temporal) herniation occurs when? Which specific part and of which lobe?
MEDIAL aspect of the TEMPORAL lobe is compressed against the free margin of the tentorium
146
in hemispheric lesions, where does the eyes deviate towards with destructive and irritative lesions
destructive: toward lesion
irritative: away from lesion
147
How does Diazepam differ from Alprazolam in terms of half-life, active metabolites and rate of onset?
Diazepam: long half-life (\>100 hr); has active metabolite; very fast onset
- Alprazolam: short half-life (12-15); no active metabolite; fast onset
148
Morphology of red neurons
- Shrinkage of cell body - Pyknosis of nucleus - Dissapearance of nucleolus - Loss of nissl substance - Intense eosinophilia of the cytoplasm
149
What is the most comon location for debris causing Benign Positional Vertigo?
How is this diagnosed?
Direction of Nystagmus?
- **Posterior** semicircular canal
- Use **Dix Hallpike** manuever, nystagmus provoked w/ affected ear down
- **Nystagmus** = torsional
150
Between ESR and CRP, which rises and falls quicker?
**CRP**
151
Medullary infarcts are usually small and clinically silent except when they occur in the which 3 settings?
- **Gaucher disease**
- **Dysbarism** (i.e., the "bends")
- **Sickle cell anemia**
152
Frontal gaze centers deviate eyes where?
Pontine gaze centers deviate eyes where?
- **Frontal** gaze centers deviate eyes to **opposite** side (i.e., if right area is dysfunctional, there is no drive to the left, so eyes will be looking right)
- **Pontine** gaze centers deviate eyes to **same** side
153
what is the dosing and patient age for the anti-hypertensives (guanfacine and clonidine)
what titration is necessary with these medications
every day dosing
pts 6+
downward dose titration over 1+ weeks do reduce risk of rebound hypertension
154
what form does desoxyn come in
tablet
155
What are the symptoms of a stroke occuring in the brainstem?
- CN findings w/ contralateral hemisensory or hemimotor sx's
- Vertigo
156
what is the one \***liquid**\* extended release amphetamine based stimulant
**dyanavel XR**
157
List 5 benzodiazepines that are short to intermediate acting?
- Alprazolam
- Temazepam
- Triazolam
- Oxazepam
- Midazolam
158
What is seen with a tonic-clonic seizure, how long does it last, and how does the patient feel afterwards?
- Bilateral extension followed by symmetrical jerking of extremities
- Loss of consciousness
- Lasts 1-3 mins
- Post-ictal state
159
What is the effect of barbituates on CYP450 enzymes?
Can induce CYP450 enzymes
160
What is Decorticate vs. Decerebrate posturing and where is the lesion for each?
- **Decorticate** = arms flexed, legs extended (**hemispheric**)
- **DEcrebrate** = all extremities **E**xtended (**brainstem**)
161
What are the **two** _anticholinergic agents_ that are coadministered with AChE inhibitors to minimize adverse cholinergic effects (bradycardia, bronchoconstriction, salivation, nausea, vomiting) at muscarinic AChRs?
**Atropine**
**glycopyrrolate**
162
galantamine should not be used in what patients
those with end-stage kidney dz or severe haptic impairment
163
what extended release methylphenidate-based stimulants used to treat ADHD come in liquid form
**\*quillivant XR\***
164
How do fluctuation of sx's differ between generalized anxiety and those of panic anxiety disorder?
Sx's of **generalized** anxiety fluctuate **more** than those of panic anxiety
165
how to test proprioception in peripheral neuropathy?
romberg maneuver
166
What is the prototype short-acting barbituate that is used for insomnia?
Secobarbital
167
Which AE's are associated with **Pegloticase** and how can they be managed?
- **Infusion rxns** i.e., fever, chills, rash, angioedema, bronchospasm, hypo- or HTN
- Need to **premedicate** w/ **glucocorticoids** and **anti-histamines**
168
When is the typical onset of Friedrich's Ataxia and what are the common findings?
- Onset before age 20 (young persons disease)
- Gait ataxia w/ absent tendon relfexes in legs and muscle weakness
- Extensor plantar responses
- Pes cavus
- Kyphoscoliosis
169
sensory C7
3rd digit
170
What are the 3 intial steps of managing a comatose patient?
1) **A:** insure patent airways
2) **B:** insure breathing and adequate oxygenation
3) **C:** insure adequate circulation and control any active bleeding
\*Stabilize neck, get C-spine films if trauma suspected
171
The effects of nondepolarizing neuromuscular blocking agents are reversed how?
Addition of an **acetylcholine esterase (AChE) inhibitors**
172
Which cell type is evident 12-24 hrs after acute CNS hypoxic/ischemic insult?
Red neurons ("red dead guys")
173
Which AChE inhibitor is commonly used to reverse neuromuscular blocking drug-induced paralysis?
**Neostigmine**
174
CSF analysis via lumbar puncture will have what diagnostic findings in a patient with MS?
Presence of oligoclonal bands and/or increased IgG index/synthesis rate
175
Where is Spondylosis most commonly seen?
Can lead to?
Early signs?
- Cervical region
- Can lead to **myelopathy** (spondylotic)
- Unexplained gait impairment or imbalance often **an early symptom**
176
benefits of vaping compared to cigarettes
- less expensive
- tastes better
- less smell/stains on fingers
- less teeth staining
- sense of smell and taste improve
- social interactions increase
- less lung damage
177
Where does the seizure activity begin with Partial Seizures?
Activity begins on **one side of the brain**
178
DTR loss in patella vs achilles
patella = L4
achilles= S1
179
What does conjugate vs. dysconjugate gaze imply about the brainstem?
- **Conjugate** implies brainstem **intact**
- **Dysconjugate** implies brainstem **lesion**
180
Which state of altered consciousness is represented by arousal only to noxious stimuli and not enviornmental, only rudimentary awareness (i.e., purposeful motor response)?
Stupor
181
Which 4 structures are considered peripheral/labyrinthine for the maintenance of equilibrium?
1. Utricle
2. Saccule
3. Semicircular canals
4. Vestibular nerve
182
Rule of thumb for lesion location when pupils are enlarged on one side?
**Parasympathetic** division (usually CN III)
183
Which endocrine abnormality can manifest as secondary OA; which joints most often affected?
- **Hyperparathyroidism**
- **Wrist, MCP**
184
What are the symptoms of a stroke occuring in the cerebellum?
- Ipsilater ataxia
- Vertigo
- Nystagmus
185
list the extended release amphetamine based stimulants used to treat ADHD
- adderall XR
- dexedrine
- dyanavel XR
- Vyvanse
- mydayis
186
Which agents are co-administered with AChE inhibitors during reversal of the effects of neuromuscular blocking agents to minimize adverse cholinergic effects?
**Anticholinergic agents** (e.g., **atropine**, **glycopyrrolate**) are coadministered with AChE inhibitors to minimize adverse cholinergic effects (bradycardia, bronchoconstriction, salivation, nausea, vomiting) at muscarinic AChRs
187
What is the response of astrocytes to neuronal injury?
- Hypertrophy of the cytoplasm, accumulation of GFAP, and hyperplasia
188
What is the criteria for restricting type vs. binge-eating/purging ty of anorexia nervosa?
- **Restricting type**: _3 months_ of no binging or purging, but excessive exercising, fasting, dieting
- **Binge/purge type**: _3 months_ of binging and purging behaviors: self-induced vomiting, misuse of laxatives, diuretics, enemas
189
What is the criteria for diagnosing a major depressive **episode**?
- At least **5** of the following for a **_2-week period_** with at least _one_ either (1) depressed mood or (2) loss of interest or pleasure
- **SIG E CAPS**
190
Treatment for MuSK syndrome of myasthenia gravis
poor response to anticholinesterase meds, thymectomy
191
In mycobacterial osteomyelitis the organisms usually originate from where?
**_Blood_** borne, originating from a focus of active visceral disease during initial stages of primary infection; can be direct extension
192
weakness in shoulder abduction seen in which nerve roots
C5-C6
193
What are the dopamine-associated AE's of the first gen. antipsychotics?
## Footnote
- **Hyper**prolactinemia (tuberoinfundibular pathway): **amenorrhea**, **galactorrhea**, **gynecomasta**, and ↓ libido
- **Extrapyramidal sx's** and **Tardive Dyskinesia**
194
What is the morphology of alveolar rhabdomyosarcomas?
- Networks of **fibrous septae** dividing cells into **clusters** and **aggregates**; centers are **dischohesive**
- Resemblance to **pulmonary alveoli**
- **Cross striations** are **NOT** a common feature
195
What are the risk factors for OA?
Age \>55
Female
Obesity
physical labor
genetic mutation
trauma/ joint loading
196
What is seen in the brains of individuals with CTE (dementia pugilistica) during a post-mortem autopsy?
- Atrophic w/ enlarged ventricles
- Accumulation of tau-containing neurofibrillary tangles
- Characteristic pattern involving superficial frontal and temporal lobe cortex
- Depigmentation of the substantia niagra
197
pain in prox arm is seen in what nerve root
C6
198
What is the most common cause of bacterial meningitis in adult, infants, and young children?
***Streptococcus pneumoniae***
199
gabapentin is a pregnancy category C
remember that
200
which dementia drug comes as a twice-daily capsule, a twice-daily solution, and **a 24 hour transdermal patch**
**rivastigmine**
201
What is seen with an Absence (petit mal) seizure, how long does it last, how does the patient feel after?
- Nonresponsive staring, rapid blinking, chewing, clonic hand motions
- Loss of consciousness
- Lasts 10-30 sec
- **No** post-ictal state
\***Abrupt onset and abrupt end**
202
compare mononeuropathy, mononeuropathy multiplex, and polyneuropathy
mononeuropathy: one nerve affectedmononeuropathy multiplex: several nerves affected in different areaspolyneuropathy: sx are diffuse and bilateral
203
why should aspirin not be used in children with chicken pox or influenza?
due to reye syndrome
204
In children the periosteum is loosely attached to the cortex, so what is often seen in the acute phase of osteomyelitis?
Sizable **subperiosteal abscesses** may form, which **dissect** for long distances along the bony surface
205
What is the treatment and 5-year survival with Ewing Sarcomas?
- Tx w/ neoadjuvant chemotherapy followed by surgical excision with or without radiation
- 5-year survival of 75% and long-term cure in 50%
206
pain in scapula and shoulder is seen in what nerve roots
C5-C8
207
list the other biphosphonates (other than alendronate) and how they are administered
- risedronate (PO)
- ibandronate (PO, IV)
- tiludronate (PO)
- **zolendronic acid (IV)**
208
How do you treat DMD?
Steroids
209
What will examination of someone with Spondylotic Myelopathy show?
- Spastic tone in legs
- Increase knee/ankle jerks
- Babinski signs
- Variable sensory deficits
210
What must be known when making the call of brain death?
Cause of the coma **should be known**, it **MUST** be adequate to explain the clinical picture, and it **MUST** be **irreversible**
211
Age of onset for Ataxia-Telangiectasia?
Common findings?
- Disease of **infancy** (\<4 yr. of age)
- **Progressive pancerebellar degeneration** involving nystagmus, dysarthria, and gait, limb and trunk ataxia
- Choreoathetosis, loss of vibration and position sense in legs, **areflexia**, and disorders of voluntary eye movements
212
the ring finger dermatome map is divided in:
C7 and C8 (ulnar side)
213
What is the MOA of Disulfiram?
- Irreversibly inhibits **aldehyde dehydrogenase** leading to buildup of aldehyde
- Causing extreme discomfort in pt's who drink alcoholic beverages: flushing, throbing HA, N/V, sweating, hypotension, and confusion
214
RA labs (RF, Anti ccp, Acute phase reactants)? Which is each indicative of?
RF – 70% with RA. (specificity is limited because other diseases are associated with RF)
Anti CCP – antibodies in 70% with RA but has a specificity of 95% - **more predictive for erosive disease**
Acute phase reactants – ESR, CRP 75% elevated in RA. **Use to monitor treatment response**
215
indications for rivastigmine
1) mild to moderate dementia of Alzheimer's type
2) **mild to moderate dementia associated w/ Parkinson's dz**
216
Treatment for Meniere's Disease?
- Sodium restriction
- Diuretics: thiazdies, furosemide
- Sugery: endolymphatic sac decompression
217
MOA clonidine
alpha 2 adrenergic agonistblocks transmission of pain signals from periphery to brain
218
Where do entecapone and tolcapone act in the nervous system?
1. Tolcapone is central and peripheral acting; entacapone is peripheral acting only
219
cholinesterase inhibitors should be prescribed with caution when the patient is already taking what other drugs
drugs that induce bradycardia or alter AV nodal conduction
- beta blockers
- calcium channel blockers
- lacosamide
220
What is the characteristic signs of someone with diffuse/metabolic injury of the nervous system (i.e., motor signs, consciousness, breathing, and presentation)?
- Confusion and stupor commonly precede motor signs
- **Motor signs** usually **symmetrical**
- **Pupillary** rxns usually **preserved**
- **Asterixis**, myoclonus, tremor, seizures common
- Acid-base imbalance with hyper or hypoventilation
- Fluctuating level of consciousness
221
What is the ASO titer?
Evidence of a preceding group A streptococcal infection
Acute rheumatic factor (ARF) resulting from autoimmune reaction to infection of strep A
May cause post-streptococcal **reactive arthritis**; affects **small joints** (symmetric)
222
pain seen in medial forearm is seen in which nerve root
C8
223
Myasthenia Gravis: presyn, synaptic, or postsyn?
Postsynaptic
224
Simple partial seizure is characterized by what?
focal motor or sensory activity
no LOC
lasting only seconds and no post-ictal state
225
What are some of the cardiac complications associated with Anorexia Nervosa?
Bradycardia, HYPOtension, QT dispersion, Cardiac atrophy, and Mitral Valve Prolapse
226
how does Sarin and VX (nerve gases) work? outcomes?
inhibit AChE at NMJ to cause end organ overstimulation (cholinergic crisis)
onset within mins - hrs (vapor or liquid)
death by resp failure):
227
What is the most important aspect of the evaluation for a patient being seen regularly for bulimia and anorexia nervosa?
**\*Discuss suicidality**
228
MC identifiable cause of neuropathy in US is? nerves affected?
Diabetes Mellitus
CN 3 and 6 mostly
also has Bells palsy commonly
229
list the non-stimulant
atomoxetine/strattera ER
230
LGI1 Encephalitis most commonly affects who?
Occurs more commonly in **men (2:1)**
231
What kind of seizures is **LGI1 Encephalitis** associated with? Do they respond to antiepileptic drugs?
Typically involves **faciobrachial dystonic seizures**
– These are **brief seizures** involving o**ne side of the face** and **the arm on the same side**, often **occurring frequently**, sometimes hundreds of times per day.
– These seizures often **do not** respond to antiepileptic drugs alone and may require immunotherapy to abate.
232
Which anti-psychotic is indicated for tx of recurrent suicidal behavior?
## Footnote
**Clozapine**
233
define negative reinforcement and give an example
removing something to increase a behavior
ex: kid doing the dishes to stop his mom's nagging
ex: taking away plastic sheets to increase nights w/o bed-wetting
234
What do overdose effects of ethanol look like?
Slurred speech, drunken behavior, dilated pupils, weak and rapid pulse, clammy skin, shallow respiration, coma, death
235
which dementia drugs comes as a twice-daily tablet or solution and an extended-release once-daily capsule
galantamine
236
what extended release methylphenidate-based stimulants used to treat ADHD come in tablet form
concerta
- contempla XR-ODT
237
Plain radiographs of Ewing Sarcoma will show what; what is characteristic of the **_periosteal rxn_** of these tumors?
- **Destructive lytic tumor** w/ permeative margins that **extends** into surrounding soft tissues
- Produces layers of reactive bone deposited in an **_ONION SKIN_** like fashion
238
What are the most common soft tissue sarcomas of childhood and adolescence?
**Embryonal** and **alveolar** type of rhabdomyosarcoma
239
Tx for sarin and vx (nerve gases)
decontamination (remove clothes, clean skin w/ water and sodium hypochlorite)
resp support
atropine
benzos for seizures
240
How is rasburicase different from pegloticase?
**nonpegylated** recombinant uricase for prevention of **acute uric acid nephropathy** due to **tumor lysis syndrome** in patient with **high-risk lymphoma or leukemia**
241
What is the inheritance pattern of the mild form of osteopetrosis and what are the clinical features?
- **_Autosomal dominant_**
- Dx in **adolescence or adulthood** --\> repeated fractures
- **Mild** CN defecits and anemia
242
Rule of thumb for lesion location when pupils are enlarged bilaterally?
**Bilateral** CN III lesion, post-ictal, or intoxications
243
Why do absent or unequal pupillary responses imply a brainstem lesion?
Nuclei/tracts controlling pupils are **anatomically** adjacent to **ARAS**
244
Guillan-Barre:
ascending motor paralysis; symmetric
no/minimal sensory symptoms/signs
245
What is the criteria for Cyclothymic disorder?
- Characterized as **dysthymic** disorder (**_milder_ depression**) w/ intermittent **hypomanic** episodes
- **≥2 years (adults) and 1 year (children)** experiencing **repeated** episodes of **hypomania** and **depression** (not severe enough to meet criteria for major depressive episode)
246
LEMS is often associated with
Small cell lung cancer
247
HIV neuropathy is in what patients with what CD4 counts?
30-50% AIDS pts (CD4\<200)
248
What is the MOA of the first gen. anti-psychotics?
**Block** dopamine **(D2) _post_-synaptic** receptors
249
Which opioid receptor antagonist can be used in the acute setting of an OD?
**Naloxone**
250
CRP monitors what?
Assesment of disease activity and proinfammatory cytokine release
251
bupropion
252
What do imaging studies of OA show?
* **Asymmetric joint** – space narrowing
* **Subchondral sclerosis** – thickening
* Osteophytes and **marginal lipping**
* **Bone cysts**
* **Joint mice** (loose particles)
253
EMG findings of LEMS:
low amplitude motor responses that facilitate (increase) after brief period of exercise; incremental response on fast repetitive stimulation
254
What parts of the brain involved with generalized seiures?
Both sides of the brain
255
what part of the brain is affected if the patient has central neurogenic hyperventilation
midbrain
256
Peroneal mononeuropathy at fibular head, results of nerve conduction studies:
@ankle and below knee = normal but ABOVE knee get significantly abnormal nerve conduction (decreased amplitude and conduct velocity
257
What are the two forms of autoimmune encephalitis?
**NMDA Encephalitis**
**LGI1 Encephalitis**
258
In **acquired** syphillis (T. Pallidum and T. pertenue) when do the bone lesions typically present and how?
- May begin in early **tertiary** stage, _2-5 years after initial diagnosis_
- **Saddle nose**, palate and extremities (esp. **long tubular** **bones** like **tibia**)
259
Treatment of botulism
ICU monitoring w/ resp support and general medical care
| (ventilation, feeding tube; wait till toxin leaves!)
260
define negative punishment and give an example
removing something to decrease a behavior
ex: taking away someone's freedom and putting them in jail to decrease criminal behavior
261
Simple partial seizure
pt is aware (conscious)
262
Progressive Bulbar Palsy is due to selective involvement of what?
Motor nuclei of the lower CN's
263
Which test can be done to clinically document someone as being brain dead?
- Apnea test
- Disconnect ventilator --\> Catheter w/ 100% O2 and observe chest wall and abdomen for movement
- No respiratory movements **for 8 mins** (PCO2 \>60mmHg
264
Earlier the onset of bulbar symptoms in ALS, the (**Longer or shorter**) the course of the disease?
**Shorter**
265
Why is it important to ask about family hx of mood disorders?
**50%** of pt's with **bipolar disorder** have a 1st-degree relative with a _mood disorder_
266
what extra measures does the rivastigmine patch require once prescribed
dose adjustments for hepatic impairment and low body weight
267
What symptoms would result from Subfalcine herniation with compression of the ACA?
Contralateral lower extremity weakness
268
Which level of the pons is assessed with the Oculocephalic manuever (Doll's Eyes) vs. Caloric (oculovestibular) reflex test?
- **Oculocephalic** = mid pons; assess CN III, IV, and VI
- **Caloric** = lower pons
269
what form does adderall XR come in
capsule
270
sensory C5 location
lateral arm
271
How do you tx depression that is tx resistant?
**Electroconvulsive Therapy (ECT)**
272
What 2 congenital conditions cause ventricular system obstruction?
1) Acqueductal stenosis 2) Dandy-Walker malformation
273
Dysthymic disorder can be more difficult to tx what are some options?
**CBT** + **Pharm**: SSRIs, SNRIs, MAOIs
274
**Apomorphine** uses
Injected subcutaneously for quick, temporary relief of off-periods of akinesia in patients on dopaminergic therapy (clinical benefits within 10 minutes)
275
What are some of the common AE's associated with the second gen. antipsychotics?
**Weight gain** and **Metabolic Sydrome**: hyperglycemia/insulin resistance, hyperlipidemia
276
MOA memantine
* *antagonist of NMDA type glutamate receptor**
- binds to the intra-pore magnesium site, blocking the NMDA receptor from being activated
(glutamate may contribute to pathogenesis of Alzheimer's dz)
277
What is pseduogout? And where does it deposit? And what does Xray show?
**Calcium pyrophosphate deposition** (Pseudogout)
– **Hands/knees**
– **X-rays show cartilage calcification** (chondrocalcinosis)
278
sensory C8
4th + 5th digit
279
weakness in elbow flexion seen in:
C6
280
sensory symptoms of peripheral nerve dz have **pain/burning** sensations..these are secondary to what?
small unmyelinated fiber dz (w/ dysesthesia, hyperalgesia, hyperpathia)
281
Why is the cortex not typically affected in medullary infarcts (osteonecrosis)?
Due to its **collateral** blood flow
282
if a pt has myasthenic crisis, what should you do?
stop anticholinesterase meds and use PLEX, IVIg
283
In practice, will oftens see elements of which type of edema(s)? What morphological characteristics of the brain will be seen and this can lead to?
- Both vasogenic and cytotoxic edema - Gyri flattened, sulci narrowed, and ventricles compressed ---\> Herniation!
284
define positive reinforcement and give an example
adding something to increase a behavior
ex: giving candy to a kid so they keep cleaning their room
285
What is the MOA of Naltrexone?
- µ-opioid receptor antagonist (long-acting)
- ↓ craving for alcohol and the rate of relapse to either drinking **alcohol dependence for the short-term** (12 weeks)
286
elbow, wrist, and finger EXTENSION weakness in:
C7
287
How can non-adherence to antipsychotic meds be managed; which 6 agents can be used? \*test Q\*
## Footnote
- Manage w/ **L**ong-**A**cting **I**njectable **A**gents (**LAIA's**)
- **Haloperidol** decanoate
- **Fluphenazine** decanoate
- **R**isperidone **+ O**lanzapine **+ A**ripiprazole **+ P**aliperidone (**ROAP**)
288
What type of signs/sx's seen with ALS?
- **_Mixed upper_** (_spasticity_, _hyperreflexia_, _Babinski sign_)
and
- **LMN** (_atrophy_, _fasciculations_) signs
289
list the immediate release amphetamine based stimulants used to treat ADHD
290
Criteria for SLE as an FYI
* Malar rash – Erythema, malar eminence spare nasolabial folds (butterfly rash)
* Discoid rash – Erythematous patches • Photosensitivity – Rash due to sunlight
* Oral ulcers – May include nasopharyngeal ulcers; usually painless • Arthritis – 2 or more peripheral joints, tender, swelling/effusion; non erosive arthritis • Serositis – Pleuritis, rub or plerual effusion or pericarditis, ECG changes, rub or pericardia effusion
291
What is Cytotoxic edema and when is it seen?
- Increase in INTRAcellular fluid secondary to neuronal, glial, or endothelial cell membrane injury - Generalized hypoxic/ischemic insult or w/ metabolic derangment
292
Secondarily generalized seizure
symptoms that are initially associated with a preservation of consciousness that then evolves into a loss of consciousness and convulsions
293
common sites of radial mononeuropathy
axilla-crutch palsy
humerus/spiral groove- saturday night palsy (MC)
supinator (posterior interosseous branch)
wrist (superficial radial sensory branch)
294
How is the Phase II desensitizing block by Succinylcholine reversed?
Acetylcholinesterase inhibitors
295
Diagnosis of major depressive **disorder** requires what?
Presence of **one or more** major depressive **episodes** and the **_absence_** of any manic, hypomanic, or mixed episodes
296
Which imaging modalty is the best for bony abnormalities (trabecular, cortical bone), erosions, fractures, degenerative or inflammatory arthritis?
**CT**
297
What is the classic triad of clinical findings exhibited by patients with normal pressure hydrocephalus?
- "Wet, wacky, and wobbly" - Often exhibit a magnetic gait - Dementia-like sx's; impaired recognition (often not severe) - Urinary incontinence; appears late in illness and is generally of the spastic hyperreflexic, increased-urgency type
298
How does Ewing Sarcoma typically present?
- **Painful** enlargin mass; frequently **tender, warm, and swollen**
- Some have **systemic findings** that _mimic_ **infection** --\> including **fever, ↑ ESR**, anemia, and leukocytosis!
299
what impairment seen with large myelinated sensory fibers in peripheral nerve disease?
```
light touch (cotton swab)
two point discrimination
vibration (128 hz tuning fork)
joint position sense
```
300
which dementia drug comes as a once-daily tablet and a once-daily disintegrating sublingual tablet
donezapil
301
Extensive ischemic damage of both white and gray matter leading to large cystic lesions during the perinatal period is known as?
Risk factor for developing?
- Multicystic encephalopathy
- Cerebral Palsy
302
list the **sustained release** methylphenidate-based stimulants used to treat ADHD
- ritalin SR
- desoxyn
303
what part of the brain is affected if the patient has ataxic respirations
medullary respiratory centers
304
Osteopetrosis is also known as what?
**Marble bone disease** and **Albers-Schonberg disease**
305
Typical presenting sx's of (progressive) spinal muscular atrophy?
- Often begins with **symmetric upper extremity involvement**
- **Weakness + atrophy + respiratory difficulty**
306
In terms of clinically isolated syndrome (CIS) what is a monofocal episode as a differential diagnosis for MS?
Person experiences a **single** neurologic sign or symptom that's causes by a **single lesion** (i.e., optic neuritis in one eye)
307
The homogenous pattern of ANA is indicative of what antibody?
**Histone antibody** \>95% drug induced lupus
308
Consciousness depends on arousal of the cerebral cortex by the \_\_\_\_\_\_\_\_\_\_\_\_
**Brainstem** Ascending Reticular Activating System **(ARAS)**
309
pain in ant lat thigh, knee, and medial calf seen in:
L4
310
What does the presence of Homer-Wright rosettes in Ewing Sarcomas indicate?
A greater degree of **neuroectodermal differentiation**
## Footnote
**(PTEN)**
311
Anti-DS DNA antibody is seen in 50% of what? How is ANA?
**SLE**
ANA sucks and even normal patients can have it elevated
312
what form does dexedrine come in
capsule
XR amphetemine
313
incidence of Myasthenia gravis happens in who?
younger women and older men; most sporadic
314
When is Mycobacterial osteomyelitis detected?
What are the clinical symptoms of it?
Could go **years** undetected
Sx: Low grade fever, localized pain, chills, and weight loss
315
Which markers, if positive, have a 99.5% specificity for RA?
**Anti-CCP + RF (+)**
316
Charcot-Marie-Tooth neuropathy type 1A main info:
MC- demyelinating
AD
chromsoome 17
pes cavus
distal symmetric atrophy (Legs\>arms)
onset 1st and 2nd decade
**EMG: slowing of motor nerve conduction velocities (demyelination)**
317
How many signs/sx's must be present for diagnosis of a panic attack and how long do they last?
- Discrete period of intense fear or discomfort, in which **≥4** of the following developed abruptly: **Palpitations, sweating, trembling, sensation of SOB, chest pain, dizziness, fear of losing control/dying, parasthesias, chills or hot flashes**
- Reach **peak** within **10 mins** and usually last **\<25 mins**
318
Unexplained fever vs. localized pain are more common findings of osteomyelitis in which age group (adults or children)?
- **Unexplained fever** = **children**
- **_Localized pain_** = **adults**
319
what extended release methylphenidate-based stimulants used to treat ADHD come in capsule form
- aptensio XR
- focalin XR
- metadate CD
- ritalin LA
320
What are the AE's associated with bisphosphonates?
- **Esophagitis**: minimized by taking meds with 8oz of water
- **Osteonecrosis** of the **jaw**
- **Atypical femur fractures**
321
pain in dorsal thigh, lat calf seen in
L5
322
What is the clinical indication for using Pegloticase?
Tx of **chronic gout** in those _refractory_ to conventional therapy
323
What are the **essential** elements of the neurological examination for a patient with stupor?
- Pupillary responses
- Corneal reflex
- Extaocular movements
- Cough/gag reflex
- Motor responses
- Respiratory pattern
\*Start at highest CN and work your way down
324
What are two frequent pathogens for osteomyeltits?
***H influenzae***, ***group B strep*** (agalactiae)
325
What is the difference between a tonic and a clonic seizure?
- Tonic = Muscle stiffness, rigidity (think increased tone)
- Clonic = Repetitive, jerking movements
326
What is the prognosis of patients with NMDA encephalitis?
Although these patients can present with severe deficits, many will improve with aggressive treatment, though improvement may take a long time (e.g. 1 year)
327
what presents with a stocking/glove sensory loss?
polyneuropathy (diffuse symmetrical usually)
328
What is the characteristic appearance of old traumatic lesions on the surface of the brain?
Where is the morphology most commonly seen?
- Depressed, retracted, yellowish-brown patches involving the crest of the gyri (plaque jaune)
- Most commonly located at the sites of countercoup injuries
329
How does Vestibular Neuronitis differ from BPV?
- Spontaneous attack of vertigo, typically lasting up to 2 weeks (findings similar to BPV)
- But is **NOT** typically positonal
330
When CIS patients have multiple demyelinating lesions on MRI, they have a \_\_\_\_\_\_\_\_\_\_% chance of developing MS within several years
60-80%
\*High risk
331
Epsidural hematoma etiology and features?
Lens shaped
RAPIDLY DEVELOPING
Middle meningeal artery (arteries)
332
Pathway of ethanol metabolism?
Ethanol -\> **Alcohol dehydrogenase** -\> Acetylaldehyde -\> **Aldehyde dehydrogenase** -\> Acetic acid
333
describe how nicotine can affect the adolescent brain
nicotine interferes with the maturity of the prefrontal cortex --\> affects its neuroplasticity --\> leads to alterations in:
- gene expression
- cell structure
- intracellular signaling
- synaptic pruning
- axon myelination
\*- memory and attention
334
What type of syndrome can be caused by Vitamine E deficiency?
Spinocerebellar similiar to **Friedrich's**
which is
- Gait ataxia w/ absent tendon relfexes in legs and muscle weakness
- Extensor plantar responses
- Pes cavus
- Kyphoscoliosis
335
A subfalcine herniation may lead to compression of the ________ artery leading to infarction
Anterior Cerebral artery (ACA)
336
What is the tx for PTSD?
- **SSRIs** and **Venlafaxine**
- **CBT**
337
Which deficits predominate in (progressive) spinal muscular atrophy; mean age of onset?
- Mean age = **64 y/o**
- **LMN (** focal weakness, muscle atrophy, decreased muscle stretch reflexes and tone) **deficits predominate** due to degeneration of **anterior horn cell**
- **NO upper motor neuron involvement**
338
What is the prototype ultra-short acting barbituate used in anesthesia?
Thiopental
339
Convergence nystagmus or Retractory nystagmus indicates a lesions where?
Mesencephalon
340
Herniation is displacement of brain tissue due to _____ or \_\_\_\_\_\_
Mass effect or Increased intracranial pressure
341
What age does Oculocutaneous telangiectasia usually appear?
Common findings?
- Usually appears in teen years
- Immunological impairment (decreased IgA and IgE) usually evident later on and manifested by **recurrent sinopulmonary infections**
- Changes of skin and hair, hypogonadism, and insulin resistance
342
an autoimmune attack against what occurs in lambert-eaton myasthenic syndrome (LEMS)?
**voltage-gated calcium channels**
343
describe classical conditioning to treat phobias
relaxation activity (CS) + object of phobia (UCS) --\> relaxation (UCR)
object of phobia (CS) --\> relaxation (CR)
344
What is the location of the lesion if the eyes have a **ping-pong nystagmus**?
Bihemispheric, midbrain
345
MOA sumatriptan
5-HT agonist
346
What lab value will be extremely elevated for DMD?
CK
347
in the caloric test, what happens with cold and hot bilateral irrigation
cold: the eyes deviate downward
hot: eyes deviate upward
348
What must be ruled out before making the call that someone is brain dead?
- Sedative intoxication
- Hypothermia (\<90F)
- Neuromuscular blockage
- Shock
\*All can mimic brain death\*
349
What is the inheritance pattern of the severe infantile form of osteopetrosis and what are the clinical features?
- **_Autosomal recessive_**
- Usually evident in utero or soon after birth
- **Fracture**, **anemia**, and **hydrocephaly** --\> post-partum mortality
- Those who survive have CN defects: **optic atrophy**, **deafness**, and **facial paralysis**
350
What is the prognosis of (progressive) spinal muscular atrophy?
Survival rate ≥_15 years_ (**better w/ earlier age of onset**)
351
Where do **alveolar** rhabomyosarcomas arise?
Where do **embryonal** rhabomyosarcomas arise?
Both arise in **kids** and usually found growing in **siuses**, head and neck, and **GU tract (especially vaginas)**
**Grape like appearance\***
352
DTR loss in triceps seen in
C7
353
What are the (**3)** Cholinesterase inhibitors antagonize nondepolarizing blockade by increasing amount of ACh at NMJ?
**Neostigmine**
**pyridostigmine**
**edrophonium**
354
list the anti-hypertensives for attention
- clonidine/kapvay ER
- guanfacine/intuniv ER
355
how does stupor and coma affect the oculocephalic maneuver
moving head side to side: eyes move with head turn
moving head up and down: eyes move with head movement
356
What is an important prognostic finding associated with tx of Ewing Sarcoma?
Amount of **_chemotherapy-induced necrosis_**
357
Formulation of amphetemines:
Adzenys XR-ODT
3:1 ratio of d-amphetamine & L amphetamine isomers
358
what extended release methylphenidate-based stimulants used to treat ADHD come in chewable tablet form
**\*quillichew\***
359
What is a tonsillar herniation and why is it life threatening?
- Displacement of the cerebellar tonsils through the foramen magnum
- Can can brainstem compression and compromises vital respiratory and cardiac centers in the medulla
360
How are the OA affected joints characterized? How's the ROM?
The finding most consistent with the diagnosis of osteoarthritis is a **cool joint effusion**
The effusion is typically **not** warm to touch or shows erythema over the site
Diffuse MCP involvement or thoracic spine pain **are not** typical features of OA.
**Normal range of motion is not a likely finding in OA.**
361
What is Rheumatoid arthritis?
Autoimmune, chronic systemic
* Inflammatory disease, symmetrical
* Targets synovial tissues, diarthrodial joints
* Polyarthritis, extra-articular features
* Idiopathic
362
Complex partial
pt loses consciousness
363
What are the symptoms of a stroke occuring in the left hemisphere?
- **Aphasia** (Loss of ability to understand/express speech)
- Right sided sensory and motor symptoms
- Right visual field cut
364
define the unconditioned stimulus and response and the conditioned stimulus and response in the pavlov's dog example
UCS: meat powder
UCR: salivation
CS: bell ringing
CR: salivation
\*unconditioned response and conditioned response are always the same\*
365
How many episodes and for what duration must they occur for diagnosis of Binge Eating Disorder?
Occur on average **1x/week** for **≥3 months**
366
What is the inhertance pattern for DMD?
X linked recessive
367
In which type of RA will rheumatoid factor (RF) be present 100% of the time? How about early/regular RA?
**"Nodular" RA is 100%**
70% patients with regular RA
368
Fibrous dysplasia is due to mutations in what?
Gain of function somatic mutation ***GNAS1***
369
other tests can perform on myasthenia gravis
tensilon test - ptosis typically disappears after injection (not for long); atropine = antidote to side effects (bradycardia)
ice bag test - apply to ptotic lids for 2 mins and can see 2 mm improvement (+)
370
Describe the Gower maneuver and discuss its clinical significance
Clinically, it is a sign of severe **proximal muscle weakness**
First legs have to be pulled up under the body and the weight shifts to hands and feet
The hips are then thrust into the aire as the knees are straightened and the hands are brought close to the legs
They extend their trunk by the hands and get up
371
What occurs if cholinesterase inhibitors are given during the phase I depolarizing block of Succinylcholine?
**Potentiate** the block; not reversal
372
How and when does skeletal syphillis (T. Pallidum and T. Pertenue) present when congenital?
- Appear about **5th** month gestation and _fully developed_ at birth
- **Saber shin**: massive reactive periosteal bone deposition on **medial** and anterior surfaces of the tibia
373
Regardless of etiology, medullary infarcts (osteonecrosis) are geographic and involve which parts of bone?
**Trabecular** bone & **marrow**
374
what form does atomoxetine/strattera ER come in
capsule
375
Bisphsphonates are structural analogs of what; what is their MOA?
- Structural analogs of **pyrophosphate**, normal component of bone
- Incorporated into bone, then **_inhibit_ bone** **resorption** by **↓↓** both the _number_ and _activity_ of osteoclasts
376
Perinatal ischemic lesions of the cerebral cortex leading to thinned-out, gliotic gyri is known as?
Ulegyria
377
Sellegeline uses
1. slows the breakdown of dopamine and prolongs the antiparkinsonian effects of levodopa; **may reduce mild on-off or wearing-off phenomena**; adjunctive therapy in patients with declining or fluctuating response to levodopa
378
A disorder of the brain and spinal cord characterized by a tendency for periods of increasing and decreasing symptoms and signs (exacerbation and remissions), which result from loss of myelin at multiple sites in the CNS, defines what?
MS
379
What is diffuse vs. focal mass effect that is associated with herniation?
- Diffuse: generalzied brain edema - Focal: tumors, abscesses, or hemorrhages
380
what is the oculocephalic maneuver?
doll's eye
- passive horizontal head rotation --\> eyes move horizontally opposite
- passive vertical head rotation --\> eyes move vertically opposite
381
What is a big difference about patients perceptions of their actions with OCPD vs. OCD?
- **OCPD**: pt does **NOT** perceive they have a problem
- **OCD**: they know their compulsions and obsessions are not reasonable (most of the time)
382
Stress incontinence antidepressant
duloxetine
383
Ewing sarcoma is most often seen in which age group and has a predilection for which ethnicity?
- 80% arise in pt's **\<20 y/o** with slightly more **males** affected
- Striking predilection for **whites**, while _blacks/asians_ rarely affected
384
What is a secondary generalized (partial onset) seizure (symptoms, consciousness, timeline, post-ictal)?
- Starts on one side of brain and then progresses to **bilateral tonic-clonic activity**
- Loss of consciousness
- Lasts 1-3 mins
- Post-ictal state
385
What is the Erosive subtype of OA; seen most often in whom?
- Affects **DIP** and **PIP** joints; more painful than typical hand OA
- More common in **women**
386
sensory nerve action potentials in plexopathy vs. radiculopathy
```
plexopathy = abnormal
radiculopathy = normal
```
387
Which 2 second-gen antipsychotics have the least amount of AE's with it?
**Aripiprazole** and **Ziprasidone**
388
Which 4 drugs can be used in the tx of acute alcohol withdrawal syndrome?
Diazepam
Lorazepam
Oxazepam
Thiamine
389
what form does methylin come in
**\*liquid\***
390
What is the only second-gen anti-psychotic that is assoc. with hyperprolactinemia (like the first-gen's)?
## Footnote
**Risperidone**
391
Guillan barre treatment
plasmapharesis, IVIG
392
what form does Zenzidi (dextroamphetamine) come in?
Tablet
IR Amphetamine
393
What is seen commonly with epiphyseal infection (osteomyelitis) in infants?
Spread thru the **articular surface** or **along capsular** and **tendoligamentous insertions** into joints ---\> **septic** or **suppurative arthritis**
394
MOA aspirin
irreversible Inhibition of COX
395
Etiologies of Presyncope?
What can make it worse?
- Arrythmia, hypotension, vasovagal excess, pulmonary emboli, drugs
- Aggravated by: increased temperature, prolonged standng, large meals, and deconditioning
396
What are the two genetic abnormalities that lead to DMD? And what is the pathogenesis behind this?
1) Frameshift mutation in the dystrophin gene
2) Dystrophin is absent or totally non functional
Dystrophin occurs nearly everywhere in the body, without it muscle membrane tears, necrosis occurs, fibrosis develops, etc
397
XR-ODT %eges
50% IR & 50% XR
398
Which benzodiazepine has the fastest rate of onset?
Diazepam
399
What are the OA bone characteristics?
Altered **chondrocyte** function
**Loss/thinning of cartilage**
Subchondral **bone thickening** (sclerosis)
**Remodeling** of bone
**Marginal spurs** (osteophytes)
**Cystic** changes to subchondral bone
Mild reactive **synovitis**
400
What joints are affected in OA? And how is the pain characterized?
OA usually affects **weight bearing joints** and frequently used joints; **hips**, **knees**, **spine**, hands (**DIP**, **PIP**, 1st CMC – thumb base)
**Pain worse with activity**; alleviated with rest
• Morning stiffness (about 30 minutes)
401
What is the most effective tx for depression and is often used in tx resistant cases?
**Electroconvulsive Therapy (ECT)**
402
What is the seen in the acute phase of osteomyelitis?
Bacteria proliferate and induce **neutrophilic** inflammatory rxn; **necrosis** of bone cells and marrow ensues within first **48 hours**
403
Prognosis of ALS?
* Relentlessly progressive disease without remissions, relapses, or stable plateaus.
* Death from respiratory failure, pneumonia (aspiration), pulmonary embolus.
* Mean duration of symptoms 4 years (27-43 months).
* Death within 2 to 5 years.
404
Anticentromere antibody is for what?
Scleroderma **CREST**
Calcinosis, raynauds, esophageal dysmotility, sclerodactyly, and telangiectasia
405
what is the rule of thumb for anisocaria?
if it's the large pupil --\> should fail to constrict to light
if it's the small pupil --\> should fail to dilate in the dark
406
PNS signs
LMN: flaccid one, atrophic, sometimes fasciculations, hypoactive DTRs, no plantar reflex; hands and feet sensory loss
407
what form does ritalin come in
tablet
408
in brainstem lesions, were does the eye deviate towards with destructive lesions
away from the lesion
409
Enlargement of the entire ventricular system ("symmetric dilation") due to accumulation of CSF not being properly absorbed at the dural sinus level is known as?
Communicating ("non-obstructive") hydrocephalus
410
**sensory symptoms** of peripheral nerve dz have **paresthesias**...these are secondary to what?
large myelinated fiber dz (pins and needles)
411
What are the characteristics of Meniere's Disease and who is most often affected?
Hallmarks?
- Recurrent episodes of spontaneous vertigo, lasting minutes to hours
- **Low frequency hearing loss** = **Hallmark**
- Tinnitus and **aural fullness**
- Woman are 3x more affected
412
Which predisposing factor incrases the risk for severe RA?
**Genetic factors: HLA-DRB4/ DRB 0401/4**
413
if you have a paraneoplastic etiology of peripheral neuropathy it is most likley
pure sensory neuropathy (dorsal ganglionopathy)
414
What is the prototype long-acting barbituate that is used for seizures?
Phenobarbital
415
Treatment for Benign Postional Vertigo?
- Often resolves on its own within a few weeks
- Positional exercises helpful: **Sermont manuever**
- Meds such as: vestibular suppressants, antiemetics, and anxiolytics
416
what form does Evekeo (amphetamine sulfate) come in?
capsule
| (IR Amphetamine)
417
Most cases of osteonecrosis (avascular necrosis) are due to what 2 etiologies?
- **Fractures** or **corticosteroid** tx
- May also be seen w/ **bisphosphonate tx** (especially jaw!)
418
Impaired consciousness means involvement of what 3 things?
1. Diffuse or bilateral impairment of both cerebral hemispheres, or
2. Failure of brainstem ARAS, or
3. BOTH
419
describe how behavioral therapy for phobias work
pairing relaxation with a feared stimulus to induce calming
feared stimulus + relaxation --\> decreased anxiety
feared stimulus --\> relaxation
420
Synovial inflammation with hypertrophy and effusion is seen with what?
Osteoarthritis
421
define positive punishment and give an example
adding something to decrease a behavior
ex: child kneels on rice to decrease lying
422
Rule of thumb for lesion location when pupils are constricted?
**Sympathetic** division (hypothalamus, carotid)
423
What can mimic infectious encephalitis and what is the time line like for that entity?
**Autoimmune encephalitis** should be considered in patients with rapidly progressive (usually \< 6weeks) encephalopathy or psychiatric disturbance, especially if **seizures** also present. Fever may or may not be present. Some of these entities have overlap with paraneoplastic syndromes and may be associated with tumors.
424
What labratory studies will be abnormal in NMDA encephalitis?
**At least one** of the following laboratory study results:
– **Abnormal EEG** (focal or diffuse slow or disorganized activity, epileptic activity, or extreme delta brush)
– **CSF with pleocytosis** or **oligoclonal bands** and/or **NMDA receptor antibodies**
425
What is the tx for normal grief (bereavement)?
**Grief counseling**; do NOT give them antidepressants
426
onset of activity, what patients, and schedule for non-stimulants
onset: 1-4 weeks
patients: those intolerant of stimulant effects or those resistant to using stimulants
- schedule: non-scheduled, refills and samples allowed up to 1 year
427
What are the 5 first-gen. aka typical/conventional antipsychotics?
## Footnote
- Chlorprom**azine** (low-potency)
- Fluphen**azine** (high-potency)
- Thiorid**azine** (low-potency)
- Haloperidol (high-potency)
- Thiothixene (high-potency)
428
Which intracranial location is most susceptible to a direct parenchymal injury which results from trauma to the head?
Crests of the gyri = greatest amt. of force
429
list the immediate release methylphenidate-based stimulants used to treat ADHD
- focalin
- methylin
- ritalin
430
Subfalcine herniation involves displacement of the _______ under the \_\_\_\_\_\_\_
Cingulate gyrus under the falx cerebri
431
What are the 3 high-potency first gen. antipsychotic agents and what AE's are they more associated with?
## Footnote
- **Fluphenazine** + **Haloperidol** + **Thiothixene**
- _More_ **movement (EPS)** and **endocrine effects** (**prolactin**)
432
what extended release methylphenidate-based stimulants used to treat ADHD come in transdermal patch form
**\*daytrana\***
433
What is seen in the chronic phase (after first week) of osteomyelitis; what does the term involucrum refer to?
- **Chronic** inflammatory cells release **cytokines** that stimulate **osteoclastic** resorption, ingrowth of fibrous tissue and deposition of reactive bone at the periphery
- **Newly deposited bone** can form a **shell** of living tissue, known as **_involucum_**, around the segment of devitalized infected bone
434
Secondary injury associated seen in subarachnoid hemorrhage is often associated with what?
Etiology?
Vasospasm
Arterial venous malformaiton/aneurysms
435
What are the criteria for a hypomanic episode?
- Similar to a _manic episode_ but is **less severe**
- Episodes last **≥4 consecutive days** and **_no_** psychotic features
436
Which deficits prevail in Primary Lateral Sclerosis; what are the signs/sx's?
- **Upper motor neuron (corticospinal)** deficits prevail
**- Weakness, spasticity, hyperreflexia, Babinski signs**
Babinski: when the big toe bends up and back to the top of the foot and the other toes fan out
437
What is the histo like for mycobacterial osteomyelitis?
**Caseous** necrosis and **granulomas**
438
Which first gen. antipsychotic is associated with more prolactin elevation, sedation, anticholinergic effects, orthostatic hypotension and a dose-dependent retinitis pigmentosa?
## Footnote
**Thioridazine**
439
Which state of altered consciousness is represented by disorientation, stimuli misinterpretation, and hallucinations (visual)?
Delirium
440
A sense of impending loss of unconsciousness often associated with pallor, sweating, visual dimming or constricted fields, is known as?
Presyncope
441
What are the 3 P's associated with pinpoint pupils?
1) **P**ontine lesion
2) o**P**iates
3) **P**ilocarpine
442
Which underlying GI disease can lead to secondary OA; which joints most often affected?
- **Hemochromatosis**
- **2nd/3rd MCP joints and wrist**
443
EMG findings of Myasthenia gravis
decremental response on repetitive stimulation; increased "jitter" on single fiber EMG
444
diagnostic criteria for bulimia nervosa
- recurrent episodes of binge eating
- recurrent inappropriate compensatory behavior to prevent weight gain
- events at least 2x/week for three months
445
What are the criteria for persistence of brain death?
- **6 hours** w/ confirmatory (flat) EEG: performed to tech. standards of AEES
- **12 hours** w/o a confirmatory isoelectric EEG
- **24 hours** for anoxic brain injury w/o confirmatory isoelectric EEG
446
**tolcapone** and **entacapone MOA**
1. COMT metabolizes levodopa to 3-*O*-methyldopa, which competes with levodopa for transport across the intestinal mucosa and the blood-brain barrier
2. COMT inhibitors (**tolcapone** and **entacapone**) prolong the activity of levodopa by inhibiting its peripheral metabolism, which decreases clearance and increases bioavailability
447
What is osteonecrosis?
What part of the bone does it involve?
**Infection** of the bone and marrow
Medullary cavity or involves the medulla and the cortex
448
What is seen with an Atonic seizure, duration, and how does patient feel after?
- Sudden loss of muscle tone --\> head drops or patient collapses
- Loss of consciousness
- Variable duration
- Post-ictal state
449
**Apomorphine** MOA
dopamine agonist at dopamine D2 receptors
450
The syndrome associated with Copper deficiency can present very similar to what?
B12 deficiency
451
giving predisnone for myasth. gravis
is a treatment but make sure to give high initial dose ; can exacerbate
452
What are the 2 low-potency first gen. antipsychotic agents and what AE's are they more associated with?
## Footnote
- **Chlorpromazine** and **Thioridazine**
- _More_ **sedation, hypotension**, and ↓ **seizure-threshold**
453
what nerves are tested in the corneal test
5 and 7
454
Pathway of methanol metabolism?
Methanol -\> **Alcohol dehydrogenase** -\> Formaldehyde -\> **Aldehyde dehydrogenase** -\> formic acid
455
What are some of the rare AE's associated with the first and second gen. antipsychotics?
## Footnote
- **QTc prolongation** and **negative inotropic effects** --\> ↑ risk for women, elderly and those on anti-arrhythmics
- **Seizures**: with first gen.
456
Ewing Sarcoma is a malignant bone tumor characterized by what?Primitive round cells without obvious differentiation
Primitive **blue round cells** without obvious differentiation
457
Treatment of LEMS
first tx/look for malignancy
AChE inhibitors
Amifampridine
immunosuppression
IvIg
458
Which genetic aberrations and translocations are associated with **alveolar** rhabdomyosarcomas?
Fusion of **FOXO1** with either **PAX3** = **(2;13)** or **PAX7 = (1;13)**
459
Characteristic morphology of fibrous dysplasia includes what?
**Curvilinear shapes** of the **trabeculae of woven** **bone** mimic **_Chinese characters_** and bone lacks prominent osteoblastic **rimming**
460
What lab values are indicative of BMD?
CK at diagnosis is usually elevated…but not as reliably as it is in DMD
461
Which first generation antipsychotic is more often associated with weight gain, DM, and hypercholesterolemia?
## Footnote
**Chlorpromazine**
462
Lambert-Eaton: presyn, synaptic, or postsyn?
Presynaptic
463
MOA non-stimulants for ADHD treatment
enhance neurotransmitter transmission via:
- inhibit NE pre-synaptic reuptake
- agonist of CNS alpha 2A adrenergic receptors
464
what form does Vyvanse come in
capsule
XR amphetemine
465
What can cause B12 deficiency?
- Malabsorption syndromes
- Surgery
- Drugs (H2 receptor antagonists)
- **Nitrous oxide use** (whip-its!!!)
- Fish tapeworm
466
What is the criteria for diagnosis of a Manic Episode?
- Abnormally and persistently elevated, expansile, or irritable mood lasting **_at least 1 week_** with at least **3** of the following:
- Manics **DIG FAST**
**D- Distractability**
**I- Impulsive**
**G- Grandiosity**
**F- Flight of ideas**
**A- Agitation**
**S- Decreased need for sleep**
**T- Talkative**
467
EMG/NCV tool:
can broadly classify into axonal or demyelinating; rarely leads to specific dx; may provide localization of particular nerves, may provide indication of severity
468
what form does ritalin SR come in
tablet
469
indications ergotamine
drug of choice for terminating ongoing migraine attack
470
what impairment seen with small unmyelinated sensory fibers in PND?
```
temperature perception
pain perception (pin prick)
```
471
describe the caloric stimulation test and what nerves it tests
COWS
1) pour cold water in left ear --\> eyes should slowly move to right and quick movement to the left (left nystagmus)
2) pour cold water in right ear --\> eyes should slowly move to left and quick movement to the right (right nystagmus)
coma pt: eyes will deviate away and then stay there
nerves 8 (stimulation from the water to the brainstem), 6 (abducting the eye), 3
472
finger abduction and finger FLEXION weakness seen in
C8
473
What are 3 possible consequences of progression of transtentorial herniations?
1) Compression of CN III ---\> pupillary dilation; eye is "down and out" (ipsilateral to lesion) 2) Compression of PCA ---\> ischemia of primary visual cortex 3) Large herniation may compress contralateral cerebral peduncle ---\> hemiparesis ipsilateral to side of herniation = Kernohan notch = "false localizing sign"
474
MOA dihydroergotamine
blocks inflammation associated w/ trigeminal vascular system and suppresses release of CGRP
475
what is apneustic breathing and in what conditions is it seen
476
Alcohol is metabolized via what type of kinetics?
Zero-order kinetics; rate of metabolism remains constant and is independent of concentration or amount of chemical
477
Treatment of multifocual motor neuropathy
IVIG
478
What are specific interventions that can be done to reduce ICP?
- Elevate head of bed
- Intubate and hyperventilate to PCO2 of 20 mmHg
- Use **mannitol** for **ischemic lesions**
- Use **decardron** for **tumor, abscess**, and perhaps cerebral hemorrhage
479
The **pathophysiology** of ALS is due to degeneration of which 4 things?
- **Betz cell**
- **Lower brainstem nuclei**
- **Descending corticospinal tracts**
- **Anterior horn cells**
Etiology unknown
480
What is the origin of most osteomyelitis in both healthy children and adults?What is the origin of most osteomyelitis in both healthy children and adults?
- Children: **hematogenous** spread from trivial **mucosal** injuries i.e., defecation or vigorous chewing of hard foods or from minor infections of skin
- Adults: more often arise as complication of **open fractures**, **surgical procedures**, and **diabetic infections** of the feet
481
cholinesterase inhibitors are contraindicated in what patients
those with baseline bradycardia or known cardiac conduction system disease (sick sinus syndrome, incomplete heart block)
--\> due to risk of syncope, falls, and fractures
482
sensory C6
1+2nd digit; lateral arm
483
A positive Romberg test indicates what?
- A somatosensory dysfunction (proprioception)
\*With the eyes open, three sensory systems provide input to the cerebellum to maintain truncal stability. These are vision, proprioception, and vestibular sense. When pt closes eyes during Romberg test you remove that visual sense.
484
What would an Xray for RA do?
How about CT?
X-rays of hands and feet – **detect symmetrical involvement of MCP/MTP joints**; erosions
• CT – more sensitive detecting **erosions**
485
What is the inheritance pattern for Becker's Muscle Dystrophy?
X linked recessive
486
Traumatic Brain Injury (TBI)
associated diminished or altered state of consciousness. Brain function is temporarily or permanently impaired and structural damage may or may not be detectable with current technology from repeated head blows from external mechanical forces
487
What are 3 types of Parial Seizures?
1. Simple partial
2. Complex partial
3. Secondarily generalized (partial onset)
488
What is commonly seen preceding brainstem dysfunction (4 D's) due to a subtentorial mass lesion?
1. Dysequilibrium
2. Dysarthria
3. Dysphagia
4. Diplopia
\*Vertigo
489
What defines unresponsivness in someone who is truly brain dead?
Absence of?
- Unresponsive to **ALL** sensory input, including pain and speech
- **Absent brainstem reflexes**
490
How does the the levels of Rheumatoid Factor (RF) correlate with a disease process?
**High levels** associated with **aggressive dz**, **joint erosions**, and **worse prognosis**
491
What is typical clnical presentation of ALS?
Age at onset 20-60 years, most common after age of **50**. First sign often hand clumsiness or impaired dexterity with mild **wasting/weakness of hand intrinsics**. Eventually, other hand/arm become involved. **Weakness/atrophy spreads proximally in arms**. Before long, legs become similarly affected. Later, **atrophic weakness spreads to tongue, pharynx, and muscles of respiration**. Accompanying symptoms include **fasciculations**, cramps, drooling, weight loss.
492
What is Hydrocephalus Ex-Vacuo? Whom is it seen in? What is the CSF pressure?
- Compensatory increase in ventricular volume secondary to loss of brain parenchyma - Atrophy with increasing age, stroke or other injury, chronic neurodegenerative disease - CSF pressure is NORMAL!!!
493
Botulism is what kind of paralysis?
descending
494
What is the tx for Cyclothymic Disorder?
- **Mood-stabilizing drugs**
- **Supportive psychotherapy**: CBT
495
MOA ergotamine
blocks inflammation associated w/ trigeminal vascular system and suppresses release of CGRP
496
What antibiotics are usually given for meningitis?
**Vancomycin** plus a third generation **cephalosporin**
497
**Selegiline MOA**
**Selective irreversible MAO-B inhibitor** (inhibits MAO-A at high doses); slows the breakdown of dopamine and prolongs the antiparkinsonian effects of levodopa
498
how does nicotine use in adolescence affect health later in life
can cause behavioral disturbances later in life such as substance abuse and mental health problems
499
Most common cause of recurrent vertigo and characteristics?
- Benign Positional Vertigo
- Brief recurrent episodes of vertigo triggered by **changes in head positon**
500
Large, pressure producing supratentorial mass lesions can cause coma how?
- Dysfunction in the **upper ARAS**
- Downward herniation of the brain to **compress the ARAS**
501
In terms of clinically isolated syndrome (CIS) what is a multifocal episode as a differential diagnosis for MS?
AKA?
- aka Acute Disseminated Encephalomyelitis (ADEM)
- Person experiences **more than one** sign or symptom caused by lesions in **more than one place** (i.e., optic neuritis in one eye plus hemiparesis)
502
What are the sensory sx's seen with ALS?
How is the bladder affected in ALS?
How are the eyes in ALS?
**NONE**
No extraocular involvment
Bowel/bladder normal
503
What is fibrous dysplasia and what has it been linked to?
- Benign tumor likened to **localized developmental arrest**
- **ALL** components of **normal bone present**, but they **do not differentiate** into mature structures
504
How does ESR change with age? And what's it mainly used for?
Rises with age and used to **monitor disease activity/progression** especially polymyalgia rheumatica and GCA
505
Rheumatoid Factor (RF) can positive in which conditions?
- Healthy pt's and pt's \>60 y/o
- **Sjogrens syndrome** and **SLE**
- Sarcoidosis, malignancy, and lung disease
- **Cryoglobulinemia**
- **Primary biliary cirrhosis**
- **Mixed CT diseases**
506
What is the most common cause of noncommunicating (obstructive) hydrocephalus in the neonate/infant?
Aqueductal stenosis
507
indications for donazepil
treatment of dementia of the Alzheimer's type
- mild, moderate, and severe
508
Monostotic fibrous dysplasia most often occur when and affects which bone; presenting sx's?
- Early adolescence and equally in boys and girls; typically asymptomatic
- The femur, tibia, ribs, jawbones, calvarium, and humerus = most commonly affected
509
What is Vasogenic edema and is often seen following what?
- Increased EXTRAcellular fluid due to BBB disruption and increased vascular permeability - Fluid shifts from INTRAvascular compartments to INTERcellular spaces - Can be either localized (i.e., adjacent to neoplasms or inflammation) or generalied often follows ischemic injury
510
What is the MOA and use of Acamprosate?
- Weak NMDA-receptor antagonist and GABAa receptor agonist
- Reduces short-term and **long-term relaspse rates** (more than 6 months)
511
What type of hemorrhage is seen in the germinal matrix of premature infants?
Often found near which junction; may extend where and cause?
- Intraparenchymal hemorrhage
- Junction between **thalamus** and **caudate nucleus**
- May extend into ventricles ---\> subarachnoid space ---\> hydrocephalus (obstructive)
512
During a general medical exam of the skin of a patient with impaired consciousness what should be considered with dry skin?
- Hypothyroid
- Drugs (anticholinergics, TCA's)
513
Myasthenia crisis vs. cholinergic crisis
Myasthenic crisis: rapid deterioration of dz itself; may occur spontaneously or after infection, certain drugs; aspiration, diffuse weakness, resp failure possible
cholinergic crisis: rare; rapid increase in weakness from excess anticholinesterase meds; N&V, COLIC, DIARRHEAL, brady; MIOSIS and/or FASCICULATIONS (big clues)
514
Acquired demyelinating polyneuropathies
Acute: Guillan-Barre
Chronic- chronic inflammatory demyelinating polyneuropathy
515
What are 4 hematologic (CBC) indicators of SLE?
- **Hemolytic anemia** w/ reitculocytosis
or
- **Leukopenia** (\<4000/mm3 total)
or
- **Lymphopenia** (\<1500/mm3 total) on 2+ occasions
or
- **Thrombocytopenia** (\<100,000/mm3)
516
What are the 2 classes + agents used to tx the extrapyramidal sx's (EPS) of first gen. antipsychotics?
## Footnote
- **Anticholinergic** agents: **benztropine** + **trihexyphenidyl**
- **Antihistamine** agents: **diphenhydramine**
517
indications tramadol
moderate to moderately severe painmore effective than codeine, less effective than morphine
518
Why is the direction of nystagmus provoked by the anterior type of Benign Postional Vertigo significant?
- This is a more **rare** form
- Dix Hallpike will causes a **downbeat** (vertical) nystagmus, which is usually only seen in **central lesions.**
- Must **carefully assess** to rule out brainstem or cerebellar lesions!
519
With central dysfunction leading to dysequilibrium what is the vertigo like, duration of nystagmus, direction of nystagmus, and neuro symptoms?
- **Mild** vertigo
- **Persistence** of nystagmus, which can be in **vertical** direction
- Usually some **neuro symptoms**
520
pain in post thigh and post calf is seen in
S1
521
peripheral neuropathy = same as
polyneuropathy
522
MOA butorphanol
prototype for the agonist-antagonist opioids used to treat mild to moderate pain
523
After exercise, what happens with strength in LEMS?
strength improves w/ exericse
524
Which type of anxiety disorder is most often seen in assoc. w/ anorexia nervosa?
**Obsessive compulsive disorder**
525
What are the characteristics of Mal de Debarquement ("Sickness of Disembarkment")?
Duration?
Treatment?
- Illusion of movement as an after effect of travel (**sea**, car, train)
- Rocking, swaying feeling after getting off a boat
- Duration = usually \<24 hours; sometimes longer
- Tx: meclizine, scopolamine, benzodiazepines (dizziness meds)
526
precautions flurbiprofen
may slow or delay healing
527
Common cause of death in someone with Friedrich's Ataxia?
Cardiomyopathy
528
warnings and precautions for rivastigmine
1) dose should be titrated as prescribed and re-initiated at the lowest dose if interrupted for more than a few days
2) weight should be monitored during patch therapy
529
What is seen with diffuse idiopathic skeletal hyperostosis (**DISH**); what are the criteria?
- **Calcification** and **ossification** of **spinal ligaments** i.e., anterior longitudinal ligament and enthesis (tendon/lig. attachement to bone)
- **_No_** **SI joints involvement**\*
- Ossifications of **at least 4 contiguous vertebral levels**, usually on the _right_ side of spine
530
list the extended release methylphenidate-based stimulants used to treat ADHD
- aptensio XR
- concerta
- comtempla XR-ODT
- daytrana
- focalin XR
- metadate CD
- ritalin LA
- quillichew
- quillivant XR
531
Hip involvement in OA most commonly manifests as what sx?
Groin pain
532
Which second-gen. antipsychotic is associated with agranulocytosis and requires monitoring of WBC; REMS program?
## Footnote
**Clozapine**
533
What type of injury is associated with localized vs. generalized vasogenic edema?
- Localized: adjacent to inflammation or neoplasms - Generazlied: follow ischemic injury
534
What is the criteria for diagnosing a major depressive **episode**?
- At least **5** of the following for a **_2-week period_** with at least one either (1) depressed mood or (2) loss of interest or pleasure (**SIG E CAPS**)
**S**- sleep disturbance
**I**-interest lost
**G**- Guilt or feelings of worthlessness
**E**- Energy loss/fatigue
**C**- concentration problems
**A**- Appetite/weight changes
**P**-Psychomotor retardation
**S**- Suicidal
535
What is the first sign and the first symptom of a Cerebellopontine Angle Tumor?
- **First symptom** = hearing loss (CN VIII)
- **First sign** = absent corneal reflex (loss of CN V and VII)
536
What is the clinical presentation of BMD kids?
**1. Proximal symmetric muscle weakness**
**2. Neck flexor muscle strength is preserved**
**3. Presents later in life, usually not earlier than 5yo**
4. Less severe disease than DMD, slower progression
5. Usually ambulatory into their 20’s, sometimes longer
6. **Cognitive dysfunction usually not present**
7. Independent walking until late teens or early 20’s
8. Life expectancy between **fourth and sixth decades**
537
indications for galantamine
treatment of mild to moderate dementia of the Alzheimer's type
538
Entecapone and tolcapone can be helpful in what situations?
May be helpful in patients receiving levodopa who have developed response fluctuations
539
If meningitis is suspected, what should you do immediately?
**Blood culture**/lumbar puncture STAT
Start on **Dexamethasone** and **empirical antiobiotic** therapy
540
What is seen with a Myoclonic Seizure, duration, and how does patient feel after?
- **Brief**, rapid **symmetrical** jerking of extremities and/or torso
- Loss of consciousness
- Lasts \< **few seconds**
- **Minimal** post-ictal state
541
What is an important consideration before prescribing pt Naltrexone for tx of alcohol and opiate dependence?
Must be **opioid-free** before initiating therapy because naltrexone can precipitate an **acute withdrawal syndrome**
542
MOA opioids
bind to opioid receptors in the CNS causing inhibition of ascending nerve pathway, altering the perception of and response to pain and producing generalized CNS depression
543
3 main adverse effects of opioids
- CNS depression
- constipation
- hypotension
544
what is the black box warning for opioids
serious, life-threatening, or fatal respiratory depression may occur
carbon dioxide retension
545
list the acute adverse effects of opioid use
- respiratory depression
- N/V
- pruritis
- urticaria
- constipation
- urinary retention
- delirium
- sedation
- myoclonus
- seizures
546
warnings/precautions naloxone
acute opioid withdrawal: naloxone causes release of catecholamines which may precipitate acute withdrawal or unmask pain
547
warnings/precautions naltrexone
- accidental opioid OD (pts on naltrexone may respond to lower opioid doses than previously used, so using opioids again could cause OD)
- acute opioid withdrawal
548
Discuss four (4) benefits of Integrated Healthcare
- **Improved** patient experience and outcomes
- **Decreased** healthcare expenditures
- **Improved** **access to care**
- **Improved** provider satisfaction
549
Describe what an effective integrated care team looks like.
550
Explain four (4) functions that a Behavioral Health Consultant can provide to assist a physician in his or her day-to-day practice.
- Assessment, Education, Brief Intervention, Referral
- Warm hand-offs
- Chronic illness, Mental health disorders
- Prevention
- Quality improvement and quality assurance
551
What is mononeuritis multiplex?
**describes a disease process that damages several nerves in a haphazard fashion**
552
Mononeuritis multiplex describes a disease process that damages several nerves in a haphazard fashion and is commonly due to what?
## Footnote
**Vasculitis i.e., polyarteritis nodosum**
553
What is PAN's symptoms so you recognize it?
systemic vasculitis of the small and medium-sized vessels, which leads to tissue ischemia; most commonly involving skin, peripheral nerves, muscles, joints, gastrointestinal tract, and kidneys
Nonspecific symptoms: fever, abdominal, muscle, and joint pain
Renal involvement: hypertension, renal impairment
Coronary artery involvement; increased risk of myocardial infarction
Skin involvement: rash, ulcerations, nodules
Neurological involvement: polyneuropathy (mononeuritis multiplex), stroke
GI involvement: abdominal pain, melena, nausea, vomiting
Usually spares the lungs
554
What is Gullian- Barre characterized as?
**Acute inflammatory demyelinating polyneuropathy**
555
Histologic features of Guillain-Barre Syndrome are characterized by what?
**_Inflammation_** and **_demyelination_** of s**pinal nerve roots** and **peripheral nerves** = **_radiculoneuropathy_**
556
2/3's of Guillain-Barre Syndrome cases are preceded by what; which etiologies have been implicated?
- Acute, **influenza-like illness**
- ***_Campylobacter Jejuni_***, CMV, EBV, and Mycoplasma penumoniae, or prior vaccination
557
Morphologically what is the most prominent lesion seen in Guillain-Barre Syndrome? What else do you see?
**Segmental** **demyelination** affecting **peripheral nerves**
Also see **perivenular & endoneurial infiltration** by lymphs, macrophages and a few plasma cells
558
What antibodies do you see with Guillain-Barre?
Anti-myelin antibodies
559
Clinical presentation of Guillain-Barre Syndrome is dominated by what signs/sx's; what characteristic CSF finding will there be?
**- Ascending paralysis and areflexia**
**- DTR's lost early in the process**
- **↑ CSF protein** levels w/ little or no CSF pleocytosis (inflammatory cells remain confined to the roots) (no cells in csf)
560
How is Guillain-Barre Syndrome managed clinically?
**Plasmapheresis** and **IV Ig**
561
What is the most common acquired inflammatory peripheral neuropathy?
**_Chronic_** inflammatory **Demyelinating Poly(radiculo)neuropathy**
562
Chronic inflammatory Demyelinating Poly(radiculo)neuropathy is characterized by what type of neuropathy?
_Symmetrical_ mixed **sensorimotor** **polyneuropathy** that persists for **2 months or more**
563
Which 2 features of Chronic inflammatory Demyelinating Poly(radiculo)neuropathy distinguish it from Guillain-Barre?
**Time course** (presence at least 2 months) and **actually responds to steroids** (unlike GB)
564
How is Chronic inflammatory Demyelinating Poly(radiculo)neuropathy treated?
**_Glucocorticoids_** + IVIg + plasmapheresis
565
Upon sural nerve biopsy in patient with Chronic inflammatory Demyelinating Poly(radiculo)neuropathy what is a characteristic finding?
**Onion-bulbs**: excessive proliferation --\> **_multiple layers of Schwann cells_** wrap around an axon like the layers of an onion
566
What is the most common pattern of peripheral neuropathy seen with Diabetes?
**Ascending** distal **symmetric sensorimotor** polyneuropathy
567
Prevalence of peripheral neuropathy associated with diabetes is dependent on what?
**Duration** of the disease
568
Biopsies of the affected peripheral nerves/arterioles in diabetes will show what finding and with what stain?
**Endoneurial arterioles** show _thickening, hyalinization_, and intense **PAS-(+)** of their walls + extensive reduplication of basement membranes
569
Diabetic peripheral neuropathy is characterized by a relative loss of which size and type of nerve fibers?
**Small** myelinated and **unmyelinated** fibers
570
What are "positive sx's" associated with diabetic peripheral neuropathy? What are other clinical symptoms?
**Paresthesias** and **dyesthesias** = **painful sensations**
571
Other than peripheral neuropathy, what is another manifestation of diabetic nervous system dysfunction that is often seen?
**Autonomic dysfunction**: postural hypotension, incomplete bladder emptying (↑ infections) and sexual dysfunction
572
Uremic neuropathy seen in setting of renal failure is a distal, symmetric neuropathy often associated w/ what signs and sx's?
**Muscle cramps + distal dysesthesias + ↓ DTRs**
573
Disorders of NMJs present with what?
Painless muscle weakness
574
Myastheina Gravis is associated with autoantibodies against what?
**- ACh receptors on post-synaptic membrane (85% cases)**
- Muscle-specific receptor tyrosine kinase (15%)
575
There is a strong association with AChR autoantibodies seen in Myathenia Gravis and which abnormalities?
Thymic abnormalities: **Thymoma** and **Thymic hyperplasia**
576
Myasthenia gravis patients with AChR autoantibodies usually present with what signs/sx's?
- **Fluctuating weakness** that **worsens** with **exertion** and over course of day
- **Diplopia and ptosis due to involvement of extra-ocular muscles**
577
What electrophysiologic findings help distinguish Myasthenia Gravis and Lambert-Eaton Myasthenic Syndrome?
- **M.G**. = **Diminished** muscle responses after repeated stimulation
- **L.E.M.S** = **Increased** muscle response after repeated stimulation
578
What is 1st line tx for Myasthenia Gravis and what other tx's can be used to control the sx's?
- 1st line = **Acetylcholinesterase inhibitors**
- **Plasmapheresis** and **immunosuppressives** (glucocorticoids, cyclosporine, rituximab) --\> ↓ autoAb titers
579
Lambert-Eaton Myasthenic Syndrome is an autoimmune disorder due to what?
**_Antibodies_** block ACh **_release_** by inhibiting **_pre_**-synaptic Ca2+ channel
580
50% of Lambert-Eaton Myasthenic Syndrome cases are associated with what underlying condition?
Malignancy; most often **small-cell carcinoma of lung**
581
Pt's with Lambert-Eaton Myasthenic Syndrome typically present with what sx's?
**Weakness of the extremities** and **autonomic** dysfunction
582
Clostridium botulinum, botox has what affect on the NMJ?
Blocks release of ACh
583
Curare muscle relaxant has what effect on on the NMJ?
blocks AChR → flaccid paralysis
584
Which drugs are associated with slowly progressive muscle weakness which predominantly affects type I fibers?
Chloroquine and hydroxychloroquine
585
How do the type of mutations of Dystrophin differ between Duchenne and Becker muscular dystrophy?
- Duchenne: deletions or frame shift mutations --\> total absence
- Becker: synthesis of a truncated version, which retains some function
586
What will immunohistochemical staining for dystrophin show in Duchenne vs. Becker muscular dystrophy?
- Duchenne: absence of normal sarcolemmal staining pattern
- Becker: shows reduced staining
587
What morphological changes are seen with disease progression in Duchenne and Becker muscular dystrophy?
Muscle tissue is replaced by collagen and fat cells = Fatty replacement or change"
588
Where does weakness associated with Duchenne muscular dystrophy begin and how does it progress?
- Begins in pelvic girdles ---\> extends to shoulder girdles
- Pseudohypertrophy of calves often present
- Wheel-chair bound around age 9.5
589
Which lab value can aid in the diagnosis of Duchenne and Becker muscular dystrophy?
↑↑↑ CK
590
What is a key feature of Myotonic Dystrophy?
**Myotonia**: **sustained involuntary contraction** of a group of muscles; can be elicited by **percussion on thenar eminence**
591
Myotonic dystrophy is caused by what?
Expansions of **CTG** triplet repeats in **3'-noncoding region of DMPK gene**
592
How does Myotonic Dystrophy present signs and sx's?
**Gait**, then **atrophy of facial muscles** = **ptosis** and **"hatchet face,"** frontal balding, cataracts, cardiomyopathy
593
Malignant hyperthermia is associated with what mutations?
***RYR1*** mutation
594
What can trigger hyperthermia? And how do those agents do it?
Halogenated inhalational agents
• Anesthetic triggers ↑efflux of Ca⁺² from sarcoplasmic reticulum→ **tetany & excessive heat production**
595
Malignant hyperthermia leads to what clinical symptoms?
**hypermetabolic state**: **tachycardia** + tachypnea + muscle spasms and later **hyperpyrexia**
596
Pt's with anxiety, type A behavior, hostility, anger, and acute mental stress are at an increased risk for what?
- 2-fold ↑ risk of incident **MI and CAD**-related mortality
- Strongest evidence shows ↑ incidence of **depression**
597
Asthmatics are at an increased risk for which psych disorders?
- **Panic disorder** or agoraphobia
- Fear of dyspnea can trigger asthma attacks and high levels of **anxiety**
598
Diabetes is associated with an increased risk for what psych conditions?
Frustration, loneliness, dejection, and **_depression_**
599
What are some of the psych sx's associated with hyponatremia?
Confusion, lethargy, personality changes
600
Which psych sx's are very common with pancreatic carcinoma?
Depression, lethargy, **anhedonia**, apathy, and **↓ energy**
601
Acute intermittent porphyria psych symptoms?
Abdominal pain with psychosis, paranoia, acute depression, agitation, visual hallucinations
602
What are some of the psych sx's associated with hepatic encephalopathy?
Euphoria, disinhibition, psychosis, and depression
603
What are some of the psych sx's associated with frontal lobe tumors?
- Mood changes + irritability + facetiousness
- Impaired judgement + Impaired memory
- Delirium
- Loss of speech
- Loss of smell
604
What medication should be avoided in elderly hospitalized pt's especially those with delirium?
AVOID benzodiazepines
605
Which commonly used drug is associated with mania + psychosis (hallucinations)?
Corticosteroids
606
Personality disorders consist of an enduring pattern on inner experience and behavior that deviates markedly from the expectations of the individul's culture. This pattern is manifested in 2 (or more) of which 4 areas?
- **Cognition**: perception and interpretation of self, others and events
- **Affect**: the range, intensity, liability, and appropriateness of emotional response
- **Interpersonal functioning**
- **Impulse control**
607
ule of thumb is that personality disorder diagnosis cannot be made until which age?
≥18 y/o
608
Which personality disorder is characterized by irrational suspicions and mistrust of others?
Paranoid personality disorder (Cluster A)
609
Which personality disorder is characterized by a lack interest in social relationships, seeing no point in sharing time with others, little pleasure in activities, and appear indifferent to the praise or criticism of others?
Schizoid personality disorder (Cluster A)
610
What are the characteristics of Schizotypal personality disorder (cluster A)?
- **Odd behavior, beliefs and/or magical thinking** (in contrast to Schizoid)
- Vague, circumstantial, or stereotyped speech
- **Excessive social anxiety** that does not diminish with familiarity
611
Which personality disorder is characterized by repeated violations of the law, pervasive lying and deception, physical aggressiveness, reckless disregard for safety of self or others, irresponsibility, and lack or remorse?
Antisocial personality disorder (Cluster B)
612
Antisocial personality disorder is 3x more common in which sex?
Males
613
Pt's with antisocial personality disorder are at risk for what 4 co-morbid conditions?
- Anxiety disorders
- Substance abuse
- Somatization disorder
- Pathologic gambling
614
What are the major characteristics of Borderline personality disorder (Cluster B)?
- **Unstable mood** and interpersonal relationships; intense anger
- **Fear of abandonment**
- Sense of emotional emptiness
- **Impulsivity in ≥2** potentially self-damaging areas i.e., sex, substance abuse, etc..
- Recurrent suicidal behaviors or threats or self-mutilation
**3x more common in women**
615
What is important to keep in mind when tx pt with Borderline personality disorder?
- Need to set **rigid boundaries**!
- These pt's are very manipulative; **will often "split" staff members**
616
Which personality disorder is characterized by attention-seeking, dramatic speech and emotional exprssion, shallow and labile emotions, and sexual provocativity?
Histrionic personality disorder (Cluster B)
617
Pt's with Narcissistic personality disorder are at risk for what 3 co-morbidities?
- Anorexia nervosa
- Substance abuse
- Depression
618
Pt's with Borderline personality disorder have a high rate of what co-morbid condition?
Major depression
619
How does Avoidant personality disorder (cluster C) differ from Schizoid (cluster A)?
They desire relationships with others
620
Which personality disorder is characterized by hypersensitivity to rejection and criticism, socially inhibited, timid, feelings of inadequacy and a desire for relationships with others?
Avoidant personality disorder (Cluster C)
621
Which personality disorder is characterizd by being preoccupied w/ perfectionism and control, lack of flexibility or openness, often stingy as well as stubborn?
Obsessive-Compulsive personality disorder (Cluster C)
622
What is the best treatment for Borderline Personality Disorder?
**Dialectial Behavior therapy (DBT)**
Learn coping skills and impulse control and focus on stabilizing self destructive behavior
623
Which drug can be used off-label for impulse control in pt's with personality disorders?
## Footnote
**Valproic acid**
624
Treatment of personality disorders
-tricyclic antidepressants and monoamine oxidase inhibitors (MAOIs) are usually not prescribed
**-selective serotonin reuptake inhibitors (SSRIs) and newer antidepressants are safe and reasonably effective**
-less effective than in patients with uncomplicated major depression -anticonvulsants: Valproic acid
625
What is psychodynamic therapy?
examines the ways that patients perceive events,
assumption that perceptions are shaped by early life
identify perceptual distortions and their historical sources
facilitate the development of more adaptive modes of perception and response
frequency from several times a week to once a month; it makes use of transference
626
Cognitive Behavior Therapy [CBT]
deals with how people think about their world and their perception of it
typically limited to episodes of 6-20 weeks, once weekly
627
What is the diagnostic criteria for Schizophrenia?
- **≥2 of the following for most of 1 month; at least 1 of the first 3 sx's**
- Delusions
- Hallucinations
- Disorganized speech
- Grossly disorganized or catatonic behavior
- Negative sx's
628
What is criteria for Delusional Disorder?
- Presence of **1+ delusions** with duration of **1 month**
- Functioning is NOT impaired and behavior is not obviously bizarre or odd
629
What is the diagnostic criteria for Brief Psychotic Disorder?
- Presence of **1+** of the following sx's. At least one being the first 3.
- Delusions, Hallucinations, Disorganized speech, Grossly disorganized or catatonic behavior
**- Duration of an episode is at least 1 day but \<1 month**
630
What is the diagnostic criteria for Schizophrenifrom Disorder?
- **Two** (or more) of the following, each presnt for a significant portion of time during a **1-month period**. At least one must be the first 3 sx's:
- Delusions, Hallucinations, Disorganized Speech, Grossly disorganized or catatonic behavior, Negative sx's
- **Episode must last at least 1 month but \<6 months**
631
What is the diagnostic criteria for Schizoaffective Disorder?
- An **uninterrupted** period of illness during which there is a **major mood disorder** (major depressive or manic) concurrent with criterion A of schizophrenia
- **Delusions or hallucinations** for **≥2** weeks in absence of **major mood episode** (depressive or manic)
632
There must be the presence of one or both of which 2 sx's for diagnosis of substance/medication-induced psychotic disorder?
- Delusions
- Hallucinations
633
What is the timeline for Schizophrenia vs. Schizophreniform vs. Brief Psychotic Disorder?
- Schizophrenia: \>6 months
- Schizophreniform: 1-6 months
- Brief psychotic disorder: \<1 month
634
what is the diagnostic criteria for PTSD
Duration of disturbance and symptoms is more than 1 month
1) person exposed to a traumatic event in which both were present:
- person experienced/witness actual or threatened death/injury/integrity of self or others
2) persistent re-experience of event
3) persistent avoidance of stimuli associated with trauma
4) persistent increased arousal (difficulty sleeping, irritability, hypervigilance)
5) negative cognitions
635
treatment for PTSD
* *- drug of choice: SSRIs**
- cognitive processing thearpy
- eye movement desensitization and reprocessing (EMDR)
**\*\*avoid addictive prescriptions like benzos**
636
define acute distress disorder
sx similar to PTSD but lasting 3 days to 1 month after trauma exposure
637
define adjustment disorder
development of emotional/behavioral sx in response to an identifiable stressor
- occurs within 3 months of stressor
- results in significant distress out of proportion to severity of stressor or impairment of function
## Footnote
**does not persist past 6 months**
638
time period for adjustment disorder
occurs within 3 months of stressor, usually does not persist beyond 6 months
639
