Clinical NS - Dr. Hon Flashcards
(147 cards)
1
Q
3rd leading cause of death in USA
A
Stroke
2
Q
What % of stroke survivors will have another stroke?
A
25%
3
Q
In what age group do more women die from stroke than MI?
A
Under 45 years of age
4
Q
Types of stroke (2) and their properties:
A
- Hemorrhagic stroke (20%)
- Intracerebral hemorrhage (cortical vs. sub-cortical)
- Subarachnoid hemorrhage - Ischemic stroke (80%)
- Large artery atherosclerosis with thromboembolism
- Lacunar (small vessel) disease
- Embolism from the heart
- Nonatherosclerotic vasculopathy (i.e. Syphilis)
- Hypercoagulable states (i.e. “Sticky Blood Syndromes”)
5
Q
Risk factors for stroke
A
Increasing age Previous TIA or stroke Atherosclerosis (HTN, smoking, DM, hyperlipidemia) Cardiac disorders - Valvular heart disease - Dysrhythmias (atrial fib, etc.) - Mural thrombus - Atrial myxoma (tumor) - Endocarditis (infection) - Interatrial septal abnormalities Drug abuse (IV drug abuse, cocaine, amphetamines) Oral contraceptives Pregnancy/Postpartum period Fibromuscular dysplasia Hypercoaguable states (thrombocytosis, polycythemia, sickle cell disease, leukocytosis, etc.) Inflammatory disorders (giant cell arteritis, SLE, polyarteritis nodosa, AIDS, syphilis, etc.) Migraine
6
Q
Blood color for hemorrhagic stroke vs. ischemic stroke (hemorrhagic conversion after ischemia)
A
Hemorrhagic stroke (20%): merlot Ischemic stroke (80%) with hemorrhagic conversion: rose
7
Q
Cause of death for 50% of stroke patients:
A
Med. complications: pneumonia, UTI, PE, DVT, decubitus ulcers
8
Q
Hypertension treatment during stroke
A
If this is an ischemic stroke (NOT HEMORRHAGIC), you will not treat the HTN - this is because the ischemic area of the brain may have lost autoregulatory function and may be hypotensive (despite systemic HTN).
NORMOTENSION is bad for patients with HTN experiencing ischemic stroke!
9
Q
NIH stroke scale to predict risk of hemorrhage
A
Normal score: 0
Coma: 4
Score 20: 17% chance of hemorrhage
10
Q
Evaluation and acute treatment of stroke
A
ABCs
Elevate head of bead to 30 degrees
O2 @ 2L vis nasal cannula
Vitals, IV, height/weight
Labs, EKG
Determine if there is a fever, and if there is- treat it
Obtain history and compare that to findings on CT (may take several hours for new findings to appear on CT)
Last known “normal”, medications (i.e. Warfarin), hx of trauma
11
Q
Ddx for stroke
A
Seizure post-ictal period, migraine headache, hypoglycemia, etc.
12
Q
When to use t-PA
A
Good: within 3 hours of onset of symptoms
Better: within 1.5 hours
13
Q
Improvement with t-PA administration
A
With t-PA: 31-50% with complete or nearly complete recovery at 3 months
Without t-PA (placebo): 20-38% with same findings
14
Q
Anticoagulation with heparin- efficacy
A
Early studies said 50% reduction in neuro worsening especially in stroke-in-progression or TIA patients
But
Methodologically flawed and high risk of hemorrhagic conversion during ischemic stroke without proven benefit
15
Q
Why you can still given heparin today
A
To prevent DVT in immobilized patients with acute stroke
16
Q
Other conditions for which to give anticoagulation agents:
A
A fib MI Prosthetic valve Atrial septal defect Hypercoagulable state Large vessel disease Aortic arch disease
17
Q
Other anticoagulation agents
A
Aspirin Aggrenox Ticlid Plavix Persantine Warfarin Low MW heparin
18
Q
Paroxysmal disorders in Neuro:
A
Migraine
Seizures
Syncope
Dizziness
19
Q
% positive findings for epilepsy on a single EEG
A
All types: 40%
Generalized tonic-clonic: 20%
Petit mal: 90%
Partial: 30%
20
Q
% positive findings on EEG for 3 slee-deprived EEGs
A
85%
So, you can’t ALWAYS catch it on EEG
21
Q
Most important piece of information when diagnosing epilepsy
A
History of the event by an eyewitness
22
Q
Partial/Focal seizure types (3):
A
- Simple partial
- Complex partial
- Secondary generalized (with a partial onset)
23
Q
Generalized seizure types (7+):
A
- Absence (petit mal)
- Tonic-clonic
- Myoclonic
- Tonic
- Clonic
- Atonic
- Clonic-tonic-clonic
24
Q
Characteristics of simple partial seizures
A
Focal motor or sensory activity
NO LOC
Lasts seconds
NO POST-ICTAL STATE
25
Characteristics of complex partial seizures
```
Nonresponsive staring
Preceding aura is possible (impending doom feeling)
Automatisms
LOC
Lasts 1-3 minutes
Post-ictal state present
```
26
Characteristics of secondary generalized seizures
Bilateral tonic-clonic activity
LOC
Lasts 1-3 minutes
Post-ictal state present, possibly for even hours
27
Characteristics of absence (petit mal) seizures
Nonresponsive staring
Rapid blinging, chewing, clonic hand motions
LOC
Lasts 10-30 seconds
NO POST-ICTAL STATE (mind "just stops and restarts later")
28
Difference between complex focal and absence seizure types
Complex focal seizures last longer (1-3 min vs. 10-30 seconds)
Complex seizures have a post-ictal state but absence seizures do not
Complex seizures have a preceding aura associated with them
29
Characteristics of tonic-clonic seizures
Bilateral extension of extremities followed by symmetrical jerking of extremities
LOC
Lasts 1-3 min
Post-ictal state present
30
Characteristics of atonic seizures
Sudden loss of muscle tone/Head drops/Patient collapses
LOC
Variable duration in time
Post-ictal state present (for up to an hour post-event)
31
Characteristics of myoclonic seizures
Brief, rapid symmetrical jerking of extrem. and/or torso
LOC
Lasts only a few seconds
MINIMAL POST-ICTAL STATE
32
Drugs for treatment of partial/focal seizures and SECONDARY generalized seizures
```
Phenytoin
Carbamazepine
Valproic Acid*
Phenobarbital
Gabapentin
Lamotrigine*
Topiramate*
Gabatril
Felbamate
Oxcarbamazepine
Levetiracetam*
Zonisamide*
Perampanel*
```
33
Drugs that can work for both partial/focal seizures + seconary generalized seizures AND primary generalized seizures:
```
Valproic acid
Levetiracetam
Lamotrigine
Topiramate
Perampanel
Zonisamide
```
34
Combination of AEDs with synergistic action for treating seizures
Valproic acid + Lamotrigine
35
Drugs for treating PRIMARY generalized seizures
```
Ethosuxamide (absence seizures only)
Valproic acid (backup if ethosuxamide doesn't work; also used for primary tonic-clonic seizures)
Lamotrigine
Leviteracetam
Topiramate
Perampanel
```
36
Status epilepticus
Prolonged seizures (>10 minutes) or repeated seizures without recovery in between
37
Treatment for status epilepticus
```
ABCs
IV line
Labs
Non-contrast head CT
Gain history
Give a benzodiazepine (i.e. Lorazepam 2-4 mg IV) - this will buy time, but need to give another longer-lasting AED
```
38
Other options for treatment of status epilepticus
First give this: Lorazepam 2-4 mg IV (or other benzodiazepine)
Then give these:
Fosphenytoin IV or IM
Phenobarbital IV (must intubate if IV, can give IM to avoid need for intubation due to respiratory depression)
Valproic acid
Next best options:
Midazolam IV or Propofol IV
Last resort:
Pentobarbital coma
39
What to give if patient doesn't respond to normal treatment for status epilepticus
Try giving an oral anticonvulsant via NG tube
| i.e. Carbamazepine
40
General principles of treating patients with epilepsy:
Use monotherapy
Consider drug-drug interactions (ex. OCPs and carbamazepine --> 4x higher risk of OCP failure)
Consider long-term side effects (ex. Bone loss on "old" AEDs like carbamazepine, phenytoin)
41
What AED do you avoid in pregnant women at all costs?
Valproic acid - highly teratogenic!
42
AEDs in pregnancy
Use whatever drug controls mom's seizures.
New drugs have fewer side effects.
Women of child-bearing age should be on folic acid supplement because AEDs are folate-depleting.
43
Syncope vs. Seizure
Syncope: pallor, sweating, abnormal head sensation, lightheadedness, positionally related (hypotension on standing), slow in onset (allows time to get into a safe position), brief unconsciousness, no post-ictal state
Seizure: causes tongue injury, urine/bowel incontinence, tonic/clonic movements, post-ictal state is present
44
Multiple Sclerosis defined
A disorder of the brain and spinal cord characterized by a tendency for periods of increasing and decreasing symptoms and signs (exacerbations & remissions), which result from loss of nerve tract insulation (myelin) at multiple sites in the CNS
45
Common MS signs and symptoms
```
Paresthesias
Gait disturbance (transverse myelitis)
Weakness
Visual loss (optic neuritis)
Urinary difficulty
Dysarthria
Hemiparesis
```
46
4 types of MS
```
Relapsing remitting (45-50%)
Secondary progressive (20-25%)
Primary progressive (15-20%)
Benign (10-15%)
```
47
MS characteristics
Disease of CNS
Diagnosed in 20s and 30s
Characetrized by periods of exacerbation and remission
No test can confirm this diagnosis alone
48
Causes of MS
Unknown
Genetic susceptibility (3-5% risk if 1st degree relative with MS)
Childhood event or illness may sensitize immune system to attack CNS myelin, followed by adult event triggering disease (i.e. Viral infection, post-partum period)
49
Epidemiology of MS
Women:Men ratio 1.5:1
Women have a more favorable course
Onset between 15-50 YO
Earlier onset indicates more favorable course
Risk determined before 14 YO
More cases in temperate climates than in tropical climates
50
Studies used to make diagnosis of MS
MRI head neck spine- see ovoid lesions of high signal around ventricles in white matter and also in spinal cord, acute lesions may enhance (on T2 weighted imaging)
Multimodality evoked potentials
LP for CSF analysis- presence of oligoclonal bands &/or increased IgG index/synthesis rate
51
Drugs for maintenance of MS (to decrease frequency/severity of exacerbations and to slow disease progression) -- drugs used in relapsing remitting MS:
Avonex, Rebif (Interferon Beta-1A)
Betaseron (Interferon Beta-1B)
Copaxone (Glatirimer acetate)
52
Drugs for chronic progressive MS
Betaseron (Interferon Beta-1B)
| Others (methotrexate, cyclophosphamide, novantrone, azothiaprine)
53
Drug to treat acute exacerbation of MS
High dose corticosteroids (solumedrol 1 g IV QD for 5 days followed by a prednisone taper)
This will reduce length but not severity of exacerbation and will not alter outcome of this disease progression
54
DDX for MS
- MS
- ADEM - acute disseminated encephalomyelitis s/p infection or immunization (but if ADEM, won't recur)
- Autoimmune disease (i.e. SLE with cerebritis, CNS vasculitis, or polyarteritis nodosa with transverse myelitis)
- B12 deficiency
- Lymphoma, leukemia with CNS involvement (improves on steroids just like MS, so hard to differentiate until biopsy taken)
- Spinocerebellar ataxia
- Vascular malformations (i.e. Spinal cord AVM)
- Infections (i.e. HIV, EBV, CMV, West Nile, Lyme, Syphilis)
- Granulomatous disease (i.e. Sarcoidosis)
- Metachromatic leukodystrophy, adrenomyeloleukodystrophy
- Neuromyelitis optica (Devic's disease, which was once thought to be a variant of MS)
55
Symptomatic treatment of MS: spasticity
Spasticity: baclofen, tizanidine, botox injections, etc.
56
Symptomatic treatment of MS: Intention Tremor
Intention tremor: propanolol, primidone, clonzepam
57
Symptomatic treatment of MS: Urinary Urgency
Urinary urgency: Oxybutynin, Detrol LA
58
Symptomatic treatment of MS: Urinary Retention
Urinary retention: Bethanechol
59
Symptomatic treatment of MS: Painful dysesthesias (tingling that is painful or stinging)
Painful dysesthesias: carbamazepine, oxcarbamazepine, gabapentin, phenytoin, baclofen
60
Symptomatic treatment of MS: Fatigue
Fatigue: amantadine, modafinil, fluoxitine, buproprion, pemoline, exercise
61
Primary headache (benign headche disorders) classification
– Migraine (with or without aura)
– Tension type
– Cluster HA
– Post-traumatic HA
– Drug rebound HA
– Other
62
Associated symptoms for headache
N/V
Anorexia
Photo-/Phonophobia
Others (diarrhea, watery eyes, ptosis/miosis, stuffy/runny nose)
63
Triggers for headache
Hormones (menstrual cycle, OCPs, hormone therapy)
Diet
Stress (i.e. When stress goes down/letdown periods)
Environmental changes (i.e. Weather, seasons, travel, sleep)
Sensory stimuli (i.e. Smells/perfumes, lighting in stores)
64
What other medications cause headaches for patients?
Proton pump inhibitors
| Acyclovir
65
Neuro exam components for patient c/o HA
```
Neck flexion
Check for bruits in head/neck
Optic fundi, pupils, visual fields
Thorough CN exam
Motor power in limbs
Muscle reflexes
Plantar responses
Sensory exam
Coordination
Gait
```
66
Worrisome signs in a HA patient that may indicate a HA of pathological origin (Secondary HA):
```
Worst HA
Onset of HA after 50 YO
Atypical HA for patient
HA with fever
Abrupt onset fever (max intensity in seconds, not minutes)
Subacute HA with progressive worsening over time
Drowsiness, confusion, memory impairment
Weakness, ataxia, loss of coordination
Paresthesias, sensory loss, paralysis
Abnormal medical or neuro exam
```
67
What does the brain do when insulted?
Swell
Bleed
Sieze
68
Who to scan when c/o HA
As a general rule, many physicians (including neurologists) believe that any person with HA should have a one-time, thorough neuroimaging study (CT head with and w/o contrast or MRI of the head)
69
What scans/tests to do when patient has a worrisome HA complaint
CT head w/ and w/o contrast
MRI
LP
Arteriogram
70
How often does CT miss a subarachnoid hemorrhage
5-10% of the time
71
Primary HA disorders
```
Common migraine (migraine without aura)
Classic migraine (migraine with aura)
Tension-type HA
Cluster HA
```
72
Common migraine characteristics
Moderate to severe intensity
Inhibits daily activities, aggravated by activity
Onset in late teens/20s, prevalence peaks at 35-40
More common in females (3:1)
1-4 attacks/month
Last 4-72 hrs, typically 12-24 hrs
Located unilaterally
Throbbing, sharp, pressure-type pain
Prodrome: mood changes, myalgias, food cravings, yawning
Postdrome: fatigue, irritability, "fog"
No aura
73
Common migraine associated symptoms
N/V
Photophobia
Phonophobia
Also diarrhea, red eyes, stuffy nose, lacrimation, miosis, ptosis
74
Cluster HA characteristics
Severe, excruciating
Prohibits daily activities
Onset in 20s-50s
More common in males (6:1)
Recently has been associated with obstructive sleep apnea
Episodic type: 1+ attacks/day for 6-8 weeks
Chronic type: several attacks/week without remission
Last 30 min-2 hours
Located 100% unilaterally, typically over orbit/temporal area
Non-throbbing, excruciating, sharp, boring, penetrating pain
Prodrome possible (mild burning in nose/eyes)
No aura
Patient is pacing, frenetic, and rocking
75
Classic migraine characteristics
```
Aura present (usually 15-30 min, sometimes longer)
Commonly, aura includes visual symptoms like scotoma and scintillations
```
76
Tension-type HA characteristics
Mild to moderate
Does not inhibit daily activities
Variable age of onset, but generally 20-40 YO
More common in females (3:2)
Episodic: 15 days/month (*** but consider chronic migraine HA in DDX because this sounds more likely)
Located bilaterally- bifrontal, bioccipital, neck, shoulders, band-like pressure around the top of the head
Dull, aching, squeezing pressure
NO prodrome or aura
Behavior is not generally affected
77
Cluster HA associated symptoms
Ipsilateral ptosis, miosis, conjunctival injection, lacrimation, stuffed/runny nose
78
Acute treatment of migraine HA
```
OTC analgesics
NSAIDs
Isometheptene (midrin)
Butalbital (fioricet)
Opioids, but now debatable d/t crisis
DHE nasal spray
Triptans (5HT1 agonists)
- Sumatriptan (Imitrex)
- Zolmitriptan (Zomig)
- Naratriptan (Amerge)
- Rizatriptan (Maxalt)
- Almotriptan (Axert)
- Frovotriptan (Frova)
```
79
Triptans contraindications/cautions
Documented or strong risk factors for ischemic heart disease, other cardiovascular, cerebrovascular or peripheral vascular disease, Raynaud’s syndrome, uncontrolled HTN, hemiplegic or basilar migraine, severe renal or hepatic impairment, use within 24 hr. of tx. with ergotamines, MAOI’s, or other 5-HT1 agonists
80
What drug is used to break the cycle of a prolonged migraine or weeks of frequent migraines?
Prednisone taper
81
Prevention of common and classic migraine HAs:
Antidepressants
- TCAs (e.g. amitriptyline, nortriptyline)
- SSRI’s (e.g. Prozac, Zoloft, Paxil, Celexa)
- MAOI’s (e.g. Phenelzine)
Beta-Blockers (e.g. propranolol)
Calcium Channel Blockers (e.g. verapamil)
Anticonvulsants (e.g. valproic acid, gabapentin, topiramate, tigabine)
Ergotalkaloids (ergotomine + phenobarbital)
NSAID’s (e.g. ASA, naproxen)
Muscle relaxants (e.g. Zanaflex)
Methysergide (Sansert)
82
Non-prescription tx of migraine HAs
```
Exercise
Stop smoking
HA education
Riboflavin
Magnesium
Biofeedback/relaxation
```
83
Acute tx of tension HA
OTC analgesics
NSAIDs
Opioids
Midrin
84
Preventive tx for tension HAs
```
Antidepressants
- TCAs, SSRIs, MAOIs
Muscle relaxants
Anticonvulsants
Botox injections
Ergot alkaloids (DHE to break the cycle of chronic daily HA)
```
85
Acute tx of cluster HAs
```
DHE 1 mg or Ergotamine 2 mg SL
Lidocaine 4% intranasal
Narcotics
Oxygen 100% 8L/min by MASK ONLY
Sumatriptan 6 mg SQ
```
86
Preventive tx for cluster HAs
```
Calcium channel blocker
Anticonvulsant
Lithium
Indomethacin
Predisone x 10-14 days
Capsaicin (intranasal TID x 1 week)
Ergotamine tartrate
```
87
BOARDS questions for HAs (trigeminal neuralgia drug, SUNCT drug, and paroxysmal hemicrania drug):
What drug for trigeminal neuralgia? Carbamazepine or Oxcarbamazepine
What drug for SUNCT syndrome? Lamotrigine
What drug for paroxysmal hemicrania? Indomethacin
88
Two classes of movement disorders
1. Bradykinetic
| 2. Hyperkinetic
89
Bradykinetic disorders- the most common parkinsonism that encompasses... (4):
* Idiopathic Parkinson’s Disease
* Postencephalitic (Von Economo’s encephalitis)
* Toxin-induced (Manganese, Canbon Disulfide, Carbon Monoxide)
* MPTP (meperidine analogue)
90
Idiopathic Parkinson's disease cause
Caused by a depletion of DA in the nigrostriatal system which disrupts the balance of ACh and Dopamine
91
Cardinal signs of Idiopathic Parkinson's disease
CARDINAL FEATURES:
1. Tremor - u/l at first, resting tremor, "pill-rolling", mouth or chin tremor (NOT head or voice tremor!)
2. Rigidity - increased resistance to passive movement ("cogwheel rigidity")
3. Bradykinesia - slowness of movement, difficulty initiating movement
92
Other features of idiopathic Parkinson's disease:
- Postural instability (cannot correct changes in balance and posture)
- Hypophonia (quiet voice)
- Hypomimia (reptilian stare/gaze)
- Less blinking
- Myerson's sign - glabellar reflex
- Gait disturbance - slow, stooped forward, small steps, festinating gait
- Dysautonomia (orthostatic hypotension, incontinence, impotence, anhydrosis)
- Dementia (many patients)
93
Other akinetic rigid syndromes:
Progressive Supranuclear Palsy (PSP)
Shy-Drager Syndrome (MSA: multiple systems atrophy)
Cortical basal (ganglionic) degeneration (CBD)
94
Feature of PSP
Bradykinesia and rigidity
| LOSS OF VOLUNTARY CONTOR OF EYE MOVEMENTS (won't move eyes around to keep focus on an object when you move head around)
95
MSA core features (Shy-Drager syndrome)
Bradykinesia and rigidity
PRONOUNCED autonomic dysfunction
No tremor
96
Cortical Basal (Ganglionic) Degeneration (CBD) characteristics
Bradykinesia and rigidity
CHARACTERIZED BY BOTH CORTICAL AND BASAL GANGLIONIC DYSFXN
Also see cortical sensory loss, apraxia, myoclonus, or aphasia
97
Do anti-parkinsonian meds work for "other" akinetic rigid syndromes (PSP, MSA, CBD)
Not really, but don't have other drugs to give/use so use them at high doses to try them anyway
98
Anti-parkinsonian treatments (7):
* Dopamine Agonists (e.g. Bromocriptine, Pergolide, Pramipexole, Ropinirole)
* Levodopa (e.g. Sinemet)
* COMT inhibitors (e.g. COMTAN)
* Anticholinergics (e.g. benztropine, trihexyphenidyl)
* MAO-B inhibitor (selegiline)
* Amantadine
* Surgery – most common is Deep Brain Stimulation (DBS), but hard to catch them in that window of time!
99
Hyperkinetic Movement Disorder characteristics (5):
```
Chorea
Athetosis
Dystonia
Ballism
Tic
```
100
Chorea defined:
Chorea - irregular, brief, “dancelike” movements; may be incorporated into purposeful movements
101
Athetosis defined:
Athetosis – writhing, sinuous movements (often occur in combination with chorea)
102
Dystonia defined:
Dystonia – sustained muscles contractions that produce twisting and repetitive movments and abnormal postures
103
Ballism defined:
Ballism – large amplitude, flinging movement, usually from the proximal part of an extremity.
104
Tic defined:
Tic – brief, rapid, repetitive, seemingly purposeless stereoptyped action that may involve single or multiple muscle groups
105
Chorea disorders:
Huntington's disease
| Syndenham's chorea
106
Huntington's disease characteristics:
– Gradual onset and progression of chorea and dementia caused by inherited trinucleotide repeat gene defect on chromosome 4.
– Autosomal dominant inheritance, so offspring of affected person has 50% chance of inheriting disease.
– Prevalence is 5 per 100,000
– Symptoms generally begin between age 30-50, with avg. lifespan 15 yr. after onset of sx.
– Genetic testing provides accurate and definitive means to establish dx. Caution: multiple ethical issues!
– No known prevention or cure
107
Syndenham's chorea characteristics:
– Occurs mainly in children and adolescents as a complication of a previous infection with group A hemolytic streptococcus. May be a form of arteritis.
– Characterized by unilateral choreiform movements which when mild can be confused forrestlessness or fidgeting. May also see behavioral changes.
– Treatment: bedrest and antibiotics
108
Huntington's disease characteristics:
– Gradual onset and progression of chorea and dementia caused by inherited trinucleotide repeat gene defect on chromosome 4.
– Autosomal dominant inheritance, so offspring of affected person has 50% chance of inheriting disease.
– Prevalence is 5 per 100,000
– Symptoms generally begin between age 30-50, with avg. lifespan 15 yr. after onset of sx.
– Genetic testing provides accurate and definitive means to establish dx. Caution: multiple ethical issues!
– No known prevention or cure
109
Dystonia disorders:
Idiopathic torsion dystonia
Focal torsion dystonia
Wilson's disease
110
Idiopathic torsion dystonia characteristics:
– Characterized by dystonic movements and postures without other signs.
– May be inherited as AD, AR, or X-linked recessive disorder
– Onset may be in childhood or later life, but remains throughout life.
– May see variety of clinical findings:
• Torticollis (cervical dystonia) – neck twisted to one side
• Blepharospasm – spontaneous involuntary forced closure of eyelids
• Oromandibular dystonia – spasms of muscles around mouth
• Arm may be held in hyperpronated position with wrist flexed and fingers extended (Wendy hands)
• Leg may be held in extension, with pronation and inversion of the foot.
– Treatment:
• Low doses levodopa
• Anticholinergics
• Benzodiazepines
• Neuroleptic Drugs
• Baclofen
• Carbamazepine
111
Idiopathic torsion dystonia characteristics:
– Characterized by dystonic movements and postures without other signs.
– May be inherited as AD, AR, or X-linked recessive disorder
– Onset may be in childhood or later life, but remains throughout life.
– May see variety of clinical findings:
• Torticollis (cervical dystonia) – neck twisted to one side
• Blepharospasm – spontaneous involuntary forced closure of eyelids
• Oromandibular dystonia – spasms of muscles around mouth
• Arm may be held in hyperpronated position with wrist flexed and fingers extended.
• Leg may be held in extension, with pronation and inversion of the foot.
112
Focal torsion dystonia characteristics:
```
ONLY ONE OF THE FOLLOWING:
– Dystonia confined to FOCAL area
– Blepharospasm
– Oromandibular dystonia
– Spasmodic Torticollis
– Writer’s cramp:
• Dystonic posturing of hand and forearm when used for specific task (e.g. writing, using tools or utensils)
```
113
Wilson's disease characteristics:
If you catch it, you can reverse it... If you don't you can die!
– Autosomal recessive disorder of COPPER metabolism that produces
NEUROLOGIC AND HEPATIC dysfunction, localized to chromosome 13
– Decreased binding of copper to cerruloplasmin --> leads to large free copper deposits in tissues
– Usually presents in CHILDHOOD or young adult life
```
Clinical features: BOTH bradykinetic and hyperkinetic features present:
• Resting or postural tremor
• Choreiform movements
• Rigidity
• Bradykinesia
• dysarthria, dysphagia
• Ataxia
• Personality / Behavioral changes
• Dementia (if untreated)
• Psychosis / Hallucinations
```
114
Wison's disease treatment:
Treatment:
• Penicillamine (copper chelating agent)
• Restriction of dietary copper
115
Wilson's disease diagnosis:
```
Diagnosis:
• Based on history, exam findings
• Increased amounts of copper excretion in 24 hr. urine collection
• Decreased serum cerruloplasmin level
• Kayser-Fleischer Ring on eye exam
```
116
Idiopathic torsion dystonia treatment:
```
– Treatment:
• Low doses levodopa
• Anticholinergics
• Benzodiazepines
• Neuroleptic Drugs
• Baclofen
• Carbamazepine
```
117
Focal torsion dystonia treatment:
– Medications the same as for Idiopathic Torsion Dystonia
– ***BOTOX (Botulinum Toxin) injections may also be helpful to weaken involved muscles: probably treatment of choice for focal dystonia
118
Tic disorder types and characteristics:
- Single motor tics (i.e. blinking, coughing, throat clearing), often as a benign entity
- Gilles De La Tourette's syndrome: chronic motor and vocal tics with onset before age 21
119
Tourette's syndrome characteristics:
- Most cases sporadic
- Most common in males
- Vocal tics: barks, hisses, grunts, coughing, throat clearing
- Also: vulgar/obscene speech, parroting speech of others, imitation of others' movements, repetition of words or phrases
120
Tourette's syndrome treatment:
Clonidine
Haldol
Phenothiazines
121
Essential tremor (benign familiar tremor) clinical presentation:
- Postural or kinetic tremor of both hands and may involve head or voice
- Possible in early adulthood, but often not until later in life
- Usually progresses slowly over years to decades (much slower progression than PD patients will have!)
- Usually does not result in significant disability, but social embarrassment is common. As disease progresses, can impair coordination
- EtOH often decreases the tremor temporarily
122
Essential tremor treatment:
Beta blockers
Primidone
Benzodiazepines
Deep brain stimulation
123
Definition of dementia:
– A decline in memory and at least one other cognitive function (aphasia, apraxia, agnosia, or a decline in an executive function i.e. planning, organizing, sequencing, or abstracting)
– This decline impairs social or occupational functioning in comparison with previous functioning. The deficits should not occur exclusively during the course of delirium and should not be accounted for by another psychiatric condition, such as depression or schizophrenia
124
Incidence and prevalence of dementia:
10% of people >65 YO
| 30% of people >85 YO
125
Causes of dementia:
```
Degenerative
Vascular
Infectious
Psychiatric
Toxic/Metabolic
Traumatic
Tumors
Other
```
126
Degenerative causes of dementia (can be definitively determined at autopsy):
```
• Alzheimer’s Disease (80% of all cases)!!!!
• (Diffuse) Lewy body disease
• Parkinson’s disease
• Frontotemporal lobar degeneration
– Frontotemporal dementia (Pick’s)
– Progressive nonfluent aphasia
– Semantic dementia
• Progressive supranuclear palsy
• Corticobasal degeneration
• Multiple systems atrophy
• Huntington’s disease
• Olivopontocerebellar degeneration
```
127
Vascular causes of dementia:
```
Multiple infarction
Single stroke
Binswanger's disease
Vasculitis
Subarachenoid hemorrhage
```
128
Infectious causes of dementia:
```
Fungal meningitis
Syphilis
AIDS dementia
CJD (Jakob-Creutzfeldt disease)
Post-herpes simplex encephalitis
```
129
Psychiatric causes of dementia:
```
Depression
Alcohol abuse
Drug-related disorder
Personality disorder
Anxiety disorder
```
130
Toxic/Metabolic causes of dementia:
```
Vitamin B 12 deficiency
Thyroid deficiency
System failure: liver, renal, cardiac, respiratory
Heavy metals
Toxins (glue sniffing)
```
131
Traumatic causes of dementia:
```
Subdural hematoma
Closed head injury
Open head injury
CTE (chronic traumatic encephalopathy)
Anoxic brain injury
```
132
Tumors that cause dementia:
Glioblastoma
Lymphoma
Metastatic tumor
133
Other causes of dementia:
Symptomatic hydrocephalus
134
Evaluation of a patient with dementia:
History (from caregiver) is essential and may require a separate interview to obtain!
Short term memory issues?
Rapid/slow progression?
Functioning of patient - decline?
Safety of patient at home?
H/O head injury, toxin exposure, infection, psych issues?
Family hx of dementia?
135
Examination for dementia: scales (2)
Folstein MMSE
| MOCA
136
Recommended lab studies for the diagnosis of Alzheimer's disease:
CBC, CMP, ESR, Thyroid fxn studies, B12 level, RPR, CT or MRI of the head
```
Selectively can also perform:
EEG - for CJD, encephalitis, seizures
LP - for CA, infection, vasculitis, NPH
CXR
HIV testing
Drug screen
SPECT or PET scan
Heavy metal screen
```
137
Treatment of AD:
```
ACh inhibitors
- Donepezil
- Rivastigmine
- Galantamine
NMDA receptor antagonist
- Memantine
B complex, lipid-lowering agents, aspirin
```
138
Mild cognitive impairment (MCI)
Memory complaints NOTED BY THE PATIENT
Abnormal memory for age, but do not meet criteria for dementia [... yet]
Probably a precursor to AD (5x more likely to develop AD than those without MCI)
- Treatment with galantamine, rivastigmine, donepezil and other AChE inhibitors to delay the onset of AD
139
Criteria for clinical diagnosis of AD:
Clinical exam (MMSE or MOCA)
Deficits in 2 or more areas of cognition
Progressive worsening of memory or other cognitive function
No change of consciousness
Most often in patients >65 YO
An absence of systemic disorders or other brain diseases that would account for the progressive loss of memory and cognition
140
Supporting findings in diagnosis of AD:
– Progressive deterioration of specific cognitive functions such as aphasia, apraxia, or agnosia
– Impaired activities of daily living and altered patterns of behavior
– Family history of similar disorders, particularly if confirmed
neuropathologically
– Normal L.P.
– EEG: normal or mild generalized slowing
– Progressive atrophy documented by MRI or CT brain
141
Criteria for probable vascular dementia:
Cerebrovascular disease defined by the presence of focal signs on neurologic examination, such as hemiparesis, lower facial
weakness, Babinski sign, sensory deficit, hemianopia, consistent with stroke
A relation between the two previous disorders manifested orinferred by the presence of one or more of the following:
• Onset of dementia within 3 months after a recognized stroke
• Abrupt deterioration in cognitive functions OR
• Fluctuating, stepwise progression of cognitive deficits
142
(Diffuse) Lewy Body Disease characteristics:
Tetrad of symptoms:
- Dementia
- Parkinsonian symptoms (generally bradykinesia and rigidity but NO tremor)
- Prominent psychotic symptoms (usually visual hallucinations)
- Extreme sensitivity to antipsychotic agents
Also progresses more rapidly than AD
Symptoms will vary a great deal from one day to the next
Up to 81% of patients have periods of increased confusion that can last days or weeks (mimics delirium closely)
143
Diffuse Lewy Body Disease characteristics (other):
– Mild to moderate parkinsonian features often present early in disease.
– Bradykinesia, rigidity and falls are often prominent.
– Tremor is often absent.
– Response to Levodopa is often poor.
– Dysautonomia is common.
– Psychotic symptoms are much more common and occur earlier (!!!) in diffuse Lewy body disease than in Alzheimer’s disease.
--> Visual hallucinations are often children or small animals and are not particularly bothersome to patient
144
Diffuse Lewy Body Disease and anti-psychotic agents:
Beware!!!
Most patients with Diffuse Lewy body disease experience
severe, potentially life-threatening adverse reactions if treated with
antipsychotic agents. If an antipsychotic is truly needed, use one of the newer agents (e.g. Seroquel or Zyprexa), not the older drugs
145
PD vs. Diffuse Lewy Body Disease/Dementia:
Parkinson’s disease:
– Midbrain (!) Lewy bodies
– Executive dementia sometimes occurs LATE in illness
– Resting tremor usually PRESENT
– Autonomic dysfunction SOMETIMES
– Hallucinations only IN RESPONSE TO antiparkinsonian drugs
Diffuse Lewy body disease:
– Cortical (!) Lewy bodies
– Cortical dementia always occurs EARLY in illness
– Resting tremor usually ABSENT
– Autonomic dysfunction PROMINENT
– Hallucinations common IN ABSENCE OF antiparkinsonian drugs
146
Frontotemporal degeneration:
– Includes several forms of dementia characterized by a slowly progressive deterioration of social skills and changes in personality, along with impairment of intellect, memory, and
language.
– The disease varies greatly in the way it affects individuals, but often see common core of symptoms:
• Loss of memory
• Lack of spontaneity
• Difficulty in thinking or concentrating
• Disturbances of speech
– Other symptoms include: gradual emotional dullness, loss of
moral judgment, and progressive dementia.
– Usually affects individuals between ages of 40 & 60.
– Patients typically have atrophy of the frontal and temporal lobesof the brain.
– No cure. Length of progression 2-10 years.
147
Normal pressure hydrocephalus:
```
Triad:
- dementia
- gait disturbance
- urinary incontinence
Potentially reversible if ventriculoperitoneal shunting is performed (gait disturbance is the most likely to get reversed if shunt is inserted)
```
