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Neuro > Miscellaneous Neurologic Disorders - Exam 4 > Flashcards

Miscellaneous Neurologic Disorders - Exam 4 Flashcards

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1
Q

What is multiple sclerosis? What are the 4 characteristic features?

A

An autoimmune disorder resulting in damage to the neurons of the CNS

aka the body attacks myelin sheaths

chronic inflammation
demyelination
gliosis (plaques or scarring)
neuronal loss

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2
Q

What is the proposed etiology behind MS?

A

an environmental agent or event (eg, viral or bacterial infection, exposure to chemicals, lack of sun exposure) occurs in the presence of a genetic predisposition to immune dysfunction resulting in an autoimmune attack on the CNS neurons

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3
Q

In the pathophys behind MS: Insult to immune system results in development of _______ (inflammatory T cells, B cells, and macrophages) that cross the BBB. An inflammatory reaction occurs ______ around the axon resulting in what is know as an _____. The body attempts to ______ leads to a build up of rubbery or hardened _______

A

autoreactive lymphocytes

breaking down the myelin

MS “lesion”

remyelination

plaques (sclerosis)

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4
Q

MS more common in males or females? what is the typical age of onset? MC ethnicity?

A

more common in females

between 20-40 years of age
RRMS - average age 28-31
PPMS - average age 40

white people in the northern US

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5
Q

What are the risk factors for MS?

A

genetics
low vit D
EBV exposure
smoking

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6
Q

How does MS present? **What is the important phrase to remember here? What are some sensory symptoms a pt might complain about?

A

abrupt or insidious “attacks” that range from asymptomatic to severe that are separated in time and space and s/s are increased when the body temperature increases

sensory symptoms include: paresthesias, hypethesia (decreased sensation, numbness), unpleasant sensation, pain, shock-like sensation radiating down the back of the legs

s/s rarely follow dermatomes and sometimes the patient describes them as “weird”

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7
Q

What is it called when the s/s worsen by increased body temp?

A

Uhthoff Phenomenon

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8
Q

What are shock-like sensation radiating down back of legs called? Where do they RARELY present?

A

Lhermitte’s symptoms

rarely down the arms

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9
Q

What are some eye conditions that are associated with MS?

A

optic neuritis: usually unilateral, blurred vision, pain with EOM movements and central field disturbances, decreased color perception

RAPD: unequal response to pupillary constriction with swinging light test

optic disc may be normal or atrophied

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10
Q

How will the motor symptoms of MS present?

A

weakness: worse with exercise, facial weakness that may resemble Bell’s palsy

spasticity: if due to UMN lesions

hyperreflexia

+babinski sign

intention tremor

dysarthria: slurred speech

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11
Q

What cranial nerve is involved? Name 2 other s/s that can also be seen on the face with MS.

A

Trigeminal neuralgia - CN V
same s/s as idiopathic TN but often bilateral, occurring before age 50

facial myokymia: involuntary twitching or contraction of the facial muscles

glossopharyngeal neuralgia: intense electric shock-like pain in the posterior pharynx, tongue or ear triggered by swallowing or without warning

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12
Q

How does the trigeminal neuralgia seen with MS differ from idiopathic TN?

A

MS: bilateral and before the age of 50

idiopathic TN: unilateral and after the age of 40

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13
Q

What is facial myokymia? What CN? What condition?

A

involuntary twitching or contraction of the facial muscles

CN VII

seen in MS

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14
Q

What is glossopharyngeal neuralgia? What condition is it associated with?

A

intense electric shock-like pain in the posterior pharynx, tongue or ear triggered by swallowing or without warning

MS

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15
Q

What are the GU complaints seen with MS?

A

detrusor hyperreflexia

detrusor sphincter dyssynergia: difficulty starting and stopping urine stream, hesitancy, urinary retention, overflow incontinence, recurrent infections

bowel changes: constipation or fecal urgency/incontinence

can also have sexual dysfunction in both men and women

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16
Q

Can also see ______, ______ , _____ and _____ with MS

A

mild cognitive dysfunction

depression

fatigue

vertigo

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17
Q

Describe the timing of MS symptoms? They are usually precipitated by _____ or _______

A

Neurological symptoms lasting 10-120 seconds and occuring 5-40 x/d

Precipitated by hyperventilation or movement

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18
Q

What do they contribute the paroxysmal symptoms of MS to? Give some MS paroxysmal symptoms

A

Thought to be a result of spontaneous discharges from neurons at the edge of demyelinated plaques

Lhermitte symptoms, tonic contractions, dysarthria, ataxia, sensory disturbances

these are usually self limiting after weeks to months

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19
Q

What are the 5 types of MS? Which 2 are associated with the very early stages? Which 3 describe the pattern of symptoms?

A

very early:
Clinically isolated syndrome (CIS)
Radiologically Isolated Syndrome (RIS)

types of MS patterns:
RRMS
SPMS
PPMS

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20
Q

What is clinically isolated syndrome? How long does it have to last?

A

the first MS attack

must last for at least 24 hours, is characteristic of multiple sclerosis but does not yet meet the criteria for a diagnosis of MS

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21
Q

What is Radiologically isolated syndrome (CIS)?

A

evidence of MS on brain MRI before “clinical attack” is present

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22
Q

What is the MC type of MS? describe the pattern of symptoms. What is happening in between attacks?

A

Relapsing-remitting MS (RRMS)

discrete attacks of neurological dysfunction that evolves over days-wks (relapse), followed by partial or complete recovery over wks to months (remission)

between attacks patients are neurologically stable

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23
Q

What is secondary progressive MS?

A

starts as RRMS², at some point in the course of the disease a deterioration in function occurs unassociated with the acute attacks

aka quit seeing improvement

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24
Q

What is primary progressive MS?

A

a steady decline in function from disease onset and LACKS the relapse-remission characteristics of other forms of MS

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25
What is the diagnostic criteria for MS? What is the timing?
2 or more episodes of symptoms and 2 or more signs that reflect pathology in different areas anatomically. lesions on MRI can act as the 2nd sign if necessary Symptoms must last > 24 hours and must be separated by at least a month in recurrence
26
What will the lesions on brain MRI with gadolinium contrast look like for a pt with MS?
Acute MS lesions: larger with ill-defined margins. (lasts appx 1 month) Chronic lesions: smaller with sharper margins
27
When would you use evoked potentials as a dx tool in MS? If the test is +, what will it show?
indicated for asymptomatic patients and are most effective in assessment of clinically uninvolved pathways a marked delay in latency of a specific stimulation which is diagnostic in an asymptomatic patients
28
When would you order a CSF analysis in an MS pt? What would you expect to find?
Indicated in patients with CIS or an atypical presentation (e.g. MRI is nondiagnostic) Oligoclonal bands present in 90-95% Increased levels of intrathecal synthesized IgG (70–90%) mildly elevated WBC (< 75 cells/μL) CSF protein is normal to elevated
29
What are the some red flag findings that would make you think this is NOT MS?
symptoms localized to the posterior fossa (ataxia and imbalance), craniocervical junction (neck pain, headache, balance problems, voice changes, difficulty swallowing, respiratory problems or sleep apnea, motor speech issues such as difficulty articulating, compression of the spinal cord, spasticity, twisted or rotated neck) or spinal cord (sensory and motor symptoms only) patient is <15 or >60 years of age clinical course is progressive from onset patient has NEVER experienced visual, sensory, or bladder symptoms diagnostic findings (e.g., MRI, CSF, or EPs) are atypical
30
What is the tx for acute exacerbations? What is the pt does not respond to the first treatment?
glucocorticoids: High-dose IV or oral methylprednisolone: reduce severity and shorten duration of attacks Plasma exchange to get rid of the antibodies
31
What is the first line tx for disease modifying treatment in MS? What is their MOA?
Monoclonal antibodies function to decrease the AI response
32
**What do you need to screen for before starting a pt on monoclonal antibodies? **Why?
screen for immunity to Hepatitis A,B,C and VZV **BBW: risk of progressive multifocal leukoencephalopathy (PML) an opportunistic viral infection (John Cunningham [JC] virus) of the brain that usually leads to death or severe disability
33
Need to assess if a pt has ______ before starting monoclonal antibodies. Do you want to result to be positive or negative? Why?
JC antibodies patients who are seronegative are less likely to develop PML
34
**Which monoclonal antibody is the only agent indicated for PPMS? What are the rest of the agents indicated for?
ocrelizumab (Ocrevus) rest are indicated for RRMS
35
What is the 2nd line tx for disease modifying therapy in MS?
Fumarates and Sphingosine 1-phosphate receptor (S1PR) modulators
36
______ MOA not fully known - modulates proinflammatory and anti-inflammatory cytokines. Has anti-oxidative properties. what do they end in?
fumarates fumarates
37
_____ MOA binds to the lymphocyte surface (within the lymph nodes/spleen) preventing the release of lymphocytes into the PNS in turn preventing migration to the CNS. What do they end in?
Sphingosine 1-phosphate receptor (S1PR) modulators - end is "mod"
38
What is the 3rd line tx in MS? What is the MOA?
Platform Injection Therapy: Glatiramer Acetate Interferon β Works in a variety of ways to suppress the immune system
39
What is used in MS to treat the ataxia/tremor? What is used to treat severe spasticity and spasms?
Clonazepam, propranolol, primidone Thalamotomy and deep-brain stimulation - mixed results when utilized severe: Baclofen oral or intrathecal pump if severe
40
______ is MS specific med that is used to treat the symptomatic LE weakness when it interferes with ambulation. What is the MOA?
dalfampridine (Ampyra) K+ channel blocker
41
What factors favor a BETTER prognosis in MS?
ON or sensory symptoms at onset fewer than two relapses in the first year of illness minimal impairment after 5 years
42
What factors favor a WORSE prognosis in MS?
truncal ataxia action tremor pyramidal symptoms primary progressive disease
43
What are the 4 pt education points needed in MS?
1. maintaining an optimistic outlook 2. a healthy diet 3. regular exercise without excessive overheating as tolerated. swimming is often well-tolerated because of the cooling effect of cold water 4. correct vitamin D deficiency
44
A patient with RRMS presents with complaints of increased weakness and double vision that began this morning. On exam you note a temperature of 103.2. Work-up reveals the patient is positive for Influenza A. Should we start high dose steroids to treat the symptoms of her acute MS?
not recommended to tx because the pt is experiencing "pseudo-exacerbation" due to the increase in body temperature not actually a true MS acute episode. Tx the flu and MS symptoms should improve
45
What is the key description of cerebral palsy? What is the underlying cause?
An umbrella term used to describe a group of disorders that cause permanent, NON-PROGRESSIVE motor dysfunction affecting muscle tone, posture, and/or movement Results from an insult to the developing brain during birth, delivery, or in the perinatal period
46
______ is the leading cause of childhood disability. _____ are congenital and _____ are acquired
cerebral palsy 70-90% are congenital 10-30% are acquired
47
What are the risk factors for cerebral palsy? which ones are modifiable?
Genetic abnormality toxic or infectious etiology vascular insufficiency Modifiable risks: maternal smoking, maternal heavy alcohol use, maternal obesity
48
Gross motor delay in first year of life Abnormal muscle tone - most often hypotonia followed by spasticity Definite hand preference before 12 months of age Asymmetric crawling or failure to crawl Growth disturbances (weight/height) Hyperreflexia Some cases - persistent primitive reflexes What am I?
cerebral palsy
49
What are the different ways CP is classified? Which on is the MC?
Based upon predominant impairment AND location of disability spastic- MC 70-80% dyskinetic ataxic AND (see picture)
50
What type of CP? Increased tone Hyperreflexia Muscle contractures how common is it?
spastic MC- 70-80%
51
What type of CP? Involuntary movements Hyper- or hypotonia Trouble maintaining an upright position Variable degree of dysarthria Intellectual disability how common is it?
dyskinetic CP less common :10-15%
52
What type of CP? Impaired balance and coordination Speech typically is slow, jerky, and explosive Hypotonia Poor motor skills trouble writing, typing, using scissors Difficulty with auditory and visual processing how common is it?
ataxic CP least common: less than 5% of cases
53
How do you dx cerebral palsy? at what age?
Diagnosis is clinical - based upon history and PE confirming dx is best deferred until after age 2 due to postnatal brain development
54
_____ are used most often in unstable neonates who are unable to be transported for MRI/CT when looking for CP
cranial US
55
______ is the best imaging modality after 2-3 weeks of age for CP. What is the imaging is normal?
brain MRI Normal neuroimaging studies do not exclude a clinical diagnosis of CP, but may indicate a genetic or metabolic etiology
56
Name 3 additional tests used in CP. Why would you order each?
57
What is the management for CP?
no specific tx for CP so manage all the presenting symptoms individually need to consult: physical, occupational and speech therapy
58
______ is a procedure used in CP to decrease spasticity. What is a brief summary of the procedure?
Selective dorsal rhizotomy surgical ablation of 70-90% of the dorsal nerve roots
59
Might need to consult _____ and _____ in a child with CP. Why?
ortho and ophthalmology because muscles can get so tight that they pull bones out of the socket and strabismus in the eyes. also higher rates of cataracts
60
What are 3 organ systems that have frequent complications due to CP?
Respiratory- risk of aspiration pneumonia, lung/bronchopulmonary dysplasia, bronchiolitis, asthma Gastrointestinal - reflux, constipation Skin - decubitus ulcers
61
What is the prognosis with CP?
related to the severity of the condition and their individual complications 25% have mild impairment that doesn't affect ambulation, self care or other activities 50% are able to achieve complete independence 25% are unable to walk
62
What is complex regional pain syndrome? What is it characterized by? What does it often follow? What age range?
An inflammatory disorder affecting a region of the body (most often an extremity) characterized by pain, swelling, limited ROM, vasomotor instability, skin changes and patchy bone demineralization often follows trauma but does NOT reflect the severity of the trauma 30-60 years old
63
What are the 3 stages of complex regional pain syndrome? How long does each last?
acute, subacute and chronic acute: lasts approx 3 months subacute: 3months-1 year chronic: 1+ year after disease onset
64
What s/s are present during the acute stage of CRPS? What makes the pain worse? ______ of the affected limb due to a lack of use
pain is so severe it prevents the use of limb swelling, erythema and edema of the extremity with dependance, hyperhidrosis, skin cool to touch neuropathic: burning, cutting, searing, pressure, tearing constant and disproportionate to the precipitant injury pain increases with: loud noises, stress, light touch, active or passive motion bone demineralization
65
What are some s/s of the subacute phase of CRPS?
persistent severe pain and fixed edema cyanosis or pallor dry atrophic skin atrophy of the subcutaneous tissue joint fibrosis - stiffness and limited use further bony demineralization
66
What are some s/s of chronic phase of CRPS?
symptoms can last for years before abating or may be permanent pain is variable *edema resolves* skin is dry, pale, cool and shiny - flexion and extension creases become absent joint fibrosis persists leading to loss of function and stiffness bone demineralization become osteoporosis
67
**What is the criteria called for dx CRPS?
Budapest Criteria
68
What should be included as part of your work-up for CRPS? What are you looking for?
xray: looking for signs of demineralization bone scintigraphy (bone scan): assesses bone metabolism in patients with active bone resorption, there will be increased radiotracer uptake in areas of demineralization
69
a bone scintigraphy (bone scan) areas with ______ radiotracer uptake indicate _______
increased radiotracer uptake in areas of demineralization
70
What is the first line tx CRPS?
first line tx -> Physical and occupational therapy as soon as dx is made Psychosocial and behavioral therapy
71
Why do you give pain meds in CRPS? Which ones? What can you give if evidence of bone demineralization?
Goal - to achieve better compliance with PT/OT 1. start with NSAIDs 2. add neuropathic meds (gabapentin (Neurontin), pregabalin (Lyrica) or TCA 3. topical agents: lidocaine or capsaicin refer to pain management!! for injections add bisphosphonates
72
What is an important pt education point for CRPS?
explain the long term benefits of PT and OT to improve compliance !!! pt will experience an increase in pain with therapy. Remind them of the long term goal of regaining use of the affected limb
73
What is the prognosis with CRPS? How often does it reoccur?
Uncertain and highly variable Most patients report some degree of prolonged disability Recurrence is common: 10-30% Seen most frequently in younger patients
74
What is Amyotrophic Lateral Sclerosis (ALS)? What is it characterized by?
A fatal neurodegenerative disease of the lower AND upper motor neurons characterized by progressive loss of motor function progressive loss of motor function
75
What is the "lateral sclerosis" in ALS?
changes seen in the lateral columns of the spinal cord where the UMN degenerate and are replaced by fibrous astrocytes (gliosis)
76
What is the pathophys behind ALS? part 1 Impaired ____ and _____ release toxins. Impaired _____ leads to _____ at the synaptic cleft. Excessive _____ leads to increased uptake of _____.
astrocytes and microglia astrocyte increased glutamate glutamate Ca+
77
What is the pathophys behind ALS? part 2 _____ inside the neuron inhibits the ____ removal. Excessive _____ leads to neurodegeneration via ______. _______ form intracellular aggregates worsening _______ and turn into ______ which results in neuronal death
Mitochondrial dysfunction calcium calcium oxidative stress Mutant proteins oxidative stress neurofilaments
78
What is the etiology behind ALS?
UNKNOWN!! family component in 5-10% of cases but SPORADIC is the key thing to remember
79
What are the risk factors for ALS? How do you differentiate ALS from all other motor neuron diseases?
risk increased with age - peaks in mid 70’s family hx smoking ALS effects both UMN and LMN
80
What s/s start first in ALS? What is the MC initial symptom?
symptoms start very slow and progressive and will reflect the body segment of the affected UMN lesions extremity dysfunction: tripping, stumbling, trouble running, foot/wrist drop, reduced finger dexterity
81
________ is also extremely common in ALS. How will it present?
bulbar dysfunction Slurred speech, hoarseness, decreased volume of speech, nasal dysphonia, drooling** involuntary laughing or crying
82
Eventually, ALS patients will start to show signs of _____ and _____
UMN lesions: Babinski, hyperreflexia, spasticity, hypertonia, clasp-knife rigidity¹, pronator drift LMN lesions: muscle fasciculation, hypotonia, hyporeflexia with generalized weakness
83
How do you dx ALS? What will electromygraphy show?
clinical dx! all labs and imaging will be normal electromyography: measures the electrical activity in your muscles to evaluate how well your muscles and nerves are working will show features of acute and chronic denervation and reinnervation
84
______ is a disease modifying agent in ALS that has been shown to extend the life of a pt. How does it work?
Riluzole (Rilutek) inhibits glutamate release
85
_______ is a disease modifying agent in ALS that slows the functional deterioration. What is the MOA? ______ to get the best result
Edaravone (Radicava IV or ORS) a free radical scavenger that reduces oxidative stress best results if started early in course of disease
86
______ is the gene silencing therapy in ALS and is only helpful in patients with the mutation in the _____ gene
Tofersen (Qalsody) superoxide dismutase-1 (SOD1)
87
What is the MOA of Tofersen (Qalsody)? What is the outcome?
binds to the SOD1 mRNA, resulting in degradation and prevention of SOD1 protein synthesis - ultimately decreasing oxidative stress reduces progression of disease
88
______ was the ALS drug that was taken off the market
Sodium phenylbutyrate-taurursodiol (Relyvrio)
89
What is the tx for drooling in ALS?
Anticholinergics (amitriptyline, scopolamine) Sympathomimetics (pseudoephedrine) Salivary gland irradiation Botulinum toxin type B
90
_____ is needed at night for ALS pts to help with ventilation. ______ if difficulties with swallowing/appetite. may need ______ to help sustain nutrition
BiPAP at night speech therapy and nutritionist (PEG) tube placement
91
Why does an ALS pt need to see PT/OT?
maintain a low impact exercise regimen to maintain ROM and tone, prevent contractures
92
_____ is the median survival of an ALS pt. What is the MC cause of death?
3 years aspiration pneumonia
93
_______ is an acute condition resulting in global cerebral dysfunction in the _________
Toxic-Metabolic Encephalopathy absence of primary structural brain disease
94
What is the pathophys behind toxic-metabolic encephalopathy?
Dysfunction of the ascending reticular activating system and/or its projections to the cerebral cortex, leading to impairment of arousal and/or awareness
95
What are risk factors for Toxic-Metabolic Encephalopathy?
ICU placement Older patients Underlying dementia
96
Cognitive dysfunction ranging from confusion to delirium or coma Seizures Exaggerated physiologic tremor Asterixis - flapping tremor Myoclonus Presence of (+) Babinski, brisk DTRs What am I? What is the most common etiology?
Toxic-Metabolic Encephalopathy sepsis
97
What needs to be part of the work-up for Toxic-Metabolic Encephalopathy?
CT scan/MRI: rule out ddx when focal signs are present on PE or when subdural hematoma is suggested in the history Electroencephalography (EEG): confirms global cerebral dysfunction labs
98
What is the prognosis for Toxic-Metabolic Encephalopathy?
Can be reversible if recognized and treated promptly Neurological improvement is slower than that of underlying condition
99
Neurofibromatosis (NF) A genetic disorder characterized by _____ on _____ that results from a _____ or ____ of _______
tumor formation nerve tissue mutation or deletion tumor suppressor genes
100
What is the clinical presentation of neurofibromatosis type 1? When does it appear?
-greater than 6 Cafe au lait spots - freckling of the axilla or groin: appears by age 3-5 - Lisch nodules - neurofibromas - diminished bone mineral density - optic glioma - learning disability -larger than normal head size -short stature -elevated blood pressure
101
What are Lisch nodules? What dz are they associated with?
Gold-tan-brown dome-shaped gelatinous masses on the surface of the iris Neurofibromatosis Type 1
102
Describe the neurofibromas seen in type 1.
usually SOFT and pea sized lesions, NOT TENDER OR PAINFUL can be on or under the skin but can grow deep inside the body
103
What is Plexiform neurofibroma?
tumor formation on a nerve plexus, can lead to disfigurement
104
Describe a pt's bones with Neurofibromatosis Type 1?
diminished bone mineral density¹, abnormal bone growth results in scoliosis or bowing of the lower legs
105
What will optic gliomas present like in Neurofibromatosis Type 1?
visual disturbances, proptosis (tumor pushes the eye outward) compression on pituitary gland
106
Why is it common to see elevated blood pressure in Neurofibromatosis Type 1?
often due to renal artery stenosis or pheochromocytoma may develop during childhood
107
**What is the criteria to dx neurofibromatosis type 1?
Requires at least 2 of the 7 NIH criteria
108
What is the tx for Neurofibromatosis Type 1?
no specific tx- tx all individual manifestations as they arise refer to NF clinic
109
What is the complication from Neurofibromatosis Type 1?
Malignant peripheral nerve sheath tumors (MPNSTs) (aka neurosarcomas) a rare aggressive type of (lifetime risk of 10%).
110
What are the 3 red flags for a malignant tumors in Neurofibromatosis Type 1?
significant and persistent pain within the lesion transition of lesion from soft to hard rapid growth of a nodule
111
What is the life expectancy of a pt with NF type 1?
Life expectancy in NF1 is approximately 8 years less than the general population usually caused by a malignant tumor
112
What is the clinical presentation of NF2-SWN? Is there a strong family link? When do symptoms appear?
tumors of both the CNS and PNS with LESS prominent cutaneous manifestations than NF-1 50% are new mutation- family hx is often negative! s/s onset usually in early adult years (20-25)
113
What are the MC lesions in NF2-SWN? What are the clinical findings?
benign, slow-growing tumors of the vestibulocochlear nerve gradual hearing loss, tinnitus ataxia headaches
114
Besides the ear, name 3 additional places NF2-SWN lesions will appear?
Meningiomas - often multiple Spinal tumors - pain, radiculopathy, weakness, paresthesias Cataracts - visual impairment
115
What additional tests should you order if you suspect NF2-SWN?
genetic testing MRI of brain and spinal to assess tumor burden refer to ophthalmology for eye exam
116
a pt with NF2-SWN should be referred to _____ and annual exams by what 3 specialilties?
NF clinic for chronic management audiologist, ophthalmologist and neurologist
117
What is Schwannomatosis (Non NF2 Related)? What is the key difference?
A rare condition resulting in multiple painful noncutaneous schwannomas (a benign tumor of the nerve sheath) affecting the peripheral nerves, spinal roots, and cranial nerves without vestibular involvement WITHOUT VESTIBULAR INVOLVEMENT This one is PAINFUL!!
118
What are the clinical findings in non NF2 related Schwannomatosis?
focal pain - neuropathic and nociceptive features - may be disabling neuropathic: hot, cold, cutting, crushing, burning nociceptive: sharp, aching, or throbbing palpable mass - usually nontender/nonpainful focal numbness and weakness muscle atrophy PAINFUL!!
119
What do you need to do next when working a pt up for Schwannomatosis?
genetic testing MRI w and w/o of brain and spine and may do whole body MRI to determine extent of disease
120

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