Neuro 2 Flashcards

1
Q

Characteristic feature of NMJ disorders?

A

Muscle weakness in the absence of UMN or LMN signs

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2
Q

Presentation of MG?

A

Fluctuating and fatiguable extraocular (eg, diplopia, ptosis) and bulbar (eg, dysarthria, dysphagia) muscle weakness as well as symmetrical proximal weakness of the neck and extremities (upper>lower)

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3
Q

Pathogenesis of MG?

A

Autoantibodies against acetylcholine receptors in the motor end plate

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4
Q

List the 3 major types of muscular dystrophy.

A
  1. Duchenne
  2. Becker
  3. Myotonic
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5
Q

Compare the genetics of the 3 major types of muscular dystrophy.

A

Duchenne and Becker: X-linked recessive deletion of dystrophin gene on chr Xp21

Myotonic: AD expansion of a CTG trinulceotide repeat in DMPK gene on chr 19q 13.3

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6
Q

Compare the presentations of the 3 major types of muscular dystrophy

A
  1. Duchenne: onset age 2-3 of progressive weakness, Gower maneuver, calf pseudohypertrophy
  2. Becker: onset age 5-15, milder weakness compared to Duchenne
  3. Myotonic: onset age 12-30, facial weakness, hand grip, myotonia, dysphagias
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7
Q

Compare the comorbidities of the 3 major types of muscular dystrophy.

A
  1. Duchenne: scoliosis and cardiomyopathy
  2. Becker: cardiomyopathy
  3. Arrhythmias, cataracts, balding, testicular atrophy/infertility
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8
Q

Compare the prognosis of the 3 major types of muscular dystrophy.

A
  1. Duchenne: wheelchair dependent by adolescence, death by 20-30 from respiratory or heart failure
  2. Becker: death by 40-50 from heart failure
  3. Death from respiratory or heart failure depending on age of onset
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9
Q

Risk factors for a febrile seizure?

A

Fever from mild viral illness

Family history

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10
Q

Dx criteria for febrile seizure?

A

Typically age 3 months to 5 years
No previous afebrile seizure
No meningitis or encephalitis
No acute metabolic cause

Simple febrile seizures are generalized, last <15 minutes, and do not recur within 24 hours

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11
Q

Manage febrile seizure?

A

Abortive therapy if 5+ minutes

Reassurance

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12
Q

Prognosis of febrile seizure?

A

Normal development/intelligence
~30% risk of recurrence
<5% risk of epilepsy

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13
Q

Next step in a simple febrile seizure after treating the fever?

A

No further evaluation or treatment required; discharge home with education and supportive care

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14
Q

Frequent, prolonged, focal febrile seizures may be concerning for ___.

A

Dravet syndrome

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15
Q

When is hospitalization and observation required in a patient with a febrile seizure?

A

If they have not returned to neurologic baseline

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16
Q

Features of essential tremor?

A

Bilateral action tremor of the hands, usually without leg involvement
Possible isolated head tremor without dystonia
Usually no other neurologic signs
Relieved with alcohol in many cases

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17
Q

Features of tremor in Parkinson’s disease?

A

Resting tremor that decreases with voluntary movement
Usually involves legs and hands
Facial involvement is less common

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18
Q

Features of cerebellar tremor?

A

Usually associated with ataxia, dysmetria, or gait disorder

Tremor increases steadily as the hand reaches its target

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19
Q

Features of a physiologic tremor?

A

Low amplitude, not visible under normal conditions
Acute onset with increased sympathetic activity (drugs, hyperthryoidism, anxiety, caffeine)
Usually worse with movement, can involve face and extremities

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20
Q

Rx essential tremor?

A

Propranolol, especially helpful if patient has coexistent hypertension

Other options - anticonvulsants (primidone and topiramate)

Benzos are effective but should be restricted de to potential for dependence

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21
Q

Rx Parkinson disease?

A

Carbidopa/levodopa

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22
Q

True or false - caffeine use is correlated with the severity of essential tremor

A

False

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23
Q

Presentation of basal ganglia (puaminal) hemorrhage?

A

Sudden focal neurologic deficits that gradually worsen over minutes to hours, progresses to headache, N/V, AMS due to increased ICP

Almost always involves the adjacent internal capsule -> contralateral hemiparesis and hemianesthesia (disruption of corticospinal and somatosensory fibers in the posterior limb)

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24
Q

Most common cause of spontaneous deep intracereberal hemorrhage?

A

Hypertensive vasculopathy involving the small penetrating branches of the major cerebral arteries

Chronic HTN leads to the formation of Charcot-Bouchard aneurysms, which may rupture and bleed within the deep brain structures

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25
ACA deep branches supply blood to what structures?
Head of caudate nucleus and the anterior limb of the internal capsule
26
MCA deep branches supply blood to what structures?
Lentiform nucleus = putamen and globu pallidus
27
PCA deep branches supply blood to what structures?
Thalamus
28
Anterior choroidal artery supplies blood to what structure?
Internal capsule (posterior limb)
29
What is the most common cause of spontaneous lobar/cortical hemorrhage in the elderly?
Cerebral amyloid angiopathy
30
Most common cause of intraparenchymal hemorrhage in children?
AV malformation
31
Common cause of subarachnoid hemorrhage?
Ruptured saccular (berry) aneurysms
32
Bridging vein tears cause ___.
Subdural hematoms
33
Mengingeal artery tears cause ___.
Epidural hematomas
34
Risk factors for subdural hematoma?
Elderly and alcoholics (cerebral atrophy, increased fall risk) Infants (thin-walled vessels) Anticoagulant use
35
Clinical presentation of acute subdural hematoma?
Gradual onset 1-2 days after injury Impaired consciousness, confusion Headache, N/V (increased ICP)
36
Clinical presentation of chronic subdural hematoma?
Insidious onset weeks after injury | Headache, somnolence, confusion, lightheadedness, focal neuro deficits
37
Dx chronic subdural hematoma?
Head CT with crescent shaped hyperdensity (acute) or hypodensity (chronic) crossing suture liones
38
Rx subdural hematoma?
Reverse/discontinue anticoagulants | Surgical evacuation of symptomatic or large bleeds
39
Neuroimaging findings of cardioembolic stroke?
Multiple lesions at the grey-white matter jnction
40
Cause of lacunar stroke?
Chronic HTN causes hyalinosis of small penetrating branches of the major cerebral arteries, leading to lacunar stroke in deep brain structures
41
Cause of normal-pressure hydrocephalus?
Decreased CSF resorption by the arachnoid granulations
42
Imaging findings of NPH?
Ventricular enlargement that is out of proportion to sulci enlargement
43
Cause of diffuse axonal injury?
Result of trauma acceleration/deceleration shearing forces that diffusely damage axons in the brain
44
Causes of myoclonus?
Genetic disorders Seizures Medications Prolonged hypoxia
45
What is myoclonus status epilepticus?
Acute form of post-hypoxic myoclonus that typically develops within 24 hours after the initial hypoxic insult while the patient is still unconscious
46
Characteristics of myoclonus status epilepticus?
Generalized, often symmetric, myoclonus that typically involves the axial, limb, and facial muscles; intermittent eye opening, upward gaze deviation, and swallowing problems
47
What is Lance-Adams syndrome?
Chronic form of PHM, presents days to weeks after the initial insult once the patient has regained consciousness
48
Characteristics of Lance-Adams syndrome?
Focal Exacerbated by action Negative myoclonus can also occur, leading patients to drop things or fall
49
What should be obtained to distinguish between seizure and PHM?
EEG
50
Management of acute and chronic PHM?
Antiepilpetics and supportive care
51
DDx - myopathy
1. Glucocorticoid-induced myopathy 2. Polymyalgia rheumatic 3. Inflammatory myopathies 4. Statin-induced myopathy 5. Hypothyroid myopathy
52
Clinical features of glucocorticoid-induced myopathy?
Progressive proximal muscle weakness and atrophy WITHOUT PAIN or tenderness Lower extremity muscles are more involved (lower before upper)
53
Clinical features of polymyalgia rheumatica
Muscle PAIN AND STIFFNESS in the shoulder and pelvic girdle NORMAL MUSCLE STRENGTH Tenderness with decreased ROM at the shoulder, neck, and hip Responds rapidly to glucocorticoids
54
Clinical features of inflammatory myopathies?
Muscle pain, tenderness, and proximal muscle weakness | Skin rash and inflammatory arthritis may be present
55
Clinical features of statin-induced myopathy?
Prominent muscle pain/tenderness with or without weakness | Rare rhabdomyolysis
56
Clinical features of hypothyroid myopathy?
Muscle pain, cramps, and weakness involving the proximal muscles Delayed tendon reflexes and myoedema Ocasional rhabdomyolysis Features of hypothyroidism
57
Compare the ESR levels in the different types of myopathies.
Normal: glucocorticoid-induced, statin-induced, hypothyroid Elevated: polymyalgia rheumatica, inflammatory
58
Compare the CK levels in the different types of myopathies.
Normal: glucocorticoid, polymyalgia rheumatica Elevated: inflammatory, statin-induced, hypothyroid
59
What is the most frequent precipitant of GBS?
Campylobacter jejuni | Other precipitants include HSV, Mycoplasma, H. influenzae
60
What is the most common cause of fatal sporadic encephalitis in the US?
Herpes encephalitis
61
Presentation of herpes encephalitis?
``` Acute onset (<1 week duration) of focal neurological findings (altered mentation, focal CN deficits, ataxia, hyperreflexia, focal seizures) Fever (90% of patients) Behavioral changes, including hypomania, Kluver-Bucy (hyperphagia, hypersexuality), amnesia ```
62
CSF findings in HSV encephalitis?
Lymphocytic pleocytosis Increased number of erythrocytes (hemorrhagic destruction of temporal lobes) Elevated protein levels Low glucose levels are generally not seen
63
Brain imaging findings in HSV encephalitis?
Temporal lobe lesions (MRI preferred to CT)
64
EEG findings in HSV encephalitis?
Focal findings (prominent intermittent high amplitude slow waves) occur in >70-80% of patients and can be used in some cases as corroborative evidence
65
Dx HSV encephalitis?
PCR analysis of HSV DNA in CSF (highly sensitive and specific) -> gold standard
66
Rx HSV encephalitis?
IV acyclovir
67
Classic CSF findings in cryptococcal meningitis?
Elevated opening pressure Low leukocytes with mononuclear predominance Slightly elevated proteins Low glucose levels
68
Dx cryptococcal meningitis?
India ink preparation
69
Classic CSF findings in bacterial meningitis?
Markedly increased PMNs and decreased glucose levels
70
Clinical features of post-concussive syndrome?
Headache, confusion, amnesia, difficulty concentrating/multitasking, vertigo, mood alteration, sleep disturbance, anxiety
71
What is the classic symptom triad of NPH?
Cognitive impairment Urinary incontinence Gait abnormalities Other symptoms may include depressed affect (frontal lobe compression) and UMN signs in lower extremities
72
Dx NPH?
Neuroimaging shows ventriculomegaly out of proportion to the sulci Confirm with LP -> normal opening pressure and transient clinical improvement following high-volume CSF removal
73
Rx NPH?
Ventriculoperitoneal shunt
74
How might OTC cold medications provoke a warfarin-associated intracerebral hemorrhage?
Contain acetaminophen -> potentiates anticoagulant effect of warfarin Decongestants contain phenylephrine -> may elevate BP
75
Management of warfarin-associated intracerebral hemorrhage?
Reverse anticoagulation immediately to reduce the risk of death and permanent disability Initial therapy -> IV vitamin K (sustained response, takes 12-24 hours to be effective by promoting clotting factor synthesis in the liver + Prothrombin complex concentrate (PCC) -> vitamin K-dependent clotting factors (eg, II, VII, IX, X) that rapidly reverse warfarin
76
If PCC is not available for reversing warfarin, what can be given?
Fresh frozen plasma
77
Why should LP be avoided in patients with acute intracerebral hemorrhage?
Risk of inducing herniation as a result of increased ICP
78
When is protamine sulfate indicated?
When urgent reversal of heparin is needed
79
Sources of foodborne botulism?
``` Improperly canned foods Aged seafood (eg, cured fish) ```
80
Pathogenesis of foodborne botulism?
Toxin inhibits presynaptic ACh release at NMJ
81
Presentation of foodborne botulism?
Prodrome: GI discomfort, dry mouth, sore throat Acute onset within 36 hours of ingestion: - Bilateral cranial neuropathies -> blurred vision (due to fixed pupillary dilation), diplopia, facial weakness, dysarthria, dysphagia - Symmetric descending muscle weakness - Diaphragmatic weakness with respiratory failure
82
Dx foodborne botulism?
Serum analysis for toxin
83
Rx foodborne botulism?
Equine serum heptavalent botulinum antitoxin for adults and children >1 y/o Supportive care
84
Presentation of cholinergic toxicity caused by organophosphate poisoning?
Bradycardia, miosis, bronchospasm, vomiting, diarrhea
85
Rx cholinergic toxicity? MOA?
Atropine and pralidoxime Atropine blocks peripheral effects of ACh at muscarinic receptors Pralidoxime aids in the reactivation fo AChE
86
What is the edrophonium test used to diagnose?
MG (temporarily improves muscle weakness as a short-acting AChE inhibitor)
87
Presentation of tick paralysis?
Paresthesias and fatigue followed by ascending muscle paralysis
88
Rx GBS and MG crisis?
Plasmapheresis