Neurology Notes Flashcards

You may prefer our related Brainscape-certified flashcards:
1
Q

3 general etiologies of stroke and which one has the worst prognosis

A

Emboli

Thrombi

Hemorrhage (worst prognosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Stroke affecting anterior circulation — possible area of deficits?

A

Leg/foot

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Stroke affecting middle cerebral artery — possible area of deficits?

A

Face/arm/speech

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Stroke affecting posterior circulation, which is composed vertebral arteries that come to form the basilar artery — possible deficits?

A

Cerebellar dysfunction, change in mental status (syncope), blindness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Regardless of presentation, in the acute phase of a stroke (within 30 minutes of presentation and within 6 hours of symptoms) the goal is rapid identification and intervention if possible. What is the first step in workup?

A

CT scan without contrast — to rule out hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

The first step in workup for stroke is CT without contrast to r/o hemorrhage. At that point, intervention can be considered. After initial presentation (day 2), additional testing is required to assess for cause. ______ can be done to assess cardiac valves, ________ for carotid stenosis, and ______ to assess for afib.

A

2D echo; carotid duplex; ECG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

If the workup for stroke is uncertain, _____ is the best radiographic test to confirm stroke but is not needed.

________ is a test that can replace the carotid ultrasound and can identify causes of ischemia such as vasculitis

A

MRI

CT angiogram

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Pt presents with focal neurologic deficit and you suspect stroke. CT scan confirms hemorrhagic stroke. What is the next step in management?

A

Neurosurgery — will decide to coil, clip, or craniotomy

ICU

BP control with goal systolic of <150

FFP if there is derangement in INR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Pt presents with thrombotic stroke and you determine it was d/t carotid stenosis. U/S of carotids shows >70% stenosis. What is the next step in management?

A

Carotid endarterectomy (or stent)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Terminology: the infarcted are is the area of brain affected by stroke that cannot be saved. The ______ is the surrounding area that can be saved with intervention

A

Penumbra

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Indications and contraindications for tPA following stroke

A

Indications:
Thrombotic/embolic stroke only
Symptom onset <3 hours

Contraindications:
History of brain bleed
History of head trauma
Surgery in the last 21 days

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Most strokes occur and it’s too late to do anything for them, so preventing another becomes crucial. _____ is the mainstay tx unless the pt has an allergy. If there is an allergy, use ________.

If there’s a stroke on the mainstay tx above, add a second agent, usually _______. Everyone gets an antiplatelet within 24 hours unless they get tPA.

A

ASA; clopidogrel

Dipyridamole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are some conditions that require medical management to prevent stroke?

A

Dyslipidemia — high potency statin

Diabetes — keep a1c <7%

HTN — goal BP <130/<80

Smoking — cessation counseling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

tPA’s use for strokes is greatly restricted but can actually rescue ischemic tissue and preserve the penumbra. The risk of transforming an ischemic stroke into a hemorrhagic one is high so caution must be used. What are 3 other physiologic parameters that you can control to allow the at-risk penumbra to recover?

A

Keep O2 sat >95%

Tight glucose control 60-100

Blood pressure - permissive hypertension (goal <220/120)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

If patients present within 4.5 hours of symptom onset, or 3 hours for pts with ________, they can be considered for tPA

A

Diabetes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

If stroke occurs due to carotid stenosis, a carotid endarterectomy can be performed. While carotid stenting can be performed, this should be reserved for pts who CANNOT undergo surgery. Stenting and endarterectomy should be performed in the acute setting, always within ________. A stuttering stroke (TIA) or an evolving stroke should prompt more emergent intervention

A

2 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

If a pt has a stroke and they are determined to have afib, they need to be on anticoagulation. If warfarin is used, the goal INR is ______. It doesn’t matter whether you use warfarin or the non-vitamin K anticoagulants (NOAC), except that NOACs cannot be used in ______ afib.

A

2-3

Valvular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

If a pt has a stroke and they are determined to have afib, they need to be on anticoagulation. If warfarin is used, the goal INR is 2-3. It doesn’t matter whether you use warfarin or the non-vitamin K anticoagulants (NOAC), except that NOACs cannot be used in valvular afib. Which option for anticoagulant requires a bridge?

A

Neither!

Often, initiation of warfarin requires a heparin bridge. However, afib is one time where you definitely do NOT need to bridge (unless there is another indication like a mechanical valve)

NOTE that if a thrombus is found — you must bridge to warfarin!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Warfarin or NOAC should be used when pt has chronic afib with CHADS2 score of 2+. What are the elements of the CHADS2 score for embolic stroke prevention?

A
CHF
HTN
Age >65
DM
Stroke (worth 2 pts)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Hemorrhagic strokes due to subarachnoid hemorrhage or intracranial hemorrhage may present with cushing’s reflex, which is what?

A

Bradycardia + HTN

[impending herniation]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

A grand mal seizure presents with tonic clonic convulsions, bowel/bladder incontinence, and tongue biting. There’s a loss of consciousness, but it is the __________ that separates a seizure from alternative causes of LOC

A

Post-ictal confusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

A pt presenting with a seizure that is not actively seizing requires what 3 tests for workup?

A

CT

VITAMINS mnemonic

EEG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

If a pt presents with a seizure, is post-ictal, or has entered status epilepticus, they need to be treated as a medical emergency. What steps must immediately be followed (i.e., one after another if the prior step does not work)?

A
  1. IV/IM Benzos (lorazepam/diazepam)
  2. Fosphenytoin
  3. Midazolam
  4. Propofol
  5. Phenobarbital
  6. Draw labs and reverse any underlying defects
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

The VITAMINS mnemonic helps determine cause of a seizure — what does it stand for?

A

Vascular — stroke, AVM, hemorrhage

Infection — encephalitis, meningitis

Trauma — MVA, TBI

Autoimmune — lupus, vasculitis, arthritis

Metabolic — Na, Ca, Mg, O2, glucose

Idiopathic

Neoplasm

Sychiatric — faking it or iatrogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

3 antiseizure medications considered broad specturm and first line therapy

A

Valproate
Lamotrigine
Levetiracetam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What test is used to diagnose a seizure and the location of origination?

A

EEG

Look for spike and waves indicative of organized neuronal firing (which is abnormal for an awake adult). Note that 24 hr video monitoring may be required to catch the seizure and its manifestations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Partial seizures involve a specific complaint while generalized indicate total brain involvement. What is the difference in treatment options?

A

Partial = Carbamazepine or Phenytoin

Generalized = Valproate or Lamotrigine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Difference between complex vs. simple seizures

A

Complex = with LOC

Simple = without LOC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Tx for atonic seizures

A

Valproate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Define atonic seizures

A

Loss of tone without loss of consciousness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Define absence seizures

A

Loss of consciousness without loss of tone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Tx for absence seizures

A

Ethosuximide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Jerky muscles indicates myoclonic seizures, what is the tx of choice?

A

Valproate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Tx of choice for trigeminal neuralgia

A

Carbamezepine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Parkinson’s is caused by a loss of ______ neurons within the _______. This essentially eliminates the “go” signal, preventing the initiation of movement.

A

Dopaminergic; substantia nigra

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Classic symptoms of parkinsons

A

Bradykinesia (difficulty initiating movement)
Cog-wheel rigidity
Resting pill-rolling tremor
Gait disturbances/postural instability

Pt will have difficulty with get-up-and-go test, and will walk with shuffling steps. A board buzz word is a mask-like expressionless face

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Tremor etiologies

A
Parkinsons
Essential
Huntingtons
Delirium tremens
Cerebellar dysfunction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

T/F: MRI of the substantia nigra to show degeneration is needed to confirm dx of Parkinsons

A

False

Parkinsons is a clinical dx. Imaging may be utilized to r/o something else (CT for bleeding, MRI for stroke), but these are not needed for diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Treatment of Parkinsons is about the “go” signal which is dopamine. The stop signal is acetylcholine. Dopamine agonists are the mainstay of therapy for young, functional people (<70, maintained function). Bromocriptine is an older dirtier dopamine agonist, so the 2 better options are ______ and _____

A

Ropinirole

Pramipexole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

While dopamine agonists are the mainstay of therapy for Parkinsons in young, functional people (<70, maintained function), __________ is the mainstay of therapy for everyone else.

A

Levodopa-carbidopa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Why is levidopa combined with carbidopa for the tx of Parkinsons?

A

Levodopa CAN cross the BBB while carbidopa cannot. Carbidopa prevents the conversion of L-dopa into dopamine peripherally, meaning that more levodopa gets to the brain and more dopamine is created

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Eventually, levodopa-carbidopa begins to wear off in the tx of Parkinsons. The _______ and _______ are brought in as L-C begins to fail. There’s no way to determine how to add them

A

COMT-inhibitors (capones); MAO-B inhibitors (Selegiline)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Acetylcholine-R antagonists like _______ could theoretically work to tx Parkinsons, but the acetylcholine side effects are not worth it for the elderly and the effect is weak. Use this on young people who have tremor only

A

Benztropine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Side effects of dopamine agonists used in Parkinsons

A

Hypotension

Psychosis — schizophrenic sxs with hallucinations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Anti-influenza drug sometimes used to tx Parkinsons in functional pts >60 y/o

A

Amantadine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Tremor that is absent at rest but worsens with movement. There’s typically a family history of tremor and it’s often a man.

A

Essential tremor (aka Familial tremor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Tx option for essential tremor

A

Beta blockers

48
Q

Tremor that’s absent at rest but worsens the closer to the target the finger gets. Usually due to physical lesion (stroke, atrophy, etc)

A

Intention tremor (indicates cerebellar dysfunction)

49
Q

_______ is an autosomal dominant genetic disease caused by trinucleotide repeats. The more repeats the person has, the earlier the disease sets in. Most people begin to exhibit symptoms near middle age (30-60). It also exhibits ________, a phenomenon in which disease occurs earlier and earlier in subsequent generations as the number of trinucleotide repeats expands. ________ is purposeless ballistic movements. The prognosis is terrible as it leads to dementia, psychosis, and often death by suicide. There is no treatment.

A

Huntington’s; anticipation; chorea

50
Q

Headache red flags

A

New headache at age >50
Suddent crescendo headache
Fever + headache
Focal neurologic deficits

51
Q

Most common cause of headache; usually b/l vice-like pain that radiates from the front to the back/neck and aggravated by exercise

A

Tension headache

52
Q

Headache characterized by severe unilateral pain that has autonomic symptoms (rhinorrhea, lacrimation, conjunctival injection, Horner’s) and tend to occur over and over for a period of weeks, then pts go symptom-free for months at a time

A

Cluster headaches

53
Q

When cluster headache is diagnosed, a(n) _______ should be obtained to rule out other diseases that mimic cluster. _______ is first line treatment and is often sufficient to abort attacks. __________ can be used if that fails.

A

MRI; Oxygen; Sumatriptan

54
Q

Prophylaxis for cluster headaches

A

Calcium channel blockers like verapamil

55
Q

POUND mnemonic for migraines

A
Pulsatile
One day in duration
Unilateral
Nausea and vomiting
Disabling

Any 3 is diagnostic

56
Q

Type of headache with vascular pathogenesis (arterial vasodilation)

A

Migraine

57
Q

If migraines are severe, start tx with Triptan or an ergot. In what comorbidity should these tx be avoided?

A

CAD, due to tendency to cause vasospasm

58
Q

Prophylactic agents for tx of migraine

A

Beta blockers (propranalol)
CCBs (Verapamil or diltiazem)
Anticonvulsants (valproic acid, topiramate)

59
Q

What is pseudotumor cerebri?

A

Also called idiopathic intracranial HTN

Intracranial pressures are elevated, but there is no tumor. Almost all pts are women, obese, and of child-bearing age. There is a strong association with OCPs, but other causes include vitamin A, isotretinoin, and glucocorticoid withdrawal. You will see classic signs of intracranial HTN like papilledema. An LP will reveal opening pressure >25 cmH2O and the tap will relieve the headache.

60
Q

Tx for idiopathic intracranial HTN (pseudotumor cerebri)

A

First line tx is acetazolamide

Refractory disease is tx with ventriculoperitoneal (VP) shunts or serial LPs

61
Q

Sumatriptan and fiorcet are often used for acute abortion of migraine headaches. What 3 drugs are included in fiorcet?

A

Butalbital
Acetaminophen
Caffeine

62
Q

Warning symptoms of spinal cord compression

A
Urinary sxs (incontinence or retention)
Sexual dysfunction (ED or priapism)
B/l lower extremity weakness
Sensory deficits in a dermatome
Fever
Hx of cancer
63
Q

If any alarm symptoms for spinal cord compression are present, give _________ immediately. The first imaging to obtain is an x-ray, if positive, leave it at that. If negative, get an MRI. Most things will respond to radiation or surgery.

A

Dexamethasone

64
Q

In a pt with suspected musculoskeletal back pain, what are some things to look for to r/o herniated disc?

A

Pts with herniation will have a lightening or shooting pain down the leg (“sciatica”) exacerbated by hip flexion, movement, cough, or activity

Assess plantar flexion (L4) and dorsiflexion (L5), the common nerves impinged by a bulging disc

65
Q

Which is a better tx for disc herniation - conservative management or neurosurgery?

A

Neurosurgery has better results at 6 months, but both are the same at 1 year

66
Q

If you’ve found a pt that might have a herniation but it is an elderly male, consider that they may have a(n) _________, a simple bone growth into the exit of a nerve root. Get an x-ray then MRI to r/o compression fracture. In this case, between neurosurgery and conservative management, the better tx is __________

A

Osteophyte; neurosurgery

67
Q

Cause of back pain typically found in an elderly pt presenting with a unique form of sciatica. There’s often leg and butt pain that sounds like claudication but is positional (increased symptoms when upright and with exercise; relieved when hunched over). Do an MRI to confirm and surgery to fix.

A

Spinal stenosis

68
Q

A pocket of CSF bulges into the anterior cord that produces back pain and loss of pain/temp, sparing proprioception. As it expands motor and sensation will be compromised. MRI diagnosis it. Surgery corrects it.

A

Syringomyelia

69
Q

If a pt presents with back pain and has a hx of HTN, CAD, and smoking, they might have a AAA. With AAA, the _________________ artery can be affected. It produces a spastic paralysis and a loss of _________. The back pain is from the visceral compression. It can be screened via ultrasound. If there are neuro symptoms, it also requires an MRI and surgery

A

Anterior spinal artery; proprioception

70
Q

Mini mental status exam or mini cognitive assessment can be used to assess for dementia. What constitutes an abnormal score on MMS?

A

<22/30

71
Q

What are some conditions that may mimic dementia?

A

Hypothyroid

B12 deficiency

Subdural hematoma

Syphilis

Uremia

Cirrhosis

Pseudodementia (depression)

72
Q

Most common cause of organic dementia; linked with neurofibrillary tangles, neurotic plaques, and amyloid deposition. CT scan shows diffuse cortical atrophy. Linked to chromosome 21

A

Alzheimers

73
Q

Once reversible causes of dementia have been ruled out and you dx Alzheimers disease, what are tx options?

A

Cholinesterase inhibitors like tacrine or donepezil (Aricept)

These will slow progression but do not reverse disease. Death usually occurs w/i 5-10 years, typically d/t secondary cause like PNA.

74
Q

Condition that in contrast to alzheimers disease, personality and social graces are lost FIRST while memory remains intact. Pts may be violent and/or hypersexual. While Alzheimers shows cortical atrophy, this condition has frontal and hypothalamic degeneration. Diagnosis is clinical and there is no tx

A

Pick’s disease

75
Q

Most common means of acquiring prion disease

A

Sporadic mutation

76
Q

Organic and reversible cause of dementia often seen in elderly pts with an ataxic gait, urinary incontinence, and dementia. Get a CT or MRI to evaluate

A

Normal pressure hydrocephalus

77
Q

Tx for normal pressure hydrocephalus

A

The problem is increased CSF, so do serial LPs to take off extra fluid, then fit them for a VP shunt

78
Q

____________ dementia is essentially Parkinson’s disease with dementia and visual hallucinations. The two differ only by the time of onset but the pathology is the same. Look for Parkinsonian symptoms and dementia

A

Lewy-Body dementia

79
Q

What is the progression of memory loss in Alzheimers?

A

Short term memory first
Long term memory next
Social graces last

80
Q

Mild alzheimers disease may be treated with acetylcholinesterase inhibitors - what are the 3 primary drugs used?

A

Donepezil
Rivastigmine
Galantamine

81
Q

Drug of choice for treatment of severe alzheimers disease

A

Memantine

82
Q

In vertigo, a pt will sense movement where none exists. This will present as either the room spinning, or being unsteady on their feet. Once vertigo is established, it is critical to differentiate between central and peripheral causes. Central causes usually involve cranial nerve deficits. What are central causes of vertigo?

A

CVA
Posterior fossa tumor
Multiple sclerosis
Medications

83
Q

In vertigo, a pt will sense movement where none exists. This will present as either the room spinning, or being unsteady on their feet. Once vertigo is established, it is critical to differentiate between central and peripheral causes. What are peripheral causes of vertigo?

A

Labyrinthitis/vestibular neuritis
Meniere’s disease
BPPV

84
Q

When it comes to central causes of vertigo, whether it’s vertebrobasilar insufficiency or a posterior fossa tumor, the main problem is the structural lesion compressing on or eating away at the cerebellum and brainstem. If there are focal neuro deficits, it’s almost pathognomonic for a central lesion. The first test to order is a(n) _______. If that is normal, follow it with a(n) ______.

A

MRI; MRA

85
Q

Vertigo due to BPPV can be reproduced by the _________ maneuver. Movement exercises dislodge and break up the otolith, curing the pt of the disease. _______ maneuver can be done in office and is often curative

A

Dix-Hallpike; Epley

86
Q

Suspect this disease in a pt with vertigo, nausea/vomiting, and hearing symptoms that occur 4 weeks after a URI (pharyngitis, otitis, sinusitis). It’s a dx of exclusion because pontine strokes and tumors mimic the chronic nature of the vertigo and the URI often goes unnoticed.

A

Labyrinthitis/vestibular neuritis

87
Q

If labyrinthitis/vestibular neuritis is dx early, give ______ within 72 hrs. The disease will resolve in months, but balance and hearing may be compromised for those months. _______ can provide symptomatic relief for vertigo episodes

A

Steroids; meclizine

88
Q

Peripheral cause of vertigo presenting along with hearing loss, fullness, or tinnitus that’s unrelated to movement. Like BPPV, it’s acute, but the vertigo persists, lasting ~30 minutes

A

Meniere’s disease

89
Q

Tx for Meniere’s disease

A

Diuretics

Low salt diet

90
Q

Triad of Meniere’s disease

A

Vertigo
Hearing loss
Tinnitus

91
Q

Pt c/o right-sided hearing loss. Weber test lateralizes a louder sound to the left. Rinne test reveals pt hears sound better when tuning fork is held to pinna than when placed against mastoid bone. What type of hearing loss?

A

Sensorineural

92
Q

Pt c/o right-sided hearing loss. Weber test lateralizes a louder sound to the right. Rinne test reveals pt hears sound better when tuning fork is held to mastoid bone than when placed against pinna. What type of hearing loss?

A

Conductive

93
Q

______ is a state of unconciousness of depressed cerebral function such that there is no response to internal or external stimuli and is by definition reversible

A

Coma

94
Q

Causes of coma

A

LITERALLY ANYTHING

Toxins (EtOH, benzos, opiate)

Electrolyte abnormalities

Endocrine (hypothyroid, thiamine)

Hypoxic/ischemic encephalopathy (drowning, cardiac arrest)

Trauma (diffuse axonal injury)

Brainstem pathology (hemorrhage, infarction)

95
Q

What are some elements of the comprehensive workup for coma?

A

CMP
CT scan
LP
EEG

96
Q

During comprehensive workup for coma, a “coma cocktail” is often given, what does this usually include?

A

Thiamine
D50
Oxygen
Naloxone

97
Q

Condition in which pt has a flat EEG but opens their eyes or has positive caloric test. The pt has no arousal but can move, display pain, and have sleep-wake cycles. Nonetheless, the personality is gone. They’ll never recover and will require tube feeds and institutionalized care for life

A

Persistent vegetative state

98
Q

Condition in which cerebral EEG shows no activity — no arousal, no sleep-wake cycle, no drive to breathe, no intact neural reflexes (caloric reflex and corneal reflex absent)

A

Brain death

[note: 2 doctors must confirm brain death prior to removal of life support]

99
Q

Condition in which pt looks like they’re in persistent vegetative state, but they have full awareness. They’re able to communicate with eye movements. There is no recovery. MRI confirms dx

A

Locked-in syndrome

100
Q

2 major causes of locked-in syndrome

A

Basilar artery infarct
Central pontine myelinolysis

[affects the pons, the site where both motor and sensory tracts pass]

101
Q

Normal response to cold water caloric test

A

Cold water in right ear results in eyes moving to the right with nystagmus to the left

[use COWS mnemonic referring to quick beating movements of nystagmus — cold/opposite, warm/same]

102
Q

Chronic progressive disease of unknown etiology that produces asymmetric UMN and LMN lesions, generally sparing the eyes. Look for atrophy and fasciculations of the tongue and extremities comingled with upward Babinski and hyperreflexia of extremities associated with emotional lability and weight loss though sphincter tone is maintained; associated with superoxide dismutase in 10%

A

Amyotrophic lateral sclerosis

103
Q

When ALS is suspected, r/o spinal lesions with a CT/MRI/spinal x-ray and confirm the diagnosis with ______

A

EMG

104
Q

Autoimmune disease targeting post-synaptic ACh receptors causing fatigability most commonly affecting eyes (diplopia, ptosis), and throat (swallowing). Fatigue is relieved by rest

A

Myasthenia gravis

105
Q

Initial test vs. best test for myasthenia gravis

A

Initial = Anti-ACh receptor antibody (nearly 100% specific with clinical symptoms)

Best = EMG (shows decreased amplitude on repeated stim)

106
Q

________ is first line therapy for myasthenia gravis, thereby increasing acetylcholine concentration. If the disease is associated with thymoma (confirmed by chest CT), thymectomy may be curative.

If the weakness compromises life functions like eating and breathing, then give either _____ or _______.

Finally, refractory disease is treated with ________ or disease-modifying agents such as azathioprine.

A

Pyridostigmine

IVIG; plasmapheresis

Prednisone

107
Q

Paraneoplastic syndrome producing antibodies against presynaptic calcium channels which inhibits release of ACh-vesicles, producing proximal muscle weakness that improves with repeated use

A

Lambert-Eaton syndrome

108
Q

How is Lambert-Eaton syndrome diagnosed?

A

Clinical diagnosis is sufficient, but antibodies should be checked

A CT of the chest should be done to identify the small cell cancer causing the disease

The best test is an EMG showing improvement with repetitive use

109
Q

Tx for Lambert Eaton syndrome

A

Tx underlying small cell cancer with chemo, radiation, and/or resection

If cure is not possible, control sxs with prednisone

110
Q

Demyelinating autoimmune disease that produces an ascending paralysis 1-3 weeks after diarrhea (campylobacter) or flu vaccine. There’s always hyporeflexia while paresthesia and autonomic dysregulation may or may not be present

A

Guillain barre syndrome

111
Q

How is guillain barre diagnosed?

A

Look for evidence of autoimmune processes in CSF with an LP — you will see lots of proteins and very few cells

Confirmation with EMG and nerve conduction velocity test showing decreased nerve conduction velocity

112
Q

Treatment for guillain barre syndrome

A

IVIG or plasmapheresis to eliminate the causative IgG Ab response against myelin

NEVER GIVE STEROIDS

113
Q

Autoimmune demyelinating disease defined by neurologic symptoms separated by time and space; the primary complaint is often blurry vision/diplopia (from optic neuritis)

A

Multiple sclerosis

114
Q

What testing should you do if you suspect multiple sclerosis?

A

MRI — look for periventricular plaques, multiple lesions, or lesions in corpus callosum.

Because it’s often relapsing-remitting, MRI may be non-diagnostic. In this case, an LP with pleocytosis and oligoclonal IgG or evoked potentials may be done

115
Q

Tx for multiple sclerosis

A

Chronic management with interferon, fingolimod, glatiramer

Acute flares get steroids

Symptomatic relief for urinary retention (bethanechol), incontinence (amitriptyline), and spasticity (baclofen)

116
Q

Medication that may slightly prolong life in ALS

A

Riluzole