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Deja reivew USMLE 2 > Demyelinating Diseases > Flashcards

Demyelinating Diseases Flashcards

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USMLE® Step 1 flashcards
1
Q

Delerium or Dementia?

Usually a rapid onset

A

Delerium

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2
Q

Delerium or Dementia?

Characterized by memory loss

A

Dementia (think deMEMtia)

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3
Q

Delerium or Dementia?

Associated with disturbances in sleep-wake cycle

A

Delirium

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4
Q

Delerium or Dementia?

Often irreversibel

A

Dementia

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5
Q

Delerium or Dementia?

Associated with changes in sensorium (hallucinations and illusions)

A

Delirium

(but can sometimes be present in dementia)

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6
Q

Delerium or Dementia?

Inattentiveness

A

Delerium

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7
Q

Delerium or Dementia?

Waxing and waning level of consciousness

A

Delirium

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8
Q

Name four major causes of delirium.

A

“HIDE”

  1. Hypoxia
  2. Infection (often UTIs) and ICU psychosis
  3. Drugs (anticholinergics, opioids, steroids, barbiturates), and withdrawal (especially EtOH)
  4. Electrolyte and Endocrine causes
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9
Q

Which two syndromes are classically seen in alcoholics experiencing delirium?

A
  1. Wernicke’s encephalopathy
  2. Korsakoff’s psychosis
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10
Q
A
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11
Q

What is the primary cause of these Wernicke’s ensephalopathy and Korsakoff’s psychosis?

A

Thiamine deficiency

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12
Q

List four important steps in the evaluation of a patient with new-onset delirium

A
  1. Check vitals (including O2 saturation).
  2. Check med list.
  3. Check lab values.
  4. Check for occult infection.
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13
Q

Name other common etiologies for dementia:

A

“DEMENTIASS”
Degenerative diseases (Parkinson, Huntington)
Endocrine (thyroid, pituitary, parathyroid)
Metabolic (electrolytes, glucose, hepatorenal dysfunction, ethanol)
Exogenous (CO poisoning, drugs, heavy metals)
Neoplastic
Traumatic
Infectious (encephalitis, meningitis, cerebral abscess, syphilis, prions, HIV, Lyme)
Affective disorders (ie, pseudodementia)
Stroke (multi-infarct dementia, ischemia, vasculitis)

Note: vascular causes account for 10% of dementias.

Structural (normal pressure hydrocephalus [NPH])

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14
Q

Dementia where death occurs 5-10 years after the onset of cognitive decline.

A

Alzheimer

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15
Q

Dementia accompanied by changes in personality, speech disturbance, and extrapyramidal signs

A

Pick

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16
Q

Triad of chorea, behavioral changes, and dementia

A

Huntington

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17
Q

Most common cause of dementia; donzepil may be helpful

A

Alzheimer

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18
Q

Dementia where the risk factors are identical to those of cerebrovascular disease

A

Vascular or multi-infarct

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19
Q

Dementia with difficulty with vertical gaze

A

Progressive supranuclear palsy

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20
Q

Dementia with frontotemporal atrophy

A

Pick

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21
Q

Progressive dementia, urinary incontinence, gait disorder

A

Normal pressure hydrocephalus

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22
Q

Rapidly progressive dementia associated with pyramidal, extrapyramidal, and cerebellar motor decline, myoclonus, and increased startle response

A

Creutzfeldt-Jakob

23
Q

What are the differences between these Wernicke and Korsakoff?

A

Wernicke’s (ataxia, ophthalmoplegia, nystagmus, confusion) is the early manifestation and progresses to Korsakoff’s (memory loss and confabulation) if left untreated.

24
Q

What is the treatment course for delirium?

A

Address the underlying cause(s); neuroleptics for agitation

25
Q

What cognitive disorder is characterized by progressive, global intellectual impairment?

A

Dementia

26
Q

What is pseudodementia?

A

Signs/symptoms of dementia secondary to depression; reversible w/ treatment

27
Q

What is the most common etiology for dementia?

A

Dementia of Alzheimer type (DAT) — 70%-80% of cases

28
Q

What treatable causes of dementia must be ruled out?

A

Vitamin B12 deficiency, thyroid/ parathyroid disorders, uremia, syphilis, tumors (brain), NPH

29
Q

Dementia associated with apolipoprotein E4 (ApoE4), amyloid precursor protein, presenilin, and a2-macroglobulin genes

A

Alzheimer

30
Q

Risk for this type of dementia reduced with appropriate antihypertensive and antiplatelet medications

A

Vascular or multi-infarct

31
Q

Cognitive impairment, extrapyramidal signs, and early visual hallucinations

A

Dementia with Lewy bodies

32
Q

Insidious onset of difficulties with the activities of daily living and cognitive decline in the absence of other neurologic deficits

A

Alzheimer

33
Q

Stepwise dementia in a patient with focal neurologic deficits

A

Vascular or multi-infarct

34
Q

What are the common symptoms of amyotrophic lateral sclerosis (ALS)?

A

Asymmetric, slowly progressive limb, bulbar weakness with fasciculations (ie, difficulty swallowing)

35
Q

What are the classic signs of ALS?

A

Upper motor neuron (UMN) signs (spasticity, hyperreflexia, clonus, upgoing toes, frontal reflexes) and lower motor neuron (LMN) signs (flaccid paralysis, fasciculations)

36
Q

What are the common EMG abnormalities in ALS?

A

Denervation potentials in at least three limbs

37
Q

What is the common presentation of multiple sclerosis (MS)?

A

“Symptoms separated in time and space”; may include limb weakness, spasticity, optic nerve dysfunction, internuclear ophthalmoplegia, paresthesias, tremor, urinary retention, and vertigo

38
Q

What are the classic radiologic abnormalities on magnetic resonance imaging (MRI) in a patient with MS?

A

Periventricular white matter lesions

39
Q

What are the classic CSF abnormalities in a patient with MS?

A

Oligoclonal bands and mononuclear pleocytosis

40
Q

What class of medications can be used during exacerbations?

A

Steroids

41
Q

What class of medications can be used to prolong periods of remission for MS?

A

Immunosuppressants (cyclophosphamide, azathioprine, methotrexate) and immunomodulators (β-interferon and copaxone)

42
Q

Most common demyelinating disorder

A

MS

43
Q

Ascending paralysis, facial diplegia, and autonomic dysfunction

A

Guillain-Barré syndrome

44
Q

Loss of myelin from globoid and peripheral neurons

A

Krabbe disease

45
Q

Charcot’s triad (intention tremor, scanning speech, and nystagmus)

A

MS

46
Q

Autosomal recessive (AR) disease → progressive paralysis, dementia, ataxia; fatal in early childhood

A

Metachromatic leukodystrophy

47
Q

Spinal lesions typically occur in the white matter of the cervical cord

A

MS

48
Q

Postviral autoimmune syndrome causing demyelination of peripheral nerves, especially motor fibers

A

Guillain-Barré syndrome

49
Q

May present with intranuclear ophthalmoplegia (medial longitudinal fasciculus [MLF] syndrome) or sudden visual loss due to optic neuritis

A

MS

50
Q

Albuminocytologic dissociation (↑ CSF protein with normal cell count)

A

Guillain-Barré syndrome

51
Q

Rapidly fatal AR disease of childhood associated with globoid bodies in white matter due to deficiency of β-galactocerebrosidase

A

Krabbe disease

52
Q

Steroids are contraindicated for this demeyelinating disease.

A

Guillain-Barré syndrome

53
Q

Demeyelinating disease that is a genetic disorder causing accumulation of very-long-chain fatty acids resulting in behavioral and a diverse array of changes neurologic deficits

A

Adrenoleukodystrophy

54
Q

Demeyelinating disease with a history of upper respiratory infection (URI) or immunization 1 week prior

A

Guillain-Barré syndrome

Deja reivew USMLE 2 (14 decks)

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