Demyelinating Diseases

Question 1

Delerium or Dementia?

Usually a rapid onset

Answer 1

Delerium

Question 2

Delerium or Dementia?

Characterized by memory loss

Answer 2

Dementia (think deMEMtia)

Question 3

Delerium or Dementia?

Associated with disturbances in sleep-wake cycle

Answer 3

Delirium

Question 4

Delerium or Dementia?

Often irreversibel

Answer 4

Dementia

Question 5

Delerium or Dementia?

Associated with changes in sensorium (hallucinations and illusions)

Answer 5

Delirium

(but can sometimes be present in dementia)

Question 6

Delerium or Dementia?

Inattentiveness

Answer 6

Delerium

Question 7

Delerium or Dementia?

Waxing and waning level of consciousness

Answer 7

Delirium

Question 8

Name four major causes of delirium.

Answer 8

“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

Question 9

Which two syndromes are classically seen in alcoholics experiencing delirium?

Answer 9

1. Wernicke’s encephalopathy
2. Korsakoff’s psychosis

Question 11

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

Answer 11

Thiamine deficiency

Question 12

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

Answer 12

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

Question 13

Name other common etiologies for dementia:

Answer 13

“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])

Question 14

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

Answer 14

Alzheimer

Question 15

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

Answer 15

Pick

Question 16

Triad of chorea, behavioral changes, and dementia

Answer 16

Huntington

Question 17

Most common cause of dementia; donzepil may be helpful

Answer 17

Alzheimer

Question 18

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

Answer 18

Vascular or multi-infarct

Question 19

Dementia with difficulty with vertical gaze

Answer 19

Progressive supranuclear palsy

Question 20

Dementia with frontotemporal atrophy

Answer 20

Pick

Question 21

Progressive dementia, urinary incontinence, gait disorder

Answer 21

Normal pressure hydrocephalus

Question 22

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

Answer 22

Creutzfeldt-Jakob

Question 23

What are the differences between these Wernicke and Korsakoff?

Answer 23

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

Question 24

What is the treatment course for delirium?

Answer 24

Address the underlying cause(s); neuroleptics for agitation

Question 25

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

Answer 25

Dementia

Question 26

What is pseudodementia?

Answer 26

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

Question 27

What is the most common etiology for dementia?

Answer 27

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

Question 28

What treatable causes of dementia must be ruled out?

Answer 28

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

Question 29

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

Answer 29

Alzheimer

Question 30

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

Answer 30

Vascular or multi-infarct

Question 31

Cognitive impairment, extrapyramidal signs, and early visual hallucinations

Answer 31

Dementia with Lewy bodies

Question 32

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

Answer 32

Alzheimer

Question 33

Stepwise dementia in a patient with focal neurologic deficits

Answer 33

Vascular or multi-infarct

Question 34

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

Answer 34

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

Question 35

What are the classic signs of ALS?

Answer 35

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

Question 36

What are the common EMG abnormalities in ALS?

Answer 36

Denervation potentials in at least three limbs

Question 37

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

Answer 37

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

Question 38

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

Answer 38

Periventricular white matter lesions

Question 39

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

Answer 39

Oligoclonal bands and mononuclear pleocytosis

Question 40

What class of medications can be used during exacerbations?

Answer 40

Steroids

Question 41

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

Answer 41

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

Question 42

Most common demyelinating disorder

Answer 42

MS

Question 43

Ascending paralysis, facial diplegia, and autonomic dysfunction

Answer 43

Guillain-Barré syndrome

Question 44

Loss of myelin from globoid and peripheral neurons

Answer 44

Krabbe disease

Question 45

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

Answer 45

MS

Question 46

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

Answer 46

Metachromatic leukodystrophy

Question 47

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

Answer 47

MS

Question 48

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

Answer 48

Guillain-Barré syndrome

Question 49

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

Answer 49

MS

Question 50

Albuminocytologic dissociation (↑ CSF protein with normal cell count)

Answer 50

Guillain-Barré syndrome

Question 51

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

Answer 51

Krabbe disease

Question 52

Steroids are contraindicated for this demeyelinating disease.

Answer 52

Guillain-Barré syndrome

Question 53

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

Answer 53

Adrenoleukodystrophy

Question 54

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

Answer 54

Guillain-Barré syndrome