Demyelinating Diseases Flashcards
Delerium or Dementia?
Usually a rapid onset
Delerium
Delerium or Dementia?
Characterized by memory loss
Dementia (think deMEMtia)
Delerium or Dementia?
Associated with disturbances in sleep-wake cycle
Delirium
Delerium or Dementia?
Often irreversibel
Dementia
Delerium or Dementia?
Associated with changes in sensorium (hallucinations and illusions)
Delirium
(but can sometimes be present in dementia)
Delerium or Dementia?
Inattentiveness
Delerium
Delerium or Dementia?
Waxing and waning level of consciousness
Delirium
Name four major causes of delirium.
“HIDE”
- Hypoxia
- Infection (often UTIs) and ICU psychosis
- Drugs (anticholinergics, opioids, steroids, barbiturates), and withdrawal (especially EtOH)
- Electrolyte and Endocrine causes
Which two syndromes are classically seen in alcoholics experiencing delirium?
- Wernicke’s encephalopathy
- Korsakoff’s psychosis
What is the primary cause of these Wernicke’s ensephalopathy and Korsakoff’s psychosis?
Thiamine deficiency
List four important steps in the evaluation of a patient with new-onset delirium
- Check vitals (including O2 saturation).
- Check med list.
- Check lab values.
- Check for occult infection.
Name other common etiologies for dementia:
“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])
Dementia where death occurs 5-10 years after the onset of cognitive decline.
Alzheimer
Dementia accompanied by changes in personality, speech disturbance, and extrapyramidal signs
Pick
Triad of chorea, behavioral changes, and dementia
Huntington
Most common cause of dementia; donzepil may be helpful
Alzheimer
Dementia where the risk factors are identical to those of cerebrovascular disease
Vascular or multi-infarct
Dementia with difficulty with vertical gaze
Progressive supranuclear palsy
Dementia with frontotemporal atrophy
Pick
Progressive dementia, urinary incontinence, gait disorder
Normal pressure hydrocephalus
Rapidly progressive dementia associated with pyramidal, extrapyramidal, and cerebellar motor decline, myoclonus, and increased startle response
Creutzfeldt-Jakob
What are the differences between these Wernicke and Korsakoff?
Wernicke’s (ataxia, ophthalmoplegia, nystagmus, confusion) is the early manifestation and progresses to Korsakoff’s (memory loss and confabulation) if left untreated.
What is the treatment course for delirium?
Address the underlying cause(s); neuroleptics for agitation
What cognitive disorder is characterized by progressive, global intellectual impairment?
Dementia
What is pseudodementia?
Signs/symptoms of dementia secondary to depression; reversible w/ treatment
What is the most common etiology for dementia?
Dementia of Alzheimer type (DAT) — 70%-80% of cases
What treatable causes of dementia must be ruled out?
Vitamin B12 deficiency, thyroid/ parathyroid disorders, uremia, syphilis, tumors (brain), NPH
Dementia associated with apolipoprotein E4 (ApoE4), amyloid precursor protein, presenilin, and a2-macroglobulin genes
Alzheimer
Risk for this type of dementia reduced with appropriate antihypertensive and antiplatelet medications
Vascular or multi-infarct
Cognitive impairment, extrapyramidal signs, and early visual hallucinations
Dementia with Lewy bodies
Insidious onset of difficulties with the activities of daily living and cognitive decline in the absence of other neurologic deficits
Alzheimer
Stepwise dementia in a patient with focal neurologic deficits
Vascular or multi-infarct
What are the common symptoms of amyotrophic lateral sclerosis (ALS)?
Asymmetric, slowly progressive limb, bulbar weakness with fasciculations (ie, difficulty swallowing)
What are the classic signs of ALS?
Upper motor neuron (UMN) signs (spasticity, hyperreflexia, clonus, upgoing toes, frontal reflexes) and lower motor neuron (LMN) signs (flaccid paralysis, fasciculations)
What are the common EMG abnormalities in ALS?
Denervation potentials in at least three limbs
What is the common presentation of multiple sclerosis (MS)?
“Symptoms separated in time and space”; may include limb weakness, spasticity, optic nerve dysfunction, internuclear ophthalmoplegia, paresthesias, tremor, urinary retention, and vertigo
What are the classic radiologic abnormalities on magnetic resonance imaging (MRI) in a patient with MS?
Periventricular white matter lesions
What are the classic CSF abnormalities in a patient with MS?
Oligoclonal bands and mononuclear pleocytosis
What class of medications can be used during exacerbations?
Steroids
What class of medications can be used to prolong periods of remission for MS?
Immunosuppressants (cyclophosphamide, azathioprine, methotrexate) and immunomodulators (β-interferon and copaxone)
Most common demyelinating disorder
MS
Ascending paralysis, facial diplegia, and autonomic dysfunction
Guillain-Barré syndrome
Loss of myelin from globoid and peripheral neurons
Krabbe disease
Charcot’s triad (intention tremor, scanning speech, and nystagmus)
MS
Autosomal recessive (AR) disease → progressive paralysis, dementia, ataxia; fatal in early childhood
Metachromatic leukodystrophy
Spinal lesions typically occur in the white matter of the cervical cord
MS
Postviral autoimmune syndrome causing demyelination of peripheral nerves, especially motor fibers
Guillain-Barré syndrome
May present with intranuclear ophthalmoplegia (medial longitudinal fasciculus [MLF] syndrome) or sudden visual loss due to optic neuritis
MS
Albuminocytologic dissociation (↑ CSF protein with normal cell count)
Guillain-Barré syndrome
Rapidly fatal AR disease of childhood associated with globoid bodies in white matter due to deficiency of β-galactocerebrosidase
Krabbe disease
Steroids are contraindicated for this demeyelinating disease.
Guillain-Barré syndrome
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
Adrenoleukodystrophy
Demeyelinating disease with a history of upper respiratory infection (URI) or immunization 1 week prior
Guillain-Barré syndrome
