Behavioral Neurology Flashcards

1
Q

hyperintensity of the caudate and putamen; cortical ribbon on MRI

A

CJD

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2
Q
  • rapidly progressive mental deterioration
  • myoclonus (provoked by startle)
  • extrapyramidal signs (hypokinesia, rigidity)
  • cerebellar manifestations (nystagmus, ataxia)
  • visual disturbance
  • akinetic mutism
A

CJD

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3
Q

positive 14-3-3 protein in CSF

A

CJD

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4
Q

Pathology: loss of neurons, spongiform degeneration, or plaques positive for PrPSc

A

CJD

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5
Q

Clinical features of CJD

A
  • rapidly progressive mental deterioration
  • myoclonus (provoked by startle)
  • extrapyramidal signs (hypokinesia, rigidity)
  • cerebellar manifestations (nystagmus, ataxia)
  • visual disturbance
  • akinetic mutism
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6
Q

What clinical symptoms are most compatible with the disease process seen in the picture?

a) Dementia, social disinhibition
b) Memory loss, language deficits, visuospatial impairment
c) Acute course of hypersalivation, hydrophobia, encephalopathy & death
d) Writhing fluid movements, ataxia, psychosis
e) Parkinsonism, hallucinations, neuroleptic sensitivity

A

Lewy Body Dementia

e) Parkinsonism, hallucinations, neuroleptic sensitivity

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7
Q

What clinical symptoms are most compatible with the disease process seen in the picture?

a) Dementia, social disinhibition
b) Memory loss, language deficits, visuospatial impairment
c) Acute course of hypersalivation, hydrophobia, encephalopathy & death
d) Writhing fluid movements, ataxia, psychosis
e) Parkinsonism, hallucinations, neuroleptic sensitivity

A

Alzheimer’s Disease

b) Memory loss, language deficits, visuospatial impairment

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8
Q

What are 5 things that can be seen on pathology in Alzheimer’s Disease?

A

Neuritic Plaques

Neurofibrillary Tangles

Hirano Bodies

Neuronal Granulovacuolar degeneration

Deposition of Amyloid in blood vessel walls

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9
Q

What clinical symptoms are most compatible with the disease process seen in the picture?

a) Dementia, social disinhibition
b) Memory loss, language deficits, visuospatial impairment
c) Acute course of hypersalivation, hydrophobia, encephalopathy & death
d) Writhing fluid movements, ataxia, psychosis
e) Parkinsonism, hallucinations, neuroleptic sensitivity

A

Rabies

= Negri Bodies

c) Acute course of hypersalivation, hydrophobia, encephalopathy & death

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10
Q

What is the pathology pictured?

A

Negri Bodies

seen in Rabies

Acute course of hypersalivation, hydrophobia, encephalopathy & death

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11
Q

What clinical symptoms are most compatible with the disease process seen in the picture?

a) Dementia, social disinhibition
b) Memory loss, language deficits, visuospatial impairment
c) Acute course of hypersalivation, hydrophobia, encephalopathy & death
d) Writhing fluid movements, ataxia, psychosis
e) Parkinsonism, hallucinations, neuroleptic sensitivity

A

Pick’s Disease (FTD)

a) Dementia, social disinhibition

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12
Q

What clinical symptoms are most compatible with the disease process seen in the picture?

a) Dementia, social disinhibition
b) Memory loss, language deficits, visuospatial impairment
c) Acute course of hypersalivation, hydrophobia, encephalopathy & death
d) Writhing fluid movements, ataxia, psychosis
e) Parkinsonism, hallucinations, neuroleptic sensitivity

A

Caudate Atrophy

seen in Huntington’s Disease

d) Writhing fluid movements, ataxia, psychosis

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13
Q

Man presented with visual hallucinations and altered mentation for 6 months before becoming bed-bound. A brain biopsy was conducted. What statement about the patient’s disease is most accurate?

a) It’s treatable w/ PCN
b) Immunosuppression is a primary risk factor for this
c) The patient had signs of Parkinsonism
d) This disease was caused by a tick
e) CSF would likely reveal 14-3-3 positivity

A

Spongiform change - prion disease

e) CSF would likely reveal 14-3-3 positivity

spongiform change, gliosis, & neuronal loss = classic triad

Misfolding maybe induced by mutation or exposure of normal cellular prion protein to pathogenic prions;

Misfolding alters 2ndary structure so that the protein becomes highly resistant to chemical or thermal methods of sterilization.

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14
Q

36YOF s/p gastric bypass who 3 months later developed confusion, feeling off balanced and shaky eyes. Her brain biopsy is shown.

  1. What did she have?
  2. What could have prevented her symptoms?
  3. What structure was affected?
  4. What nucleus of the thalamus is the damage connected to?
A

Wernicke’s Encephalopathy

Thiamine intake

Mammillary Bodies

Anterior Nucleus

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15
Q

What are the alpha-synucleinopathies?

A
L = LBD
A = alpha synucleinopathies
M = MSA
P = PD

All the rest are tauopathies

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16
Q
  1. What region of the brain is most often affected in dementia with Lewy bodies?
  2. What regiong of the brain is most often affected in Alzheimer disease?
A
  1. Posterior parietal-occipital functional defect
  2. Temporal parietal functional defect