Neurodegenerative Diseases Flashcards

1
Q

Alzheimer’s disease

A

Present with early memory ad visuospatial problems

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2
Q

Frontotemporal dementia (FTD)

A

Present with early behavioral, executive and language problems

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3
Q

Lewy Body dementia

A

Present with early Parkinsonian features, PSYCHOSIS, fluctuating consciousness

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4
Q

Parkinson’s disease

A

Present with tremor, rigidity and bradykinesia

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5
Q

Progressive supranuclear palsy (PSP)

A

Present with bradykinesia, rigidity, falls, abnormal vertical eye movements
Fall early in disease than other ND dx

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6
Q

Amyotrophic lateral sclerosis

A

Present with weakness and atrophy, fasciculations, and the combination of both upper and lower motor neuron signs

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7
Q

Prion diseases (Creutzfeldt-Jakob)

A

Present with rapidly progressive dementia with myoclonus (a prion disease)

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8
Q

What is a Prion?

A

A proteinaceous infectious particle, lacks nucleic acid

The prion protein PrP appears to be the sole constituent of these agents. Normal PrP is expressed in most human tissues, with the highest levels found in the CNS. A prion diseae is a CONFORMSTIONAL CHANGE in the protein

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9
Q

Presentation of Prion Diseases

A

Present with ataxia, abnormal movement, and neuropsychiatric features (depression, executive decision making, memory, etc)

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10
Q

Tell me some extra-scary things about Prion Diseases

A

It is sporadic, heritable AND transmissible

ALWAYS fatal

Can happen REALLY fast (ie CJD can go from onset to death in only 3 months)

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11
Q

Gerstmann-Straussler-Scheinker Syndrome

A

*Autosomal dominant
*1 to 10 cases per 100 million population per year
*Cerebellar findings (Clumsiness, incoordination, and gait ataxia
Myoclonus is usually absent in GSS)
*The presence of dementia, and its severity, varies among affected families and individuals within the same family

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12
Q

The neurochemistry in various degenerative diseases

A

Tau :FTD, PSP

Synuclein: Parkinson’s

Ubiquitin : ALS

Polyglutamine: Huntington’s

Prion protein: CJD

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