Miscellaneous Flashcards

1
Q

Huntingtons AOE

A

If from

Mother- 40-50 y

Father- <20 y + SEIZURES. (ANTICIPATION+)

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2
Q

Genetics Huntingtons

A

Chr 4 SHORT ARM

CAG repeats > 40

AD

2 successive generations affected

Larger the defect more severe / more earlier the symptoms

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3
Q

C/f huntingtons

CDCP

A

CHOREA

DEMENTIA

COGWHEEL RIGIDITY

PERSONALITY D/O

Memory not impaired until late

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4
Q

Brain and blood ix in HC

A

Atrophy of STRIATUM***

DA increased

AcH and GABA decreased

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5
Q

Rx HC

A

DA depletor- TETRABENZENE

Da antagonist- HALOPERIDOL

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6
Q

Westphal variant of HC is a ddx of which disease

A

PARKINSONS

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7
Q

Predisposing factors to WERNICKES ENCEPHALOPATHY

A

ALCOHOL

STARVATION- (Renal dialysis/ cancer / HIV / Hyper emesis)

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8
Q

Pathophysiology of WE

A

Thiamine (B1) def—Is a cofactor for

TRANSKETOLASE
PYRUVATE KINASE
aKG

Therefore—TCA cycle inhibited—glucose accumulation—NEUROTOXICITY

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9
Q

C/f of WE (GOA)

A

Global Confusion / Psychosis

Opthalmoplegia
(LR palsy/ Conjugate gaze palsy/ ptosis rare)

Ataxia
(Dt poly neuropathy and Cerebellar inv)

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10
Q

MRI Brain in WE

A

Acute- 3 & 4 Periventricular Petechial Hge

Chronic- Mamillary Body atrophy

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11
Q

Rx WE

A

Thiamine replacement

100mg iv x 14d

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12
Q

Recovery sequence of WE

A

OAG

Confusion may be persistent

Glu infusion—ppt Wernickes

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13
Q

KORSAKOFF PSYCHOSIS c/f, circuit name

A

Dementia + CONFABULATION

PAPEZ CIRCUIT**

Mamillary body (SMALL PETECH HGE**)

Anterior Nucleus of Thalamus— LESION+

Peri aqueductal Grey matter

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14
Q

NPH pressure range

A

150-200

N= 100-180

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15
Q

Cause of NPH

A

SAH

Meningitis

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16
Q

Cf NPH

G.U.D

A

Gait problems (MAGNETIC GAIT)

U. incontinence

Dementia (mild)

NO CORTICAL ATROPHY

17
Q

Which nucleus is responsible for amnesia in Korsakoff syndrome

A

DORSOMEDIAL NUCLEUS OF THALAMUS

18
Q

MILLER FISCHER TEST IS DONE IN

A

NPH

Remove 30ml csf—- improvement in Ataxia (1st to improve) and cognition??—-the do a VP SHUNT

19
Q

Hallevorden spatz other name

A

PKAN (Pantothenate kinase assoc neuro degeneration

)

20
Q

Inheritance of PKAN

A

AR

21
Q

Pathology of PKAN

A

Fe accumulation in BASAL GANGLIA

GP
Substantia nigra

RED NUCLEI***

22
Q

Cf of PKAN

A

Dementia

Cognitive impairment

Involuntary spasticity