Miscellaneous Flashcards
Huntingtons AOE
If from
Mother- 40-50 y
Father- <20 y + SEIZURES. (ANTICIPATION+)
Genetics Huntingtons
Chr 4 SHORT ARM
CAG repeats > 40
AD
2 successive generations affected
Larger the defect more severe / more earlier the symptoms
C/f huntingtons
CDCP
CHOREA
DEMENTIA
COGWHEEL RIGIDITY
PERSONALITY D/O
Memory not impaired until late
Brain and blood ix in HC
Atrophy of STRIATUM***
DA increased
AcH and GABA decreased
Rx HC
DA depletor- TETRABENZENE
Da antagonist- HALOPERIDOL
Westphal variant of HC is a ddx of which disease
PARKINSONS
Predisposing factors to WERNICKES ENCEPHALOPATHY
ALCOHOL
STARVATION- (Renal dialysis/ cancer / HIV / Hyper emesis)
Pathophysiology of WE
Thiamine (B1) def—Is a cofactor for
TRANSKETOLASE
PYRUVATE KINASE
aKG
Therefore—TCA cycle inhibited—glucose accumulation—NEUROTOXICITY
C/f of WE (GOA)
Global Confusion / Psychosis
Opthalmoplegia
(LR palsy/ Conjugate gaze palsy/ ptosis rare)
Ataxia
(Dt poly neuropathy and Cerebellar inv)
MRI Brain in WE
Acute- 3 & 4 Periventricular Petechial Hge
Chronic- Mamillary Body atrophy
Rx WE
Thiamine replacement
100mg iv x 14d
Recovery sequence of WE
OAG
Confusion may be persistent
Glu infusion—ppt Wernickes
KORSAKOFF PSYCHOSIS c/f, circuit name
Dementia + CONFABULATION
PAPEZ CIRCUIT**
Mamillary body (SMALL PETECH HGE**)
Anterior Nucleus of Thalamus— LESION+
Peri aqueductal Grey matter
NPH pressure range
150-200
N= 100-180
Cause of NPH
SAH
Meningitis