nbme Q

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nbme Q

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11 Posts

lq2006
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Topics: 43
Posts: 382

05/17/08 - 10:59 PM

A full-term male newborn has enlargement of head circumference (3 cm greater than 99% of age range). Body weight is appropriate for gestational age. The cranial sutures are separated. Ultrasonography of the head shows enlargement of the lateral ventricles and thinning of the cerebral cortex. The newborn's maternal uncle had similar abnormalities. Further anatomic studies are most likely to show which of the following?
A ) Absence of the foramina of Luschka
B ) Cerebellar astrocytoma
C ) Holoprosencephaly
D ) Neurofibromatosis
E ) Stenosis of the aqueduct of Sylvius
F ) Tuberous sclerosis

mukho
Forum Elite

Topics: 6
Posts: 556

05/17/08 - 11:18 PM

E

Aqueductal stenosis is the commonest cause of congenital hydrocephalus and is x-linked

Edited by mukho on 05/18/08 - 09:41 PM. Reason: explanation

Vietnamese
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05/18/08 - 07:18 PM

B- Turcot's syndrome ?

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lq2006
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Topics: 43
Posts: 382

05/20/08 - 03:17 PM

I don't know the answer. The given one is F.

godfather1212m
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05/20/08 - 05:38 PM

I think it is E,

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liliaeliz
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05/22/08 - 09:08 PM

e

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vnty
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Topics: 24
Posts: 87

05/22/08 - 09:42 PM

Tuberous Sclerosis - Pediatrics: Genetics and Metabolic Disease
...develop in the Monro foramen and may cause signs and symptoms of hydrocephalus and increased intracranial pressure as they enlarge.

DrAlex_76
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Topics: 120
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06/02/08 - 12:51 PM

autosomal dominat trait, hydrocephalus-----> Tuberous sclerosis

doc-o8
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Topics: 35
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06/20/08 - 10:08 PM

does tuberous sclerosis present in neonates? confused

mukho
Forum Elite

Topics: 6
Posts: 556

06/20/08 - 10:33 PM

#10

tuberous sclerosis can present at any age, but during neonatal period ash leaf macules are the feature noted and later others
corical tubers usually dont result in obs hydrocephalus, it is the predisposition to subpendymal astrocytoma that occurs nears foramen of monroe and presents with hydrocephalous---anyways both occur later

the ans i still think is aqueductal stenosis--commonest cause, neonatal presentation and X linked

dralkesh
Forum Newbie

Topics: 0
Posts: 2

06/26/08 - 10:14 AM

#11

Ans is E its most common cause and X linked recessive

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