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Medicine

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Neurology

Question
3 out of 12
 

Neurofibromatosis type-II is associated with (select three options): (PGI Nov 2012)



A B/L acoustic schwannoma

B Multiple cafe-au-Iait spots

C Chromosome-22

D Lisch nodule

E Posterior subcapsular lenticular cataract

Ans. A

B/L acoustic schwannoma

C . Chromosome-22

E . Posterior subcapsular lenticular cataract

Neurofibromatosis (NF) or Von Recklinghausen disease is a neurocutaneous disorder with 'autosomal dominant' inheritance. There are two distinct forms of NF.

Neurofibromatosis – I

Neurofibromatosis – II (Chromosome 22)

Most prevalent type (90%)

Features

1). Cafe-au-Iait macules

2). Axillary or inguinal freckling

3). iris Lisch nodules

4). Neurofibromas

5). A distinctive osseous lesion such as sphenoid dysplasia or cortical thinning of long bone, with or without pseudoarthrosis.

6). Optic gliomas.

Accounts for 10% of cases of NF

Features

1). Bilateral acoustic neuromas are the most distinctive tumors in patients with NF-2).

2). Meningioma

3). Glioma

4). Juvenile posterior subcapsular opacity

Summary of Types of neurofibromatosis

Type I

Peripheral form

Multiple cutaneous neurofibromas, soft papillomas, café-au-lait patches, axillary freckling, iris fibromas, plexiform neurofibromas, spinal neurofibromas, aqueduct stenosis, scoliosis, endocrine tumours.

Type II

Central form

Few or no cutaneous lesion, bilateral acoustic neuromas, cerebral and otic nerve gliomas, meningiomas, spinal neurofibromas.

Neoplasms associated with NF-I include:

CNS neoplasms

Other tumors with higher incidence than general population

1). Optic nerve glioma (commonest)

2). Meningiomas

3). Neurofibromas

4). Schwannomas

5). Astrocytomas

1). Pheochromocytomas

2). Rhabdomyosarcomas

3). Leukemia

4). Wilms’

Neurology Flashcard List

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