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Tumours of Cerebellopontine Angle

Question
18 out of 18
 

Neurofibromatosis type 2 is associated with: [AIIMS Nov 2011]



A B/L acoustic neuroma

B Cafe-au-Iait spots

C Chromosome 22

D Lisch nodule

E Posterior subcapsular lenticular cataract

Ans. A B, C, and E . B/L acoustic neuroma, cafe au lait spot, chromsome 22 and posterior subcapsular cataract

B/L acoustic neuromas are a hallmark of Neurofibromatosis 2

Neurofibromatosis Type 2 is an autosomal dominant highly penetrant condition

a. Gene for NF-2 is located on chromosome 22q.

b. Patients with NF2 present in second and third decade of life, rarely after the age of 60.

c. M/C symptom/Presenting symptom = Hearing loss

d. Skin tumors are present in nearly two thirds of patients of NF-2

"Cafe au lait spots, which are a hallmark of NF-7, are also frequently found in patients with NF2. In contrast to patients with NFl, patients with NF 2 invariably have fewer than six of these hyperpigmented lesions. Juvenile posterior sub capsular lenticular opacties are common and have been reported in up to S7% of patients with NF 2."

So as is clear from above lines-cafe an lait spots and posterior subcapsular lenticular opacity are seen in NF-2 also.

Remember: Diagnostic criteria for NF-2

I. Bilateral Acoustic neuroma

or

II. Family hisory of NF- 2 and

U/L Vestibular schwan noma/acoustic neuroma

or

III. Any two of the following:

Meningioma Glioma

Neurofibroma Schwannoma

Posterior subcapsular leticular opacity

Tumours of Cerebellopontine Angle Flashcard List

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