(MCQS 271-273) :an association with neurofibromatosis type 2 is a feature of

Correct Answer: E
There is a higher incedence of intracranial schwannomas in neurofibromatosis type 2 Acoustic neurinomas are derived from Schwann cells. They represent approximately 7% of all intracranial neoplasms. They arise twice as often from the vestibular division of the eighth nerve as from the auditory division. Initially, they produce tinnitus and a neural hearing loss. The patient complains of unsteadiness or imbalance. True vertigo is not a common complaint. The hearing loss is predominantly a high-tone loss, with greater impairment of speech discrimination than would be expected with a cochlear lesion producing the same amount of pure-tone hearing loss

Correct Answer: G
Osteonecrosis, also called avascular or ischemic necrosis, results from bone death secondary to an impaired blood supply. The femoral head is commonly involved, leading to hip-joint arthritis, but any other joint can be affected. Symptoms include pain, especially when bearing weight, limping and the eventual restricted movement of the involved joint. Osteonecrosis should be considered in young people with risk factors such as corticosteroid use, hemoglobinopathies or sickle cell disease, as well as in alcoholic and elderly diabetic patients

Correct Answer: H
Cushing's syndrome is caused by glucocorticoid excess. The classic syndrome is defined clinically: weight gain, plethora, striae, hypertension, and proximal muscle weakness. The weight gain is predominantly truncal, with increased fat deposited in a yoke-like pattern around the neck leading to the well-known dorsocervical fat pad (buffalo hump) and filling in of the supraclavicular fossae. Plethora is evident as a ruddy complexion. The striae of Cushing's syndrome are characteristically violaceous and occur in thin skin. Proximal muscle weakness is best assessed by testing the ability of the patient to rise unassisted from a squatting position


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