Sunday, July 16, 2006

Combined cranial neuropathies

A combination of 3rd, fourth, fifth and sixth dysfunction, on one side, especially if ocmbined with oculosympatheic dysfunction, suggests a parasellar lesion., infectious, neoplastic, inflammatory or vascular.

Tolosa Hunt syndrome-- THS is a syndrome of acute, painful opthalmoplegia due to idiopathic granulomatous disease in the cavernous sinus. Signs and symptoms include periorbital or hemicranial pain with ipsilateral effects of CN III, IV, V1, VI and Horners' syndrome. THS is a diagnosis of exclusion after other causes of cavernous sinus abnormality have been excluded. (see separate citation of evaluation for painful opthalmoplegia). MRI may be normal or abnormal and show enlargement of the cavernous sinus, enhancement of the adjacent dural wall, and abnormal soft tissue surrounding and narrowing the cavernous ICA. There may be extension of the soft tissue abnormality into the orbital apex, sphenoid sinus or floor of the middle cranial fossa. The etiology is unknown. Like orbital pseudotumor syndrome, oral corticosteroids are dramatically effective, often within 48 hours. Beware, as other conditions that mimic THS are also steroid responsive.

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