Claude's Syndrome

The patient is a 76 year old woman who woke one morning unable to see out of her right eye because of ptosis. She came to the Massachusetts General Hospital emergency room and was admitted. Neurological Examination: Right third nerve palsy involving the pupil Ataxia of the left hand on finger-nose test Diagnosis: Claude's Syndrome - a lesion of the third nerve fascicle in the midbrain with involvement of the superior cerebellar peduncle. Fascicular third nerve palsies are rare. Lesions of the third nerve fascicle can present as an isolated complete or incomplete third nerve palsy, in association with other neurologic signs due to involvement of adjacent midbrain structures. Four syndromes are recognized, and verified pathologically. The commonest cause is ischemia from embolic or thrombotic occlusion of perforating branches off the basilar artery. The Midbrain Syndromes are: 1.Weber's syndrome: ipsilateral third nerve palsy, plus contralateral hemiparesis including the lower face and tongue due to involvement of the cerebral peduncle. 2.Benedikt's syndrome: Ipsilateral third nerve palsy, plus contralateral tremor due to involvement of the red nucleus. 3.Nothnagel's syndrome: Ipsilateral third nerve palsy, plus ipsilateral cerebellar ataxia due to involvement of the superior cerebellar peduncle. 4.Claude's syndrome: Ipsilateral third nerve palsy (often partial) plus contralateral ataxia, asynergy, and dysdiadochokinesis due to involvement of the red nucleus and superior cerebellar peduncle. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/334