Successful Treatment of Incomplete Susac Syndrome with Simultaneous Corticosteroids and Plasmapheresis Followed by Rituximab.

We present a case report of a patient with incomplete Susac syndrome. He had cognitive impairment, corpus callosum lesions, and vestibulocochlear dysfunction on brainstem auditory evoked responses. He was treated with methylprednisolone and plasmapheresis, improved, and then, also received rituximab.

A Case of Celiac Disease, Epilepsy, and Cerebral Calcifications With Temporal Lobe Epilepsy.

This is a case report of a 55-year-old man with medication refractory right temporal lobe epilepsy since adolescence. He was found to have bilateral posterior cerebral calcifications on routine head computed tomography with confirmation on magnetic resonance imaging. He also had elevated antibody markers for celiac disease.

Myopathic dropped head syndrome: an expanding clinicopathological spectrum.

Objective: A number of neuromuscular conditions may lead to a dropped head syndrome (DHS), with some patients developing a late onset noninflammatory myopathy affecting only, or predominantly, neck extensor muscles (NEM). The cause, pathogenesis, and nosological classification of this condition are unclear. To further investigate this condition, the authors evaluated the clinical, electrodiagnostic and pathologic findings in seven patients with a myopathic DHS.

Friedreich's ataxia associated with mitochondrial myopathy: clinicopathologic report.

A 13-year-old boy with clinical and electrophysiologic findings of Friedreich's ataxia developed unusually prominent myopathy. Skeletal muscle biopsy showed mitochondrial proliferation and structural abnormalities. No mutation was found in skeletal muscle mitochondrial DNA to explain this finding.