Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism.

摘要: A 42-year-old woman suffered unexplained weight loss followed by action tremor and difficulty initiating gait. Three months after onset of symptoms, infiltrating ductal carcinoma the breast, metastatic to liver lymph nodes, was diagnosed treated briefly with cyclophosphamide, methotrexate, 5-flourouracil (5FU). Severe symmetric postural a myoclonic component developed, minimal rest tremor, severe dysarthria dysphagia, small-stepped slightly ataxic gait progressing bedbound state, widespread dystonic posturing. The latter began as typical parkinsonian posture trunk upper extremities progressed fixed painful flexion elbows wrists extension fingers neck. Sinemet, anticholinergics, baclofen, diazepam, plasmapheresis gave no benefit. patient died complications immobility 5 neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra moderate pigmented neurons. Inflammation, Lewy bodies, tumor were absent. Cerebellar Purkinje cells moderately depleted. Mild neuronal gliosis present globus pallidus cerebellar cortex. Stains for anti-human IgG, IgM, kappa, lambda negative. This, our knowledge, is first report paraneoplastic degeneration or parkinsonism.