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Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism.
Mov Disord 1989; 4(2):147-52MD

Abstract

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

Authors+Show Affiliations

Department of Neurology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School, New Brunswick 08903.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

2543919

Citation

Golbe, L I., et al. "Paraneoplastic Degeneration of the Substantia Nigra With Dystonia and Parkinsonism." Movement Disorders : Official Journal of the Movement Disorder Society, vol. 4, no. 2, 1989, pp. 147-52.
Golbe LI, Miller DC, Duvoisin RC. Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism. Mov Disord. 1989;4(2):147-52.
Golbe, L. I., Miller, D. C., & Duvoisin, R. C. (1989). Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism. Movement Disorders : Official Journal of the Movement Disorder Society, 4(2), pp. 147-52.
Golbe LI, Miller DC, Duvoisin RC. Paraneoplastic Degeneration of the Substantia Nigra With Dystonia and Parkinsonism. Mov Disord. 1989;4(2):147-52. PubMed PMID: 2543919.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Paraneoplastic degeneration of the substantia nigra with dystonia and parkinsonism. AU - Golbe,L I, AU - Miller,D C, AU - Duvoisin,R C, PY - 1989/1/1/pubmed PY - 1989/1/1/medline PY - 1989/1/1/entrez SP - 147 EP - 52 JF - Movement disorders : official journal of the Movement Disorder Society JO - Mov. Disord. VL - 4 IS - 2 N2 - 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 of the breast, metastatic to liver and lymph nodes, was diagnosed and treated briefly with cyclophosphamide, methotrexate, and 5-flourouracil (5FU). Severe symmetric action and postural tremor with a myoclonic component developed, with minimal rest tremor, severe dysarthria and dysphagia, small-stepped and slightly ataxic gait progressing to a bedbound state, and severe widespread dystonic posturing. The latter began as a typical parkinsonian posture of trunk and upper extremities and progressed to a fixed and painful flexion of the elbows and wrists and extension of fingers and neck. Sinemet, anticholinergics, baclofen, diazepam, and plasmapheresis gave no benefit. The patient died of complications of immobility 5 months after neurologic symptom onset. Autopsy revealed many pigment-laden macrophages in substantia nigra and moderate loss of pigmented neurons. Inflammation, Lewy bodies, and tumor were absent. Cerebellar Purkinje cells were moderately depleted. Mild neuronal loss and gliosis were present in globus pallidus and cerebellar cortex. Stains for anti-human IgG, IgM, kappa, and lambda were negative. This, to our knowledge, is the first report of paraneoplastic degeneration of substantia nigra or paraneoplastic parkinsonism. SN - 0885-3185 UR - https://www.unboundmedicine.com/medline/citation/2543919/Paraneoplastic_degeneration_of_the_substantia_nigra_with_dystonia_and_parkinsonism_ L2 - https://doi.org/10.1002/mds.870040206 DB - PRIME DP - Unbound Medicine ER -