Summary
The clinical and pathologic findings in a 34-year-old woman with basal ganglia degeneration and amyotrophic lateral sclerosis are reported. The duration of symptoms was 2 years. A maternal uncle had a parkinsonian syndrome with onset at 45 years of age. Neuropathologic examination revealed extensive neuronal loss and gliosis in the corpus Luysii. Nerve cell loss and gliosis also involved both parts of the globus pallidus, and the substantia nigra. The corticospinal tracts were demyelinated in the spinal cord, and neuronal loss was observed in the anterior horns. Only one similar case of pallido-luyso-nigral atrophy associated with amyotrophic lateral sclerosis has, to our knowledge, been reported previously. Such an association may represent more than a coincidental occurrence.
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References
Bogaert L Van (1946) Aspects cliniques et pathologiques des atrophies pallidales et pallido-luysiennes progressives. J Neurol Neurosurg Psychiatry 9:125–157
Boudouresques J, Toga M, Khalil R, Ali Cherif A, Pelissier JF, Gosset A (1976) Degénérescence spino-cérébelleuse tardive avec amyotrophie comportant une atteinte pallido-luysinene sévère et des lésions histologiques diffuses de sénilité (Etude anatomo-clinique d'un cas avec discussion nosographique). Rev Neurol (Paris) 132:623–638
Davison C (1954) Pallido-pyramidal disease. J Neuropathol Exp Neurol 13:50–59
Gray F, De Baecque C, Serdaru M, Escourolle R (1981) Pallidoluyso-nigral atrophy and amyotrophic lateral sclerosis. Acta Neuropathol [Suppl] VIII:348–351
Hunt JR (1917) Progressive atrophy of the globus pallidus (Primary atrophy of the pallidal system). A system disease of the paralysis agitans type, characterized by atrophy of the motor cells of the corpus striatum. A contribution to the functions of the corpus striatum. Brain 40:58–148
Jellinger K (1968) Progressive Pallidumatrophie. J Neurol Sci 6:19–44
Jellinger K (1969) Degenerations and exogenous lesions of the pallidum and striatum. In: Vinken H, Bruyn P (eds) Handbook of clinical neurology. North-Holland, Amsterdam, pp 632–693
Serratrice GT, Toga M, Pelissier JF (1983) Chronic spinal muscular atrophy and pallidonigral degeneration. Report of a case. Neurology 33:306–310
Titeca J, Van Bogaert L (1946) Heredodegenerative hemiballismus. A contribution to the question of primary atrophy of the corpus Luysii Brain 69:251–263
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Gray, F., Eizenbaum, F., Gherardi, R. et al. Luyso-pallido-nigral atrophy and amyotrophic lateral sclerosis. Acta Neuropathol 66, 78–82 (1985). https://doi.org/10.1007/BF00698300
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DOI: https://doi.org/10.1007/BF00698300