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Motor neuron disease, parkinsonism and dementia

Report of a case with diffuse Lewy body-like intracytoplasmic inclusions


The clinicopathological finding in a 49-year-old man who presented multisystemic neurological degenerative disease are reported. The patient presented, at the age of 36, distal upper limb amyotrophy and 8 years later pyramidal signs with fasciculations. In his last year of life, he suffered extrapyramidal hypertonus and mental deterioration. Neuropathological examination showed anterior spinal root and cerebral atrophy. Myelin pallor was mild and predominated on posterior cords. Anterior horn neuronal loss was noted in the spinal cord as well as Alzheimer-type changes in the brain. Inclusion bodies consistent with Lewy bodies were diffusely apparent. The peculiar clinical progression in this case and the extension of neuropathological lesions with inclusion bodies mainly in the substantia nigra and cerebral cortex are an interesting subject of discussion.

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  1. 1.

    Castaigne P, Cambier J, Escourolle R, Brunet P, Guilleminau T (1970) Dégénérescence subaiguë des neurones moteurs périphériques avec inclusions neuronales et lésions des cordons postérieurs: les limites de la sclérose latérale amyotrophique. Rev Neurol (Paris) 122:313–320

  2. 2.

    Delisle MB, Carpenter S (1984) Neurofibrillary axonal swellings and amyotrophic lateral sclerosis. J Neurol Sci 63:241–250

  3. 3.

    Dubois B, Hauw JJ, Ruberg M, Serdaru M, Javoy-Agid F, Agid Y (1985) Démence et maladie de Parkinson: correlation biochimique et anatomoclinique. Rev Neurol (Paris) 141:184–193

  4. 4.

    Eggerston DE, Sima AAF (1986) Dementia with cerebral Lewy bodies. A mesocortical dopaminergic defect? Arch Neurol 43:524–527

  5. 5.

    Escourolle R, De Recondo J, Gray F (1970) Etude anatomopathologique des syndromes parkinsoniens. In: Ajuriaguerra de J, Gauthier G (eds) Monoamines, noyaux gris centraux et syndrome de Parkinson. Masson, Paris, pp 173–229

  6. 6.

    Forno LS, Norville RL (1976) Ultrastructure of Lewy bodies in the stellate ganglion. Acta Neuropathol (Berl) 34:183–197

  7. 7.

    Forno LS, Barbour PJ, Norville RL (1978) Presenile dementia with Lewy bodies and neurofibrillary tangles. Arch Neurol 35:818–822

  8. 8.

    Forno LS, Sternberger LA, Sternberger NH, Strefling AM, Swanson K, Eng LF (1986) Reaction of Lewy bodies with antibodies to phosphorylated and non-phosphorylated neurofilaments. Neurosci Lett 64:253–258

  9. 9.

    Gibb WRG (1986) Idiopathic Parkinsons's disease and the Lewy body disorders. Neuropathol Appl Neurobiol 12:223–234

  10. 10.

    Goldman JE, Yen SH (1986) Cytoskeletal protein abnormalities in neurodegenerative diseases. Ann Neurol 19:209–223

  11. 11.

    Goldman JE, Yen SH, Chiu FC, Peress NS (1983) Lewy bodies of Parkinson's disease contain neurofilament antigens. Science 221:1082–1084

  12. 12.

    Graybiel AM, Hirsch EC, Agid YA (1987) Differences in tyrosine hydroxylase-like immunoreactivity characterize the mesostriatal innervation of striosomes and extrastriosomal matrix at maturity. Proc Natl Acad Sci USA 84:303–307

  13. 13.

    Hirano A, Kurland LT, Sayre GP (1967) Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spino-cerebellar tract involvement and hyaline inclusions in the anterior horn cells. Arch Neurol 16:232–243

  14. 14.

    Hirsch E, Ruberg M, Dardenne M, Portier MM, Javoy-Agid F, Bach JF, Agid Y (1985) Monoclonal antibodies raised against Lewy bodies in brains from subjects with Parkinson's disease. Brain Res 345:374–378

  15. 15.

    Hudson AJ (1981) Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 104:217–247

  16. 16.

    Ikeda K, Ikeda S, Yoshimura T, Kato M, Namba M (1978) Idiopathic parkinsonism with Lewy-type inclusions in cerebral cortex. A case report. Acta Neuropathol (Berl) 41:165–168

  17. 17.

    Kosaka K (1978) Lewy bodies in cerebral cortex. Report of three cases. Acta Neuropathol (Berl) 42:127–134

  18. 18.

    Kosaka K, Yoshimura M, Ikeda K, Budka H (1984) Diffuse type of Lewy body disease: progressive dementia with abundant cortical Lewy bodies and senile changes of varying degree. A new disease? Clin Neuropathol 3:185–192

  19. 19.

    Lewy FH (1912) Paralysis agitans. I. Pathologische Anatomie. In: Lewandowsky M (ed) Handbuch der Neurologie, vol 3. Springer, Berlin, pp 920–933

  20. 20.

    Mitsuyama Y, Kogoh M, Ata K (1985) Progressive dementia with motor neuron disease. An additional case report and neuropathological review of 20 cases in Japan. Eur Arch Psychiatry Neurol Sci 235:1–8

  21. 21.

    Oda M, Akagawa N, Tabuchi Y, Tanabe M (1978) A sporadic juvenile case of amyotrophic lateral sclerosis with neuronal cytoplasmic inclusion. Acta Neuropathol (Berl) 44:211–216

  22. 22.

    Ohama E, Ikuta F (1976) Parkinson's disease: distribution of Lewy bodies and monoamine neuron system. Acta Neuropathol (Berl) 34:311–319

  23. 23.

    Pappola MA (1986) Lewy bodies of Parkinson's disease. Immune electron microscopic demonstration of neurofilament antigens in constituent filaments. Arch Pathol Lab Med 110:1160–1163

  24. 24.

    Perry EK, Curtis M, Dick DJ, Candy JM, Atack JR, Bloxham CA, Blessed G, Fairbairn A, Tomlinson BE, Perry RM (1985) Cholinergic correlates of cognitive impairment in Parkinson's disease. Comparisons with Alzheimer's disease. J Neurol Neurosurg Psychiatry 48:413–421

  25. 25.

    Schmitt HP, Enser W, Heimes C (1984) Familial occurrence of amyotrophic lateral sclerosis, Parkinsonism and dementia. Ann Neurol 16:642–648

  26. 26.

    Takahashi K, Nakamura M, Okada E (1972) Hereditary amytrophic lateral sclerosis. Histochemical and electron microscopic study of hyaline inclusions in motor neurons. Arch Neurol 27:292–299

  27. 27.

    Tomonaga M (1981) Neurofibrillary tangles and Lewy bodies in the locus caeruleus neurons of the aged brain. Acta Neuropathol (Berl) 53:165–168

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Correspondence to M. B. Delisle.

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Delisle, M.B., Gorce, P., Hirsch, E. et al. Motor neuron disease, parkinsonism and dementia. Acta Neuropathol 75, 104–108 (1987).

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Key words

  • Motor neuron disease
  • Parkinsonism
  • Dementia
  • Lewy body