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Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: report of an autopsy case.
Neuropathology 2007; 27(3):295-9N

Abstract

An autopsy case of amyotrophic lateral sclerosis with dementia (ALS-D) showing clinically overt parkinsonism and severe degeneration of the substantia nigra is reported. The patient was a 78-year-old man who died after a 2-year clinical course characterized by parkinsonism that was responsive to Levodopa (L-DOPA) treatment. Motor neuron symptoms and dementia were not apparent ante-mortem. The autopsy demonstrated the severe degeneration of the substantia nigra without alpha-synucleinopathy-related changes. Finely granular mineralization of necrotic neurons was a unique finding in the substantia nigra. The mild loss of spinal anterior horn cells, the appearance of several Bunina bodies and the degeneration of the hippocampal subiculum and temporal cortex were also noted. A small number of ubiquitinated intra-cytoplasmic inclusions were found in neurons of the dentate fascia of the hippocampus and the temporal and frontal cortices. Although the degeneration of the substantia nigra is a common finding in ALS-D, patients seldom develop clinically overt parkinsonism. This case indicates that patients with ALS-D rarely present with predominantly parkinsonian clinical features and these symptoms and signs can be improved by L-DOPA treatment.

Authors+Show Affiliations

Department of Pathology, Osaka Red Cross Hospital, Tennoji, Osaka 543-8555, Japan. masa-s@sings.jpNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

17645246

Citation

Shintaku, Masayuki, et al. "Amyotrophic Lateral Sclerosis With Dementia Showing Clinical Parkinsonism and Severe Degeneration of the Substantia Nigra: Report of an Autopsy Case." Neuropathology : Official Journal of the Japanese Society of Neuropathology, vol. 27, no. 3, 2007, pp. 295-9.
Shintaku M, Oyanagi K, Kaneda D. Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: report of an autopsy case. Neuropathology. 2007;27(3):295-9.
Shintaku, M., Oyanagi, K., & Kaneda, D. (2007). Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: report of an autopsy case. Neuropathology : Official Journal of the Japanese Society of Neuropathology, 27(3), pp. 295-9.
Shintaku M, Oyanagi K, Kaneda D. Amyotrophic Lateral Sclerosis With Dementia Showing Clinical Parkinsonism and Severe Degeneration of the Substantia Nigra: Report of an Autopsy Case. Neuropathology. 2007;27(3):295-9. PubMed PMID: 17645246.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: report of an autopsy case. AU - Shintaku,Masayuki, AU - Oyanagi,Kiyomitsu, AU - Kaneda,Daita, PY - 2007/7/25/pubmed PY - 2007/9/29/medline PY - 2007/7/25/entrez SP - 295 EP - 9 JF - Neuropathology : official journal of the Japanese Society of Neuropathology JO - Neuropathology VL - 27 IS - 3 N2 - An autopsy case of amyotrophic lateral sclerosis with dementia (ALS-D) showing clinically overt parkinsonism and severe degeneration of the substantia nigra is reported. The patient was a 78-year-old man who died after a 2-year clinical course characterized by parkinsonism that was responsive to Levodopa (L-DOPA) treatment. Motor neuron symptoms and dementia were not apparent ante-mortem. The autopsy demonstrated the severe degeneration of the substantia nigra without alpha-synucleinopathy-related changes. Finely granular mineralization of necrotic neurons was a unique finding in the substantia nigra. The mild loss of spinal anterior horn cells, the appearance of several Bunina bodies and the degeneration of the hippocampal subiculum and temporal cortex were also noted. A small number of ubiquitinated intra-cytoplasmic inclusions were found in neurons of the dentate fascia of the hippocampus and the temporal and frontal cortices. Although the degeneration of the substantia nigra is a common finding in ALS-D, patients seldom develop clinically overt parkinsonism. This case indicates that patients with ALS-D rarely present with predominantly parkinsonian clinical features and these symptoms and signs can be improved by L-DOPA treatment. SN - 0919-6544 UR - https://www.unboundmedicine.com/medline/citation/17645246/Amyotrophic_lateral_sclerosis_with_dementia_showing_clinical_parkinsonism_and_severe_degeneration_of_the_substantia_nigra:_report_of_an_autopsy_case_ L2 - https://doi.org/10.1111/j.1440-1789.2007.00763.x DB - PRIME DP - Unbound Medicine ER -