Lewy body-free nigral degeneration—a case report

Ishihara, Kenji; Nonaka, Akiko; Fukui, Toshiya; Kawamura, Mitsuru; Shiota, Jun; Nakano, Imaharu

doi:10.1016/s0022-510x(02)00050-3

Cited by 2 publications

(1 citation statement)

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“…The absence of degeneration of the locus caeruleus in these cases also suggests the involvement of a mechanism different from that operating in idiopathic Parkinson disease. There are presumably several different mechanisms leading to the neuronal death of the substantia nigra and several case reports attest to the presence of primary nigral degeneration not associated with α‐synucleinopathy 16–18 . In the present case, finely granular or stellate‐shaped mineralization of necrotic neurons (encrustation) in the substantia nigra was a unique finding that has not been described previously.…”

Section: Discussionsupporting

confidence: 60%

Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: Report of an autopsy case

Shintaku¹,

Oyanagi

Kaneda

2007

Neuropathology

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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.

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Section: Discussionsupporting

confidence: 60%