Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
2007-7-24
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
0919-6544
pubmed:author
pubmed:issnType
Print
pubmed:volume
27
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
295-9
pubmed:dateRevised
2008-3-10
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
Amyotrophic lateral sclerosis with dementia showing clinical parkinsonism and severe degeneration of the substantia nigra: report of an autopsy case.
pubmed:affiliation
Department of Pathology, Osaka Red Cross Hospital, Tennoji, Osaka 543-8555, Japan. masa-s@sings.jp
pubmed:publicationType
Journal Article, Case Reports, Research Support, Non-U.S. Gov't