Abstract:
:This report describes three brothers belonging to a consanguineous family suffering from a progressive neurological disorder associated with L-2-hydroxyglutaric aciduria. Clinically this disorder is characterized by childhood onset, pyramidal signs, cerebellar and pseudobulbar syndromes and epilepsy. Pathological examination of the brain in the oldest patient, who died at the age of 30 years, showed bilateral and diffuse spongiosis with notable cystic cavitations of the cerebral white matter without abnormal storage in neurons and glial cells. We consider that these findings are related to L-2 hydroxyglutaric aciduria. To our knowledge this present case represents the first to be reported with neuropathological examination.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Larnaout A,Hentati F,Belal S,Ben Hamida C,Kaabachi N,Ben Hamida Mdoi
10.1007/BF00310381subject
Has Abstractpub_date
1994-01-01 00:00:00pages
367-70issue
4eissn
0001-6322issn
1432-0533journal_volume
88pub_type
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journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
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abstract::Using a 3H-labelled virion DNA probe applied to tissue sections, we have previously identified the precise microscopic anatomical location of herpes simplex virus (HSV) during the acute and latent stages of infection of the mouse trigeminal ganglia and central nervous system (CNS). In the present investigation, we com...
journal_title:Acta neuropathologica
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abstract::The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ...
journal_title:Acta neuropathologica
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abstract::Astrocytic alpha-synuclein-immunoreactive inclusions have recently been noted to develop in sporadic Parkinson's disease (PD). Here, the presence of immunoreactive astrocytes is reported in 14 autopsy cases with clinically diagnosed PD and a neuropathological stage of 4 or higher. The labeled astrocytes occur preferen...
journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
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journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050502
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journal_title:Acta neuropathologica
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abstract::Fifteen cases of Down syndrome between age 25-59 years were examined neuropathologically. A variety of histological methods were used to identify plaques and neurofibrillary tangles (NFT). All cases had some plaques or NFT, but their density was generally not high before the age of 40 years. Plaques and NFT tended to ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
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更新日期:1989-01-01 00:00:00
abstract::To identify antigenic differences between gliomas and normal brain, we have immunohistochemically studied the expression of lymphocyte adhesion molecules (ICAM-1, ICAM-2, ICAM-3, VCAM-1, E-selectin and CD58), epidermal growth factor receptor (EGFR) and extracellular matrix proteins (collagen IV, fibronectin, laminin, ...
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journal_title:Acta neuropathologica
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更新日期:1995-01-01 00:00:00
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