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Titolo:
Ataxia telangiectasia with vascular abnormalities in the brain parenchyma:Report of an autopsy case and literature review
Autore:
Kamiya, M; Yamanouchi, H; Yoshida, T; Arai, H; Sasaki, A; Hirato, J; Nakazato, Y; Sakazume, Y; Okamoto, K;
Indirizzi:
Gunma Univ, Sch Med, Dept Pathol 1, Maebashi, Gumma 3718511, Japan Gunma Univ Maebashi Gumma Japan 3718511 1, Maebashi, Gumma 3718511, Japan Gunma Univ, Sch Med, Dept Neurol, Maebashi, Gumma 3718511, Japan Gunma Univ Maebashi Gumma Japan 3718511 l, Maebashi, Gumma 3718511, Japan Dokkyo Univ, Sch Med, Dept Pediat, Mibu, Tochigi 32102, Japan Dokkyo UnivMibu Tochigi Japan 32102 t Pediat, Mibu, Tochigi 32102, Japan
Titolo Testata:
PATHOLOGY INTERNATIONAL
fascicolo: 4, volume: 51, anno: 2001,
pagine: 271 - 276
SICI:
1320-5463(200104)51:4<271:ATWVAI>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Keywords:
ataxia telangiectasia; coagulative necrosis; spongy degeneration; vascular abnormality;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
21
Recensione:
Indirizzi per estratti:
Indirizzo: Kamiya, M Gunma Univ, Sch Med, Dept Pathol 1, 3-39-22 Showa-machi, Maebashi, Gumma 3718511, Japan Gunma Univ 3-39-22 Showa-machi Maebashi Gumma Japan3718511 apan
Citazione:
M. Kamiya et al., "Ataxia telangiectasia with vascular abnormalities in the brain parenchyma:Report of an autopsy case and literature review", PATHOL INT, 51(4), 2001, pp. 271-276

Abstract

A 25-year-old man was admitted to the Department of Neurology, Gunma University Hospital, in June 1997. He had an intellectual disability and had suffered from repeated Infection since childhood. Cerebellar ataxia had developed at 19 years of age and he had been clinically diagnosed with ataxia telangiectasia (AT) comprising cerebellar ataxia and oculocutaneous telangiectasia at 24 years of age. He died from pneumonia and renal failure at 26 years of age. Neuropathological examination revealed Purkinje cell loss and atrophy of the dentate nuclei in the cerebellum, anterior horn-cell atrophy and demyelination of the gracile fasciculi in the spinal cord, and the existence of nucleocytomegalic cells in the anterior pituitary gland. These neuropathological findings correlated with previously reported cases of AT. In addition, spongy degeneration was found, predominantly around the blood vessels in the cerebral cortex. Diffuse spongy degeneration and multiple foci of coagulative necrosis with calcification were noted in the white matter. Abnormal vasculature was noted in both degenerative and necrotic areas in the cerebral cortex and in the white matter. The vessels at the center of the areas of spongy degeneration in the cerebral cortex had irregularly arranged and enlarged smooth-muscle-cell nuclei and a distorted, narrow lumen. The vessels present in the white matter were hyalinized. To our knowledge, these vascular abnormalities in the brain parenchyma have not been reported previously.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/01/20 alle ore 01:27:01