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Titolo:
DISORDER OF CEREBELLAR FOLIATION IN WALKERS LISSENCEPHALY AND NEU-LAXOVA SYNDROME
Autore:
LYON G; RAYMOND G; MOGAMI K; GADISSEUX JF; DELLAGIUSTINA E;
Indirizzi:
UCL,SCH MED,FAC MED,NEUROL DEV LAB,52 AVE EM MOUNIER B-1200 BRUSSELS BELGIUM
Titolo Testata:
Journal of neuropathology and experimental neurology
fascicolo: 6, volume: 52, anno: 1993,
pagine: 633 - 639
SICI:
0022-3069(1993)52:6<633:DOCFIW>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
CONGENITAL MUSCULAR-DYSTROPHY; EXTERNAL GRANULE CELLS; WARBURG SYNDROME; TRANSPLANTATION; DYSPLASIA; FETUS; RAT;
Keywords:
BRAIN DEVELOPMENT; CEREBELLAR GRANULE CELLS; CEREBELLAR MALFORMATION; EXTERNAL BASAL LAMINA; EXTRACELLULAR MATRIX; NEU-LAXOVA SYNDROME; WALKERS LISSENCEPHALY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
24
Recensione:
Indirizzi per estratti:
Citazione:
G. Lyon et al., "DISORDER OF CEREBELLAR FOLIATION IN WALKERS LISSENCEPHALY AND NEU-LAXOVA SYNDROME", Journal of neuropathology and experimental neurology, 52(6), 1993, pp. 633-639

Abstract

A diffuse disorder of cerebellar foliation was found in eight infantsand one fetus with Walker's lissencephaly. The cerebellar cortex consisted of fused and irregularly distorted folia. In the white matter, trilaminated rings of cortex were concentrically arranged around blood vessels and mesenchymal tissue. The normal relative position of the different classes of cortical nerve cells was preserved. Cells of the external granular layer invaded the meninges and migrated along penetrating blood vessels. We believe that this foliation disorder is caused by a defect in the external basal lamina that allows adjacent folia to be fused and sulci obliterated by intrameningeal ectopias of external granule layer cells. Physical forces applied during development probably contribute to the distortion of the gyral pattern. There was a volumetric reduction of the neocerebellum, which might also be a consequence of the basal lamina defect. The cerebellum of a fetus with the Neu-Laxova syndrome showed the same abnormalities as in Walker's lissencephaly. It is postulated that these two conditions belong to a class of prenatal developmental disorders that involves a defect of the extracellular matrix.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/04/20 alle ore 19:54:47