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Titolo:
Etiology and pathophysiology of autistic behavior: Clues from two cases with an unusual variant of neuroaxonal dystrophy
Autore:
Weidenheim, KM; Goodman, L; Dickson, DW; Gillberg, C; Rastam, M; Rapin, I;
Indirizzi:
Montefiore Med Ctr, Dept Pathol, Bronx, NY 10467 USA Montefiore Med Ctr Bronx NY USA 10467 r, Dept Pathol, Bronx, NY 10467 USA Albert Einstein Coll Med, Dept Neurol, Bronx, NY USA Albert Einstein Coll Med Bronx NY USA ll Med, Dept Neurol, Bronx, NY USA Univ Gothenburg, Dept Child & Adolescent Psychiat, Gothenburg, Sweden UnivGothenburg Gothenburg Sweden olescent Psychiat, Gothenburg, Sweden
Titolo Testata:
JOURNAL OF CHILD NEUROLOGY
fascicolo: 11, volume: 16, anno: 2001,
pagine: 809 - 819
SICI:
0883-0738(200111)16:11<809:EAPOAB>2.0.ZU;2-6
Fonte:
ISI
Lingua:
ENG
Soggetto:
N-ACETYLGALACTOSAMINIDASE DEFICIENCY; OBSESSIVE-COMPULSIVE DISORDER; ORBITAL FRONTAL-CORTEX; MENTAL-RETARDATION; INFANTILE-AUTISM; SPECTRUM DISORDERS; CELL COUNTS; CHILDREN; INVOLVEMENT; CHILDHOOD;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
79
Recensione:
Indirizzi per estratti:
Indirizzo: Weidenheim, KM Montefiore Med Ctr, Dept Pathol, 111 E 210th St,Cent 2, Bronx, NY 10467 USA Montefiore Med Ctr 111 E 210th St,Cent 2 Bronx NY USA 10467
Citazione:
K.M. Weidenheim et al., "Etiology and pathophysiology of autistic behavior: Clues from two cases with an unusual variant of neuroaxonal dystrophy", J CHILD NEU, 16(11), 2001, pp. 809-819

Abstract

Two unrelated individuals with autistic behavior had numerous swollen axonterminals (spheroids) located in specific brain regions relevant to their behavioral symptoms. Spheroids are characteristic of neuroaxonal dystrophy,but the clinical profile and anatomic distribution of the lesions in thesetwo patients differed from those of previously described patients with neuroaxonal dystrophy. Spheroids were numerous in the sensory nuclei of the spinal cord and medulla, specific nuclei and the reticular formation of the brainstem tegmentum, hypothalamus, anterior and dorsomedial thalamus, hippocampus, and cingulate and orbitofrontal cortices. Spheroids were sparse in the primary and association cortices and basal ganglia and absent in the hemispheric white matter. Cerebellar atrophy was present in both cases but associated with spheroids in only one case. These cases represent a new variant of neuroaxonal dystrophy in which behavioral symptoms characteristic of autism dominated the clinical picture. Neuroaxonal dystrophy should be included in the list of diseases that may be found in persons with autism.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 02:45:03