Neurogenic arthrogryposis multiplex congenita: clinical and MRI findings.
Pediatr Neurol
; 9(5): 343-8, 1993.
Article
em En
| MEDLINE
| ID: mdl-8292208
ABSTRACT
A clinical and magnetic resonance imaging (MRI) study on a selected group of 11 children, with a diagnosis of neurogenic arthrogryposis multiplex congenita (AMC) based on clinical, electromyographic, and muscle biopsy findings, is presented to determine the extent of central nervous system involvement in AMC. Family history, pregnancy, perinatal problems, other abnormalities, and epileptic seizures were reviewed. Neurologic examination, electroencephalography, intellectual assessment, and MRI study both of spinal cord and brain were performed. The clinical and laboratory findings disclosed evidence of spinal cord lesions with involvement of anterior horn cell function in all patients, and impairment of cerebral function in 5 patients. MRI revealed spinal cord atrophy in 3 patients, diffuse atrophy in 2 patients, and involved thoraco-lumbar segments in 1 patient. Cranial MRI studies demonstrated features of developmental brain abnormalities in 3 patients, cortical frontal atrophy in 2, and was normal in 4. In neurogenic AMC patients, MRI examination of the spinal cord and brain may help to clarify the pathogenesis of the disease and is helpful for prognostic and therapeutic purposes.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Artrogripose
/
Imageamento por Ressonância Magnética
/
Doenças Neuromusculares
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
/
Pregnancy
Idioma:
En
Revista:
Pediatr Neurol
Assunto da revista:
NEUROLOGIA
/
PEDIATRIA
Ano de publicação:
1993
Tipo de documento:
Article
País de afiliação:
Itália