Neurogenic arthrogryposis multiplex congenita: Clinical and MRI findings

Ermellina Fedrizzi, Gabriella Botteon, Michelina Inverno, Elisa Ciceri, Ludovico D'Incerti, Federica Dworzak

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)343-348
Number of pages6
JournalPediatric Neurology
Volume9
Issue number5
DOIs
Publication statusPublished - 1993

Fingerprint

Arthrogryposis
Magnetic Resonance Imaging
Spinal Cord
Atrophy
Brain
Anterior Horn Cells
Reproductive History
Neurologic Examination
Electroencephalography
Epilepsy
Central Nervous System
Biopsy
Muscles

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Neurogenic arthrogryposis multiplex congenita : Clinical and MRI findings. / Fedrizzi, Ermellina; Botteon, Gabriella; Inverno, Michelina; Ciceri, Elisa; D'Incerti, Ludovico; Dworzak, Federica.

In: Pediatric Neurology, Vol. 9, No. 5, 1993, p. 343-348.

Research output: Contribution to journalArticle

Fedrizzi, E, Botteon, G, Inverno, M, Ciceri, E, D'Incerti, L & Dworzak, F 1993, 'Neurogenic arthrogryposis multiplex congenita: Clinical and MRI findings', Pediatric Neurology, vol. 9, no. 5, pp. 343-348. https://doi.org/10.1016/0887-8994(93)90102-I
Fedrizzi, Ermellina ; Botteon, Gabriella ; Inverno, Michelina ; Ciceri, Elisa ; D'Incerti, Ludovico ; Dworzak, Federica. / Neurogenic arthrogryposis multiplex congenita : Clinical and MRI findings. In: Pediatric Neurology. 1993 ; Vol. 9, No. 5. pp. 343-348.
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