Neurological manifestations of Ehlers-Danlos syndrome

T. Mathew, S. Sinha, A. B. Taly, G. R. Arunodaya, S. G. Srikanth

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Ehlers-Danlos Syndrome (EDS) is more identified for its cutaneous features but its neurological manifestations have not received the focused attention. Four patients of Ehlers-Danlos Syndrome (EDS) with neurological manifestations were evaluated for phenotypic data. These four men were from three families and two had consanguineous parentage. The mean age at onset and presentation of neurological symptoms were 10.5 years and 19 years respectively. Patient 1 presented with bilateral optic atrophy, sensorineural deafness, cerebellar ataxia and neuropathy. Patient 2 had marfanoid habitus, chorea and cerebellar ataxia. Patient 3 had action and percussion myotonia, wasting and weakness of sternocleidomastoid and distal limb muscles. Patient 4 had action myotonia, mirror movements of both hands and neuropathy. MRI of brain showed right parietal polymicrogyria. Neuroaxis involvement at multiple levels in EDS may have prognostic significance.

Original languageEnglish
Pages (from-to)339-341
Number of pages3
JournalNeurology India
Volume53
Issue number3
Publication statusPublished - Jul 2005

Keywords

  • Ataxia
  • Chorea
  • Ehlers-Danlos syndrome
  • Mirror movement
  • Myotonia
  • Neurological manifestations
  • Polymicrogyria

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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  • Cite this

    Mathew, T., Sinha, S., Taly, A. B., Arunodaya, G. R., & Srikanth, S. G. (2005). Neurological manifestations of Ehlers-Danlos syndrome. Neurology India, 53(3), 339-341.