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Year : 2005  |  Volume : 53  |  Issue : 3  |  Page : 339--341

Neurological manifestations of Ehlers-Danlos syndrome

1 Departments of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India
2 Departments of Neuroradiology, National Institute of Mental Health and Neurosciences, Bangalore, India

Correspondence Address:
A B Taly
National Institute of Mental Health, Bangalore – 560 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.16938

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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.


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Online since 20th March '04
Published by Wolters Kluwer - Medknow