Neurological manifestations of Ehlers-Danlos Syndrome.
Neurol India
;
2005 Sep; 53(3): 339-41
Article
in English
| IMSEAR
| ID: sea-121246
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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Skin
/
Humans
/
Male
/
Magnetic Resonance Imaging
/
Adolescent
/
Adult
/
Ehlers-Danlos Syndrome
/
Myotonia
/
Nervous System Diseases
Type of study:
Prognostic study
Language:
English
Journal:
Neurol India
Year:
2005
Type:
Article
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