Document Detail


Menkes syndrome presenting as myoclonic seizures: neuroimaging and EEG observations.
MedLine Citation:
PMID:  17621528     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The authors report an 11-month-old boy with Menkes kinky hair disease who presented with global delay in acquiring milestones and repeated myoclonic jerks. He had scanty, hypopigmented scalp hairs with steely wool-like texture and intervening zones of alopecia. There was low serum ceruloplasmin (5 mg/dL) and copper (24.2 microg/dL). Neuroimaging of the brain revealed marked cerebral atrophy and significant delayed myelination. Magnetic resonance angiography showed tortuous cerebral and neck blood vessels. There was poor therapeutic response to symptomatic treatment.
Authors:
P S Bindu; S Sinha; A B Taly; J M E Kovur; N Gayathri; G R Arunodaya
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of child neurology     Volume:  22     ISSN:  0883-0738     ISO Abbreviation:  J. Child Neurol.     Publication Date:  2007 Apr 
Date Detail:
Created Date:  2007-07-10     Completed Date:  2007-08-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  452-5     Citation Subset:  IM    
Affiliation:
Department of Neurology, NIMHANS, Karnataka, India.
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MeSH Terms
Descriptor/Qualifier:
Electroencephalography / methods
Epilepsies, Myoclonic / etiology*,  pathology*
Humans
Infant
Magnetic Resonance Imaging / methods
Male
Menkes Kinky Hair Syndrome / complications*

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