Advanced Search

Journal Navigation

Journal Home

Subscriptions

Archive

Contact Us

Table of Contents

Click here for more information

Click here to sign up for SAGE Journal Email Alerts today!

Sign In to gain access to subscriptions and/or personal tools.
Journal of Child Neurology
This Article
Right arrow Full Text (PDF)
Right arrow References
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Saved Citations
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Request Reprints
Right arrow Add to My Marked Citations
Citing Articles
Right arrow Citing Articles via Google Scholar
Right arrow Citing Articles via Scopus
Google Scholar
Right arrow Articles by Bindu, P.S.
Right arrow Articles by Arunodaya, G.R.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Bindu, P.S.
Right arrow Articles by Arunodaya, G.R.
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Menkes Syndrome Presenting as Myoclonic Seizures: Neuroimaging and EEG Observations

P.S. Bindu, DM

Department of Neurology, NIMHANS, Karnataka, India

S. Sinha, DM

Department of Neurology, NIMHANS, Karnataka, India, sanjib_sinha2004{at}yahoo.co.in

A.B. Taly, DM

Department of Neurology, NIMHANS, Karnataka, India

J.M.E. Kovur, MD

Department of Neuroradiology, NIMHANS, Karnataka, India

N. Gayathri, PhD

Department of Neuropathology, NIMHANS, Karnataka, India

G.R. Arunodaya, DM

Department of Neurology, NIMHANS, Karnataka, India

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 µg/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.

Key Words: Menkes kinky hair disease • myoclonic jerks

Journal of Child Neurology, Vol. 22, No. 4, 452-455 (2007)
DOI: 10.1177/0883073807301931
Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?