Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Specialty Sites
    • COVID-19
    • Practice Current
    • Practice Buzz
    • Without Borders
    • Equity, Diversity and Inclusion
    • Innovations in Care Delivery
  • Collections
    • Topics A-Z
    • Residents & Fellows
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Specialty Sites
    • COVID-19
    • Practice Current
    • Practice Buzz
    • Without Borders
    • Equity, Diversity and Inclusion
    • Innovations in Care Delivery
  • Collections
    • Topics A-Z
    • Residents & Fellows
    • Infographics
    • Patient Pages
    • Null Hypothesis
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Residents & Fellows

Share

April 09, 2019; 92 (15 Supplement) May 8, 2019

Epilepsy in Alternating Hemiplegia of Childhood: Characteristics, Neuroimaging, and Response to Therapy (P4.6-069)

Julie Uchitel, Ashley Helseth, Lyndsey Prange, Melissa McLean, Ryan Ghusayni, Monisha Sachdev, Arsen Hunanyan, Mohamad Mikati
First published April 16, 2019,
Julie Uchitel
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ashley Helseth
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Lyndsey Prange
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Melissa McLean
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ryan Ghusayni
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Monisha Sachdev
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Arsen Hunanyan
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Mohamad Mikati
1Duke University Medical Center, Division of Pediatric Neurology Durham NC United States
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Citation
Epilepsy in Alternating Hemiplegia of Childhood: Characteristics, Neuroimaging, and Response to Therapy (P4.6-069)
Julie Uchitel, Ashley Helseth, Lyndsey Prange, Melissa McLean, Ryan Ghusayni, Monisha Sachdev, Arsen Hunanyan, Mohamad Mikati
Neurology Apr 2019, 92 (15 Supplement) P4.6-069;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
0

Share

  • Article
  • Info & Disclosures
Loading

Abstract

Objective: Describe the manifestations of epilepsy in Alternating Hemiplegia of Childhood (AHC) and our experience in managing it.

Background: AHC is characterized by recurrent episodes of hemiplegia, double hemiplegia, dystonia, and, frequently, epilepsy. However, the characteristics of epilepsy in AHC have yet to be described in detail.

Design/Methods: We analyzed clinical and electrophysiological data of a cohort of 50 consecutive AHC patients who underwent systematic evaluations as per our clinical pathway.

Results: 31/50 had epilepsy (seizure age of onset: 1.89±3.04 years). In seven, seizures preceded other AHC spells. Eighteen (58%) were drug resistant. Seventeen had focal seizures, frontal more frequently than temporal, then parietal, and occipital. Eleven had primary generalized seizures: 11 generalized tonic-clonic (GTC), 3 myoclonic, and 2 absence. Three had GTCs of unknown onset. Therapies included antiepileptics (31/31), ketogenic diet (6/31), repurposed drugs (11/31) and pulse steroids (2/31), and had mixed results. Vagal nerve stimulation resulted in >50% reduction in seizure frequency in 5/6. Although the majority had epileptiform EEGs, 7 initially had normal EEGs that later became epileptiform. MRI, normal in the majority, showed in some cerebral atrophy (3/31), cerebellar atrophy (3/31) or abnormal spectroscopy (4/5, all had prior status epilepticus-SE). Eleven (35%) had SE, refractory or super refractory in all. 8/11 had multiple SE episodes. 3/11 had regression after SE.26/50 also had, previously not characterized, spells we termed Reduced Awareness Spells (RAS), consisting of isolated lethargy, drowsiness, staring, and reduced responsiveness without concomitant clinical epileptic manifestations or EEG changes.

Conclusions: Epilepsy in AHC: 1) is focal or generalized, 2) can be the initial manifestation of AHC, 3) manifests high frequency of drug resistance and of often recurrent refractory SE that at times is followed by regression, and 4) RAS, likely non-epileptic in nature, occur frequently.

Disclosure: Dr. Uchitel has nothing to disclose. Dr. Helseth has nothing to disclose. Dr. Prange has nothing to disclose. Dr. McLean has nothing to disclose. Dr. Ghusayni has nothing to disclose. Dr. Sachdev has nothing to disclose. Dr. Hunanyan has nothing to disclose. Dr. Mikati has nothing to disclose.

Disputes & Debates: Rapid online correspondence

No comments have been published for this article.
Comment

NOTE: All authors' disclosures must be entered and current in our database before comments can be posted. Enter and update disclosures at http://submit.neurology.org. Exception: replies to comments concerning an article you originally authored do not require updated disclosures.

  • Stay timely. Submit only on articles published within the last 8 weeks.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • 200 words maximum.
  • 5 references maximum. Reference 1 must be the article on which you are commenting.
  • 5 authors maximum. Exception: replies can include all original authors of the article.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Disputes & Debates Submission Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
  • Info & Disclosures
Advertisement

Related Articles

  • No related articles found.

Alert Me

  • Alert me when eletters are published
Neurology: 96 (3)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2021 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise