This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Leber hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by acute or subacute bilateral visual loss usually in young men. However, LHON also occurs in those with other neurologic abnormalities,1 such as epilepsy, peripheral neuropathy, tremor, dementia, dystonia, parkinsonism, and multiple sclerosis–like illness. The disease in those patients has been called “Leber plus.”2 We report a patient with Leber plus associated with chorea and dementia resembling Huntington disease (HD).
Case report.
A 37-year-old woman sought treatment for choreic movement and mental deterioration. She developed normally and had relatively good grades in school. At age 24 years, she showed an involuntary movement in her hands and became restless and negligent of housekeeping. At approximately age 30 years, her choreic movement spread to the upper extremities and trunk. By age 34 years, she was unable to walk or do most housekeeping tasks. She also …
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Alert Me
Recommended articles
Clinical Course of a Cohort in the Cuban Epidemic Optic and Peripheral Neuropathy
Mitochondrial DNA haplogroups and mutations in children with acquired central demyelination
Clinical features of MS associated with Leber hereditary optic neuropathy mtDNA mutations
Homoplasmy, heteroplasmy, and mitochondrial dystonia