Huntington chorea presenting with motor neuron disease.
Arch Neurol
; 68(5): 650-2, 2011 May.
Article
in En
| MEDLINE
| ID: mdl-21555641
ABSTRACT
BACKGROUND:
There have been a few case reports of motor neuron disease in association with Huntington disease (HD).OBJECTIVE:
To describe a patient presenting with prominent fasciculations, chorea, and possible amyotrophic lateral sclerosis (ALS) in whom genetic testing revealed HD mutation.DESIGN:
Case report.SETTING:
University of Texas Southwestern Medical Center, Dallas. Patient A 69-year-old man with chorea and fasciculations.INTERVENTIONS:
Genetic and electrophysiologic testing. MAIN OUTCOMEMEASURES:
Genetic test result, electrophysiologic test result, and physical examination.RESULTS:
A 69-year-old man with long-standing depression and failing memory presented with muscle twitches of 8 months' duration. He was found to have choreoathetoid movements and distal weakness on neurological examination. Electrophysiologic studies revealed evidence of motor neuron disease. Genetic test showed CAG repeat of 40 on chromosome 4, confirming the diagnosis of HD.CONCLUSION:
Motor neuron disease can rarely occur in patients with HD and could be one of its presenting features.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Chromosomes, Human, Pair 4
/
Motor Neuron Disease
/
Huntington Disease
Type of study:
Diagnostic_studies
/
Prognostic_studies
Limits:
Aged
/
Humans
/
Male
Language:
En
Journal:
Arch Neurol
Year:
2011
Document type:
Article
Affiliation country:
Estados Unidos