Lumbar puncture alleviates chorea in a patient with Huntington's disease and normal pressure hydrocephalus.
A 44-year-old African-American male was admitted to our hospital after a suicide attempt. He had depression, poor cognitive function, choreiform movements, difficulty pronouncing words, and difficulty walking. His symptoms had worsened markedly over several months. Chorea lead to genetic testing that confirmed a diagnosis of Huntington Disease (HD). A CT scan of the head showed wider ventricles than is typical of HD. The head CT and gait change suggested normal pressure hydrocephalus (NPH). Lumbar puncture (LP) led to improved neuropsychologic test scores and walking thereby supporting the diagnosis of NPH. Surprisingly, the LP also led to an 80% improvement of chorea. There are two other reports of an association between HD and NPH. NPH should be considered in HD patients with atypical symptoms, such as the inability to walk or rapid progression, as its treatment may lead to improved cognition, gait, and chorea.
Shirani
Peyman
P
Department of Neurology, Baylor College of Medicine, Houston, TX, USA.
Salamone
Alicia R
AR
Lahijani
Elham
E
York
Michele K
MK
Schulz
Paul E
PE
D000328:Adult; D002819:Chorea / Q000150:complications / Q000628:therapy*; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000628:therapy*; D006850:Hydrocephalus, Normal Pressure / Q000150:complications / Q000628:therapy*; D008297:Male; D013129:Spinal Puncture