TY - JOUR
T1 - Levodopa responsive parkinsonism in an adult with Huntington's disease
AU - Racette, Brad A.
AU - Perlmutter, Joel S.
PY - 1998/10
Y1 - 1998/10
N2 - A patient is reported on with Huntington's disease who, as an adult, first developed severe parkinsonism with bradykinesia, rigidity, postural instability and festinating gait. His clinical signs were similar to those of the Westphal variant of Huntington's disease except that he also had resting tremor and a supranuclear gaze palsy. Magnetic resonance imaging showed caudate and putamen atrophy. Genetic analysis disclosed 49 triple CAG repeats in allele 1 and 17 in allele 2 confirming the diagnosis of Huntington's disease. Treatment with levodopa produced substantial functional motor improvement with a 17 point reduction in the unified Parkinson's disease rating scale (UPDRS) motor subscale including reduction of tremor, bradykinesia, and postural instability. This is the first report of a patient with adult onset Huntington's disease with parkinsonism responsive to levodopa.
AB - A patient is reported on with Huntington's disease who, as an adult, first developed severe parkinsonism with bradykinesia, rigidity, postural instability and festinating gait. His clinical signs were similar to those of the Westphal variant of Huntington's disease except that he also had resting tremor and a supranuclear gaze palsy. Magnetic resonance imaging showed caudate and putamen atrophy. Genetic analysis disclosed 49 triple CAG repeats in allele 1 and 17 in allele 2 confirming the diagnosis of Huntington's disease. Treatment with levodopa produced substantial functional motor improvement with a 17 point reduction in the unified Parkinson's disease rating scale (UPDRS) motor subscale including reduction of tremor, bradykinesia, and postural instability. This is the first report of a patient with adult onset Huntington's disease with parkinsonism responsive to levodopa.
KW - Huntington's disease
KW - Levodopa
KW - Parkinsonism
UR - http://www.scopus.com/inward/record.url?scp=0031785792&partnerID=8YFLogxK
U2 - 10.1136/jnnp.65.4.577
DO - 10.1136/jnnp.65.4.577
M3 - Article
C2 - 9771791
AN - SCOPUS:0031785792
SN - 0022-3050
VL - 65
SP - 577
EP - 579
JO - Journal of Neurology Neurosurgery and Psychiatry
JF - Journal of Neurology Neurosurgery and Psychiatry
IS - 4
ER -