Objective:

Background: Type IV spinocerebellar ataxia type 3 (SCA3) could present as pure parkinsonism. A bilateral subthalamic nucleus deep brain stimulation (STN-DBS) has been reported to improve parkinsonism in one SCA2 patient with resistance to levodopa therapy. Here we report the first case of positive STN-DBS effect in a young-onset pure parkinsonian SCA3 patient with levodopa-induced motor fluctuation and pathological gambling.

Methods:

Results: A 39-year-old male presented with typical features of Parkinson disease including unilateral resting tremor, rigidity and bradykinesia and excellent response to levodopa and dopamine agonist in the first three years. Prominent wearing-off phenomenon and drug-induced dyskinesia develop 2 years after medication. Apparent pathological gambling occurred during the third year. Genetic diagnosis of SCA3 [CAG 15/66* (N: <= 44, P: > 52)] was made at age 33. The brain MRI showed minimal vermis atrophy. Tc99m-TRODAT-1 SPECT showed an asymmetric reduction of uptake in the bilateral putamen. Due to the apparent motor fluctuation and gambling behaviour, DBS was introduced at age of 37. The UPDRS Part III motor score was 39 at pre-DBS phase, 5 at post-DBS 6 months under levodopa 800mg/d and entacapone 800mg/d, and 9 at post-DBS 22 months under levodopa 300mg/d and Selegiline 30mg/d. Neither impulsive control disorder nor motor complications were observed. The stimulation parameters of DBS on left STN were 3.7 V, 130 Hz, 90-μsec pulse width, contact 3 negative, case positive; on right STN: 3.5 V, 130 Hz, 90-μsec pulse width, contact 11 negative, case positive. There were no DBS-related complications. Poor performance in one leg standing indicating mild cerebellar ataxia was noted 6 years after the diagnosis.

Conclusions: STN-DBS could be considered as an alternative treatment for pure parkinsonism of SCA3 patients particularly in those with severe dopa-related motor or non-motor complications. A longitudinal follow-up study is warranted to evaluate the continuous effect of DBS and new-onset atypical manifestations.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/stn-dbs-responsive-motor-complication-and-pathological-gambling-in-type-iv-sca3/