SCA6 presenting parkinsonism without ataxia - A case report

Shinichi Takeshima, Ikuko Takeda, Keitaro Kobatake, Toru Yamashita, Koji Abe, Masaru Kuriyama

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A 57-year-old man was admitted to our hospital because of bradykinesia. He was diagnosed with Parkinson disease (Hoehn and Yahr grade 2) and administered levodopa at the maximum dose of 800 mg. However, his condition did not improve. While his symptoms were responsive to levodopa therapy, the sensitivity to the drug was poor. Brain MRI revealed atrophy of the upper vermis and cerebral hemispheres, and brain SPECT revealed low perfusion in both parietal lobes. I123-metaiodobenzylguanidine scintigraphy showed a decrease in the heart/mediastinum ratio. Striatal dopamine transporter (DAT) density was evaluated using I123-FP-CIT. The patient showed moderately reduced DAT density, which suggested nigrostriatal dopaminergic damage. His mother was found to have pure cerebellar ataxia without parkinsonism, and her two siblings also had celebellar type of multiple system atrophy (MSA-C) and progressive supranuclear palsy, respectively. Genetic testing revealed that the patient, his mother and the uncle with MSA-C had spinocerebellar ataxia type 6 (SCA6). SCA6 presenting parkinsonism without ataxia is very rare and important for the pathomechanism of disease.

Original languageEnglish
Pages (from-to)243-247
Number of pages5
JournalClinical Neurology
Volume55
Issue number4
Publication statusPublished - 2015

Fingerprint

Spinocerebellar Ataxias
Dopamine Plasma Membrane Transport Proteins
Parkinsonian Disorders
Levodopa
Ataxia
Mothers
Progressive Supranuclear Palsy
Multiple System Atrophy
Corpus Striatum
Cerebellar Ataxia
Hypokinesia
Parietal Lobe
Brain
Cerebrum
Mediastinum
Genetic Testing
Single-Photon Emission-Computed Tomography
Radionuclide Imaging
Atrophy
Parkinson Disease

Keywords

  • Dat scan
  • Levodopa response
  • Nigrostriatum
  • Parkinsonism
  • Spinocerebellar ataxia type 6 (sca6)

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Takeshima, S., Takeda, I., Kobatake, K., Yamashita, T., Abe, K., & Kuriyama, M. (2015). SCA6 presenting parkinsonism without ataxia - A case report. Clinical Neurology, 55(4), 243-247.

SCA6 presenting parkinsonism without ataxia - A case report. / Takeshima, Shinichi; Takeda, Ikuko; Kobatake, Keitaro; Yamashita, Toru; Abe, Koji; Kuriyama, Masaru.

In: Clinical Neurology, Vol. 55, No. 4, 2015, p. 243-247.

Research output: Contribution to journalArticle

Takeshima, S, Takeda, I, Kobatake, K, Yamashita, T, Abe, K & Kuriyama, M 2015, 'SCA6 presenting parkinsonism without ataxia - A case report', Clinical Neurology, vol. 55, no. 4, pp. 243-247.
Takeshima S, Takeda I, Kobatake K, Yamashita T, Abe K, Kuriyama M. SCA6 presenting parkinsonism without ataxia - A case report. Clinical Neurology. 2015;55(4):243-247.
Takeshima, Shinichi ; Takeda, Ikuko ; Kobatake, Keitaro ; Yamashita, Toru ; Abe, Koji ; Kuriyama, Masaru. / SCA6 presenting parkinsonism without ataxia - A case report. In: Clinical Neurology. 2015 ; Vol. 55, No. 4. pp. 243-247.
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