Majalah Kedokteran Bandung
Vol 54, No 1 (2022)

Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy

Siti Aminah Sobana (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Iin Pusparini (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Nushrotul Lailiyya (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Ahmad Rizal Ganiem (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Uni Gamayani (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Yusuf Wibisono (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Fathul Huda (Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital Bandung)
Yunia Sribudiani (Division of Biochemistry and Molecular Biology, Department of Biomedical Sciences, Faculty of Medicine Universitas Padjadjaran, Bandung)
Tri Hanggono Achmad (Division of Biochemistry and Molecular Biology, Department of Biomedical Sciences, Faculty of Medicine Universitas Padjadjaran, Bandung)



Article Info

Publish Date
30 Mar 2022

Abstract

Spinocerebellar ataxia (SCA) 3 is a neurodegenerative disease which involves cerebellum and extra cerebellum. Neuropathy in SCA3 manifests in various ways, including axonal and demyelination lesions in sensory and motor nerves. There has not been any study that describes the peripheral neuropathy characteristics of SCA3 patients in Indonesia at the time of this publication. This paper reports a case of a 43-year-old male with known spinocerebellar ataxia 3 presented with hereditary ataxia and mild numbness in both palms since two years before. No abnormalities were found during the sensory examination. The NCS showed severe axonal demyelinating sensorimotor peripheral neuropathy. In magnetic resonance imaging (MRI), an atrophy in the cerebellum with cerebral multiple lacunar infarction was identified. Electrophysiological results revealed profound axonal lesion in peripheral nerves. To conclude, peripheral neuropathy in SCA3 represents the dominance of axonal lesions in motor nerves.

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Journal Info

Abbrev

mkb

Publisher

Subject

Medicine & Pharmacology

Description

Majalah Kedokteran Bandung (MKB)/Bandung Medical Journal publishes peer-reviewed original articles and case reports in basic medical research, clinical research, and applied medical science. This journal is published quarterly (March, June, September, and December) by Faculty of Medicine Universitas ...