Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy

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 ch...

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Main Authors: Siti Aminah Sobana, Iin Pusparini, Nushrotul Lailiyya, Ahmad Rizal Ganiem, Uni Gamayani, Yusuf Wibisono, Fathul Huda, Yunia Sribudiani, Tri Hanggono Achmad
Format: Article
Language:English
Published: Universitas Padjajaran 2022-03-01
Series:Majalah Kedokteran Bandung
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Online Access:http://journal.fk.unpad.ac.id/index.php/mkb/article/view/2394
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author Siti Aminah Sobana
Iin Pusparini
Nushrotul Lailiyya
Ahmad Rizal Ganiem
Uni Gamayani
Yusuf Wibisono
Fathul Huda
Yunia Sribudiani
Tri Hanggono Achmad
author_facet Siti Aminah Sobana
Iin Pusparini
Nushrotul Lailiyya
Ahmad Rizal Ganiem
Uni Gamayani
Yusuf Wibisono
Fathul Huda
Yunia Sribudiani
Tri Hanggono Achmad
author_sort Siti Aminah Sobana
collection DOAJ
description 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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spelling doaj.art-9c25b22f9df34d058c53978e1df454c42022-12-22T00:37:58ZengUniversitas PadjajaranMajalah Kedokteran Bandung0126-074X2338-62232022-03-01541576210.15395/mkb.v54n1.23941412Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral NeuropathySiti Aminah Sobana0Iin Pusparini1Nushrotul Lailiyya2Ahmad Rizal Ganiem3Uni Gamayani4Yusuf Wibisono5Fathul Huda6Yunia Sribudiani7Tri Hanggono Achmad8Department of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDepartment of Neurology, Faculty of Medicine Universitas Padjadjaran/Dr. Hasan Sadikin General Hospital BandungDivision of Biochemistry and Molecular Biology, Department of Biomedical Sciences, Faculty of Medicine Universitas Padjadjaran, BandungDivision of Biochemistry and Molecular Biology, Department of Biomedical Sciences, Faculty of Medicine Universitas Padjadjaran, BandungSpinocerebellar 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.http://journal.fk.unpad.ac.id/index.php/mkb/article/view/2394ncs, peripheral neuropathy, sca3
spellingShingle Siti Aminah Sobana
Iin Pusparini
Nushrotul Lailiyya
Ahmad Rizal Ganiem
Uni Gamayani
Yusuf Wibisono
Fathul Huda
Yunia Sribudiani
Tri Hanggono Achmad
Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
Majalah Kedokteran Bandung
ncs, peripheral neuropathy, sca3
title Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
title_full Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
title_fullStr Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
title_full_unstemmed Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
title_short Spinocerebellar Ataxia 3 (SCA3) Patient with Peripheral Neuropathy
title_sort spinocerebellar ataxia 3 sca3 patient with peripheral neuropathy
topic ncs, peripheral neuropathy, sca3
url http://journal.fk.unpad.ac.id/index.php/mkb/article/view/2394
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