• Title/Summary/Keyword: ataxia

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Isolation of porcine respiratory coronavirus from pig showing ataxia (보행실조증 자돈의 뇌조직에서 돼지 호흡기 코로나바이러스의 분리)

  • Kweon, Chang-hee;Han, Myung-guk;Lee, Jae-gil;Hwang, Eui-kyung;Kang, Yung-bai;Lee, Kwang-won
    • Korean Journal of Veterinary Research
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    • v.37 no.2
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    • pp.339-347
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    • 1997
  • A cytopathogenic virus was isolated from the brain tissues of pig showing ataxia. The biophysical, morphological and serological assay showed that the isolate belongs to a coronavirus. The differential identification of the isolate with monoclonal antibodies against A and X sites of transmissible gastroenteritis virus indicated that the virus has a characteristics of porcine respiratory coronavirus. The RT-PCR on nucleocapsid region of TGEV also showed that the isolate has the same conserved sequence. The diverse pathogenesis of PRCV and its implication in field were discussed.

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A Case of Sensory Guillain-Barre syndrome (감각성 길랑바레 증후군 1예)

  • Choi, Yong-Seok;Kim, Jung-Mee;Han, Young-Su;Cha, Kyung-Man;Han, Jeong-Ho;Cho, Eun-Kyoung;Kim, Doo-Eung
    • Annals of Clinical Neurophysiology
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    • v.6 no.1
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    • pp.57-60
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    • 2004
  • The sixty two-year-old woman was admitted with facial diplegia and ataxic gait. Neurological examination revealed areflexia and sensory ataxia with decreased sensation of position and vibration in both lower extremities. Electrophysiologic study suggest motor dominant demyelinating polyneuropathy and bilateral facial neuropathy. CSF study revealed no cells and increased proteins. After intravenous immunoglobulin therapy, sensory ataxia and electrophysiological study had markedly improved for 3 months.

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Molecular Pathogenesis of Spinocerebellar Ataxia Type 1 Disease

  • Kang, Seongman;Hong, Sunghoi
    • Molecules and Cells
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    • v.27 no.6
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    • pp.621-627
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    • 2009
  • Spinocerebellar ataxia type 1 (SCA1) is an autosomal-dominant neurodegenerative disorder characterized by ataxia and progressive motor deterioration. SCA1 is associated with an elongated polyglutamine tract in ataxin-1, the SCA1 gene product. As summarized in this review, recent studies have clarified the molecular mechanisms of SCA1 pathogenesis and provided direction for future therapeutic approaches. The nucleus is the subcellular site where misfolded mutant ataxin-1 acts to cause SCA1 disease in the cerebellum. The role of these nuclear aggregates is the subject of intensive study. Additional proteins have been identified, whose conformational alterations occurring through interactions with the polyglutamine tract itself or non-polyglutamine regions in ataxin-1 are the cause of SCA-1 cytotoxicity. Therapeutic hope comes from the observations concerning the reduction of nuclear aggregation and alleviation of the pathogenic phenotype by the application of potent inhibitors and RNA interference.

A case of recurrent Miller Fisher Syndrome (재발 Miller Fisher증후군 1예)

  • Lee, Hyo Min;Seok, Jung Im;Lee, Dong Kuck
    • Annals of Clinical Neurophysiology
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    • v.9 no.1
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    • pp.26-28
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    • 2007
  • Miller Fisher syndrome (MFS) is a variant of Guillian-Barre syndrome (GBS) characterized by the triad of ophthalmoplegia, ataxia, and areflexia. Although recurrent GBS is a well known entity, the recurrence of MFS is extremely rare. Here we report an unusual case of recurrent MFS. Initially, the patient had presented with ophthalmoplegia, ataxia, areflexia, and tingling sensation of all extremities. After resolution of the first episode, the patient presented with atypical MFS characterized by ataxia, areflexia, and tingling sensation without ophthalmoplegia.

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Pure Cerebellar Ataxia Presenting in the SCA 1 (순수 소뇌실조증의 임상 양상으로 SCA 1의 과도한 CAG 반복서열을 보인 유전성 소뇌실조증 가족 1례)

  • Song, Eun-Hyang;Lee, Chung-Seok;Kim, Woo-Jung;Kim, Doo-Eung
    • Annals of Clinical Neurophysiology
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    • v.3 no.2
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    • pp.151-155
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    • 2001
  • SCA 1 is an autosomal dominant disorder. The phenotypic manifestations of SCA 1 are not specific, and thus, the diagnosis of SCA 1 rests on molecular genetic testing. The number of CAG repeats ranges from 6-44 in normal alleles and from 39-81 repeats in disease-causing alleles(chromosomal locus 6p22-23). The main clinical features of SCA 1 are ataxia, dysarthria, ophthalmoparesis, extrapyramidal signs without retinal degeneration. A 24-year-old woman with suspected family history presented with progressive cerebellar ataxia, dysarthria, ptosis, titubation and general weakness. Brain MRI revealed a moderate cerebellar atrophy. A genomic polymerase chain reaction(PCR) analysis showed 66 repeats at the SCA 1 locus.

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Miller Fisher syndrome mimicking Wernicke encephalopathy during pregnancy

  • Seo, Jung Hwa;Kang, Mi-Ri;Yoon, Byeol-A;Ji, Ki-Hwan;Oh, Seong-il
    • Annals of Clinical Neurophysiology
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    • v.21 no.1
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    • pp.53-56
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    • 2019
  • Miller Fisher syndrome (MFS) is characterized by ataxia, areflexia, and ophthalmoparesis. Here we present a case of MFS mimicking Wernicke encephalopathy (WE) during pregnancy. A 31-year-old woman at 8 weeks of gestation presented with diplopia and ataxia after experiencing nausea and vomiting for several weeks. We initiated thiamine based on a suspicion of WE, which produced no clear effects. However, her symptoms began to improve following intravenous immunoglobulin treatment, and other findings finally lead to a diagnosis of MFS. Because ataxia and ophthalmoparesis can be misdiagnosed as WE during pregnancy, clinicians should consider MFS in the differential diagnosis.

The Clinical Study on Spinocerebellar Ataxia with Cerebellar Atrophy (Cerebellar Atrophy로 인해 유발된 Spinocerebellar Ataria 환아(患兒) 1례(例) 보고(報告))

  • Ha, Kwang-Su;Kim, Sun-Mi;Ha, Su-Yun;Song, In-Sun;Lee, Jeong-Lim
    • The Journal of Pediatrics of Korean Medicine
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    • v.19 no.2
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    • pp.41-50
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    • 2005
  • Objective : The autosomal dominent cerebellar ataxia(ADCA) is an unusal familial herediatary disorder that has been called olivopontoerebellar atrophy. Recently ADCA referred to as spinocerebellar ataxia(SCA) by molecular genetic characteristics. The purpose of this study is to focus on the improvement of clinical symptoms in SCA patient by oriental medical treatment. Materials & Methods : We experienced a case of the 6-year-old female patient with SCA and the MRI showed atrophy of cerebellum. The patient's chief symptoms come within the purview of five kinds of retardation and five kinds of flaccidity. We treated her with herb medicine (Yukmijihwang-tang gamibang), acupuncture, scalp acupuncture. After we measured the progress of general condition by MBI(Modified Bathel Index). Results : After oriental medical treatment, chief symptoms (ataxia, weakness of low extremities, dysarthria, etc.) and general condition were improved. Conclusion : We suggest that oriental medical therapy is effective to the possibility of treatment on SCA, but more clinical study and observation should be needed.

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Atlantoaxial instability with hydrocephalus in a dog

  • Jeong, Seong Mok;Choi, Hojung
    • Korean Journal of Veterinary Research
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    • v.46 no.1
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    • pp.67-70
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    • 2006
  • An 8-month-old 3.15 kg female Cocker-spaniel with history of ataxia referred to the Veterianary Medical Teaching Hospital, Chungnam National University. There were no abnormalities in CBC and serum chemical analysis. Agenesis of dens was found on dorsoventral view in cervical radiography. Compressed cervical spinal cord and enlarged cerebral ventricle were observed in magnetic resonance imaging. It was diagnosed as atlantoaxial instability with hydrocephalus. For conservative therapy, neck brace was applied and diuretics and prednisolone were administered. The dog's ataxia became better gradually.

Atypical Radiologic Manifestation of NARP Mimicking MELAS: a Case Report

  • Lee, Youdae;Lee, Donghoon;Hwang, Hokyeong
    • Investigative Magnetic Resonance Imaging
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    • v.22 no.2
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    • pp.119-122
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    • 2018
  • Neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome is a rare maternally inherited mitochondrial disorder. Radiologic findings in NARP syndrome are varied; they include cerebral and cerebellar atrophy, basal ganglia abnormalities, and on rare occasions, leukoencephalopathy. This article describes an extremely rare case of NARP syndrome mimicking mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).

A Case Report of a Patient with Wernicke's Encephalopathy Complaining of Quadriplegia, Ataxia, and Impaired Cognition Improved by Korean Medicine Treatment (한의치료로 호전되었던 사지마비, 운동실조, 인지저하를 호소하는 베르니케 뇌병증 환자 증례보고 1례)

  • Shim, Sang-song;Lee, Hyun-seung;Ahn, Jae-yoon;Chae, Han-nah;Yun, Jong-min;Moon, Byung-soon
    • The Journal of Internal Korean Medicine
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    • v.41 no.5
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    • pp.777-786
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    • 2020
  • Background: This study is a report on a case of a Wernicke's encephalopathy with quadriplegia, ataxia, and impaired cognition, whose condition was improved by Korean medicine treatment. Case report: A 51-year-old man diagnosed with Wernicke's encephalopathy was treated with acupuncture, Banhabakchulchunma-tang mixed with Ondam-tang-gami (半夏白朮天麻湯合溫膽湯加味), cupping, moxibustion, and rehabilitation. Clinical symptoms were measured with the Manual Muscle Test (MMT), Berg Balance Scale (BBS), Korean Mini Mental Status Exam (K-MMSE), Functional Independence Measure (FIM), Modified Barthel Index (MBI), and a numeric rating scale (NRS). After 22 days of treatment, his clinical symptoms showed improvement. The motor function improved (MMT Rt. side Gr. 4+G/4+G, Lt. side Gr. 4G/4+G → Rt. side Gr. 5-N/4+G, Lt. side Gr. 5-N/4+G), Ataxia was relieved (BBS 3→33), cognition improved (K-MMSE 15→27), ADL scores showed improvement (FIM 58→90, MBI 40→75), and the NRS score decreased for headache (3→0). Conclusion: Korean medicine treatment could be effective in the treatment of patients with Wernicke's encephalopathy.