• Title/Summary/Keyword: Cerebellar Ataxia

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Occupational Neurotoxic Diseases in Taiwan

  • Liu, Chi-Hung;Huang, Chu-Yun;Huang, Chin-Chang
    • Safety and Health at Work
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    • v.3 no.4
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    • pp.257-267
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    • 2012
  • Occupational neurotoxic diseases have become increasingly common in Taiwan due to industrialization. Over the past 40 years, Taiwan has transformed from an agricultural society to an industrial society. The most common neurotoxic diseases also changed from organophosphate poisoning to heavy metal intoxication, and then to organic solvent and semiconductor agent poisoning. The nervous system is particularly vulnerable to toxic agents because of its high metabolic rate. Neurological manifestations may be transient or permanent, and may range from cognitive dysfunction, cerebellar ataxia, Parkinsonism, sensorimotor neuropathy and autonomic dysfunction to neuromuscular junction disorders. This study attempts to provide a review of the major outbreaks of occupational neurotoxins from 1968 to 2012. A total of 16 occupational neurotoxins, including organophosphates, toxic gases, heavy metals, organic solvents, and other toxic chemicals, were reviewed. Peer-reviewed articles related to the electrophysiology, neuroimaging, treatment and long-term follow up of these neurotoxic diseases were also obtained. The heavy metals involved consisted of lead, manganese, organic tin, mercury, arsenic, and thallium. The organic solvents included n-hexane, toluene, mixed solvents and carbon disulfide. Toxic gases such as carbon monoxide, and hydrogen sulfide were also included, along with toxic chemicals including polychlorinated biphenyls, tetramethylammonium hydroxide, organophosphates, and dimethylamine borane. In addition we attempted to correlate these events to the timeline of industrial development in Taiwan. By researching this topic, the hope is that it may help other developing countries to improve industrial hygiene and promote occupational safety and health care during the process of industrialization.

A Case Report of Wallenberg Syndrome due to Lateral Medullary and Cerebellar Infarction Treated with Korean Medicine Treatment (외측 연수경색 및 소뇌경색에 의한 Wallenberg 증후군 환자의 한방 치험 1례)

  • Nam, Hyun-seo;Han, Seung-hee;Baek, Tae-hyun;Kim, Mikyung;Sun, Seung-ho;Jeong, Ui-min;Han, In-sik
    • The Journal of Internal Korean Medicine
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    • v.40 no.5
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    • pp.768-775
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    • 2019
  • Objective: The aim of this study was to report a case in which Korean medicine treatment with Gami-daebo-tang and acupuncture improved the clinical symptoms in a patient with Wallenberg syndrome. Methods: The patient was treated for 53 days with a Korean medicine treatment consisting of Gami-daebo-tang and acupuncture. We measured dysesthesia based on the number of affected dermatomes. Gait ataxia was estimated with the Korean version of the Berg Balance Scale (K-BBS) and the number of times that the patient had stumbled per day. Dizziness was measured with a numerical rating score (NRS). Results: After treatment, the number of affected dermatomes was decreased. The K-BBS score was improved from 52 to 56, and the frequency of stumbling decreased from 20 to 0 times per day. The NRS score of dizziness decreased from 8 to 0. Conclusion: This case showed that Korean medicine treatment might be helpful for improving the symptoms of patients with Wallenberg syndrome.

A case study of gait disturbance on early stage MSA-C treated with Korean Medicine (보행장애를 호소하는 초기 다계통 위축증(MSA-C) 환자 한의 치험 1례)

  • Choi, Jeong-Woo;Kim, Seo-Young;Kim, Ha-Ri;Jeon, Gyu-Ri;Cho, Seung-Yeon;Park, Seong-Uk;Park, Jung-Mi;Ko, Chang-Nam
    • The Journal of the Society of Stroke on Korean Medicine
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    • v.21 no.1
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    • pp.67-76
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    • 2020
  • ■ Objectives The purpose of this case is to report the effectiveness of Korean medicine in the treatment of early stage multiple system atrophy-cerebellar ataxia(MSA-C) ■ Methods The patient was treated with acupuncture, bee venom, and herbal medicine. The clinical symptoms was assessed using unified multiple system atrophy rating scale(UMSARS) and 20M gait time and steps. ■ Results After treatment, UMSARS score and 20M gait time and steps showed improvement. ■ Conclusion This study suggested that Korean medical treatment could be an effective option for treating early stage MSA-C.

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Gerstmann-Sträussler-Scheinker Disease: A Case Report (Gerstmann-Sträussler-Scheinker병: 증례 보고)

  • Minji Shin;Donghyun Kim;Young Jin Heo;Jin Wook Baek;Suyoung Yun;Hae Woong Jeong
    • Journal of the Korean Society of Radiology
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    • v.84 no.3
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    • pp.745-749
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    • 2023
  • Gerstmann-Sträussler-Scheinker (GSS) disease is a rare hereditary prion disease which is clinically characterized by a progressive cerebellar ataxia followed by cognitive impairment. We report a rare case of GSS disease in a 39-year-old male patient who complained of a progressive gait disturbance followed by dysarthria with cognitive impairment, after five months from the onset of initial symptom. His brain MRI scan revealed multifocal symmetric diffusion restricted lesions with T2/FLAIR hyperintensities in bilateral cerebral cortices, basal ganglia, and thalami. His family members also manifested similar symptoms in their 40-50s, suggesting the possibility of a genetic disease. Finally, he was genetically diagnosed with GSS disease by real-time quaking-induced conversion and prion protein (PRNP) gene sequencing test.

Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses

  • Chang, Soo Jin;Lee, Ji Hyun;Kim, Shin Hye;Lee, Joon Soo;Kim, Heung Dong;Kang, Joon Won;Lee, Young Mock;Kang, Hoon-Chul
    • Clinical and Experimental Pediatrics
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    • v.58 no.5
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    • pp.194-198
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    • 2015
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

Clinical characteristics and prognosis of acute disseminated encephalomyelitis based on the lesions on MRI (자기공명영상의 병변에 따른 급성 파종성 뇌척수염의 임상 양상과 예후)

  • Chung, Sunghoon;Park, Sungsin;Chung, Sajun
    • Clinical and Experimental Pediatrics
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    • v.50 no.9
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    • pp.891-895
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    • 2007
  • Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.