• Title/Summary/Keyword: Cerebellar Ataxia

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Possibility of Motor Speech Improvement in People With Spinocerebellar Ataxia via Intensive Speech Treatment (집중치료를 통한 소뇌운동실조증 환자의 말운동개선 가능성)

  • Park, Youngmi
    • The Journal of the Korea Contents Association
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    • v.18 no.11
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    • pp.634-642
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    • 2018
  • People with spinocerebellar ataxia, a hereditary and progressive neurogenic disorder, suffer from ataxic dysarthria due to cerebellar dystrophy. This study was designed to examine if intensive motor speech treatment yields improvement in progressive ataxic dysarthria and if then, to investigate magnitude of therapeutic effect. SPEAK $OUT!^{(R)}$ was provided to a 55-year old female diagnosed with SCA for improving motor speech functions. Magnitude of therapeutic effect was large in changes of MPT and vocal intensity across speech tasks. Small effect size was found in changes of fundamental frequency, however, large therapeutic effect was observed in changes of frequency range. In addition, improvement of vocal quality based on jitter, shimmer, and HNR was observed with large therapeutic effect size and vowel space was expanded, particularly, due to F1. Lastly, VHI scores were decreased. Intensive motor speech treatment, called as SPEAK $OUT!^{(R)}$ was effective enough to observe improvement in vocal intensity, frequency range, and vocal quality, expanding vowel space and lowering VHI scores. Based on the results of this case study, further efficacy evaluation of SPEAK $OUT!^{(R)}$ for improving progressive ataxic dysarthria in people with SCA is required.

Vertigo and Sudden Hearing Loss Caused by Pontine Infarction (현훈과 돌발성 난청을 주소로 하는 뇌교경색)

  • Hyun, Min-Kyung;Sin, Won-Yong;Kim, Mi-Rang;Shin, Hyeon-Cheol;Jeong, Ji-Cheon;Yoon, Cheol-Ho;Jeong, Byoung-Mu
    • The Journal of Internal Korean Medicine
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    • v.25 no.4
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    • pp.200-206
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    • 2004
  • Anterior inferior cerebellar artery(AICA) is a branch of the basilar artery which supplies the bloods for ventrolateral cerebellum and the lateral tegmentum of the lower two-thirds of pons. AICA occlusion can cause such symptoms as acute-onset unilateral deafness, vertigo, facial weakness and ataxia. A case of sudden hearing loss, facial palsy and vertigo caused by AICA territory infarction recently presented itself. Korean Oriental and Western medical therapy were applied in combination. Facial palsy and vertigo improved, but hearing loss did not improve. This case is reported with a brief overview of related literatures.

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Delayed Clinical Signs Associated with Cerebellar Lesion in Holstein Calf Infected with Bovine Viral Diarrhea Virus

  • Choi, Woojae;Ro, Younghye;Hong, Leegon;Kim, Eunkyung;Choe, Eunhui;Kim, Danil
    • Journal of Veterinary Clinics
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    • v.39 no.1
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    • pp.38-43
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    • 2022
  • A 5-month-old Holstein downer calf was presented, and bovine viral diarrhea virus was detected in the feces and the cerebrospinal fluid. Combined treatment of recombinant bovine somatotropin (rBST) administration and standing rehabilitation had been performed for two months, and the calf could maintain the standing position for a while. However, the symptoms were not improved, and the calf died due to ruminal tympany and aspiration pneumonia. Megaesophagus, abomasal ulcer, and a normal-sized but softened cerebellum were observed in clinical necropsy. Improvement of symptoms was confirmed by rBST, but was not reached the complete recovery of gait and standing position.

Effects of Rotarod Exercise and Electroacupuncture on Muscle Activity and Serum BDNF Level in the Ataxic Rats by the 3-Acetylpyridine (3-Acetylpyridine에 의한 운동실조 동물모델에서 로타로드 운동과 전침이 근활성도와 혈청 BDNF에 미치는 영향)

  • Rho, Min-Hee;Park, Sook-Young
    • The Journal of the Korea Contents Association
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    • v.10 no.4
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    • pp.236-246
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    • 2010
  • The 3-Acetylpyridine(3-AP) induces cerebellar injury which is chemoablation of the inferior olive nucleus. The purpose of this study was to investigate the effects of exercise(Ex) and electroacupuncture(EA) on muscle activity of hindlimb and serum brain derived neurotrophic factor(BDNF) in the ataxia rats by the 3-AP. 12-week-aged male Sprague-Dawley rats were randomly divided into the 5 groups: Control, 3-AP, 3-AP+Ex, 3-AP+EA and 3-AP+Ex+EA groups. Maximal Height Vertical Jump(MHVJ) was significantly decreased in the 3-AP compared with control group, and it was increased in Ex, EA and Ex+EA groups than 3-AP group. The muscle activity of hindlimb was significantly increased in the 3-AP groups than control group, also it was most decreased in Ex+EA group. The concentration of BDNF of the serum was significantly decreased in the Ex, EA and Ex+EA groups than 3-AP group. The results of this study showed that dynamic exercise and electroacupuncture have a positive effect on functional recovery and in the ataxia rats by the 3-AP.

Posttraumatic Giant Extradural Intradiploic Epidermoid Cysts of Posterior Cranial Fossa: Case Report and Review of the Literature

  • Enchev, Yavor;Kamenov, Bogidar;William, Alla;Karakostov, Vasil
    • Journal of Korean Neurosurgical Society
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    • v.49 no.1
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    • pp.53-57
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    • 2011
  • We reported a unique case of posttraumatic giant infratentorial extradural intradiploic epidermoid cyst. A 54-year-old male, with a previous history of an open scalp injury and underlying linear skull fracture in the left occipital region in childhood, presented with a painful subcutaneous swelling, which had been developed gradually in the same region and moderate headache, nausea, vomiting and cerebellar ataxia. The duration of symptoms on admission was 3 months. Imaging studies revealed occipital bone destruction and giant extradural intradiploic lesion. The preoperative diagnosis was giant infratentorial extradural intradiploic epidermoid cyst. Surgery achieved total removal of the lesion, which was histologically confirmed and the postoperative course was uneventful. To our knowledge, this is the first case of giant infratentorial extradural intradiploic epidermoid cyst with a traumatic etiology described in the literature.

Canine distemper virus infection in a marten (담비의 개디스템퍼 바이러스 자연 감염례)

  • Woo, Gye-Hyeong;Kim, Jae-Hoon;Jean, Young-Hwa;Lee, Nam-Il;Hwang, Seon-Wook;Seo, Il-Bok;An, Dong-Jun
    • Korean Journal of Veterinary Pathology
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    • v.3 no.1
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    • pp.61-64
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    • 1999
  • A dead marten(Martes melampus) showing cough, ataxia and convulsion of hind limb followed by seizures, was submitted for diagnosis to the Pathology Division of the National Veterinary Research and Quarantine Service. In the gross lesions, lung was congested and consolidated and meningeal blood vessels were mildly congested. Histopathologic findings were diffuse interstitial pneumonia and nonsuppurative meningoencephalitis with malacia of cerebral and cerebellar white matter. Eosinophilic inclusion bodies were observed in neurons and astrocytes and oligodendroglial cells of brain and transitional epithelium of kidney. Using FA test and PCR method, specific antigens of canine distemper virus were demonstrated in the brain.

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A case of suspected bacterial meningoencephalitis in a Miniature Pinscher dog

  • Jung, Dong-In;Park, Chul;Kang, Byeong-Teck;Yoo, Jong-Hyun;Park, Hee-Myung
    • Korean Journal of Veterinary Research
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    • v.46 no.4
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    • pp.405-408
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    • 2006
  • A 4-year-old male Miniature Pinscher was referred because of head tilt, nystagmus, and ataxia. The hemogram revealed a moderate neutrophilic leukocytosis. On magnetic resonance imaging, cerebellar inflammation was detected. And cerebrospinal fluid analysis indicated marked neutrophilic pleocytosis. Based on these results, bacterial meningoencephalitis was suspected. The clinical signs were well controlled by a combination antibiotics therapy of the third generation cephalosporins (cefotaxime and cefixime) and metronidazole. We tentatively diagnosed this case as a bacterial meningoencephalitis because clinical signs were improved after only antibiotics therapy and relapsed when stopped antibiotics administrations, even though the result of bacterial culture on communication of cerebrospinal fluid (CSF) was negative.

Craniopharyngioma with Intratumoral Hemorrhage and Superficial Siderosis

  • Oh, Jeong Hee;Park, Sung-Tae;Lim, Hyun Kyung
    • Investigative Magnetic Resonance Imaging
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    • v.22 no.4
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    • pp.249-253
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    • 2018
  • Superficial siderosis of the central nervous system (CNS) is a progressive and debilitating neurological disease manifesting sensorineural hearing loss, cerebellar ataxia, and pyramidal tract signs. Chronic extravasation of blood into the subarachnoid space results in the accumulation of hemoglobin derivate in the subpial layer of the CNS, which is toxic to the neural tissues. Craniopharyngioma is a benign third ventricle tumor, which rarely presents with tumor bleeding. We report a rare case of superficial siderosis associated with craniopharyngioma with intratumoral hemorrhage in a patient with no history of prior trauma or CNS surgery.

SLC9A6-related developmental and epileptic encephalopathy with spike-and-wave activation in sleep: A case report

  • Hye Ri Bae;Young Ok Kim
    • Journal of Genetic Medicine
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    • v.19 no.2
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    • pp.100-104
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    • 2022
  • The gene encoding solute carrier family 9 member 6 (SLC9A6) on Xq26.3 is associated with Christianson syndrome (CS) mimicking Angelman syndrome. In CS, developmental and epileptic encephalopathy (DEE) appears in about 20%, and DEE with spike-and-wave activation in sleep (SWAS) is reported only in several cases. A 10-year-old boy with DEE showed multidrug resistant focal seizures from 6 months of age. He had progressive microcephaly, regression, global developmental delay without speech, hyperkinesia, and truncal ataxia; he had a long thin face, esotropia, and happy demeanor. Brain magnetic resonance imaging demonstrated cerebellar atrophy. Electroencephalogram at 7.5 years of age showed nearly continuous diffuse paroxysms in slow wave sleep. The seizures were responsive to corticosteroids for a while. Trio whole exome sequencing exhibited a likely pathogenic variant of SLC9A6 in the proband and his asymptomatic mother: c.1194dup (p.Leu399AlafsTer12). This is a rare case report of CS with DEE-SWAS in a Korean patient.

Fatal Balamuthia Amebic Encephalitis in a Healthy Child: A Case Report with Review of Survival Cases

  • Krasaelap, A.;Prechawit, S.;Chansaenroj, J.;Punyahotra, P.;Puthanakit, T.;Chomtho, K.;Shuangshoti, S.;Amornfa, J.;Poovorawan, Y.
    • Parasites, Hosts and Diseases
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    • v.51 no.3
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    • pp.335-341
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    • 2013
  • Balamuthia mandrillaris is one of the 4 amebas in fresh water and soil that cause diseases in humans. Granulomatous amebic encephalitis (GAE), caused by B. mandrillaris, is a rare but life-threatening condition. A 4-year-old, previously healthy, Thai girl presented with progressive headache and ataxia for over a month. Neuroimaging studies showed an infiltrative mass at the right cerebellar hemisphere mimicking a malignant cerebellar tumor. The pathological finding after total mass removal revealed severe necrotizing inflammation, with presence of scattered amebic trophozoites. Cerebrospinal fluid (CSF) obtained from lumbar puncture showed evidence of non-specific inflammation without identifiable organisms. A combination of pentamidine, sulfasalazine, fluconazole, and clarithromycin had been initiated promptly before PCR confirmed the diagnosis of Balamuthia amebic encephalitis (BAE). The patient showed initial improvement after the surgery and combined medical treatment, but gradually deteriorated and died of multiple organ failure within 46 days upon admission despite early diagnosis and treatment. In addition to the case, 10 survivors of BAE reported in the PubMed database were briefly reviewed in an attempt to identify the possible factors leading to survival of the patients diagnosed with this rare disease.