• Annals of Clinical Neurophysiology
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• 제9권1호
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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.

• 대한한의학회지
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• 제20권4호
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• pp.98-105
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• 2000

Miller Fisher syndrome is characterized by acute external ophthalmoplegia, ataxia and areflexia in the abscence of significant motor or sensory deficit in the limbs and usually results in a complete recovery. Most cases have anteceding events like upper respiratory infection or other viral infections. Its accurate anatomic lesion sites and pathogenesis is still unknown. Recently we experienced a 47 year-old man who had a sudden onset of complete total ophthalmoplegia, ataxia, diplopia and whose condition was improved through Oriental medical treatment.

• Annals of Clinical Neurophysiology
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• 제2권2호
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• pp.81-88
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• 2000

한 정신병원에 장기입원한 정신분열증환자에서 계속 발생한 8명의 급성 축삭성 GBS로 추정되는 환자들의 평균연령은 38세였으며 7명이 남자였다. 모든 환자들은 급성 상행성 양쪽하지 마비나 사지마비를 보이면서 심부 건반사가 소실되었다. 이 병은 주로 여름철에 많이 발생 하였으며 전기생리학적 검사상 축삭이 주로 손상된 소견을 보였다. IVIG치료를 한 1명을 제외한 나머지 환자들은 경제적 사정상 대증요법으로 치료하였다. AMAN형태의 환자 3명 중 1명에서 임상적 호전을 보였고, AMSAN형태의 환자 5명 중 2명에서 임상적 호전을 보였다. AMSAN형태의 환자중 1명에선 10개월 뒤 같은 증상이 재발하였다.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• 제8권1호
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• pp.133-138
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• 1997

Prader-Willi 증후군(Prader-Willi Syndrome, PWS)은 $50{\sim}-70%$ 정도의 환자에서 염색체 15번에 이상이 있음이 보고된 유전적인 질환으로서, 그 특징적인 임상양상은 심한 근긴장의 저하(hypotonia), 반사소실(areflexia), 섭식의 곤란(feeding difficulty), 저체온증(hypothermia), 성기왜소증(microgenitalia), 음낭저형성증(hypoplastic scrotum), 대식증(polyphagia), 포만감의 감소와 비만, 정신운동발달의 지연, 저성선기능증(hypogonadism) 및 안면과 척추의 기형 등이다. 또한 행동상의 여러 문제들이 동반되는데 분노발작, 피부를 심하게 뜯거나 발모광, 과도한 식욕과 관련된 음식 도벽증이 나타나므로 정신과적 치료가 필요하다. 본 증례는 14세된 PWS환자로서 비만과 대식증, 도벽증, 학업부진 및 생활부적응 문제 등으로 2주간의 입원과 약 1년간의 추적기간 동안 약물요법(fluoxetine 투여)과 행동요법, 그리고 가족요법을 받아 왔다. 장기간의 Fluoxetine투여를 통하여 비만과 식욕의 조절에는 효과가 만족스럽지 않았으나 우울감과 감정문제, 자살사고 및 행동문제들은 호전되었다.

• Annals of Clinical Neurophysiology
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• 제6권1호
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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.

• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.71-73
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• 2006

Many neurologic signs are found in Miller Fisher syndrome (MFS) especially including pupillary abnormalities. But when internal ophthalmoparesis is first manifestation in MFS, diagnosis may be difficult in acute phase of illness. We report two cases of MFS presenting with internal ophthalmoplegia. Pupillary areflexia may be involved in acute phase of MFS. When acute bilateral internal ophthalmoparesis is encounted in clinical practice, initial manifestation of MFS should be included in differential diagnosis.

• Annals of Clinical Neurophysiology
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• 제5권1호
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• pp.11-15
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• 2003

Backgrounds: Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods: We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results: We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions: Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple nucleotide CAG repeats(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.

• Annals of Clinical Neurophysiology
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• 제9권1호
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• pp.19-22
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• 2007

Critical illness polyneuropathy (CIP) and critical illness myopathy (CIM) occur commonly in the patients who have been on mechanical ventilation for more than 1 week. Even in some patients diagnosed with CIP, an underlying myopathy may be the primary cause of the muscle weakness. The cormorbid status of CIP and CIM is called as critical illness polyneuropathy and critical illness myopathy (CIPNM). We describe a 56-year-old man with acute quadriparesis and areflexia after systemic inflammatory response syndrome. The diagnosis of CIPNM is important to avoid unnecessary investigations and unreasonably pessimistic prognosis. Electrophysiologic studies are essential for the diagnosis and for planning further clinical management.

• Annals of Clinical Neurophysiology
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• 제22권2호
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• pp.117-120
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• 2020

Miller Fisher syndrome (MFS) is characterized by the acute ophthalmoparesis, ataxia and areflexia. We describe the case of 70-year-old man with cardinal symptom of MFS and active pulmonary tuberculosis (Tb). A thorough evaluation led to the diagnosis of MFS and treatment with intravenous immunoglobulin (IVIg) was started. The complete resolution of ophthalmoparesis and ataxia was observed from the fourth day of IVIg treatment. This is the first report to describe a case of MFS that developed in patient pulmonary tuberculosis.

• Annals of Clinical Neurophysiology
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• 제9권2호
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• pp.89-92
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• 2007

A 33-year-old women developed weakness in all limbs 3 days prior to admission. Motor examination showed decreased strength in all limbs, but sensory examination was normal. Deep tendon reflexes were areflexia. Electrophysiological examination showed conduction blocks with nearly normal conduction velocities and terminal latencies in motor nerves and normal amplitudes and velocities in sensory nerves. Her serum was positive for IgG antibodies to gangliosides GM1, GD1b, and galactocerebroside. Acute motor conduction block neuropathy may be another variant of Guillain-Barre syndrome.