• Clinical and Experimental Pediatrics
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• 제53권4호
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• pp.607-611
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• 2010

Bickerstaff 뇌간 뇌염은(BBE) 4주 이내의 진행성이며, 비교적 대칭성으로 오는 안근 마비와 실조증, 의식 장애 또는 심부건 반사 항진 등의 임상적 특징을 가지며, 뇌간을 침범하는 타 질환을 배제하였을 때 진단할 수 있는 드문 질환이다. 혈청 또는 뇌척수액의 항 Ganglioside 항체(GM, GD and GQ) 는 때로 BBE의 진단에 도움이 되기도 하며, 뇌 자기 공명 영상, 뇌 척수액 검사, 신경 전도 검사 및 근 전도 검사 등은 진단에 크게 도움이 되지 않는다. 저자들은 안근 마비, 실조증, 언어 운동 장애, 연하 장애, 점진적 사지 마비, 의식 저하 등의 증상을 보이며 혈청과 뇌척수액에서 anti-GM1 항체의 증가를 보여 BBE로 진단하고 면역 글로불린과 스테로이드 치료 후 완치되었던 9세의 여아의 증례를 경험하였기에 문헌 고찰과 함께 이를 보고하는 바이다.

• Annals of Clinical Neurophysiology
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• 제13권1호
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• pp.13-20
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• 2011

Neuro-ophthalmological findings are common and occasionally prominent features in movement disorders. Accordingly, careful evaluation of the ocular motor functions may provide valuable information in early detection of the diseases and monitoring of the progression. Furthermore, accurate assessment of the abnormal ocular motor findings aids in understanding the pathophysiology and mechanisms of the movement disorders, and in their differential diagnosis. Ocular motility examination should include bedside evaluation and laboratory recording of the fixational abnormalities, saccades, smooth pursuit, the vestibulo-ocular reflex, optokinetic nystagmus, and vergence eye movements. In this review, we will discuss various ocular motor findings in ataxia and parkinsonian syndromes, and hyperkinetic movement disorders.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.33-36
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• 2009

Bickerstaff's brainstem encephalitis (BBE) is an autoimmune central nervous system disorder. It can occur in more limited forms and may overlap with Guillain-Barr$\acute{e}$ syndrome (GBS). A 49-year-old female presented with rapidly progressive paralytic ileus, urinary retention, deep drowsiness, ophthalmoplegia, dysarthria, ataxia, quadriparesis and hyporeflexia after viral meningitis. She was diagnosed as BBE with GBS and treated with immunoglobulin. She was completely recovered after 1 month. It is a rare case of BBE overlapping with GBS presenting with severe paralytic ileus.

• Journal of Korean Neurosurgical Society
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• 제55권2호
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• pp.92-95
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• 2014

Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.

• Journal of Genetic Medicine
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• 제10권1호
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• pp.20-26
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• 2013

Mitochondrion is an intracellular organelle with its own genome. Its function in cellular metabolism is indispensable that mitochondrial dysfunction gives rise to multisystemic failure. The manifestation is most prominent with tissues of high energy demand such as muscle and nerve. Mitochondrial myopathies occur not only by mutations in mitochondrial genome, but also by defects in nuclear genes or secondarily by toxic insult on mitochondrial replication. Currently curative treatment modality does not exist and symptomatic treatment remains mainstay. Administration of L-arginine holds great promise according to the recent reports. Advances in mitochondrial RNA import might enable a new therapeutic strategy.

• Journal of Yeungnam Medical Science
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• 제21권1호
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• pp.91-95
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• 2004

저자들은 임신 초기 산모에서 심한 임신입덧에 의해 발생된 베르니케 뇌병증 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제46권3호
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• pp.204-207
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• 2020

Benign fibro-osseous lesions occur when normal bone is replaced by cellular fibrous connective tissue and mineralized structures. One rare type of these lesions is the ossifying fibroma (OF). The aim of this study is to report an unusual case of OF in a 57-year-old female. Physical examination showed facial asymmetry without any tenderness, fluctuation, ocular pain, or ophthalmoplegia. Imaging exams revealed a solid mass involving the left maxilla and orbital floor. Surgical resection was performed without any complications or sequelae, and the histopathological results confirmed OF. Although recurrence is rare in this condition, the patient remains under follow-up.

• Annals of Clinical Neurophysiology
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• 제16권1호
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• pp.27-31
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• 2014

Thiamine deficiency can cause peripheral polyneuropathy and Wernicke's encephalopathy. Wernicke's encephalopathy is characterized by ataxia, ophthalmoplegia, nystagmus, and confusion, and typically presents acute and rapidly progressive course, whereas peripheral neuropathy associated with thiamine deficiency manifests chronic and slowly progressive one. However, acute and rapidly progressive axonal polyneuropathy combined with Wernicke's encephalopathy is quite rare and unusual. Here, we describe a patient with Wernicke's encephalopathy who presented with acute bilateral axonal neuropathy.

• Investigative Magnetic Resonance Imaging
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• 제23권2호
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• pp.172-174
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• 2019

Upon review, it is noted that recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological syndrome characterized by recurrent unilateral headaches and painful ophthalmoplegia of the ipsilateral oculomotor nerve. As seen on brain MRI, thickening and enhancement of the oculomotor cranial nerve can be observed in these cases. We experienced a case of RPON in an adult patient who showed thickening and enhancement of the oculomotor nerve on gadolinium-enhanced 3D-FLAIR image. The authors report a case of RPON with a review of the literature.

• Annals of Clinical Neurophysiology
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• 제21권2호
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• pp.105-107
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• 2019

Acute ophthalmoparesis that includes the oculomotor, trochlear, or abducens nerve may occur as an initial presentation of Miller Fisher syndrome (MFS). The symptoms of MFS or variant forms of Guillain-Barre syndrome are pathogenically related to anti-GQ1b antibodies. We report a case of a 36-year-old man with unilateral isolated abducens nerve palsy associated with anti-GM1 antibody. To the best of our knowledge, this is the first report of unilateral isolated abducens nerve palsy with positivity for anti-GM1 immunoglobulin M antibody.