• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.94-97
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• 2006

Bickerstaff's brainstem encephalitis (BBE) is a rare disorder with acute ophthalmoparesis, ataxia, consciousness disturbance, and pyramidal signs of suspected autoimmune origin. A-62-year-old woman developed acute diplopia, dysarthria, gaze-paretic nystagmus and gait ataxia. Her mental status fell subsequently into stupor. Brain MRI and nerve conduction study showed no significantly abnormal findings. Electroencephalography revealed diffuse low voltage slowings. After treating with intravenous immunoglobulin, she demonstrated rapid clinical recovery. This case suggests that immunoglobulin can be an alternative option in BBE treatment, especially when plasmapheresis and corticosteroids are difficult or contraindicated.

• Tuberculosis and Respiratory Diseases
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• 제73권5호
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• pp.273-277
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• 2012

Paraneoplastic limbic encephalitis (PLE) is a rare syndrome characterized by memory impairment, affective and behavioral disturbances and seizures. Among many different neoplasms known to cause PLE, small cell lung cancer (SCLC) is the most frequently reported. The pathogenesis is not fully understood but is believed to be autoimmune-related. We experienced a patient with typical clinical features of PLE. A 67-year-old man presented with seizure and disorientation. Brain magnetic resonance imaging demonstrated high signal intensity in the bilateral amygdala and hippocampus in flair and T2-weighted images suggestive of limbic encephalitis. Cerebrospinal fluid tapping revealed no evidence of malignant cells or infection. Positron emission tomography/computed tomography showed a lung mass with pleural effusion and a consequent biopsy confirmed the diagnosis of PLE associated with SCLC. The patient was subsequently treated with chemotherapy and neurologic symptoms gradually improved.

• Clinical and Experimental Pediatrics
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• 제54권6호
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• pp.234-240
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• 2011

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

• Clinical and Experimental Pediatrics
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• 제55권3호
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• pp.111-113
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• 2012

Hashimoto's encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.

• 대한영상의학회지
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• 제81권2호
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• pp.453-458
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• 2020

하시모토 뇌병증은 다른 대뇌 질환의 증거가 없이 항갑상선 항체의 높은 혈청 농도를 특징으로 하는 드문 자가 면역 질환이다. 하시모토 뇌병증의 자기공명영상 소견은 비특이적이고, 현재까지 미만성 또는 국소 백질 변화가 보고 된 바 있다. 저자는 증가된 항갑상선 항체와 동반하여 수막뇌염과 유사한 영상 소견을 보인 79세 여성의 하시모토 뇌병증을 경험하여 이에 대해 보고하고자 한다. 환자의 일련의 자기공명영상에서 치료 전 관찰되던 여러 T2-고강도 병변과 미만성의 뇌수막 조영증강은 스테로이드 요법 후 소실되었다.

• 대한신경집중치료학회지
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• 제11권2호
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• pp.137-142
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• 2018

Background: New-onset refractory status epilepticus (NORSE) occurs in people without a history of seizures. In these cases, the seizure causes are unclear, and the seizures are not controlled by standard treatment. Autoimmune encephalitis (AIE) can be a cause of NORSE. Cryptogenic NORSE may be associated with AIE, but antibodies associated with the condition have not yet been identified. Primary immunotherapy may not be effective for AIE. Rituximab has improved the prognosis in some cases. Case Report: We treated a cryptogenic NORSE patient with a combination of antiepileptic drugs and immunotherapy. On the 13th hospital day, the seizures were controlled, but the patient remained in a coma. The patient rapidly recovered after administration of rituximab started on the 26th hospital day. Conclusion: Rituximab may be helpful for cryptogenic NORSE patients in whom primary immunotherapy controls seizures, but fails to improve consciousness.