• 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.

• Investigative Magnetic Resonance Imaging
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• 제19권3호
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• pp.186-190
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• 2015

Acute disseminated encephalomyelitis (ADEM) is a demyelinating and inflammatory condition of the central nervous system, occurring predominantly in white matter. ADEM involving the rhombencephalon without affecting the white matter is very rare. Here, we present an unusual case of ADEM involving only the rhombencephalon in a 4-year-old Asian girl. The patient complained of pain in the right lower extremities, general weakness, ataxia, and dysarthria. The initial brain CT showed subtle ill-defined low-density lesions in the pons and medulla. On brain MRI, T2 high signal intensity (T2-HSI) lesions with mild swelling were present in the pons, both middle cerebellar peduncles, and the anterior medulla. The initial diagnosis was viral encephalitis involving the rhombencephalon. Curiously, a cerebrospinal fluid (CSF) study revealed no cellularity, and negative viral marker findings. Three weeks later, follow up brain MRI showed that the extent of the T2-HSI lesions in the brain stem had decreased. After reinvestigation, it was found that she had a prior history of upper respiratory infection. In this case, we report the very rare case of a patient showing isolated involvement of the rhombencephalon in ADEM, mimicking viral rhombencephalitis on CT and MR imaging. ADEM can involve unusual sites such as the rhombencephalon in isolation, without involvement of the white matter or deep gray matter and, therefore, should be considered even when it appears in unusual anatomical areas. Thorough history taking is important for making a correct diagnosis.

• 고신대학교 의과대학 학술지
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• 제33권2호
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• pp.257-262
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• 2018

Guillain-$Barr{\acute{e}}$ syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.

• Clinical and Experimental Pediatrics
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• 제64권3호
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• pp.103-110
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• 2021

Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.

• Annals of Clinical Neurophysiology
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• 제24권2호
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• pp.101-106
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• 2022

Neurological complications attributed to coronavirus disease-19 (COVID-19) infection have been reported including acute disseminated encephalomyelitis, Guillain-Barré syndrome, and so on. Herein, we report a 49-year-old woman presented with acute encephalopathy and paraplegia simultaneously after COVID-19 infection. Brain magnetic resonance imaging (MRI) showed symmetric hyperintense basal ganglia lesions on T2-weighted imaging. Cerebrospinal fluid pleocytosis, motor axonal neuropathy and enhancement of conus medullaris nerve roots on spine MRI were observed. We treated her with high-dose corticosteroid and intravenous immunoglobulin.

• Clinical and Experimental Pediatrics
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• 제50권9호
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• pp.891-895
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• 2007

목 적 : ADEM은 급성 중추 신경계의 탈수초성 질환으로 병태 생리학적 기전은 확실히 밝혀지지 않았으나 대부분 바이러스 감염 또는 예방 접종 후 수일에서 수주일 경과 한 후에 발병하며, 면역학적 기전이 관여 하는 것으로 알려져 있다. 임상 양상과 예후에 있어서도 다양하게 나타나며, 대부분은 단상성의 경과를 보이는 양성 질환으로 알려져 있다. 뇌 자기공명영상 병변은 전형적인 경우 주로 백질에서만 관찰되나 심부 회백질을 침범하는 경우도 드물지 않게 보고 되고 있다. 이에 다양한 임상 증상과 예후가 뇌 병변의 위치에 의한 것이라 생각되어, 후향적으로 자기공명영상 소견과 의무기록지를 통하여 조사하였다. 방 법 : 1995년 1월부터 2003년 8월까지 경희대학교 소아과에 ADEM으로 입원한 환자 21명을 의무 기록을 토대로 조사하였다. 자기공명영상 소견에 따라 1군은 백질에 국한된 다중 또는 단일 병변, 뇌실 주위 및 소뇌의 병변도 포함된 경우로 분류하였으며, 2군은 심부 회백질 또는 피질 병변으로 백질을 침범하는 경우로 분류하였다. 두 군간에 선행 증상이나, 발병 시 신경학적 증상, 뇌 척수액 소견, 신경학적 후유증을 비교분석 하였다. 결 과 : 두 군에 있어 선행 증상의 빈도를 보면 아무런 선행 증상 없이 발병한 경우가 38.1%로 가장 많았으며 상부 호흡기 감염 증상이 28.6%였고, 두 군간에 선행 증상에 대한 통계학적 차이는 없었다. 발병 당시 증상을 보면 경련이 76.2%, 두통과 구토 증상이 47.6%, 의식의 변화가 38.1%로 관찰되었으며 두 군간에 통계학적 차이가 없었다. 검사 결과 백혈구 증다증이 38.1%, 뇌척수액 소견상 세포 증다증이 76.2%에서 보였으며, 뇌척수액 내 단백질 증가가 28.6%에서 관찰되었으며 두 군간에 통계학적 차이는 없었다. 예후에 있어서도 15명인 71.4%가 정상화 되었으며 지속된 경련은 19%, 운동 장애는 19%에서 보였으며 두 군간에 통계학적 차이가 없었다. 결 론 : 이번 연구에서 병변의 차이에 따른 임상 양상과 예후에 있어 차이가 없는 것으로 관찰되나, 다양한 증상과 다양한 병변 및 예후를 보이고 있어 수많은 다른 면역학적 기전을 가진 이종성 질환이 아닌지 의심된다. 아직까지 ADEM의 진단 기준은 임상 증상과 자기공명영상 소견에 의존하여 다소 불분명하다. 이에 ADEM에 대한 연구가 더 필요할 것이라 생각되며, 아울러 초기 자기공명영상 검사와 추적 관찰에 있어 좀더 세심한 주의와 연구가 필요하겠다.