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MFN2 유전자 돌연변이가 발견된 축삭형 Charcot-Marie-Tooth 환자들의 하지 MRI 특성
서범천,심동석,오지영,김상범,김성준,정기화,황정희,박기덕,김승민,선우일남,최병옥 대한신경과학회 2007 대한신경과학회지 Vol.25 No.1
Background: Mutations in mitofusin2 (MFN2) are a major underlying cause of axonal Charcot-Marie-Tooth neuropathy (CMT). It has been reported that patients with an early age of onset (<10 years, EO) show more severe clinical phenotypes than those of patients with a later age at onset (≥10 years, LO) in CMT2A with MFN2 mutations. There are few studies about CMT patients with MRI studies and we performed leg MRIs for better understanding of CMT2A. Methods: We identified 19 patients (EO=10; LO=9) with MFN2 mutations. We used functional disability scales and CMT neuropathy scales for the grading of disability. Nerve conduction studies and MRIs of the lower leg were performed in all patients. Results: We confirmed that EO had more severe leg muscle involvement than LO by leg MRI. In 7 out of 9 in LO, there were some degree of asymmetric leg muscle weakness and MRI findings explained the nature of asymmetry, that is, asymmetric cross-sectional areas or fatty infiltration. MRI of EO showed marked fatty infiltration on all three compartments whereas that of LO showed rather selective involvement of the posterior compartment. These results were well correlated with clinical findings that in LO, five patients could not do toe walking whereas only one could not do heel walking. Conclusions: MRI of the leg may be a useful tool for evaluating axonal CMT neuropathy, and asymmetric leg muscle weakness may be the characteristics of an axonal CMT. In addition, more prominent involvement of the posterior leg in LO is a very interesting phenomenon, which is in contrast to the length-dependent involvement in congenital demyelinating neuropathy.
서범천,최영철,김승민,최병옥,심동석,이동현,선우일남 대한신경과학회 2006 대한신경과학회지 Vol.24 No.6
Bethlem myopathy (BM) is an early-onset benign autosomal dominant myopathy characterized by proximal muscle weakness and multiple contractures. It is caused by mutations in the three genes encoding collagen VI, which is a ubiquitous extracellular matrix protein forming a microfibrillar network in close association with the basement membrane. Here, we present a family with BM which is the first reported Korean case.
서범천,김승민,김흥동,신하영,조정희,선우일날 대한신경과학회 2005 대한신경과학회지 Vol.23 No.3
We report 3 cases of recurrent acute disseminated encephalomyelitis (ADEM) known as multiphasic disseminated encephalomyelitis (MDEM). The 3 cases presented with fever, headache, neck stiffness, abdominal pain, seizure and decreased mentality as well as focal neurologic deficits, which showed relatively good steroid responsiveness. Brain MRI revealed variable sized, multifocal, subcortical white matter lesions with gray matter involvement. The CSF study showed mild pleocytosis with negative oligoclonal band.
서범천 대한의사협회 2021 대한의사협회지 Vol.64 No.7
Background: Neuropathic pain is defined as pain arising as a direct consequence of a lesion or disease affecting the somatosensory system either at the peripheral or central level. In most cases, neuropathic pain is associated with poor general health and has a problem of suboptimal response to medical treatment. This review will discuss the neurologic and non-neurologic conditions that cause neuropathic pain and the results of epidemiologic studies on neuropathic pain. Current Concepts: Epidemiology would be a useful clinical tool for designing management and prevention strategies for various neuropathic pain syndromes. Validated neuropathic pain screening questionnaires are widely used as useful tools for the epidemiologic study of neuropathic pain. There are also validated Korean versions of these questionnaires. The overall prevalence of neuropathic pain was estimated at 6.9-10%. Common neuropathic pain syndromes include diabetic neuropathy, herpes zoster, and trigeminal neuralgia. In addition, neuropathic pain can also occur in central nervous system disorders such as spinal cord injury or stroke, and other conditions like cancerous diseases, intervertebral disc disease, and joint diseases. Discussion and Conclusion: Neuropathic pain does not respond well to medical treatment, which leaves both patients and physicians are less satisfied with such treatments. Therefore, physicians must identify the causes of the pain, explain them to the patient, and proceed with the treatment together with patients.
박용식,서범천,김용범,정필욱,문희수,윤원태,김봉제,윤병석,정용균,정하늘,김근현 대한임상신경생리학회 2012 Annals of Clinical Neurophysiology Vol.14 No.1
Neurolymphomatosis is a complication of lymphoma and initial presentation of cranial nerve involvement has been rarely reported. We describe a patient with neurolymphomatosis who presented as facial palsy mimicking steroid responsive Bell’s palsy, a common and benign condition. This case shows that even minor neurologic deficit of lymphoma patient should not be absolutely regarded as a benign condition.
대상포진 후 발생한 복벽의 가성 탈장 - 전기 생리학적 검사와 영상학적 검사의 소견
김봉제,서범천,김용범,정필욱,문희수,윤원태,진동관,박용식,윤병석 대한근전도전기진단의학회 2010 대한근전도 전기진단의학회지 Vol.12 No.2
Background: Segmental zoster paresis occurs only 1-5 percent patient with herpes zoster. Although, thoracic area is the most frequent region of zoster, zoster paresis involving thoracic myotome is relativelyrare. Furthermore, it is difficult to assess the clinical and electrophysiological findings. Case: A 76-year-old man visited neurology department due to right abdominal lancinating post-herpetic pain. We could see the old skin lesion of zoster and right lower abdominal protrusion on examination and abdominal CT revealed mild right abdominal wall distension without visible other intraabdominal pathology. Nerve conduction study of intercostal nerve demonstrated low compound motor action potentials (CMAPs) on right T10, T11 compared with left one, and needle electromyography showed mild denervation potentials in right rectus abdominis (T10-T11 myotome) and T10 paraspinal muscle. Conclusion: Abdominal pseudohernia could be presented in patient with zoster in lower thoracic level and electrophysiologic study help to define pathologic status of post-herpetic pseudohernia.