소아 중추 및 말초신경계의 급성 병합 탈수초질환: 단독 탈수초질환과의 비교
Acute Combined Central and Peripheral Demyelination in Children: in Comparison with Isolated Demyelinating Disease
3
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
목적: 본 연구는 소아 중추 및 말초신경계의 급성 병합 탈수초질환의 발병률과 임상적 특징을 조사하고, 중추 혹은 말초신경계의 단독 탈수초질환과의 비교 분석을 하고자 하였다. 방법: 부산대어린이병원에서 2010년부터 2015년 사이에 급성 탈수초질환 소아들의 의무기록을 후향적으로 조사하였다. 임상증상, 자기공명영상 및 신경전도검사 소견을 바탕으로 진단 기준에 합당한 96명을 선정하였다. 이들을 중추신경계 단독 탈수초질환군(A군, 60명), 말초신경계 단독 탈수초질환군(B군, 30명), 그리고 병합 탈수초질환군(C군, 6명)으로 분류하였다. 결과: 세 군 사이 비교분석을 하였고, 2013-2015년 동안의 발병율(A군, 45.0%; B군, 43.3%; C군, 83.3%; P=0.024), 중환자실 입실 비율(A군, 8.3%; B군, 26.7%; C군, 50.0%; P=0.027), 전체 입원 기간(A군, 9.7±11.2일; B군, 12.3±18.7일; C군, 48.3±51.2일; P=0.75), 스테로이드 치료(A군, 88.3%; B군, 10.0%; C군, 100.0%; P=0.003), 면역글로불린 투여(A군, 13.3%; B군, 100.0%; C군, 100.0%; P=0.002), 혈장교환술 시행(A군, 0.0%; B군, 3.3%; C군, 50.0%; P=0.037; P=0.037) 그리고 퇴원시 심각한 활동 장애 비율(A군, 3.3%; B군, 16.7%; C군, 33.3%; P=0.012)에서 의미 있는 차이가 있었다. 결론: 급성 탈수초질환 추정되는 경우에, 병합 탈수초질환군이 단독 탈수초질환군에 비해 불량한 경과를 보일 수 있으므로, 신경영상및 신경전도검사 등의 광범위한 검사를 통해 병합 탈수초질환에 대한정확한 진단과 신속한 치료를 함으로써, 비가역적인 신경학적 후유증을 방지할 수 있겠다.
Abstract
Purpose: This study aimed to describe the clinical characteristics and outcomes of children with acute combined central and peripheral nervous system demyelination (CCPD); and compare with the children of isolated acute central or peripheral nervous system demyelination. Methods: A retrospective chart review of 145 children with acute demyelinating disease between 2010 and 2015 was undertaken in children with younger than 18 years old. Among these, 96 fulfilled criteria (clinical features and positive neuroimaging or electromyography/nerve conduction studies) for either acute central (group A, n=60, 62.5%) or peripheral (group B, n=30, 31.3%) nervous system demyelination, or a CCPD (group C, n=6, 6.3%). Results: Significant differences among the groups (A vs B vs C) were evident for occurrence of disease between 2013-2015 (45.0% vs 43.3% vs 83.3%; P=0.024), admission to intensive care unit (8.3% vs 26.7% vs 50.0%; P=0.027), length of hospitalization (median, 9.7 vs 12.3 vs 48.3 days; P<0.001), treatment with steroids (88.3% vs 10.0 vs 100.0%; P=0.003), immunoglobulins (13.3% vs 100.0% vs 100.0%; P=0.002) and plasmapheresis (0.0% vs 3.3% vs 50.0%; P=0.037) and severe disability at discharge (3.3% vs 16.7% vs 33.3%; P=0.012). Children of group C showed good response to simultaneous use of immunoglobulin and high-dose corticosteroids and earlier try of plasmapheresis, however, two patients had moderate degree of neurological disability. Conclusion: Systemic studies using neuroimaing and electromyography/nerve conduction studies in all patients with demyelinating disease will be necessary to verify the combined or isolated disease, because CCPD might have the poorer outcome than isolated disease.
참고문헌 (25)
- 고태성, "소아 길랑-바레 증후군의 전기 생리학적 분류에 따른 예후", 대한소아신경학회지, 18(1), pp.91~99 (2010), 대한소아신경학회
- Cortese A, "Combined central and peripheral demyelination:clinical features, diagnostic findings, and treatment", J Neurol Sci, 363, pp.182~187 (2016)
- Adamovic T, "Acute combined central and peripheral nervous system demyelination in children", Pediatr Neurol, 39, pp.307~316 (2008)
- Amit R, "Acute, severe, central and peripheral nervous system combined demyelination", Pediatr Neurol, 2, pp.47~50 (1986)
- Aimoto Y, "A case of acute disseminated encephalomyelitis(ADEM)associated with demyelinating peripheral neuropathy", No To Shinkei, 48, pp.857~860 (1996)
- Katchanov J, "Acute combined central and peripheral inflammatory demyelination", J Neurol Neurosurg Psychiatry, 75, pp.1784~1786 (2004)
- Howell KB, "Concomitant transverse myelitis and acute motor axonal neuropathy in an adolescent", Pediatr Neurol, 37, pp.378~381 (2007)
- Pagano MA, "Association between acute disseminated encephalomyelitis and Guillain-Barré syndrome in an adult", Medicina, 71, pp.254~256 (2011)
- Thomas AA, "A case of acute combined central and peripheral demyelination", J Clin Neuromuscul Dis, 15, pp.112~116 (2014)
- Menon B, "Combined central and peripheral demyelination", J Neurosci Rural Pract, 5, pp.78~80 (2014)
- 김시은, "시신경염이 동반된 아급성염증탈수초다발신경병", Annals of Clinical Neurophysiology, 15(1), pp.13~18 (2013), 대한임상신경생리학회
- Lee SA, "The combined peripheral and central demyelinating disease associated with pulmonary tuberculosis", J Korean Neurol Assoc, 18, pp.240~243 (2000)
- Tenembaum S, "Acute disseminated encephalomyelitis : a long-term follow-up study of 84 pediatric patients", Neurology, 59, pp.1224~1231 (2002)
- Schwarz S, "Acute disseminated encephalomyelitis : a follow-up study of 40 adult patients", Neurology, 56, pp.1313~1318 (2001)
- Murthy SN, "Acute disseminated encephalomyelitis in children", Pediatrics, 110, pp.e21~ (2002)
- Transverse Myelitis Consortium Working Group, "Proposed diagnostic criteria and nosology of acute transverse myelitis", Neurology, 59, pp.499~505 (2002)
- Pidcock FS, "Acute transverse myelitis in childhood : center-based analysis of 47 cases", Neurology, 68, pp.1474~1480 (2007)
- The clinical profile of optic neuritis, "Experience of the optic neuritis treatment trial. Optic Neuritis Study Group", Arch Ophthalmol, 109, pp.1673~1678 (1991)
- Beck RW, "The optic neuritis treatment trial", Arch Ophthalmol, 106, pp.1051~1053 (1988)
- Klistorner A, "Axonal loss and myelin in early ON loss in postacute optic neuritis", Ann Neurol, 64, pp.325~331 (2008)
- van der Meché FG, "Chronic inflammatory demyelinating polyneuropathy. Conduction failure before and during immunoglobulin or plasma therapy", Brain, 112, pp.1563~1571 (1989)
- Jones RH Jr, "Pediatric clinical electromyography", pp.1~36 (1996), Lippincott-Raven Publishers
- Marchioni E, "Postinfectious inflammatory disorders : subgroups based on prospective follow-up", Neurology, 65, pp.1057~1065 (2005)
- Dale RC, "Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children", Brain, 123, pp.2407~2422 (2000)
- Hynson JL, "Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children", Neurology, 56, pp.1308~1312 (2001)