• Annals of Clinical Neurophysiology
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• 제3권2호
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• pp.115-121
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• 2001

The occurrence of muscle weakness in patients with sepsis or multiple organ failure managed in the intensive care unit has been recognized with increasing frequency in the last two decades. The difficulty in examining critically ill patients may explain why this complication has been only recently recognized. This weakness is due to an axonal polyneuropathy which is called critical illness polyneuropathy(CIP). It must be differentiated from myopathy or neuromuscular junction disturbance that can also occur in the intensive care setting. Neither the cause nor the exact mechanism of CIP has been elucidated. Electrophysiological studies demonstrated an acute axonal damage of the peripheral nerves. Before the recognition of CIP, these cases were usually misdiagnosed as Guillain-$Barr{\acute{e}}$ syndrome. Clinical recovery from the neuropathy is rapid and nearly complete in those patients who survive. Thus, neuropathy acquired during critical illness, although causing a delayed in weaning from ventilatory support and hospital discharge, does not worsen long-term prognosis.

• Pediatric Infection and Vaccine
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• 제23권3호
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• pp.236-239
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• 2016

길랭-바레 증후군은 약 3분의 2에서 선행 감염이 원인이 되며 면역반응 때문에 발병하는 것으로 알려져 있는데, 그 중 인플루엔자 바이러스는 비교적 드문 원인이다. 발병 기전과 관련된 항체들에 대한 보고들은 몇 차례 있었지만 길랭-바레 증후군 환자의 뇌척수액에서 인플루엔자 바이러스가 직접 검출된 증례는 없었다. 6세 여아가 내원 1주 전 인플루엔자 A로 진단된 후 oseltamivir를 복용하며 증상이 호전되었고, 내원 2일 전 두통 및 하루 전 양하지 위약감이 생겨서 응급실로 왔다. 신체 진찰, 뇌척수액 검사, 신경전도 검사, 척수 자기공명영상 등의 결과를 토대로 길랭-바래 증후군으로 진단하였고, 뇌척수액중합효소 연쇄반응 검사에서 인플루엔자 A 바이러스가 검출되었으며, 면역글로불린 정맥 투여 후 점차 증상이 호전되었다. 본 증례를 통하여 저자들은 인플루엔자 바이러스가 뇌척수액 내로 직접 침투한 것이 길랭-바레 증후군의 발생과 연관이 있을 것이라고 판단하며, 향후 그 기전에 대한 연구가 필요하겠다.

• Annals of Clinical Neurophysiology
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• 제4권2호
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• pp.119-124
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• 2002

Background : Guillain-$Barr{\acute{e}}$ syndrome(GBS) is a neurologically emergent condition, leading to respiratory insufficiency without an early and appropriate treatment. Thus, the treatment of GBS requires early diagnosis but it is difficult due to the low sensitivity of laboratory tools in the initial stage. Hoffman reflex (H-reflex) and its facilitation by Jendrassik maneuver (JM) are sensitive tools evaluating the central circuit of motor system on the spinal cord level. The aim of this study is to test whether the change of H-reflex and F-wave under the JM is able to detect the early stage of GBS and whether GBS involves the central nervous system (CNS). Material and Methods : All 7 GBS patients who showed normal or nearly normal nerve conduction study were included. The facilitation of H-reflex and changes of F-wave were calculated by measuring the percent difference of H-reflex or F-wave amplitude under JM compared to basal H-reflex of F-wave amplitude. The changes of F-wave and H-reflex in the GBS patients were compared with them of 8 healthy controls. Results : The F-wave amplitudes of both healthy controls and GBS patients did not changed under the influence of JM ($102.4{\pm}24.9%$, $108.7{\pm}29.0%$ respectively). However, the facilitation of H-wave by JM in the GBS patients was absent ($98.8{\pm}5.8%$), even though the H-reflex amplitude I the healthy controls increased under the influence of JM ($124.8{\pm}12.1%$). Conclusions : The loss of H-reflex facilitation in GBS implies that this phenomenon might be the most early change detected in the electrophysiological study and support the early diagnosis, and that GBS might include lesion in the spinal motor CNS.

• 대한한의학회지
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• 제16권1호통권29호
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• pp.118-131
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• 1995

According to the oriental medicine study on G.B.S, we obtained the result as follows : 1. G.B.S was inclined in flacid paralysis and pain and numbness(痺) in oriental medicine. 2. Etiology factors of G.B.S was classified exogenous and endogenous pathogenic factors. The formers was warmth and heat(濕熱), summer heat and dampness(暑濕), dampness and heat(濕熱), and cool and dampness(寒濕), the latter was the deficiency in both the spleen and the stomach(脾胃虛弱), deficiency of Yin(vital essence) in both the liver and kidney(肝腎陰虛) the factor of dampness and heat(濕熱) was most numerous. 3. Pathological mechanism of G.B.S was close connected with the five viscera - the spleen(脾), the stomach(胃), the liver(肝), the kidney(腎), the lung(肺) 4. Differentiation of Symptom-Complexes(辨症) in the G.B.S was consumption type of nutrient fluid due to heat symptom in the lung(肺熱傷津), fullness type of dampness and heat(濕熱侵淫), defiency type in both the spleen and the kidney(脾腎不足), deficiency type in both the spleen and the stomach(脾胃虛弱), deficiency type in the liver and the kidney(肝腎兩虛) 5. Acupuncture treatment for G.B.S was mainly Yangmoung channels of both the hand and the foot.(手足陽明經)

• Clinical and Experimental Pediatrics
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• 제53권5호
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• pp.616-622
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• 2010

Enterovirus 71 (EV71) has been recognized as a frequent cause of epidemics of hand-foot-and-mouth disease (HFMD) associated with severe neurological symptoms. In the spring of 2009, HFMD was epidemic in Korea. Severe cases with complication, including death, have been reported and it has become a public health issue. Most symptomatic EV71 infections commonly result in HFMD or herpangina. These clinical manifestations can be associated with neurologic syndromes frequently. Neurologic syndromes observed in EV71 include meningitis, meningoencephalomyelitis, poliomyelitis-like paralytic disease, Guillain-Barr$\acute{e}$ syndrome, transverse myelitis, cerebellar ataxia, opsoclonus-myoclonus syndrome, benign intracranial hypertension, and brainstem encephalitis. Examinations for EV 71 were performed from the stools, respiratory secretion or CSF of the children by realtime PCR. Gene analysis showed that most of them were caused by EV71 subgenotype C4a which was prevalent in China, 2008. Public health measures including personal and environmental hygiene, must to target daycare centers, kindergartens, and schools where highly susceptible children congregate. To prevent the spread of infection, preschools where transmission persists for more than 2 incubation periods, have been recommended for closure, and trigger criteria for voluntary closure was instituted. During closure, operators are to thoroughly clean the centers before they are allowed to reopen. In addition, parents are advised to ensure that their children adopt a high standard of personal hygiene and to keep the infected child at home until full recovery. Because the outbreaks occur in a cyclical pattern, surveillance system to predict next outbreaks and adequate public health measures to control need to be planned for future. Control of EV71 epidemics through surveillance and public health intervention needs to be maintained in Korea. Future research should focus on understanding of EV71 virulence, identification of the receptor(s) for EV71, development of antiviral agents and development of vaccine.

• Clinical and Experimental Pediatrics
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• 제58권5호
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• pp.194-198
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• 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospital, Seoul, Korea, were enrolled in this retrospective study. We diagnosed each patient on the basis of the CIDP diagnostic criteria developed in 2010 by the European Federation of Neurological Societies/Peripheral Nerve Society Guidelines. We present the cases of four pediatric patients diagnosed with CIDP to understand the variable clinical course of the disease in children. Our four patients were all between 8 and 12 years of age. Patients 1 and 2 were diagnosed with acute cerebellar ataxia or Guillain-$Barr{\acute{e}}$ syndrome as initial symptoms. While patients 1 and 4 were given only intravenous dexamethasone (0.3 mg/kg/day) for 5 days at the first episode, Patients 2 and 3 were given a combination of intravenous immunoglobulin (2 g/kg) and dexamethasone (0.3 mg/kg/day). All patients were maintained with oral prednisolone at 30 mg/day, but their clinical courses were variable in both relapse intervals and severity. We experienced variable clinical courses of CIDP in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval.

• Journal of Microbiology and Biotechnology
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• 제29권10호
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• pp.1665-1674
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• 2019

Zika virus (ZIKV) is a mosquito-transmitted, emerging Flavivirus that causes Guillain-$Barr{\acute{e}}$ syndrome and microcephaly in adults and fetuses, respectively. Since ZIKV was first isolated in 1947, severe outbreaks have occurred at various places worldwide, including Yap Island in 2007, French Polynesia in 2013, and Brazil in 2015. Although incidences of ZIKV infection and dissemination have drastically increased, the mechanisms underlying the pathogenesis of ZIKV have not been sufficiently studied. In addition, despite extensive research, the exact roles of individual ZIKV genes in the viral evasion of the host innate immune responses remain elusive. Besides, it is still possible that more than one ZIKV-encoded protein may negatively affect type I interferon (IFN) induction. Hence, in this study, we aimed to determine the modulations of the IFN promoter activity, induced by the MDA5/RIG-I signaling pathway, by over-expressing individual ZIKV genes. Our results show that two nonstructural proteins, NS2A and NS4A, significantly down-regulated the promoter activity of IFN-${\beta}$ by inhibiting multiple signaling molecules involved in the activation of IFN-${\beta}$. Interestingly, while NS2A suppressed both full-length and constitutively active RIG-I, NS4A had inhibitory activity only on full-length RIG-I. In addition, while NS2A inhibited all forms of IRF3 (full-length, regulatory domain-deficient, and constitutively active), NS4A could not inhibit constitutively active IRF3-5D. Taken together, our results showed that NS2A and NS4A play major roles as antagonists of MDA5/RIG-I-mediated IFN-${\beta}$ induction and more importantly, these two viral proteins seem to inhibit induction of the type I IFN responses in differential mechanisms. We believe this study expands our understanding regarding the mechanisms via which ZIKV controls the innate immune responses in cells and may pave the way to development of ZIKV-specific therapeutics.