Plasmapheresis in acute disseminated encephalomyelitis

High-dose corticosteroid administration has been regarded as the main therapy for acute disseminated encephalomyelitis (ADEM). However, some patients with ADEM do not respond well to this treatment. We successfully used plasmapheresis to treat 2 patients who had ADEM. We also compared our patients' symptoms and clinical outcomes to those from previous reports. Plasmapheresis may be indicated not only for severe cases that fail to improve after high-dose corticosteroid treatment but also for first-line treatment. Additional large, controlled, double-blinded trials are needed to clarify the role of plasmapheresis in ADEM.     

Reversible palinopsia and the Alice in Wonderland syndrome associated with topiramate use in migraineurs

Two patients are reported who developed palinopsia while taking topiramate for migraine prevention which resolved or decreased in frequency or duration on lower doses, but recurred or increased in frequency or duration on higher doses. Both patients had complete resolution of palinopsia when topiramate was discontinued. A third patient is described who developed the "Alice in Wonderland" syndrome about 1 week after starting topiramate for migraine prevention with complete resolution of symptoms about 1 month after stopping. Topiramate use may cause palinopsia and may be associated with the Alice in Wonderland syndrome through an unknown mechanism.     

Therapeutic plasma exchange in acute disseminated encephalomyelitis in children

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that is probably due to an autoimmune mechanism with an acute presentation and a monophasic course. The management of patients with ADEM is based on supportive therapy, corticosteroids, and intravenous immunoglobulin, and in selected cases, with therapeutic plasma exchange (TPE). The aim of our study is to evaluate the efficacy of TPE, as adjuvant therapy in pediatric patients with ADEM. We retrospectively reviewed the medical records of children with the diagnosis of ADEM between 2009 and 2011 to which TPE was indicated and were admitted in the ICU of Hospital Sant Joan de Deu (Spain). The diagnosis of ADEM was made by clinical and laboratory criteria and by the presence of compatible lesions on cranio‐spinal Magnetic Resonance Imaging (MRI). For signaling TPE, we followed the guidelines established by the American Association of Apheresis (ASFA) in 2010. Five cases were identified. The predominant neurological symptoms in our patients were: altered level of consciousness, seizures, motor deficits, cranial nerve disorders, and aphasia. Most important demyelinating lesions were located in cortical and subcortical white matter of the brain and highlighted brainstream. Patients performed between 4 and 5 sessions, with no reported side effects. Progressive clinical improvement was evident in all patients, with good neurosensory response to stimulation, cessation of seizures, and recovery of limb mobility. Nowadays, one patient's right paresis persists and another suffers epileptic seizures. None of the cases in our series presented new episodes of demyelination. Due to the suggested immune‐mediated pathogenesis of ADEM, treatment is based on immunomodulatory agents, being glucocorticoids the most important ones. The treatment can be complemented with intravenous immunoglobulin and plasmapheresis. Available data suggests that plasma exchange is beneficial in children with ADEM who fail these treatments. The good tolerance of the procedure without adverse reactions and the progressive neurological improvement detected in the reviewed cases suggest that the use of TPE in pediatric patients is a good therapeutic option when performed in an experienced center. J. Clin. Apheresis 30:335–339, 2015. © 2015 Wiley Periodicals, Inc.     

Early diagnostic magnetic resonance imaging in acute disseminated encephalomyelitis

We report two cases and review the literature concerning the importance of early magnetic resonance imaging (MRI) of the brain as a guide for the early diagnosis and treatment of acute disseminated encephalomyelitis (ADEM). A nonspecific term, ADEM refers to an acute disease that is postinfectious, parainfectious, postvaccinal, or of an unknown precipitating factor. Often when there is clinical suspicion of ADEM, MRI is not done before significant morbidity and mortality occur, despite the existence of adequate treatments. Primary care physicians should be aware of the importance of early MRI in ADEM.     

儿童急性播散性脑脊髓炎42例临床分析

目的总结儿童急性播散性脑脊髓炎的临床特征。方法回顾性分析42例急性播散性脑脊髓炎患儿的临床资料。结果 42例患儿中脑型39例,脊髓型3例;发病前有上呼吸道感染36例,疫苗接种史4例,无明显诱因者2例;临床表现为发热8例,抽搐17例,嗜睡5例,昏迷7例,性格改变15例,头痛、呕吐19例,语言障碍8例,肢体功能障碍7例,排便困难3例,脑神经受累7例;脑膜刺激征阳性14例,病理征阳性35例,肌张力增高8例;脑脊液检查异常32例,正常10例;42例均行头颅MRI检查,其中39例出现异常信号,3例无异常;16例行脊髓MRI检查,3例表现异常;应用糖皮质激素治疗后治愈37例,5例留有后遗症。结论感染和疫苗接种是儿童发生急性播散性脑脊髓炎主要诱因,MRI对其诊断有重要价值,早期应用糖皮质激素治疗可改善预后。     

Acute disseminated encephalomyelitis developed after acute herpetic gingivostomatitis

A child with acute disseminated encephalomyelitis (ADEM) developed after acute herpetic gingivostomatisis was described. Inspite of the improvement of his gingivostomatitis, his consciousness gradually deteriorated and he was admitted to Nakadori General Hospital. His consciousness level was drowsiness and increased bilateral patellar reflexes were shown. Because magnetic resonance imaging (MRI) T2-weighted scan showed areas of high signal intensity disseminated in superior portion of medulla oblongata, dorsal portion of pons, basal nuclei and thalamus, he was suspected as having ADEM. Anti-herpes simplex virus (HSV) 1 IgG and IgM antibodies elevated in both blood and cerebrospinal fluid. From these results, HSV1 infection was thought to be the preceding infection of ADEM. Methylprednisolone therapy (20 mg/kg daily) for 3 days, followed by prednisolone (2 mg/kg) was started, with an excellent response. In addition, administration of acyclovir was also continued, considering the complication of HSV encephalitis. MRI T2-weighted scan performed at 2 months later after the onset of ADEM revealed disappearance of the lesions. He was discharged without remaining disorders. It is difficult to distinguish between ADEM and HSV encephalitis because both of these diseases show various neurological symptoms. In our case, MRI was the most useful method for correct diagnosis of ADEM. We concluded that ADEM is important as a disease of central nervus system due to HSV1 infection, in addition to encephalitis.     

急性播散性脑脊髓炎MRI诊断2例

急性播散性脑脊髓炎（ＡＤＥＭ）是一组由于感染-变态反应而发生的中枢神经系统脱髓鞘疾病。临床上常继发于感染、出疹性疾病或预防接种之后。近年来,随着影像学技术的进展,ＭＲＩ检查已成为诊断的主要手段,但国内尚未见此方面的报道。我们报道２例患儿的临床资料,并结合文献分析其ＭＲＩ表现。病例例１,男,１０岁,持续发热２０余日后出现阅读困难、视物不清,同时自觉乏力,不愿活动。无头痛、呕吐、惊厥等。此次病前３天曾有轻微发热、流涕病史。查体:神志清楚,体温３７℃,脉搏１１０次／分。瞳孔散大,对光反应不良,双眼球水平震颤（ ）,视…     

Fatal acute disseminated encephalomyelitis following treated snake bite in India

Snake bite is an important cause of mortality and morbidity in India, with an estimated 35,000 to 50,000 fatal bites occurring annually. The neurological consequences of snake bite are predominantly the result of inhibition of neuromuscular transmission. We describe the first documented case of autopsy proven acute disseminated encephalomyelitis following treated snake bite in a young female.     

Role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating central nervous system disease that is associated with high morbidity and mortality. Although the recognition of ADEM may be facilitated by newer imaging techniques, the optimal treatment of this disease remains uncertain. We describe 4 patients with severe ADEM who responded to treatment that included intensive plasmapheresis. Two of the patients were in coma at the time that plasmapheresis was instituted, and all 4 patients made an excellent recovery. Immunologic studies revealed increased serum IgA levels, increased circulating immune complex levels as measured by the Raji cell assay, and decreased numbers of T and B cells prior to treatment of ADEM. These abnormalities improved following plasma exchange. Plasmapheresis appears to be effective in reversing the neuropathologic process in ADEM. The role of this treatment modality in ADEM requires further evaluation in controlled clinical trials. © 1992 Wiley-Liss, Inc.     

儿童急性播散性脑脊髓炎的临床与影像学表现

目的探讨急性播散性脑脊髓炎(ADEM)的临床与影像学表现特点。方法分析22例ADEM患儿的临床及影像学资料,并对ADEM的临床表现进行归类,观察ADEM患儿的脑MRI表现,包括病灶位置(皮层、皮层下白质、中央白质、脑室旁白质、深部灰质核团以及脑干和小脑)、大小、形态。结果 22例ADEM患儿的发病年龄平均(6.0±3.2)岁。前驱症状中以发热常见,占77.27%(17/22)。临床表现以脑病及抽搐最多见,各占45.45%(10/22);运动障碍次之,占36.36%(8/22)。影像学上,22例患儿中均可见中央白质病变;13例(13/22,59.09%)可见脑室旁病灶;9例(9/22,40.91%)可见皮层病灶;6例(6/22,27.27%)累及深部灰质核团。18例患儿急性期后1个月复查MRI,8例可见脑萎缩。结论儿童ADEM的MRI表现多样,可表现为中央白质病变,还可出现脑室旁病灶及灰质受累。影像学动态观察有助于了解疾病的变化。     

Studies on acute disseminated encephalomyelitis produced experimentally in rhesus monkeys

The factor in brain tissue which induces acute disseminated encephalomyelitis, when injected into rhesus monkeys as an emulsion with adjuvants, has been found in human, monkey, rabbit, and chicken brain but is absent from frog and fish brain. It is unaffected by fixation of the brain in formalin, by boiling, and by treatment with ultrasound. It is present in the spinal cord of 3 day old rabbits but does not appear in the rabbit cerebrum until about the 12th day of life; in this respect it parallels the laying down of myelin. Attempts to produce the encephalomyelitis passively with large quantities of serum or of cell exudates, and suspensions of cells from spleen and lymph node from monkeys with encephalomyelitis, were unsuccessful.     

6例急性播散性脑脊髓炎患者的观察与护理

探讨急性播散性脑脊髓炎患者的观察与护理.对6例急性播散性脑脊髓炎患者的护理及观察进行回顾性分析.6例患者中病死率为0%,4例基本痊愈,2例完全痊愈,其中有3例遗留轻度智能或运动障碍.通过护理干预对生命体征等严密观察处理、心理疏导、肢体功能锻炼等,促进了急性播散性脑脊髓炎患者的康复,缩短了病程,减少了并发症,降低了致残率及致死率.     

6例急性播散性脑脊髓炎患者的观察与护理

探讨急性播散性脑脊髓炎患者的观察与护理.对6例急性播散性脑脊髓炎患者的护理及观察进行回顾性分析.6例患者中病死率为0%,4例基本痊愈,2例完全痊愈,其中有3例遗留轻度智能或运动障碍.通过护理干预对生命体征等严密观察处理、心理疏导、肢体功能锻炼等,促进了急性播散性脑脊髓炎患者的康复,缩短了病程,减少了并发症,降低了致残率及致死率.

Abstract：

Objeceve To investigate the nursing intervention measures of acute disseminated encephalomyelitis(ADEM)Methods The nursing intervention and its effects on 6 patients with were analyzed retrospectively.Results After the nursing intervention,mortality was zero,4/6 of patients were basically cured,2/6 were completely cured,but3/6 remained mental retardation and dyskinesia.Conclusions Nursing intervention can pronote the rehabilitation of ADEM,shorten the course,reduce complications,decrease disabled rate and mortality.     

DWI鉴别诊断病毒性脑炎与急性播散性脑脊髓炎

目的 探讨扩散加权成像(DWI)对病毒性脑炎、急性播散性脑脊髓炎的鉴别诊断价值.方法 对10例病毒性脑炎和10例急性播散性脑脊髓炎病例行常规MRI和DWI检查.测量并计算DWI的表观扩散系数(ADC)值和相对表观扩散系数(rADC)值并以SPSS 10.0统计软件进行统计学分析.结果 病毒性脑炎平均ADC值和rADC值分别为(0.95±0.28)×10-3mm2/s和(1.07±0.31),急性播散性脑脊髓炎平均ADC值和rADC值为(1.38±0.25)×10-3mm2/s和(1.80=1=0.34)(P<0.01).结论 DWI的ADC值和rADC值能较好地鉴别病毒性脑炎与急性播散性脑脊髓炎.     

6例急性播散性脑脊髓炎患者的观察与护理

探讨急性播散性脑脊髓炎患者的观察与护理.对6例急性播散性脑脊髓炎患者的护理及观察进行回顾性分析.6例患者中病死率为0%,4例基本痊愈,2例完全痊愈,其中有3例遗留轻度智能或运动障碍.通过护理干预对生命体征等严密观察处理、心理疏导、肢体功能锻炼等,促进了急性播散性脑脊髓炎患者的康复,缩短了病程,减少了并发症,降低了致残率及致死率.