Comparison of acute flaccid myelitis and transverse myelitis in children and evaluation of diagnostic criteria

Background and purpose

Acute flaccid myelitis (AFM) and transverse myelitis (TM) are serious conditions that may be difficult to differentiate, especially at onset of disease. In this study, we compared clinical features of pediatric AFM and TM and evaluated current diagnostic criteria, aiming to improve early and accurate diagnosis.

Methods

Two cohorts of children with enterovirus D68-associated AFM and clinically diagnosed TM were compared regarding presenting clinical features, additional investigations, and outcome. Current diagnostic criteria for AFM and TM were applied to evaluate their specificity.

Results

Children with AFM (n = 21) compared to those with TM (n = 36) were younger (median 3 vs. 10 years), more often had a prodromal illness (100% vs. 39%), predominant proximal weakness (69% vs. 17%), and hyporeflexia (100% vs. 44%), and less often had sensory deficits (0% vs. 81%), bowel and/or bladder dysfunction (12% vs. 69%), and hyperreflexia (0% vs. 44%). On magnetic resonance imaging, brainstem involvement was more common in AFM (74% vs. 21%), whereas supratentorial abnormalities were only seen in TM (0% vs. 40%). When omitting the criterion of a sensory level, 11 of 15 (73%) children with AFM fulfilled the diagnostic criteria for TM. Of children with TM, four of 33 (12%) fulfilled the diagnostic criteria for probable/definite AFM.

Conclusions

Although there is considerable overlap between AFM and TM in children, we found important early differentiating clinical and diagnostic features. Meeting diagnostic criteria for AFM in children with TM and vice versa underlines the importance of thorough clinical examination and early and accurate diagnostic studies.     

The Etiological Spectrum of Acute Sensory Myelitis

Background and Purpose

Acute myelitis patients exhibiting only sensory deficits upon initial presentation are not commonly encountered in clinical practice, but they definitely exist. Since acute sensory myelitis has not been investigated previously, this study evaluated the etiological spectrum of the condition with the aim of describing the clinical characteristics thereof.

Methods

Patients with acute myelitis who presented at the Ewha Womans University Medical Center (during 1999-2012) and the National Cancer Center (during 2005-2014) with only sensory symptoms as first clinical features were enrolled in this study. Their medical records, electrophysiological and laboratory data, and MRI findings were analyzed retrospectively.

Results

Of a total of 341 acute myelitis patients, 52 (15%) were identified as having acute sensory myelitis. The male-to-female ratio of these patients was 35:17, and their age at the onset of the condition was 41.7±10.5 years (mean±SD; range, 24-72 years). Acute sensory myelitis developed in patients with multiple sclerosis (MS; 14%), neuromyelitis optica spectrum disorder (NMOSD; 17%), and acute myelitis associated with concurrent systemic diseases including Behçet''s disease and cancer (6%). Despite detailed evaluation, the etiology of 33 patients with acute myelitis could not be determined. Longitudinally extensive transverse myelitis on spinal MRI and progression of the sensory level were observed most commonly in NMOSD patients (89% and 78%, respectively); however, these patients did not exhibit sensory dissociation. Residual negative sensory symptoms were observed more frequently in NMOSD patients (33%) than in those with acute myelitis of unknown cause (24%) or MS (14%). During the long-term follow-up (4.7±2.7 years) of patients who did not undergo maintenance immunotherapy, a monophasic clinical course was common in those with acute myelitis of unknown cause (76%), but not in NMOSD or MS patients.

Conclusions

Accurate identification of the diverse nature of acute sensory myelitis may assist in patient care.     

Neurotoxin-Induced Paralysis: A Case of Tick Paralysis in a 2-Year-Old Child

BackgroundTick paralysis is an arthropod-transmitted disease causing potentially lethal progressive ascending weakness. The presenting symptoms of tick paralysis overlap those of acute inflammatory diseases of the peripheral nervous system and spinal cord; thus, the condition is often misdiagnosed, leading to unnecessary treatments and prolonged hospitalization.PatientA 2-year-old girl residing in northern New York and having no history of travel to areas endemic to ticks presented with rapidly progressing ascending paralysis, hyporeflexia, and intact sensory examination. Investigation included blood and serum toxicology screens, cerebrospinal fluid analysis, and brain imaging. With all tests negative, the child's condition was initially mistaken for botulism; however, an engorged tick was later found attached to the head skin. Following tick removal, the patient's weakness promptly improved with no additional interventions.ConclusionOur patient illustrates the importance of thorough skin examination in all cases of acute progressive weakness and the necessity to include tick paralysis in the differential diagnosis of paralysis, even in nonendemic areas.     

Acute flaccid paralysis due to West nile virus infection in adults: A paradigm shift entity

Three cases of acute flaccid paralysis (AFP) with preceding fever are described. One patient had a quadriparesis with a florid meningoencephalitic picture and the other two had asymmetric flaccid paralysis with fasciculations at the onset of illness. Magnetic resonance imaging in two cases showed prominent hyperintensitities in the spinal cord and brainstem with prominent involvement of the grey horn (polio-myelitis). Cerebrospinal fluid (CSF) polymerase chain reaction was positive for West Nile virus (WNV) in the index patient. All three cases had a positive WNV immunoglobulin M antibody in serum/CSF and significantly high titer of WNV neutralizing antibody in serum, clearly distinguishing the infection from other Flaviviridae such as Japanese encephalitis. WNV has been recognized in India for many decades; however, AFP has not been adequately described. WNV is a flavivirus that is spread by Culex mosquitoes while they take blood meals from humans and lineage 1 is capable of causing a devastating neuro-invasive disease with fatal consequences or severe morbidity. We describe the first three laboratory confirmed cases of WNV induced AFP from Kerala and briefly enumerate the salient features of this emerging threat.     

34例变异型吉兰-巴雷综合征的临床特点分析

目的 分析变异型吉兰-巴雷综合征(GBS)的临床特点。方法 回顾性分析2012年10月-2019年3月变异型GBS患者的临床表现、电生理及脑脊液特点。结果 共34例患者,包括Miller-Fisher综合征(MFS)及MFS变异亚型共12例,脑神经变异型(CNV)12例,急性感觉神经病(ASN)1例,急性泛自主神经病(APN)1例,咽-颈-臂(PCB)2例以及不能明确分型6例。34例变异型GBS患者中男20例、女14例(P=0.392); 发病年龄17～80岁,平均年龄(53.38±14.99)岁,中年(41～65岁)组所占比例最多(P=0.000); 20例有前驱事件,上呼吸道感染占65%; 首发症状以肢体麻木(38.2%)、吞咽困难(29.4%)、吐词不清(23.5%)多见; 97.1%的患者发病4周内达高峰; 需机械通气者5.9%; 疾病开始恢复的中位时间13 d,住院中位时长13.5 d; 64.7%的患者腱反射减弱或消失; 在完成腰椎穿刺检查的患者中脑脊液蛋白-细胞分离者58.6%; 发病到完善神经电生理检查的平均时间(10.70±7.32)d,85.2%神经电生理检查表现异常; 50%患者给予静脉注射免疫球蛋白(IVIg)治疗,14.7%患者给予激素治疗,8.8%患者给予免疫球蛋白联合激素治疗,除外1例患者主动要求出院,其余治疗均有效。结论 变异型GBS临床表现多样,常无典型急性四肢对称性迟缓性麻痹等症状,临床诊断需要综合判断,出现一些少见的临床表现并不能除外GBS的诊断,脑脊液和神经电生理检查可以帮助提高诊断,详尽的病史及神经系统查体尤为重要,免疫球蛋白和激素治疗有效。     

Acute Ataxia in Children: A Review of the Differential Diagnosis and Evaluation in the Emergency Department

Acute ataxia in a pediatric patient poses a diagnostic dilemma for any physician. While the most common etiologies are benign, occasional individuals require urgent intervention. Children with stroke, toxic ingestion, infection, and neuro-inflammatory disorders frequently exhibit ataxia as an essential—if not the only—presenting feature. The available retrospective research utilize inconsistent definitions of acute ataxia, precluding the ability to pool data from these studies. No prospective data exist that report on patients presenting to the emergency department with ataxia. This review examines the reported causes of ataxia and attempts to group them into distinct categories: post-infectious and inflammatory central and peripheral phenomena, toxic ingestion, neurovascular, infectious and miscellaneous. From there, we synthesize the existing literature to understand which aspects of the history, physical exam, and ancillary testing might aid in narrowing the differential diagnosis. MRI is superior to CT in detecting inflammatory or vascular insults in the posterior fossa, though CT may be necessary in emergent situations. Lumbar puncture may be deferred until after admission in most instances, with suspicion for meningitis being the major exception. There is insufficient evidence to guide laboratory evaluation of serum, testing should be ordered based on clinical judgement—recommended studies include metabolic profiles and screening labs for metabolic disorders (lactate and ammonia). All patients should be reflexively screened for toxic ingestions.     

Increased lipocortin-1 (annexin-1) expression in the sciatic nerve of Lewis rats with experimental autoimmune neuritis

Lipocortin-1 exerts a potent immunosuppressive effect on pathogenic T cells. In multiple sclerosis and experimental autoimmune encephalomyelitis levels of lipocortins are raised, suggesting their involvement in the recovery from an immunological insult or in neural regeneration. To further understand the role of lipocortins in the peripheral nervous system we have characterized lipocortin-1 levels and cellular distribution of lipocortin-1 immunoreactivity in sciatic nerves of rats with experimental autoimmune neuritis (EAN), a model of human Guillain-Barré syndrome. EAN was induced actively by immunization with bovine peripheral myelin (active EAN) or by adoptive-transfer (AT-EAN) of P2-specific T cells. Cellular infiltrates in serial and semithin cryosections were characterized by immunohistochemistry. In parallel, lipocortin-1 levels in tissue extracts were quantified by a sandwich-ELISA. Only weak lipocortin-1 immunoreactivity was found in nerves of control animals injected with non-pathogenic T cells. The majority of macrophages and lymphocytes in EAN lesions exhibited lipocortin-1 immunoreactivity. Some very heavily stained cells showed a distribution and morphology similar to ED-2-positive macrophages which were abundant during early stages of EAN. Lipocortin-1 expression in T cells and macrophages was proven by immunocytochemical studies in semithin serial sections. In tissue extracts, lipocortin-1 levels increased from 0.24 ± 0.14 μg/mg protein in controls receiving non-pathogenic T cells to a maximum of 0.55 ± 0.1 μg/mg protein in AT-EAN at the peak of disease, and then slowly decreased during clinical recovery but still remained elevated. In dose-response studies in AT-EAN, highest values of lipocortin-1 (0.71 ± 0.23 μg/mg protein) were recorded after transfer of 2 × 10⁷ T cells. Increased levels of lipocortin-1 were also measured in active EAN but occurred during the recovery phase (0.65 ± 0.27 μg/mg protein). By analogy with other immune-mediated disorders, increased lipocortin-1 expression in the inflamed sciatic nerve in EAN may exert immunoregulatory functions in-situ and contribute to the termination of the autoimmune response. Received: 20 May 1999 / Accepted: 12 July 1999     

水痘带状疱疹病毒脊髓炎1例报告并文献复习

目的：本文报告1例病前健康的70岁妇女皮肤带状疱疹后罹患水痘带状疱疹病毒脊髓炎（VZVM）患者。方法：复习国内、外关于VZVM的文献，并结合本例患者的临床症状予以回顾性分析。结果：本例患者MRI与临床脊髓受侵部位相对应。结论：阿昔洛韦是VZVM最常用的冶疗药物。VZVM患者的预后不一致。     

湖北省吉兰-巴雷综合征的分布特点及Brighton标准的价值

目的关于我国南方吉兰-巴雷综合征(GBS)的流行病学资料较少,本文旨在分析湖北省GBS的分布特点,并验证Brighton标准的诊断价值。方法收集、整理并回顾性分析2013年1月1日至2016年9月30日4年期间,湖北省内45家医院出院诊断为GBS的病例,并对其进行Brighton诊断分级。结果共收集到GBS病例581例,男性336例,中位发病年龄51岁;发病年龄呈单峰分布,46~54岁患者最多。有前驱事件者占50.9%(296/581),其中上呼吸道感染占59%(176/296)、腹泻15%(43/296);春、夏、秋、冬各不同季节发病无差异(P=0.463);湖北省各地区GBS患者所占比例有差异(P0.001),南部更多;性别及年龄无差异(P=0.906,P=0.472);581例GBS患者中包括颅神经变异型28例、Miler-Fisher综合征57例和经典型496例;经典型GBS患者中,脱髓鞘型所占比例为43.7%,轴突型24.3%;不同亚型发病年龄差异无统计学意义。Brighton分层诊断在所有经典型496例患者中1级占31.3%、2级占52.4%、3级占12.7%和4级占3.6%;在资料完善的247例患者中分别为1级62.8%、2级36.0%、3级0%和4级1.2%。结论湖北省GBS患者的好发年龄在50岁左右,男性多于女性,无季节差异;亚型以脱髓鞘型为主,明显高于我国北方;前驱因素大多数为上呼吸道感染;Brighton标准的灵敏性较高,完善的临床资料可提高诊断分级,在医疗资源相对缺乏的地区,详尽的病史及体格检查有助于提高诊断正确率。     

Detection of immune complexes in experimental allergic neuritis

Circulating immune complexes were assayed in the sera of animals with experimental allergic neuritis (EAN), and immunofluorescent staining and immunohistological examination were performed to clarify the role of immune complexes in the pathogenesis of EAN. The level of immune complexes in the sera of animals with clinical signs of EAN was from 1:32 to 1:64. Control animals showed a titer of immune complexes from 1:2 to 1:4. Animals with EAN showed deposition of immune complexes, which were detected by FITC-conjugated anti-rat IgG or C3-complement, in the vessels of the peripheral nerve. These findings suggest that immune complexes may contribute to the immunopathogenesis of EAN.     

Neuroimaging of Tuberculous Myelitis: Analysis of Ten Cases and Review of Literature

We retrospectively reviewed the clinical and neuroimaging features of 10 patients with tuberculous myelitis. The most common presenting symptoms were fever (70%) and paraplegia (60%). Bladder and bowel symptoms were present in 90% patients. On MRI, the involvement of the cervical/thoracic segment of the spinal cord was most commonly seen (90%). The most consistent finding was hyperintense signals on T2-weighted MRI. T1-weighted images showed isointense (n= 5) and hypointense (n= 4) signals in the spinal cord lesions. Post-contrast enhancement was present in 6 patients, epidural enhancement in 4 patients, and cord swelling in 2 patients. We reviewed more than 250 published cases with the diagnosis of tuberculous myelitis and radiculomyelitis with special attention to MRI findings. It is predominantly a disease of the thoracic spinal cord. Most spinal cord lesions appear as hyperintense on T2 and iso- or hypointense on T1-weighted images. MRI findings in patients with spinal cord tuberculosis have both diagnostic and prognostic significance. Cord atrophy or cavitation and the presence of syrinx on MRI may be associated with poor outcome.     

Brachial plexus neuropathy as unusual onset of diffuse neurolymphomatosis

We present a patient with a large B cell gastric lymphoma in total remission who, after 4 months, developed a fatal progressive peripheral neuropathy with an unusual early involvement of the right brachial plexus. No evidence of lymphoma was found at whole body computed tomography, magnetic resonance imaging of the head, cervical spine and right brachial plexus, bone marrow biopsy or repeated lumbar punctures. The diagnosis of neurolymphomatosis was made only at postmortem examination. Received: 21 June 2000 / Accepted in revised form: 31 August 2000     

Dysphagia and dysphonia among persons with post‐polio syndrome – a challenge in neurorehabilitation

Söderholm S, Lehtinen A, Valtonen K, Ylinen A. Dysphagia and dysphonia among persons with post‐polio syndrome – a challenge in neurorehabilitation.
Acta Neurol Scand: 2010: 122: 343–349.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective – To study the occurrence of dysphagia and dysphonia in persons with post‐polio syndrome admitted into the centre for neurological rehabilitation in Finland. Materials and methods – Fifty‐one persons with post‐polio syndrome who were rehabilitated at Käpylä Rehabilitation Centre, Helsinki, Finland, in 2003–2004 were interviewed on problems with swallowing and voice production. Pulmonary function testing and grip strength measurement were performed. A clinical assessment of oral motor and laryngeal functions was carried out for those who reported daily problems with voice production or swallowing. Results – Fifteen persons (29.4%) reported daily problems with swallowing or voice production. In the clinical assessment, the most commonly observed deficits in swallowing included decreased pharyngeal transit (n = 13) and the food catching in the throat (n = 4). The disturbance of co‐ordination of breathing and voice production was seen in 12 persons. There were no significant differences in any of the potential predictors between the groups. Conclusions – Professionals need to be aware of the routine evaluation of dysphagia and dysphonia in patients with post‐polio syndrome.     

Clinical and Radiological Features of Myelin Oligodendrocyte Glycoprotein-Associated Myelitis in Adults

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have recently been established as a biomarker for MOG-antibody-associated disease (MOGAD), which is a distinct demyelinating disease of the central nervous system. Among the diverse clinical phenotypes of MOGAD, myelitis is the second-most-common presentation in adults, followed by optic neuritis. While some features overlap, there are multiple reports of distinctive clinical and radiological features of MOG-IgG-associated myelitis, which are useful for differentiating MOGAD from both multiple sclerosis and neuromyelitis optica spectrum disorder. In this review we summarize the clinical and radiographic characteristics of MOG-IgG-associated myelitis with a particular focus on adult patients.     

Experimental neuritis induced by a mixture of neural antigens and influenza vaccines. A possible model for Guillain-Barré syndrome

We describe the development in rats of a possible model for Guillain-Barré syndrome (GBS): experimental neuritis (EN). The clinical symptoms, histopathology and the presence of antibody to nervous tissue are features that EN has in common with both GBS and experimental allergic neuritis (EAN), another GBS model. However, EN may be a more appropriate model than EAN for studying the role of autoimmune reactions in diseases such as GBS, which are triggered by various viruses or antigens, since EN depends on such agents being administered concomitantly with the syngeneic tissue.