Mycoplasma pneumoniae-associated myelitis: a comprehensive review

Myelitis is one of the most severe central nervous system complications seen in association with Mycoplasma pneumoniae infections and both acute transverse myelitis (ATM) as well as acute disseminated encephalomyelitis (ADEM) have been observed. We reviewed all available literature on cases of Mycoplasma spp. associated ATM as well as ADEM with dominant spinal cord pathology and classified those cases according to the strength of evidence implicating M. pneumoniae as the cause. A wide range of data on diagnosis, epidemiology, immunopathogenesis, clinical picture, laboratory diagnosis, neuroimaging and treatment for this rare entity is presented. The use of highly sensitive and specific molecular diagnostic techniques may assist in clearly elucidating the role of M. pneumoniae in ATM/ADEM syndromes in the near future. Immunomodulating therapies may have a role in treating such cases.     

Parainfectious myelitis: three distinct clinico-imagiological patterns with prognostic implications

Seventeen parainfectious myelitis patients were studied for site, extent and severity of lesions. Three patterns were observed each having distinct clinical, electrophysiological and MRI features: 1) focal segmentai myelitis – focal cord lesion with long tract signs and good prognosis; 2) ascending myelitis – continuous lesion from conus to mid-cord with upper and lower motor neuron signs (not necessarily spinal shock), dysautonomia and poor outcome; 3) disseminated myelitis - discrete lesions scattered throughout the cord with subtle signs in spinal segmentai distribution, above and below the transverse level and moderate outcome. Severe autonomic dysfunction, denervation of paraspinal muscles, "dense" lesion on imaging and often (but not always) the absent somatosensory evoked potentials carried poor outcome. In conclusion "parainfectious myelitis" is a better term to describe transverse myelitis, as the lesion extends to a large vertical extent. Further classification into 3 subgroups may improve understanding of anatomical and physiological dysfunction and prediction of outcome.     

117例急性横贯性脊髓炎流行病学调查与临床分析

目的 提高对急性横贯性脊髓炎(ATM)的病因、临床特征、诊断和治疗的认识.方法 建立ATM的诊断标准,对我院1992～2009年间收治的117例住院病人的临床资料进行回顾性分析.结果 117例ATM患者中发病高峰年龄为11～50岁,占全部病例的86.1%;11～20岁年龄段发病率最高,达26.9%.夏秋两季ATM发病人...     

Neuroimaging in acute transverse myelitis

Transverse myelitis is an acute inflammatory condition. A relatively rare condition, the diversity of causes makes it an important diagnostic challenge. An approach to the classification and work-up standardizes diagnostic criteria and terminology to facilitate clinical research, and forms a useful tool in the clinical work-up for patients at presentation. Its pathogenesis can be grouped into four categories. Imaging appearances can be nonspecific; however, the morphology of cord involvement, enhancement pattern, and presence of coexistent abnormalities on MR imaging can provide clues as to the causes. Neuroimaging is important in identifying subgroups that may benefit from specific treatment.     

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.     

Clinical study of childhood acute disseminated encephalomyelitis,multiple sclerosis,and acute transverse myelitis in Fukuoka Prefecture,Japan

Acute disseminated encephalomyelitis (ADEM) has recently been studied in several countries owing to the development and wide spread use of imaging technology, but few epidemiological studies of childhood ADEM have been undertaken in Asian countries. To perform a comprehensive survey of ADEM and related diseases in Japanese children, we conducted a multicenter, population-based study on childhood ADEM, multiple sclerosis, and acute isolated transverse myelitis in Fukuoka Prefecture, Japan. We identified 26 children with ADEM, 8 with multiple sclerosis, and 4 with acute transverse myelitis during 5 years between September 1998 and August 2003. The incidence of childhood ADEM under the age of 15 years was 0.64 per 100,000 person-years, mean age at onset was 5.7 years, and male–female ratio was 2.3:1. The prevalence of childhood multiple sclerosis was 1.3 per 100,000 persons. The mean age at onset of multiple sclerosis, 9.3 years, was significantly higher than that of ADEM. Nineteen (73%) and four (15%) patients with ADEM experienced antecedent infectious illnesses and vaccinations, respectively, within 1 month before the onset. Clinical and radiological findings of ADEM revealed that the frequency of seizures, mean white blood cell counts in cerebrospinal fluid, and the frequency of subcortical lesions in Fukuoka study, seemed to be higher than those in previous non-Asian studies. These findings suggest that there are ethnic or geographical differences in the incidence and clinical features of ADEM, and that there might be potent genetic or environmental risk factors for ADEM distinct from those for multiple sclerosis.     

急性横贯性脊髓炎新诊断标准和分类的临床应用

目的 探讨急性横贯性脊髓炎（ATM）新诊断标准和分类的临床应用。方法 对33例ATM患者的临床资料进行回顾性分析,并与Johes Hopkins医院神经内科制定的ATM诊断标准和分类方法进行对照。结果 （1）本组患者均为急性起病,其中有6例起病时伴发热,全部病例均出现双下肢瘫痪,病变累及胸髓最多（29例）;（2）与新标准对照,10例符合新标准分型中的伴发感染相关的ATM,7例符合与多发性硬化相关的ATN;1例符合缺血所致的ATM。结论 ATM具有起病快,病因复杂的特点,新标准可能有助于ATM的诊断、疾病分类和发病机制的探讨。     

A score that predicts aquaporin-4 IgG positivity in patients with longitudinally extensive transverse myelitis

Background and purpose

Longitudinally extensive transverse myelitis (LETM) associated with aquaporin-4 autoantibodies (AQP4-IgG) can cause severe disability. Early diagnosis and prompt treatment are critical to prevent relapses. A novel score is described based on clinical and neuroimaging characteristics that predicts AQP4-IgG positivity in patients with LETM.

Methods

Patients were enrolled both retrospectively and prospectively from multiple Italian centers. Clinical and neuroimaging characteristics of AQP4-IgG positive and negative patients were compared through univariate and multivariate analysis.

Results

Sixty-six patients were included. Twenty-seven (41%) were AQP4-IgG positive and median age at onset was 45.5 years (range 19–81, interquartile range 24). Female sex (odds ratio [OR] 17.9, 95% confidence interval [CI] 2.6–381.9; p = 0.014), tonic spasms (OR 45.6, 95% CI 3.1–2197; p = 0.017) and lesion hypointensity on T1-weighted images (OR 52.9, 95% CI 6.8–1375; p = 0.002) were independently associated with AQP4-IgG positivity. The AQP4-IgG positivity in myelitis (AIM) score predicted AQP4-IgG positivity with 85% sensitivity and 95% specificity. Positive and negative likelihood ratios were 16.6 and 0.2 respectively. The inter-rater and intra-rater agreement in the score application were both excellent.

Conclusions

The AIM score predicts AQP4-IgG positivity with good sensitivity and specificity in patients with a first episode of LETM. The score may assist clinicians in early diagnosis and treatment of AQP4-IgG positive LETM.     

Idiopathic inflammatory demyelinating disorders after acute transverse myelitis

Acute transverse myelitis (ATM) is commonly para-infectious. Recurrent ATM occurs in connective tissue diseases (CTD), infective myelitis and idiopathic inflammatory demyelinating disorders (IIDD) including multiple sclerosis (MS) and neuromyelitis optica (NMO). Previous studies might include NMO and idiopathic recurrent transverse myelitis (IRTM) as MS. The aim was to study the outcome of patients after a first attack of idiopathic ATM. Idiopathic ATM patients over a 6-year period were retrospectively studied. Known causes of myelopathy were excluded. Among 32 patients studied, 20 (63%) had single ATM attack upon follow up for 39–93 months, three developed recurrent ATM related to CTD (two systemic lupus erythematosus and one anti-Ro antibody positive) and nine (28.1%) developed recurrent neuroinflammation compatible with IIDD. Among IIDD patients, three had NMO, two restricted variant of NMO, three IRTM and one classical MS. NMO, its variant and IRTM had mean spinal MRI abnormality of 3.7, 2.1 and 3.9 vertebral segments respectively while non-recurrent ATM had 1.6 vertebral segments. Four (80%) of the five patients with NMO or its variant had poor neurological prognosis versus only one (5%) of non-recurrent ATM patients. IRTM patients had advanced mean onset age, 62 years vs. 43 years for non-recurrent ATM patients. In IIDD patients presenting with ATM as first attack of neuroinflammation, NMO and its variant (56%) were most frequent, then IRTM (33%), with classical MS (11%) the rarest. As long-term treatments for NMO are different from MS, early recognition of NMO and its variant is important for prevention of serious neurological deficits.     

Neurophysiological studies in acute transverse myelitis

A systematic evaluation of anterior horn cell, motor and sensory pathways is possible by electromyography (EMG), motor (MEPs) and somatosensory (SEPs) evoked potentials, respectively, which may provide valuable information on acute transverse myelitis (ATM). In a prospective hospital-based study, EMG, MEP and SEP studies were carried out on admission and after 3 months in 39 patients with ATM. All the patients also underwent detailed clinical evaluation, and spinal magnetic resonance imaging (MRI) was performed in 28. Outcome was defined at the end of 3 months as poor, partial or complete recovery on the basis of functional status. Spinal MRI revealed hyperintense signal changes in T2 extending for two segments to the entire spinal cord. Central motor conduction time to tibialis anterior (CMCT-TA) was more frequently abnormal (90%), followed by tibial SEP (77%). CMCT to abductor digiti minimi (ADM) was abnormal in 30% and median SEP in 15% of patients. Evidence of denervation on EMG was present in 51% of patients. The CMCT-TA improved in 48% patients and tibial SEP in 32%. Median SEP improved in all patients, and CMCT-ADM remained prolonged in two. At 3 months 2 patients had died, and 18 had poor, 10 partial and 9 complete recovery. CMCT was correlated with miscle power, tone, reflec and MRI changes. Patients' outcome of was correlated with CMCT, SEP and EMG. These results are consistent with pronounced involvement of dorsal region of spinal cord in ATM. MEP is more frequently abnormal than SEP. Received: 31 August 1999 / Received in revised form: 12 June 2000 / Accepted: 28 June 2000     

合并肺结核的长节段脊髓炎2例报告

目的探讨长节段横贯性脊髓炎(longitudinally extensive transverse myelitis,LETM)与结核杆菌感染的相关性。方法回顾性分析国内外的LETM与结核杆菌感染的相关性研究报道,就我院收治的2例合并肺结核(pulmonary tuberculosis,PTB)的LETM患者进行临床分析。结果 2例合并PTB的长节段脊髓炎患者,其中病例1,AQP4-Ab/NMO-IgG阳性,诊断为视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSDs),其EDSS最高达7.0分,给予激素联合抗结核治疗后,随访1 y后EDSS为0分,长节段脊髓病灶较治疗前显著吸收;病例2为AQP4-Ab/NMO-IgG阴性的LETM患者,其EDSS 4.5分,给予激素联合抗结核治疗后,3 m随访病情明显改善,EDSS为0分,长节段脊髓病灶较治疗前显著吸收。结论本文经验性抗结核合并激素抑制免疫反应,对此类患者具有良好的治疗作用。关于结核感染与长节段脊髓炎之间的关系,仍需要进一步研究。     

Recurrent transverse myelitis associated with collagen disease

Summary A 43-year-old woman who had four recurrences of acute transverse myelitis (ATM) at the fifth thoracic vertebral level in 7 years is reported. Her haematological and immunological abnormalities included antibodies to DNA and nRNP and markedly reduced serum complement during the course of the disease. These findings suggest systemic lupus erythematosus although she had no symptoms other than those of ATM. Methylprednisolone and prednisolone were effective in treating the ATM.     

Prognostic factors for relapse and outcome in pediatric acute transverse myelitis

ObjectiveIt may be difficult for clinicians to estimate the prognosis of pediatric acute transverse myelitis (ATM). The aim of this study was to define prognostic factors for relapsing disease and poor outcome in pediatric ATM.MethodsThis prospective cohort study included 49 children, 18 boys and 31 girls (median age 13.1 years, IQR 6.5–16.2) with a first episode of ATM. Factors associated with relapsing disease and poor outcome (Expanded Disability Status Scale (EDSS) ≥ 4) were assessed during a median follow-up of 37 months (IQR 18–75).ResultsIn total, 14 patients (29%) experienced ≥ 1 relapse(s) and nine patients (18%) had a poor outcome. Factors at onset associated with relapsing disease included higher age (16.1 vs. 11.6 years, p = 0.002), longer time to maximum severity of symptoms (5.5 vs. 3 days, p = 0.01), lower maximum EDSS score (4.0 vs. 6.5, p = 0.003), short lesion on spinal MRI (64 vs. 21%, p = 0.006), abnormalities on brain MRI (93 vs. 44%, p = 0.002) and presence of oligoclonal bands in cerebrospinal fluid (67 vs. 14%, p = 0.004). The only factor associated with poor outcome was presence of a spinal cord lesion on MRI without cervical involvement (56 vs. 14%, p = 0.02).ConclusionPediatric ATM patients presenting with clinical, radiological and laboratory features associated with multiple sclerosis (MS) are at risk for relapsing disease. In absence of these known MS risk factors at onset of disease these patients are at low risk for relapses. Only a minority of pediatric ATM patients in this cohort have a poor outcome.     

急性播散性脑脊髓炎的临床特点

目的探讨急性播散性脑脊髓炎（ADEM）的临床特点。方法对3例ADEM患者的临床表现、辅助检查、治疗及预后等进行回顾性分析。结果 3例ADEM中2例有前驱感染史,1例有手术史,首发症状分别为意识障碍、头晕和癫癇发作,头颅磁共振（MRI）见颅内多发长T1、长T2信号,呈环形或脑回样强化,累及双侧大脑、小脑和脑干,以双侧侧脑室旁和丘脑常见,经糖皮质激素治疗后病情迅速缓解。结论 ADEM是一种急性炎性脱髓鞘疾病,认识该病的临床及MRI特点可使患者得到及时诊治,改善预后。     

Magnetic resonance imaging findings in 22 cases of myelitis: comparison between patients with and without multiple sclerosis

We reviewed the magnetic resonance imaging (MRI) database of the Dent Neurologic Institute to study the abnormal findings in myelitis. We identified 22 patients, and compared non-MS-related acute transverse myelitis (ATM, n = 9), to myelitis associated with multiple sclerosis (MS-myelitis, n = 13). The ATM patients were significantly older than MS patients at the time of the myelitis diagnosis (mean age 46 vs 35, p < 0.05). ATM appeared as a "longitudinal myelitis", with fusiform cord expansion on T ₁-weighted images and intramedullary increased signal on T ₂-weighted images, each involving multiple spinal levels (mean = 7–8). However, MS-myelitis lesions appeared focal, involving significantly fewer spinal levels (mean = 1–2, p < 0.001), although the lesions were equally likely to expand the cord. Four (42%) of the ATM lesions showed abnormal, variable enhancement, whereas none of the MS myelitis lesions enhanced. Cranial MRI was more likely to be normal in ATM (78%) than in MS-myelitis patients (15%, p < 0.001). Although readily distinguishable from lesions due to MS, the various etiologies for ATM, including post-infectious ( n = 2), post-vaccination ( n = 3), and idiopathic ( n = 4) were indistinguishable on MRI. The MRI findings of an extensively lesioned, swollen cord, suspicious for an intramedullary tumor and providing a temptation for a biopsy, may reflect a non-neoplastic inflammatory disorder.     

Discriminatory features of acute transverse myelitis: a retrospective analysis of 45 patients

Acute transverse myelitis (ATM) is a pathogenetically heterogeneous inflammatory disorder of the spinal cord. Therefore, the identification of clinical and paraclinical features providing clues of the underlying etiologies is needed. The clinical presentation, blood and cerebrospinal fluid (CSF) findings as well as magnetic resonance imaging (MRI) and neurophysiological features were retrospectively analyzed in 45 unselected consecutive patients with ATM. Parainfectious ATM was diagnosed in 38% of patients. The underlying infectious agent, however, was identified only in a minority of patients. In 36% of patients, the etiology remained uncertain ("idiopathic" ATM) and in 22% ATM was the first manifestation of possible multiple sclerosis (ATM-MS) according to recently published diagnostic criteria. Spinal cord MRI showed signal alterations in 96% of the patients. In ATM-MS, monosegmental involvement of the spinal cord was most frequent while spinal cord involvement of two or more segments was more common in ATM of other etiologies. Of particular note, neurophysiological examinations showed evidence of peripheral nervous system (PNS) involvement in 27% of patients with ATM but not in patients with ATM-MS. Therefore, neurophysiological evidence of PNS involvement may provide additional discriminatory features between ATM-MS and ATM of other etiologies.     

低场FLAIR技术在急性脊髓炎中的诊断价值

目的 探讨快速液体衰减反转恢复(FLAIR)技术在急性脊髓炎中的应用价值. 方法 用0.5T超导磁共振(MR)机对52例健康者和33例急性脊髓炎患者行MR检查,扫描序列为TlM.T2WI,FLAIR序列,33例患者中23例作了T1WI增强扫描,比较FLAIR和TSE-T2WI两种序列对病灶的显示能力. 结果 33例患者33处病灶,FLAIR发现病灶33处,检出率100%,TSE-T2WI发现病灶23处.检出率69.7%:在显示病灶边缘情况方面,FLAIR显示清楚者32例,T2WI显示清楚者13例.在发现病灶及显示病灶边缘方面两者差异有统计学意义(P<0.01). 结论 FLAIR技术在脊髓炎的诊断中有较高的临床应用价值.应作为本病检查的常规方法.     

MRI and proton MR spectroscopy in acute disseminated encephalomyelitis

Introduction Acute disseminated encephalomyelitis (ADEM) is one of a group of demyelinating disorders of the central nervous system (CNS). It is said to be attributed to an overshooting immunologic response following an infection or vaccination. The clinical course and type of manifestation is heterogeneous. The early application of corticosteroids has been shown to be beneficial to outcome; thus, an early diagnosis is highly desirable.Methods The potential diagnostic value of advanced MR techniques such as proton MR spectroscopy and diffusion-weighted imaging (DWI) was investigated in two paediatric patients with ADEM, one of whom had a remitting and relapsing clinical course and presented with additional cranial nerve involvement. Proton MR spectroscopy revealed typical signs of acute demyelination, such as increased macromolecules, not found in other forms of non-necrotising pathology.Conclusion The addition of proton MR spectroscopy and DWI adds to the diagnostic power of MRI in the setting of post-infectious demyelinating disorders of the CNS or ADEM and may obviate the need for biopsy.     

Transverse myelitis following mumps in an adult - a case report with MRI correlation

This is a case report of the 2nd oldest patient reported in the literature with transverse myelitis after mumps, and the 1st with magnetic resonance imaging (MRI) correlation. He is a 38-year-old Chinese man presenting with bilateral lower limb weakness and numbness, and urinary retention starting 3 weeks after an attack of mumps parotitis. Clinically, there was mild lower limb paresis, absent plantar responses and reduced pain sensation below the umbilicus. MRI revealed cord swelling and increased T₂ signal from T7 to Til. Cerebrospinal fluid showed 23 cells/mm³ and 55 mg protein/dl. He received a 5-day course of intravenous methylprednisolone 0.5 g/d. The sensory and motor deficits improved over 2 weeks; urinary symptoms improved over the next year. Transverse myelitis following mumps is recognizable clinically and radiologically, and potentially responsive to methylprednisolone.