Transverse Myelitis in Patients with Antiphospholipid Antibodies – The Importance of Early Diagnosis and Treatment

Transverse myelitis (TM) is a rare manifestation of systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). No uniform therapeutic protocol exists for its treatment, and the prognosis is usually poor. Here we describe four patients having TM associated with antiphospholipid antibodies. Treatment measures and delay in diagnosis between symptom onset and the initiation of treatment varied between patients, but the earlier the diagnosis and the more aggressive the treatment the better was the patient’s outcome. Based on these cases and on a literature review we suggest that early aggressive treatment (usually with pulses of methylprednisolone and cyclophosphamide) might improve the prognosis of patients with TM associated with antiphospholipid antibodies. Received: 13 March 2001 / Accepted: 3 October 2001     

Occipital neuralgia as an isolated symptom of C2 myelitis

Occipital neuralgia has been attributed to lesions at a peripheral nerve or radicular level. On rare occasions, it has been associated with cervical cord lesions. We report a 55-year-old woman who presented with an isolated occipital neuralgia and was found on further investigation to have a restricted, isolated myelitis at C2 level. This represents the second reported case of occipital neuralgia due to C2 myelitis and should alert clinicians to considering cervical MRI in patients with occipital neuralgia.     

Aseptic meningo–radiculo–encephalitis presenting initially with urinary retention

We report three male patients with aseptic meningoencephalo– radiculitis presenting with acute urinary retention.Viral antibody titers for herpes types I and II and the PCR studies were negative. The cerebrospinal fluid revealed elevated myelin basic protein.The serum antibodies against a panel of gangliosides, some of which are known to be associated with acquired demyelinating neuropathies, were all negative.The magnetic resonance imaging (MRI) studies revealed spotty T2 high intensities in the basal ganglia, thalamus and brainstem in two patients. In one patient,meningeal gadolinium enhancement of the conus and cauda equina of the spinal cord was recognized. On urodynamic studies, all patients showed features of atonic bladder with or without detrusor hyperactivity. They were treated conservatively without using steroids or immunoglobulins, and made a remarkable functional recovery with the disappearance of abnormal MRI findings.However, all three were left with erectile dysfunction, and two continued to use self–intermittent catheterization at more than 3–year follow–up.There was no recurrence of symptoms. The underlying causes remain unclear, though they may represent a variant of acute disseminated encephalomyelitis.     

甲泼尼龙冲击治疗急性脊髓炎疗效分析

目的观察甲泼尼龙冲击疗法（MPPT）治疗急性脊髓炎的疗效。方法对16例急性脊髓炎患者应用甲泼尼龙冲击治疗,另对15例使用地塞米松常规治疗,对比疗效。结果 MPPT组明显优于对照组（P〈0.05）。结论 MPPT是治疗急性脊髓炎快速而安全有效的方法。     

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.     

Acute transverse myelitis with normal brain MRI

Objective To investigate the long-term risk of developing MS in patients presenting with acute transverse myelitis (ATM) and normal brain MRI scans at onset. Methods We studied 58 ATM patients with normal brain MRI at presentation for up to 5 years with serial neurologic and imaging studies. All patients underwent CSF analysis at onset which was defined positive if two or more IgG oligoclonal bands and/or elevated IgG index were present. Brain and spinal cord MRI scans were obtained every 6 months for the first 2 years, and annually thereafter unless the patient experienced a second neurologic attack different from the initial episode to confirm CDMS or there was demonstration of MRI lesions confirming dissemination in time and space to fulfill McDonald imaging criteria to diagnose MS. Results Seventeen of 58 (29%) patients developed MS of which 7 (41%) patients developed CDMS and 10 (59%) developed MS using McDonald Imaging Criteria. Mean time to CDMS by a second clinical attack was 11. 1 months compared to 19. 2 months by MRI lesions (P = 0. 03). None of the patients developed MS after 24 months of onset. All 17 patients who developed MS had positive CSF although 15 patients who had positive CSF did not develop MS during the 5 years of follow-up. Conclusions The majority of patients with ATM and normal brain MRI do not develop MS after 5 years of follow-up confirming the relatively low risk compared to patients with abnormal brain MRI scans. CSF is helpful in distinguishing patients more likely to develop MS. Compared to clinical attacks, serial imaging may not lead to an earlier diagnosis in ATM patients with normal brain MRI.     

单唾液酸四己糖神经节苷脂治疗急性脊髓炎的临床研究

目的评价单唾液酸四己糖神经节苷脂（GM-1）对急性脊髓炎的治疗作用。方法将50例急性脊髓炎患者分为治疗组35例和对照组15例,对照组采用甲基强的松龙及丙种球蛋白治疗,治疗组在对照组治疗基础上加用GM-1,治疗后观察两组患者的脊髓神经功能恢复程度并比较治疗前后核磁共振（MRI）检查结果。结果治疗组患者神经功能恢复程度明显优于对照组（P〈0．01）,MRI检查结果亦明显优于对照组。结论治疗急性脊髓炎时加用GM-1能显著提高患者的神经功能恢复程度。     

Revised diagnostic criteria for neuromyelitis optica (NMO)

We analyzed neuromyelitis optica (NMO) IgG in the serum or CSF samples from 46 patients with suspected NMO (28) and limited forms of NMO (18). One hundred and fifteen samples from multiple sclerosis (MS) patients were included as controls. The final clinical diagnosis after follow-up was 16 NMO, 12 MS, 11 transverse myelitis (TM) and seven recurrent optic neuritis (RON). NMO-IgG was detected in 62.5% of NMO, 50% of the recurrent longitudinally extensive TM, 14.3% of the RON but in none of the MS patients. The authors then compared the newly revised diagnostic criteria for NMO with the criteria published in 1999, in the 28 patients with suspected NMO. The revised criteria had higher specificity, and positive and negative predictive value (83.3% vs. 25%; 87.5% vs. 62.5; 83.3% vs. 75%), but slightly lower sensitivity (87.5% vs. 93.7%). Our study confirms NMO-IgG as a highly specific marker of NMO, and the usefulness of the revised diagnostic criteria in predicting a diagnosis of NMO.     

Longitudinally extensive transverse myelitis and Hepatitis C—a case report and literature review

Tractopathy lesions in the spinal cord associated with HCV infection, which normalized on MRI after antiviral treatment, are described. These specific MRI findings can be used in the diagnosis and treatment of secondary causes of transverse myelitis.