Recurrent Myelitis Research Articles

Validation of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorders in a cohort of South Indian patients

BackgroundNeuromyelitis Optica spectrum disorders (NMOSD) are one of the most common CNS demyelinating disorders as they will present with disabling recurrent demyelinating attacks. Hence, it is of paramount importance to diagnose early, and early diagnoses and intervention will prevent further relapses associated with NMOSD. New international consensus criteria have been proposed and studies validating its application towards diagnoses of NMOSD in south Asian population are meagre. Hence we validated the proposed International Panel for NMO Diagnosis (IPND), 2015 criteria to study the clinical, demographic profile and sero-status of patients who are presenting with core clinical symptoms of NMOSD in South India and compare it with 2006 criteria. MethodsA retrospective study was conducted in a tertiary hospital for a period of one year. Patients who had at least one core clinical feature of NMOSD were included. Demographics and clinical data were recorded and analysed. Cases were evaluated using 2015 IPND and 2006 criteria for all patients, data was analysed using SPSS. ResultsA total of 110 patients were included and 91(82.72%) patients fulfilled IPND 2015 criteria. Out of 91 patients, 70 patients were AQP4 antibody positive and 21 were negative. Out of 110, only 30 (27.2%) satisfied 2006 criteria (24 or 80% were seropositive). 2015 criteria were more sensitive in identifying 61 new NMOSD cases juxtaposed to 2006 criteria, this difference was statistically significant (P<0.05). ConclusionThe 2015 IPND criteria were more sensitive and specific than previous 2006 criteria as it covered diverse clinical manifestations of NMOSD. Applying this criteria, NMOSD could be diagnosed among patients with monophasic illness, isolated recurrent optic neuritis, isolated recurrent myelitis, cerebral syndrome, diencephalic syndrome, brainstem syndrome and area postrema syndrome, thus improving the diagnostic yield.

Vanishing “Trident Sign” in Recurrent Sarcoid Myelitis Treated with Rituximab (P1.2-064)

Vanishing “Trident Sign” in Recurrent Sarcoid Myelitis Treated with Rituximab (P1.2-064)

MOG-IgG-associated disease has a stereotypical clinical course, asymptomatic visual impairment and good treatment response.

ObjectivesWe investigated the clinical characteristics and treatment response in myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease and looked for evidence of subclinical disease.MethodsWe prospectively evaluated the frequency and pattern of relapse, tested afferent visual function and monitored treatment response in 42 south Asian patients from a single centre.ResultsEighteen patients (42.9%) had monophasic and 24 (57.1%) a relapsing course. Disease duration was longer (P<0.02) in those with a relapsing course. Median time to the second attack was prolonged (P<0.04) in patients with recurrent transverse myelitis when compared with neuromyelitis optica spectrum disorder and recurrent optic neuritis. Thirteen out of 17 patients (76.5%) initially presenting with optic neuritis developed recurrent optic neuritis later. After the first attack of transverse myelitis, 17 out of 22 (77.3%) had disease confined to the spinal cord. Optical coherence tomography detected peripapillary retinal nerve fibre layer thickness (P<0.05) and macular ganglion cell complex volume (P<0.005) abnormalities in seven out of 10 (70.0%) patients without clinical optic neuritis. Immunosuppressants induced remission in 17 out of 22 (77.3%) patients during a median follow-up of 48 months and the median Expanded Disability Status Score was 1 (range 1–10).ConclusionOur study highlighted the tendency for stereotypical attacks in MOG-IgG-associated disease, heterogeneity in clinical course among subtypes, subclinical visual impairment and the need for early and sustained immunosuppressive therapy.

Cross-modal plasticity among sensory networks in neuromyelitis optica spectrum disorders

Objective: To explore resting-state (RS) functional connectivity (FC) of the main sensory/motor networks of patients with neuromyelitis optica spectrum disorders (NMOSDs), clinically isolated optic neuritis (ON), and myelitis. Methods: Clinical evaluation and RS fMRI were obtained from 28 NMOSD, 11 recurrent ON, and 12 recurrent myelitis patients and 30 healthy controls. Between-group RS FC comparisons and correlations with motor performance were assessed (SPM12) on the main sensory/motor RS networks (RSNs) identified by independent component analysis. Functional network connectivity analysis estimated inter-network connectivity. Results: Intra- and inter-network RS FCs were reduced in RSNs associated to somatosensory modalities affected by pathology: regions of the primary visual network in ON patients, of the sensorimotor networks in myelitis patients, and of the sensorimotor and secondary visual networks in NMOSD patients. The opposite trend was observed in regions of RSNs spared by pathology: the auditory and part of visual networks in NMOSD, the secondary visual and sensorimotor networks in ON, and the primary visual network in myelitis patients. Better motor performance correlated with higher RS FC of spared RSNs. Conclusion: Sensory and motor RSN abnormalities occur in NMOSD. Loss of function within disease–target networks may elicit cross-modal plasticity across sensory networks potentially preserving clinical function.

Pregnancy Outcomes in Patients with Neuromyelitis Optica: A Systematic Review [33E

INTRODUCTION: Neuromyelitis Optica (NMO) is an antibody-mediated disorder characterized by recurrent myelitis and optic neuritis. Our objective was to systematically review pregnancy outcomes in women with NMO to aid counselling and management. METHODS: We searched MEDLINE, EMBASE, Web of Science, and PubMed from 1946 until August 2016 for relevant articles. Article selection, data extraction and quality assessment (QA), using the Joanna Briggs QA tool, were performed in duplicate. Outcomes were reported as proportions. RESULTS: We identified 2096 studies and included 22 studies describing 146 pregnancies in 112 women with NMO. QA scores for case reports and series were 7/8 and 5/10 respectively. The mean maternal age was 30±4 years and diagnosis-to-pregnancy interval was 6±4 years. De novo symptoms developed in 37%. Maternal outcomes included one postpartum death, one haemorrhagic stroke, one case of respiratory paralysis and 27 relapses, most of which involved myelitis (16) and optic neuritis (6). Relapses were treated with high-dose corticosteroids (20), plasmapheresis (15), immunosuppressants (11) and intravenous immunoglobulin (6), resulting in complete (12) or partial (10) recovery in most. In two cases symptoms worsened despite treatment, requiring emergency caesarean delivery. There were 121 (83%) livebirths, 115 (95%) of which were &gt;37 weeks, 18 pregnancy terminations, five miscarriages, two stillbirths and no adverse outcomes on postnatal follow up (average 9 months). CONCLUSION: NMO is often diagnosed in pregnancy. Although women with NMO are at risk for relapses requiring aggressive management with high-dose corticosteroids, plasma exchange and immunosuppressants, retrospective data suggest that maternal and perinatal outcomes are comparable to the general population.

Are Recurrent Myelitis a Prodromic Phase of Inflammatory Diseases or a Distinct Inflammatory Condition? (P6.412)

Are Recurrent Myelitis a Prodromic Phase of Inflammatory Diseases or a Distinct Inflammatory Condition? (P6.412)

Iatrogenic Kaposi’s sarcoma following immunosuppressive treatment of the recurrent longitudinally extensive transverse myelitis

Introduction. Iatrogenic Kaposi?s sarcoma (KS) represents a multifocal, angioproliferative tumor that develops in patients undergoing immunosuppressive treatment and is considered to be induced by activation of latent human herpes virus type 8 (HHV8) infection. The aim of this report is to present a patient with iatrogenic KS due to immunosuppressive treatment. Case outline. We present a 69-year-old male non-HIV patient, previously treated for anti-aquaporin-4 antibody negative recurrent longitudinal extensive transverse myelitis with prednisolone and azathioprine for one year. The patient developed bluish and violet plaques and nodules on his face, trunk, and extremities. Skin biopsy findings (histopathology and immunohistochemical detection of CD31 expression and anti-HHV8 antibodies in the spindle cells) confirmed the diagnosis of KS. The reduction of immunosuppression and topical treatment with imiquimod resulted in a partial but significant regression of skin lesions, but the patient had another relapse of myelitis following the cessation of azathioprine and a reduction in the dose of prednisolone. Conclusion. To the best of our knowledge, this is the first case of an inflammatory and demyelinating central nervous system disease treated with corticosteroids and azathioprine that was associated with iatrogenic KS. The efficient treatment of both conditions is highly challenging and can be troublesome in specific cases.

Study of Knowledge, Attitude, and Practice in Participants with Regular Intake of Lathyrus, But No Spastic Paraparesis.

Background and Purpose:Neurolathyrism is now a disease of the past and also the causative agent, Lathyrus sativus (Khesari Pulse or keerai in Local Hindi and Bhojpuri language) has now been proven to be harmless and has become part of the usual diet.Materials and Methods:The population at risk was screened and studied for demography, economic status, knowledge about Khesari pulse, awareness about so-called harmful effects of Khesari pulse intake in humans, and the effects of ban of Khesari pulse on the population which is using Lathyrus (Khesari) as a major source of pulse since the past 4 years through a questionnaire of three pages.Results:Nearly 97% of total screened population totally fed on Khesari pulse as their major source of food and we did not found a single case of primary walking difficulty. We did find three cases of poststroke paralysis, a case of post-GBS lower limb weakness, and a case of recurrent myelitis as a part of questionnaire-based study and then followed by personally examining the patients to confirm the diagnosis.Conclusion:Khesari pulse if consumed in smaller quantities as a part of a normal mixed diet, its nutritional values can be optimally utilized.

Poster 192: Recurrent Transverse Myelitis with Underlying Neuromyelitis Optica Triggered by West Nile Virus Infection: A Case Report.

Poster 192: Recurrent Transverse Myelitis with Underlying Neuromyelitis Optica Triggered by West Nile Virus Infection: A Case Report.

Different patterns of structural damage in neuromyelitis optica spectrum disorders, recurrent optic neuritis and recurrent myelitis (P2.331)

Different patterns of structural damage in neuromyelitis optica spectrum disorders, recurrent optic neuritis and recurrent myelitis (P2.331)

Treatment of neuromyelitis optica and neuromyelitis optica spectrum disorders with rituximab using a maintenance treatment regimen and close CD19 B cell monitoring. A six-year follow-up

Neuromyelitis optinca (NMO) represents a serious demyelinating disease of the central nervous system selectively attacking the spinal cord and optic nerve. Early differential diagnosis from multiple sclerosis is of vital importance, as NMO mandates immunosuppressive and not immunomodulatory treatment. Rituximab has been recently introduced as a treatment option for NMO. However, optimal surrogate measures and treatment intervals are still unclear.Five patients (females, mean age 54±10.21years) with NMO and NMO spectrum disorders (NMOSD) were evaluated with respect to disability and relapse rate. All patients were found positive for NMO IgG. All patients (three with NMO and two with NMOSD, 1 patient with recurrent optic neuritis and 1 patient with recurrent myelitis) had received rituximab treatment for six years. One patient with NMOSD received cyclophosphamide prior to rituximab while two were misdiagnosed as multiple sclerosis and had received interferon treatment. All received rituximab infusion of 375mg/m2 once per week for 4weeks and then every two months for the first two years and then every six months. B-cell counts were measured every two months and were kept in almost undetectable levels. No relapse was noted during the treatment period while EDSS score was improved in all patients. No severe adverse effects occurred during RTX treatment.Rituximab treatment on NMO and NMOSD patients showed significant improvement in disability and relapse-rate without any significant adverse effects.

Concurrent Demyelinating Polyneuropathy Associated with Recurrent Cervical Myelitis

Concurrent Demyelinating Polyneuropathy Associated with Recurrent Cervical Myelitis

Antibodies in acquired demyelinating disorders in children

The discovery that several demyelinating disorders in children may be associated with autoantibodies to astrocytes, myelin, and/or synaptic proteins has opened the possibility of their use as diagnostic and prognostic biomarkers. The identification of aquaporin 4 (AQP4) antibodies as specific diagnostic markers of neuromyelitis optica (NMO) led to an expansion of the clinical spectrum of this disorder to include patients with incomplete forms or with manifestations outside of the optic nerves and spinal cord. Recently, an international panel of experts proposed the use of the term NMO spectrum disorders (NMOSD) to encompass all of these phenotypes. Although rare in children, the early identification of AQP4 antibodies has important prognostic and therapeutic implications as their presence is highly predictive of relapses and accumulation of disability. More recently, antibodies to myelin oligodendrocyte glycoprotein (MOG) have been described in children and adults diagnosed with NMOSD and seronegative for AQP4 antibodies, as well as in children with other acquired demyelinating disorders. For example 60 % of children with acute demyelinating encephalomyelitis (ADEM) have MOG antibodies. Whether MOG antibodies are pathogenic or surrogate biomarkers of the disease is unclear but their identification is important for two reasons. First, patients with a first demyelinating event who are MOG antibody positive are unlikely to develop multiple sclerosis (MS); and second, the long-term persistence of MOG antibodies associates with recurrent non-MS demyelinating disorders such as NMOSD, recurrent optic neuritis or transverse myelitis, or multiphasic ADEM. Other antibodies, such as those targeting Kir 4.1 or the glycine receptor have also been described in association with pediatric MS and other demyelinating disorders but their clinical relevance, and existence, in the case of Kir 4.1, is still under investigation. The focus of this review will be the current data on the antibodies mentioned above, and the recent discovery that some children have both a demyelinating disorder and autoimmune encephalitis. The presence of antibodies against targets related to demyelinating disorders (AQP4 or MOG) and those related to autoimmune encephalitis (e.g., N-methyl-D-aspartate receptor), supports the concurrence of two autoimmune disorders in these patients.

TRANSVERSE MYELITIS IN A MYASTHENIA GRAVIS PATIENT. A CASE PRESENTATION

Myasthenia gravis (MG) is an autoimmune disease caused by the presence of immunoglobulin G (IgG)1 and IgG3 complement activating antibodies against the nicotinic acetylcholine receptor, which affects the neuromuscular junction leading to fluctuating muscle weakness due to impaired neuromuscular transmission. The association of MG and demyelinating diseases is rare, but it has been described before and it could be part of an unspecific immune activation, due to genetic susceptibility or it could just happen randomly. Demyelinating diseases (DD) in MG patients can occur as monophasic events (myelitis, optic neuritis, acute disseminated encephalomyelitis) or recurrent diseases (multiple sclerosis, recurrent transverse myelitis) and since the incidence of DD is higher in MG patients than in general population this association could be part of an autoimmune syndrome or genetically induced. We present the case of a 30 year old woman who presented with an unspecific onset of MG and after 5 months was readmitted to our unit with transverse myelitis (with negative aquaporin 4 antibodies) which regressed after 5 days of combined intravenous corticotherapy and immunoglobulin treatment.

Hypothalamic abnormality in patients with inflammatory demyelinating disorders

Background: Hypothalamic lesions in neuromyelitis optica (NMO) patients might be more specific for NMO than multiple sclerosis (MS). However, this is controversial. Objective: To characterize clinical features of patients with inflammatory demyelinating disorders (IDDs) with visible hypothalamic lesions using magnetic resonance imaging (MRI). Methods: Patients with IDDs (n = 429) were recruited retrospectively. Results: Of 52 patients with hypothalamic images enrolled, 42 were positive for aquaporin-4 (AQP4) antibodies, including 28 patients with NMO, 6 with recurrent transverse myelitis, 3 with recurrent optic neuritis, and 5 with brainstem and brain syndrome. The remaining 10 patients were anti-AQP4-negative, including 3 with MS, 3 with acute disseminated encephalomyelitis, and 4 with other disorders. In the AQP4-positive group, manifestations, including ataxia, intractable hiccup and nausea, syndrome of inappropriate antidiuretic hormone secretion and encephalopathy were more frequent in those with hypothalamic lesions than those without. Cell counts of cerebrospinal fluid in patients with hypothalamic lesions differed from patients without lesions. Brain MRI abnormalities were more frequent in brainstem and hemisphere of the hypothalamic lesion group. Conclusions: Hypothalamic lesions were observed frequently in patients with AQP4 antibodies. Clinical manifestations and paraclinical features in AQP4-positive patients with hypothalamic lesions differed from those without lesions.

Recurrent myelitis with normal imagem as an unusual systemic lupus erythematous (SLE) -probable first manifestation

Recurrent myelitis with normal imagem as an unusual systemic lupus erythematous (SLE) -probable first manifestation

Modified retrograde femoral anterolateral flap with a dual blood supply for complex destruction wound in calf

Objective To evaluate the clinical efficacy of retrograde femoral anterolateral skin flap with a dual blood supply for repairing complex destruction wound in calf. Methods From July 2011 to March 2014, 6 patients with destructive wound in calf were treated by modified retrograde femoral anterolateral flaps with a dual blood supply. The patients aged 25-56 years consisted of 4 males and 2 females. There were 4 patients with traffic injury and 2 machine crush injury. Wound ranged from 8 cm×10 cm to 10 cm×26 cm. Pedicle retrograde femoral anterolateral flap supplied by high cutaneous branches was harvested to cover the wound. Based on the principle ofbooster, flap blood flow was increased by anastomosis between lateral femoral circumflex artery and calf arteriovenous after 180 ° rotation of the skin flap. Results Flaps all survived. Markedly swollen flap was seen in 2 patients postoperatively, which impeded venous return, but significantly improved after stitches removal on one side of the flap. One-stage healing occurred in 3 patients. Good wound healing was noted in 2 patients while swelling subsided and secondary suture was done. Postoperative infection occurred in 1 patient and after debridement and dressing, the wound was finally healed. Two patients had scalded skin flap due to the careless two months after operation and healed after dressing. Follow-up lasted for 6 to 22 months with no delayed recurrent infection, myelitis and bone nonunion. Conclusion Retrograde femoral anterolateral flap with a dual blood supply may be a good option for wound repair of severe calf injury because of its normal appearance, good texture, long vascular pedicle, large surface area, high survival rate, improved flap blood supply and strong resistance to infection. Key words: Soft tissue injuries; Surgical flaps; Calf

Transverse Myelitis Associated With an Itchy Rash and HyperCKemia

Neuromyelitis optica is associated with severe neurodisability if not recognized and treated promptly. Several autoimmune disorders are associated with this condition and may vary in their presentation. It is essential that clinicians are aware of the uncommon presenting features of neuromyelitis optica and associated autoimmune conditions. A 53-year-old woman presented with nausea and vomiting and was noted to have an asymptomatic elevated creatinine kinase level, which improved with conservative management. She had a history of iron-deficiency anemia due to long-standing celiac disease that was managed with a gluten-free diet. She then presented with recurrent transverse myelitis and a vesicobullous rash over her arms and feet that was pruritic and excoriating. Skin biopsy results confirmed a clinical diagnosis of dermatitis herpetiformis and antibody test findings against aquaporin-4 were positive, leading to a diagnosis of neuromyelitis optica spectrum disorder. She was treated with methylprednisolone sodium succinate, plasma exchange, and azathioprine and has remained in remission. This report highlights the association of neuromyelitis optica with dermatitis herpetiformis, which can present even without clinical features of celiac disease. Nausea, vomiting, and asymptomatic hyperCKemia should be recognized as rare presenting features of neuromyelitis optica.

Antineutrophil Cytoplasmic Antibodies in Patients with Idiopathic Inflammatory-Demyelinating Diseases

Objective: We assessed the clinical significance of antineutrophil cytoplasm antibodies (ANCA) in patients with idiopathic inflammatory-demyelinating disease (IIDD). Methods: A consecutive cohort of 269 subjects with IIDD and 595 controls was analyzed retrospectively. Results: Among all subjects, ANCA positivity rates were low [9.5% in a perinuclear pattern (pANCA) and 2.3% in a cytoplasmic pattern (cANCA)]. One of the 117 patients with multiple sclerosis (MS) had cANCA and 2 had pANCA. Ten patients with neuromyelitis optica (NMO; 13.9%) had pANCA and 3 (4.2%) had cANCA. Four patients with recurrent longitudinal extensive transverse myelitis (RLETM; 19.0%) had pANCA and 1 (4.8%) had cANCA. In monophasic TM, 22.9% were pANCA seropositive. Among patients with brainstem syndromes, 14.3% were pANCA seropositive. Patients with NMO, RLETM or monophasic TM had higher pANCA levels than patients with MS. There was a positive association between spinal cord (SC) lesions and ANCA, and especially between longitudinal extensive transverse myelitis and ANCA. Among anti-aquaporin 4 antibody-positive patients, ANCA-positive patients (n = 16) were older and had higher Expanded Disability Status Scale scores, more antinuclear antibodies, longer SC lesions and fewer brain abnormalities than the ANCA-negative patients (n = 68). In the NMO subgroup, ANCA-positive patients were older and had more antinuclear antibodies and longer SC lesions than ANCA-negative patients. Conclusion: Among the IIDDs, we found a higher occurrence of ANCA in patients with NMO spectrum disorders than in patients with MS. Therefore, ANCA is another interesting marker of autoimmunity in IIDD patients, especially those with anti-aquaporin 4 antibody.

A clinical and radiological profile of neuromyelitis optica and spectrum disorders in an Indian cohort.

Background:There is insufficient data on the clinical and radiological features of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) from India.Objective:The objective of the following study is to examine the clinico-radiological features of NMO and NMOSD in an Indian cohort.Materials and Methods:This retrospective study included 44 consecutive patients who (1) satisfied the 2006 Wingerchuk criteria for NMO (16 seropositive and 7 seronegative); or (2) had isolated or recurrent optic neuritis (ON) with seropositivity (n = 4); or (3) had isolated or recurrent myelitis with seropositivity (n = 17).Results:The female:male ratio was 7.8:1 with median age of onset 26.5 (range 8-72). Annualized relapse rate (ARR) was comparable across all groups (F [3, 40] = 0.938 and P = 0.431). Various presentations other than ON and myelitis were noted. All 40 patients with myelitis had spinal cord lesions involving ≥3 vertebral segments during the course of the disease. Cervicomedullary involvement was seen in 32.5% (13/40) patients. Brain magnetic resonance imaging was available for 40 patients; eight of these (20%) had brain lesions in locations described in multiple sclerosis (MS), 27.5% (11/40) had lesions at sites unusual for MS and 52.5% (21/40) had normal brain imaging.Conclusion:NMO and NMOSD patients in this cohort have comparable ARR regardless of clinical presentation, supporting the emerging trend of treating all patients with immunotherapeutic agents at an early stage. Varied presentations seen in NMO and NMOSD highlight the need for a high index of suspicion for NMO in demyelinating episodes not classical for MS.