Recurrent Myelitis Research Articles

Nineteen Episodes of Recurrent Myelitis in a Woman With Neuromyelitis Optica and Systemic Lupus Erythematosus

To describe the case of a patient with systemic lupus erythematosus (SLE) and neuromyelitis optica (NMO) who experienced 19 recurrent attacks of myelitis. Case report. An outpatient neurorheumatology clinic at the Johns Hopkins Hospital devoted to care of patients with neurological manifestation of rheumatic diseases. Patient A woman with NMO and SLE. Intervention Rituximab therapy. Clinical and neuroimaging features of relapsing disease. Recurrent and increasingly severe myelitis attacks still occurred after treatment with rituximab. It may be progressively more difficult to prevent relapses and commensurate disability in patients with later stages of relapsing NMO. Recognition of NMO as a distinct diagnostic entity in patients with SLE and other rheumatic diseases is crucial, in that institution of earlier targeted immunosuppressant treatment may be more effective than later targeted immunosuppression. The cellular arm of the immune system may be recruited by pathogenic B cells and may explain why relapses may occur after treatment with B cell-depleting therapy.

Spinal cord biopsy findings of anti‐aquaporin‐4 antibody‐negative recurrent longitudinal myelitis in a patient with sicca symptoms and hepatitis C viral infection

We describe the pathological features of a spinal cord biopsy from a 69-year-old woman with anti-aquaporin-4 antibody-negative recurrent longitudinal myelitis. Spinal cord MRI showed T2 high-intensity lesions with strong gadolinium enhancement, when episodes of sensory-motor impairment were repeated. The radiological abnormality was corrected by corticosteroid administration, but improvement of the symptoms was minimal. Although the patient had sicca symptoms and fulfilled four of the diagnostic criteria for Sjögren syndrome, the diagnosis was excluded, because of infection with hepatitis C virus, an exclusion criterion of Sjögren syndrome. In the spinal cord lesions, necrotic changes affected both myelin and axons. Infiltrating lymphocytes were predominantly T-cells. The proliferation of small vessels with hyalinization and concomitant occlusive change was remarkable. These pathological findings resembled those previously reported in Sjögren syndrome. Ultrastructurally, the endothelial cells of the small vessels showed features of activated cells and contained vesiculo-tubular structures in the cytoplasm, indicating that increased blood-brain barrier (BBB) permeability might contribute to pathogenesis. We speculated that increased BBB permeability and T-cell entry in the spinal parenchyma triggered pathological reactions resulting in necrotic changes in the spinal cord. Obstruction of small vessels might add ischemic damage to the lesions. The clinical course and pathological findings indicated that damage progressed rapidly in the spinal cord and was irreversible. The lesions apparently differed from typical demyelination plaques. Faced with such spinal cord lesions, a preventive therapeutic approach is necessary to avoid attack-associated disability.

Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese

Background Antibodies to aquaporin-4 (AQP4) are found in a fraction of Japanese opticospinal multiple sclerosis (OSMS) patients. However, it remains unknown whether anti-AQP4 antibody-positive and negative OSMS patients possess an identical disease. Objective The objective of the current study was to clarify immunological differences between the two groups of patients. Methods We studied the serum antibody titers against AQP4 in 191 patients with idiopathic central nervous system demyelinating diseases and clarified their relationships with immunological parameters. Results Anti-AQP4 antibody positivity rate was higher in patients with OSMS (21/58, 36.2%), idiopathic recurrent myelitis (4/17, 23.5%), and recurrent optic neuritis (7/26, 26.9%), than in conventional MS (CMS) patients (6/90, 6.7%) and patients with other diseases (0/87). Anti-AQP4 antibody titer was significantly higher in patients with SS-A/B antibodies than in those without them. Anti-AQP4 antibody-negative OSMS patients showed significantly higher CD4+IFN-γ+IL-4−T cell percentages and intracellular IFN-γ/IL-4 ratios than anti-AQP4 antibody-positive patients, anti-AQP4 antibody-negative CMS patients, and healthy controls, and CD4+IFN-γ+IL-4−T cell percentages were negatively correlated with anti-AQP4 antibody titers. Conclusion Anti-AQP4 antibody-positive patients are immunologically distinct from anti-AQP4 antibody-negative OSMS patients owing to a Th2 shift in the former group in comparison to a Th1 shift in the latter.

MRI of the spinal cord in neuromyelitis optica and recurrent longitudinal extensive myelitis

Neuromyelitis optica (NMO) is a severe inflammatory and necrotizing disease that clinically affects the optic nerves and spinal cord in a relapsing course. We assessed the baseline and follow-up MRI characteristics of cord attacks in NMO and recurrent longitudinal extensive myelitis (RLEM). We retrospectively reviewed MRI data of 20 Afro-Caribbean patients diagnosed with either NMO or RLEM. MRI data from 51 cord or mixed attacks were evaluated, and 65 follow-up MRI studies were available for 30 baseline acute examinations. The cervical cord was involved in 63% of cases. Four attacks were limited to the brainstem. MRI of the spinal cord revealed longitudinal extensive signal abnormalities extending over three vertebral segments, associated with cord swelling in 67% of the 51 relapses. Gadolinium enhancement was observed, preferentially surrounding edema, in 69% of attacks. In the axial plane, signal abnormalities typically involved central areas of the cord. Cavitation was observed in 16% of attacks. Cord attacks recurred in the same or contiguous areas in 67% of cases. Follow-up MRI revealed a gradual decrease in cord swelling and T2 signal hyperintensity, with fragmentation of signal abnormalities in some cases. Cord atrophy was evident in 57% of the follow-up MRI. Given the poor prognosis of NMO and RLEM, radiologists need to be aware of the MRI pattern to prevent further attacks with the use of aggressive treatment.

Modification of Multiple Sclerosis Phenotypes by African Ancestry at HLA.

In those with multiple sclerosis (MS), African American individuals have a more severe disease course, an older age at onset, and more often have clinical manifestations restricted to the optic nerves and spinal cord (opticospinal MS) than white persons. To determine whether genetic variation influences clinical MS patterns. Retrospective multicenter cohort study. Six hundred seventy-three African American and 717 white patients with MS. Patients with MS were genotyped for HLA-DRB1 and HLA-DQB1 alleles. The proportion of European ancestry at HLA was estimated by genotyping single-nucleotide polymorphisms with known significant frequency differences in West African and European populations. These genotypes were correlated with the opticospinal disease phenotype, disability measures, and age at onset. Subjects with DRB1*15 alleles were twice as likely to have typical MS rather than opticospinal MS (P = .001). Of the subjects with opticospinal MS or a history of recurrent transverse myelitis who were seropositive for anti-aquaporin 4 antibodies (approximately 5%), none carried DRB1*15 alleles (P = .008). Independently of DRB1*15, African ancestry at HLA correlated with disability as measured by the Multiple Sclerosis Severity Score (P < .001) and risk of cane dependency (hazard ratio, 1.36; P < .001); DRB1*15 alleles were associated with a 2.1-year earlier age at onset (P < .001). These data indicate that the role of HLA in MS is not limited to disease susceptibility but that genes embedded in this locus also influence clinical outcomes.

Association of the HLA‐DPB1*0501 allele with anti‐aquaporin‐4 antibody positivity in Japanese patients with idiopathic central nervous system demyelinating disorders

There are two subtypes of multiple sclerosis (MS) in Asians: the opticospinal (OSMS) form that shows a selective involvement of the optic nerve and the spinal cord and the conventional (CMS) form that has disseminated lesions in the central nervous system including the cerebrum, cerebellum and brainstem. Both show distinct human leukocyte antigen (HLA) class II associations. OSMS has similar features to the relapsing form of neuromyelitis optica (NMO) in Western populations. Recently, it was shown that antibodies to aquaporin-4 (AQP4) are specifically detected in NMO patients and in some Japanese patients with OSMS or recurrent optic neuritis or myelitis. To clarify the immunogenetic background of anti-AQP4 antibody production, we studied HLA-DRB1 and -DPB1 gene polymorphisms in anti-AQP4 antibody-positive and -negative patients with idiopathic demyelinating diseases, such as MS, recurrent optic neuritis and recurrent myelitis. The phenotypic frequency of the HLA-DPB1*0501 allele was significantly increased in anti-AQP4 antibody-positive patients (89.5%, odds ratio = 4.8; 95% confidence interval = 1.6-14.3, n = 38, P(corr) = 0.032) compared with controls (64.0%, n = 125) but not in either anti-AQP4 antibody-negative OSMS (75.0%, n = 32) or CMS (69.2%, n = 52) patients. There was no significant correlation between any HLA-DRB1 allele and the existence of anti-AQP4 antibody. These findings suggest that the emergence of anti-AQP4 antibody is reinforced by the presence of the HLA-DPB1*0501 allele in Japanese.

Transverse myelitis spectrum disorders

Acute transverse myelitis (ATM) is an inflammatory demyelinating disorder that affects the spinal cord focally resulting in motor sensory and autonomic dysfunction. Establishing the diagnosis of ATM is not as difficult as determining the possible etiology. There is a difference in the perception of ATM seen in the West as compared to developing countries. In the West multiple sclerosis (MS) is the most common inflammatory disorder of the central nervous system. An attack of ATM may be the beginning of MS. However, this may not be the case in developing countries where MS is uncommon. Most often transverse myelitis is monophasic and at best represents a site-restricted form of acute disseminated encephalomyelitis (ADEM). Traditionally the combination of optic neuritis and ATM, occurring as a monophasic illness would have been called as neuromyelitis optica (NMO). Changing concepts in the definition of NMO and the discovery of a biomarker, neuromyelitis optica immunoglobulin (NMO_IgG), has changed the way relapsing autoimmune disorders are being perceived currently. A variety of idiopathic inflammatory disorders such as Japanese form of optic spinal MS, recurrent myelitis, and recurrent optic neuritis have been brought under the umbrella of neuromyelitis spectrum disorders because of the association with NMO-IgG. Complete transverse myelitis accompanied by longitudinally extensive transverse myelitis which is seronegative for this biomarker has also been reported from several countries including Japan, Australia, and India. Thus, ATM is a heterogeneous disorder with a varied clinical spectrum, etiology, and outcome.

Neuromyelitis optica: Diagnosis, pathogenesis, and treatment

Although the co-occurrence of myelitis and optic neuritis that characterizes neuromyelitis optica (NMO) was recognized over a century ago, distinguishing NMO from multiple sclerosis relied solely on clinical criteria until recently. The identification of a biomarker that has high specificity for NMO is clinically useful for distinguishing NMO from multiple sclerosis and identifying patients at high risk for recurrent myelitis and optic neuritis. That fact that the biomarker is an autoantibody that recognizes aquaporin 4 (AQP4), a water channel expressed on astrocyte podocytes, has substantially contributed to the hypothesis that NMO is a humorally mediated autoimmune disease. This review discusses the discovery of the NMO-IgG biomarker, the identification of AQP4 as its target, the clinical applications of these advances, the pathologic implications for the anti-AQP4 antibody, and advances in NMO treatment.

Optic neuritis and recurrent myelitis in a woman with systemic lupus erythematosus

A 38-year-old woman with systemic lupus erythematosus presented with headaches and bilateral hearing loss. Brain MRI was initially suggestive of small-vessel disease developing in the context of neuropsychiatric systemic lupus erythematosus. Several months later, the patient developed optic neuritis, followed by recurrent attacks of myelitis. MRI of the spine revealed multifocal regions of myelitis affecting the cervical spine. Serological evaluation revealed the presence of neuromyelitis optica-IgG antibodies. MRI of the brain was nondiagnostic for multiple sclerosis. Recurrent myelitis and optic neuritis, occurring in the context of neuromyelitis optica (also known as Devic's syndrome). The patient had recurrent attacks of myelitis despite treatment with pulse cyclophosphamide. After initiation of rituximab, the patient experienced symptomatic improvement and had no further attacks of opticospinal disease.

Primary Sjögren's Syndrome Associated with Recurrent Transverse Myelitis

Sjogren’s syndrome is a chronic autoimmune disorder due to lymphocytes infiltrations of the exocrine glands and is clinically characterized by dry eyes and dry mouth. Though Sjogren’s syndrome can affect the nervous system, acute transverse myelitis is an extremely rare CNS complication that progresses rapidly, and requires early diagnosis and therapy. Here, we report a 49-year-old female who had primary Sjogren’s syndrome that manifested itself as acute transverse myelitis. She presented with motor weakness and hypesthesia of her right lower extremity. During her first attack, MRI of the spine revealed high signal intensities on T2-weighted images in her spinal cord at the levels of C6 through T11. Symptoms improved after 5 days of intravenous methylprednisolone (1 g/day) therapy, but recurred 9 months later. She had another MRI which showed acute transverse myelitis involving the spinal cord at the levels of C2 to T8. Her symptoms remained well controlled with treatment consisting of 5 days of methylpredisolone infusion (1 g/day) and concomitant intravenous cyclophosphamide 750 mg every 4 weeks.

Poster 281: Arteriovenous Malformation Presenting as Transverse Myelitis: A Case Report

Setting: Acute inpatient rehabilitation hospital. Patient: A 59-year-old man with recurrent transverse myelitis. Case Description: Symptoms began acutely with leg weakness. Magnetic resonance imaging (MRI) revealed abnormal signal intensity from T8 to the conus. Additional workup was negative and treatment included steroids for a diagnosis of transverse myelitis. Following rehabilitation, he was community ambulating with a cane. 2 months later, neurologic progression led to treatment for a urinary tract infection; on discharge from rehabilitation he was ambulating using a walker. 3 months later, he again developed progressive weakness and was diagnosed with exacerbation of transverse myelitis secondary to a sinus infection. Additional workup included MRI and magnetic resonance angiography and led to a diagnosis of arteriovenous malformation (AVM). Ultimately, he underwent spinal angiography and surgical repair of the thoracic AVM. He was discharged to rehabilitation using a wheelchair and has not returned to independent ambulation. Assessment/Results: During his 18-month course, he suffered multiple flairs of lower-extremity weakness and varied neurologic deficit. Following surgical repair of the AVM, his motor deficit remains stable although long-term prognosis for motor improvement is currently uncertain. Discussion: Several case reports of spinal AVMs are noted in the literature. This case displays a varying and protracted course with multiple diagnosis of transverse myelitis. Once the diagnosis was made, surgical correction was performed. However, the paucity of recovery following surgical correction may be in part due to the time elapsed between onset and diagnosis, thus delaying definitive treatment, and serves to support a high index of suspicion for a spinal AVM in such cases. Conclusions: AVMs are an uncommon, but not rare, cause of neurologic deficit. As such, they should be included in the differential for neurologic deficit without definitive etiology, especially in cases with recurrent episodes.

Occurrence of CNS demyelinating disease in patients with myasthenia gravis

An increased incidence of multiple sclerosis (MS) has been reported in patients with myasthenia gravis (MG). We reviewed records of 214 patients with MG. CNS demyelinating disease (CNSDD) occurred in five patients (2.3%). CNSDD always occurred after the diagnosis of MG. Myelitis (including recurrent myelitis) was the most common CNS manifestation. All patients had antinuclear antibodies. CNSDD occurs more frequently in patients with MG and may be related to immune-modulating treatments including thymectomy.

Idiopathic inflammatory-demyelinating diseases of the central nervous system

Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Baló's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures.

Severe recurrent myelitis in patients with hepatitis C virus infection

<h3>Background and importance</h3> Alemtuzumab is a humanised monoclonal antibody that selectively targets CD52, indicated in adult patients with relapsing–remitting multiple sclerosis (RRMS). <h3>Aim and objectives</h3> To assess the effectiveness and safety of alemtuzumab for RRMS in the clinical setting. <h3>Material and methods</h3> A retrospective observational study of all patients with RRMS treated with at least one course of alemtuzumab from July 2016 to March 2019 was carried out. The drug was administered by intravenous infusion on 5 consecutive days at baseline and on 3 consecutive days 12 months later. All patients received prophylaxis with methylprednisolone, antihistamines, antipyretics and acyclovir. Alemtuzumab was started in adults with active disease, defined by clinical or imaging features despite the use of immunomodulating therapies, or having a fast and aggressive course. The variables studied were sex, age, time from diagnosis, expanded disability status scale (EDSS), previous treatment, number of cycles, adverse events (AE) and number of relapses post-treatment (NRPT). Data were collected from medical records and the electronic prescription programme. Effectiveness was evaluated in terms of NRPT with alemtuzumab. Safety was assessed by reported treatment of AE. <h3>Results</h3> Eleven patients, 63.6% women, mean age 38 (24–54) years, were included. Median time from RRMS diagnosis was 10 (4–20) years and mean baseline EDSS was 3.5 (2–5.5). Patients were previously treated with a median of 3 (2–4) drugs: interferon beta-1a (IFNβ-1a) intramuscularly (45.5%), IFNβ-1a subcutaneously (27.3%), glatiramer acetate (27.3%), natalizumab (90.9%), fingolimod (27.3%) and dimethyl fumarate (18.2%). Seven patients completed two courses of alemtuzumab, and the second course is pending in three patients. One administration was suspended due to an infusion related reaction (IRR), requiring intensive care. The mean relapse rate was 0.36 (0–2). All patients experienced IRRs: lymphopenia (63.6%) and skin disorders (72.7%). Most were mild and limited in time, except for one patient with skin rash, pruritus and oedema, requiring discontinuation of treatment. Other AE were urinary tract infection (18.2%) and herpes zoster infections (9.1%). <h3>Conclusion and relevance</h3> According to our results, alemtuzumab was effective in clinical practice due to a low relapse rate. However, further studies with a larger number of patients are needed to confirm these results. IRRs were frequent. Nevertheless, AE were mild and well tolerated. <h3>References and/or acknowledgements</h3> None. No conflict of interest.

Lupus profond et myélopathie

We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the upper and lower limbs, suggestive of a medullary lesion. MRI disclosed an image of acute myelitis at the level of the second cervical vertebra. High doses of corticosteroids were promptly administered intravenously (methylprednisolone bolus) followed by oral prednisone and hydroxychloroquine. Six months later the patient was free of neurologic or cutaneous symptoms. In the literature, systemic signs are rarely associated with lupus profundus and myelitis has never been reported. Lupus myelitis is a vascular and/or demyelinating and usually segmental lesion of the spinal cord. Neurologic symptoms are those of acute rather than chronic or recurrent transverse myelitis. The prognosis is poor with frequent and severe functional sequelae. Treatment is mainly based on high-dose systemic corticotherapy alone or combined with cyclophosphamide. Our patient responded favourably to early methylprednisolone bolus followed by oral corticosteroids and antimalarial treatment. Lupus profundus is classically of benign course with only cutaneous involvement, but it may sometimes be a sign of systemic lupus and in rare cases may be associated with severe complications.

Neuromyelitis Optica Immunoglobulin G in a Child

Neuromyelitis optica or Devic's syndrome is an uncommon demyelinating disorder that preferentially attacks the spinal cord and optic nerves. Although it is well described in adults, childhood neuromyelitis optica has rarely been reported in the literature and is frequently misdiagnosed as severe multiple sclerosis. Recently, a serum immunoglobulin G test for neuromyelitis optica has become available which may clarify and accelerate the diagnosis. This report describes a child with recurrent myelitis and an elongated spinal cord lesion who was found to have positive neuromyelitis optica autoantibody. We believe that neuromyelitis optica autoantibody testing should be performed in cases of pediatric transverse myelitis with multiple vertical segments or recurrence.

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.

Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis

We investigated whether neuromyelitis optica (NMO) IgG seropositivity at the initial presentation of longitudinally extensive transverse myelitis (LETM) predicts relapse of myelitis or development of optic neuritis. Prospective study of patients with initial LETM who were tested for the presence of NMO-IgG. Eleven of 29 patients (37.9%) were seropositive after a first attack of LETM spanning three or more vertebral segments on magnetic resonance imaging. Of 23 patients followed up for 1 year, none of 14 who were seronegative experienced a relapse or developed optic neuritis. Of 9 seropositive patients, 5 developed a second event: 4 of 9 (44%) developed recurrent transverse myelitis and 1 of 9 (11%) developed optic neuritis (p = 0.004). LETM represents an inaugural or limited form of NMO in a high proportion of patients. The 40% of patients who are seropositive for NMO-IgG are at high risk for relapse.

NMO‐IgG: A Specific Biomarker for Neuromyelitis Optica

Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that principally targets the optic nerves and spinal cord and often leads to severe disability and occasionally life threatening respiratory failure. Although its clinical manifestations overlap with those of multiple sclerosis (MS), in established cases these two conditions can be distinguished on the basis of clinical, radiological, and routine spinal fluid studies. The diagnosis in early cases or limited forms of NMO is difficult. We recently discovered a unique IgG autoantibody (NMO-IgG) that is highly specific to patients with NMO and thus a valuable diagnostic aid. Its antigen, aquaporin-4 (AQP4), is the central nervous system’s predominant water channel protein. This antibody has not yet been proven to be pathogenic, but several facts suggest that it might be, including the similarity of the immunohistochemical pattern of NMO-(AQP4) IgG binding to mouse CNS tissues to the pattern of immune complex deposition in autopsied patients’ spinal cord tissue. The spectrum of diseases identified by NMO-IgG is broader than has previously been recognized clinically and includes incomplete forms of NMO, such as recurrent transverse myelitis without optic neuritis and recurrent optic neuritis without myelitis.

Recurrent transverse myelitis following neurobrucellosis: Immunologic features and beneficial response to immunosuppression

The authors report the clinical course and immune system response of a patient with disease-associated recurrent transverse myelitis (TM) following cerebral infection with Brucellosis melitensis. The patient suffered four recurrences of his TM (each at a distinct spinal cord level) over the course of 2 years following his initial presentation, which ultimately progressed to quadriplegia. He had progressively declining cerebrospinal fluid (CSF) brucella antibody titers, suggesting a postinfectious, rather than an infectious, etiology. The authors simultaneously examined the expression of multiple cytokines in the CSF of this patient using cytokine antibody arrays and found a marked elevation of interleukin (IL)-6, IL-8, and macrophage chemoattractant protein (MCP)-1 levels relative to controls. Quantitative enzyme-linked immunosorbent assay (ELISA) analysis of the CSF confirmed a 1700-fold elevation of IL-6 and more modest elevations of IL-8 and MCP-1. IL-6 levels returned to baseline following treatment of the patient with intravenous cyclophosphamide and plasma exchange and the patient experienced a significant and sustained recovery of function.