Idiopathic severe recurrent transverse myelitis: a restricted variant of neuromyelitis optica

NMO-IgG-negative relapsing myelitis

“…Compared to these, our selection criteria additionally contemplate a 3-year followup, as well as the exclusion of patients with abnormal VEPs, and HCV infection. Indeed, recent reports classified as NMO are those recurrent myelitis with abnormal VEPs, 5,6 whereas others are identified as HCV infection in association with recurrent myelitis. 8,13 If we retrospectively apply to our patients, the criteria established by the TM Consortium Working Group, we can define our patients as definite TM based on clinical, CSF and spinal cord MRI features.…”

“…Despite the stricter selection criteria 1 and the inclusion of forms with severe motor impairment, still 30% of cases of 'idiopathic' TM were found to have a relapsing course, out of the context of MS, vasculitis, NMO, connectivitis and infections. Relapsing I-TM are reported in isolated reports and small series: [2][3][4][5] interpretations point at limited forms of NMO, 5,6 with possible subclinical optic nerve involvement, only detectable by VEPs or at a specific disease entity. 3 Compared to NMO, our patients also showed severe neurological impairment, with prominent motor and sphincter dysfunction; CSF, pleocytosis; blood barrier damage; normal IgG index; absent or transitory OBs; on MRI, prominent gray matter involvement, with LETMs occurring in 54%.…”

“…Two patients had lesions extending to the brainstem, a pattern described in an NMO series. 6 However, we believe that recurrent I-TM in our patients was distinct from limited forms of NMO, for the following reasons: unlike NMO, our patients did not show optic nerve involvement, either clinical or subclinical; on MRI, we failed to detect T1-hypointense lesions and mass effect during the acute phase, or cavitations and areas of spinal cord atrophy on follow-up; in spite of the frequent occurrence of LETMs, as many as 46% patients had monosegmental lesions, possibly multifocal, that are unusual for NMO; ultimately, NMO-IgG were absent in all patients. NMO-IgG antibodies in transverse myelitis S Ravaglia et al In this patient with post-infectious myelitis, the initial event occurred during the course of pneumonia; the first relapse occurred 14 months after the first event, 3 weeks after influenza vaccination.…”

“…Although recent studies on TM have applied this work-up, 1 isolated cases or small series of relapsing I-TM are still reported, [2][3][4] and their nosology remains controversial. [3][4][5][6] The final outcome is often characterized by severe disability, 3 and treatment options for relapse prevention are not established.…”

Clinical Reasoning: Bacterial meningitis causing a neuromyelitis optica flare

“…However, it has been shown to have clinical utility in larger studies. 5 We posit that concurrent bacterial meningitis triggered the neuromyelitis optica (NMO) flare in our patient, as her pleocytosis and hypoglycorrhachia improved after broad-spectrum antibiotics (table 1). Her presenting symptoms were similar to her prior flares, which had previously improved with immunosuppression.…”