Evidence-based guideline: clinical evaluation and treatment of transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology.

OBJECTIVE: To assess the evidence for diagnostic tests and therapies for transverse myelitis (TM) and make evidence-based recommendations. METHODS: A review of the published literature from 1966 to March 2009 was performed, with evidence-based classification of relevant articles. RECOMMENDATIONS: Level B recommendations: neuromyelitis optica (NMO)-immunoglobulin G (IgG) antibodies should be considered useful to determine TM cause in patients presenting with clinical acute complete transverse myelitis (ACTM) features. The presence of NMO-IgG antibodies (aquaporin-4-specific antibodies) should be considered useful in determining increased TM recurrence risk. Level C recommendations: in suspected TM, distinction between ACTM or acute partial transverse myelitis may be considered useful to determine TM etiology and risk for relapse (more common with APTM). Age and gender may be considered useful to determine etiology in patients presenting with TM syndrome, with spinal infarcts seen more often in older patients and more female than male patients having TM due to multiple sclerosis (MS). Brain MRI characteristics consistent with those of MS may be considered useful to predict conversion to MS after a first partial TM episode. Longer spinal lesions extending over >3 vertebral segments may be considered useful in determining NMO vs MS. CSF examination for cells and oligoclonal bands may be considered useful to determine the cause of the TM syndrome. Plasma exchange may be considered in patients with TM who fail to improve after corticosteroid treatment. Rituximab may be considered in patients with TM due to NMO to decrease the number of relapses. Level U recommendations: there is insufficient evidence to support or refute the efficacy of other TM therapies or the usefulness of ethnicity to determine the cause of a subacute myelopathy.

Transverse myelitis. Comparison with spinal cord presentations of multiple sclerosis.

We delineated the clinical and laboratory features that help distinguish acute myelopathic MS (MMS) from acute transverse myelitis (ATM), specifically testing the hypothesis that the symmetry of motor and sensory impairments at presentation can reliably distinguish between ATM and MMS. We reviewed 20 consecutive patients with ATM and 16 patients with MMS. Clinical criteria were used to assign patients to the ATM group. Patients assigned to the MMS group had onset of MS symptoms referable to the spinal cord and eventually fulfilled Poser's criteria for MS. The relative contribution of the symmetry of both motor and sensory symptoms for the accurate identification of ATM versus MMS was evaluated using a discriminant function analysis. Fifteen of 16 MMS patients and all 20 ATM patients presented with symptoms of motor dysfunction. Additionally, all patients in both groups presented with sensory complaints. MMS patients had asymmetric motor or sensory symptoms in all but one patient, whereas ATM patients exhibited symmetric weakness uniformly and symmetric sensory loss in all but one patient (statistically significant). None of the MS patients met criteria for ATM at presentation. None of the ATM patients developed MS over an average follow-up period of 4.5 years. In conclusion, MMS was easily distinguished from ATM in this study.

Magnetic resonance appearance of cerebral lymphomatoid granulomatosis.

Lymphomatoid granulomatosis is an angiocentric lymphoproliferative process that involves the lungs. In a 52-year-old-man with lymphomatoid granulomatosis who presented with encephalopathy, magnetic resonance imaging (MRI) of the brain demonstrated unusual multiple areas of enhancement that were both punctate and linear. These findings may be relatively specific for inflammation of deep cerebral vessels and have implications for MRI findings in other inflammatory cerebrovascular disorders.

Lymphocyte subsets in relapsing-remitting multiple sclerosis: a longitudinal study of B lymphocytes and T lymphocytes.

Changes in lymphocyte subset populations may provide clues to the dysimmune mechanisms involved in relapsing remitting multiple sclerosis (RRMS). The lymphocyte subgroup CD4+ CD45RA+, thought to be responsible for the induction of suppression is decreased in some patients with MS compared to controls. A possible role for another lymphocyte subset, CD19+CD5+ lymphocytes, has been proposed in autoimmune diseases and multiple sclerosis (MS). To expand this we studied CD4+CD45RA+ (T) lymphocytes and CD19+CD5+ (B) lymphocytes in nine patients with relapsing-remitting MS (RRMS) and nine controls. The patients were examined monthly for an average of ten months and nine relapses were observed in seven patients. One patient underwent monthly gadolinium enhanced magnetic resonance imaging (MRI). Normal percentages CD4+CD45RA+ lymphocytes were found in patients with RRMS. No significant abnormalities in the CD19+CD5+ lymphocyte subpopulation were noted, although a tendency for higher percentages of this subset (approaching statistical significance, P = 0.056) was detected.