Association between paroxysmal tonic spasms and neuromyelitis optica.

OBJECTIVE: To determine the frequency of the association between tonic spasms and neuromyelitis optica (NMO) at our center. DESIGN: An institutional review board-approved retrospective study of clinical, serological, and radiographic characteristics of patients with NMO. SETTING: Multiple sclerosis center. PATIENTS: Patients with NMO treated at our center between 1990 and 2008. MAIN OUTCOME MEASURE: Records were examined for documentation of tonic spasms. RESULTS: Of 110 patients with International Classification of Diseases code 341, 57 patients met diagnostic criteria for NMO. Of these, 8 patients (14%) had documented typical tonic spasms (median age at onset, 39.5 years; range, 13.8-54.2 years). Of those patients, 4 were African American, 3 were Hispanic, and 1 was white. Only 1 was male. The NMO-IgG antibody was found in 1 of 6 patients tested. Tonic spasms appeared after a mean of 24.6 months (range, 0-91 months). In 2 of 57 patients meeting NMO criteria, tonic spasms accompanied their initial episodes. Seven of 8 patients who had tonic spasms responded to treatment with carbamazepine within 1 week. CONCLUSION: Tonic spasms are associated with NMO more commonly than with multiple sclerosis and may be a presenting sign in both diseases.

Impact of rituximab on relapse rate and disability in neuromyelitis optica.

BACKGROUND: Neuromyelitis optica (NMO) is a severe demyelinating disease often leading to serious disability. Accumulating evidence now implicates humoral mechanisms in its pathogenesis. In the absence of an approved therapy, anti-inflammatory/immunosuppressant drugs have been used empirically for more than three decades. Recent evidence for a role of antibody to aquaporin-4 in the pathogenesis of NMO has led to the use of rituximab, a monoclonal antibody targeting the CD20 epitope on the entire B cell lineage. OBJECTIVES: To evaluate the impact of rituximab on the relapse rate and disability in NMO. METHODS: This is an IRB approved retrospective longitudinal study of NMO patients treated with rituximab. RESULTS: We identified 53 patients with NMO, 23 of whom had been treated with rituximab. These patients (2 males, 21 females) had a mean age of 37.1 ± 14.6 years at the time of diagnosis. Eight of the 23 treated with rituximab were treatment naïve. All 23 were scheduled to receive infusions every six or 12 months after treatment initiation with a minimum follow-up of six months (median 32.5 months, range 7-63 months). Median relapse rate declined significantly from 1.87 relapses/patient per year to 0.0 relapses/patient per year. Kurtzke Expanded Disability Status Scale (EDSS) scores stabilized or improved in all patients. Use of rituximab is associated with a significant reduction in relapses and disability in patents with NMO.

Chronic inflammatory demyelinating polyneuropathy responsive to mycophenolate mofetil therapy.

OBJECTIVE: To evaluate the efficacy of mycophenolate mofetil (MMF) in chronic inflammatory demyelinating polyneuropathy (CIDP). BACKGROUND: Evidence is growing that MMF is effective as an immunomodulatory drug in neuromuscular diseases. METHODS: A database of 184 patients with CIDP was analysed to obtain clinical, laboratory and electrophysiological information for patients with CIDP treated with MMF. RESULTS: Eight patients, who met the inclusion criteria, received MMF (mean dose 2 g/day; median duration 15.2 months). The average Neuropathy Impairment Score of the eight patients improved from baseline (72.3+/-35) after initiation of MMF therapy (37.8+/-37; p<0.001). Six of these eight patients were either able to stop concomitant medications (corticosteroid, intravenous immunoglobulin) or reduce their doses and frequency by > or = 50%. CONCLUSIONS: Our pilot data suggest that MMF appears to be an effective therapy for patients with naive or refractory CIDP, and further controlled studies are warranted for their confirmation.

Quality of life in Indian patients with rheumatoid arthritis.

PURPOSE OF STUDY: Rheumatoid arthritis (RA) is a multisystem disease with various extra-articular manifestations (EAMs). Health-related quality of life (HRQOL) issues are assuming increasing importance in chronic rheumatic diseases like RA. No data on QOL in RA is available from the Indian subcontinent. There is also a paucity of literature on the impact of EAMs on HRQOL in RA. The objective of this study was to address these lacunae. METHODS: The study group comprised 81 patients with RA from a rheumatology clinic in India. Quality of life was estimated by the generic HRQOL measure: World Health Organization quality of life instrument (WHOQOL-Bref). Disease activity in RA was measured by calculating Disease Activity Score-28 (DAS28). RESULTS: The mean HRQOL scores of the patients were 12.0+/-2.8, 13.2+/-2.7, 14.4+/-2.9 and 13.3+/-2.6 in the physical, psychological, social, and environmental domains of the WHOQOL-Bref respectively. Age, gender, disease duration, educational status, constitutional symptoms, rheumatoid factor positivity, erosions and deformities did not influence HRQOL. Disease activity had a negative influence on the physical and psychological domains. Patients with EAMs had significantly higher DAS28 scores compared to patients without EAMs. Even after adjustment for disease activity, patients with EAMs had lower HRQOL scores than patients without these features (statistically significant for physical domain). CONCLUSIONS: The physical domain of HRQOL is most affected in Indian patients with RA. Increasing disease activity and presence of EAMs worsens the quality of life.