Long-Term Remission With Low-Dose Rituximab in Myasthenia Gravis: A Retrospective Study.

OBJETIVE: Rituximab (RTX) is a therapeutic option, for patients with myasthenia gravis (MG) not responding to conventional immunosuppressive treatment. In this cohort, we evaluated long-term efficacy of RTX in the treatment of refractory generalized MG. METHODS: A retrospective study was performed in adult patients with refractory generalized MG and at least 24 months of follow-up, between January/2015 and October/2021. The Myasthenia Gravis Status and Treatment Intensity Score was used to assess outcomes, and CD19/CD20+ B-cell counts were monitored. RESULTS: Sixteen patients with MG (8 antiacetylcholine receptor+ and 8 muscle-specific antikinase+; mean age 45.5 ± 16.2 years) treated with low-dose RTX protocols were included. CD19/CD20 levels remained undetectable 12 months after induction, and no new relapses were observed during follow-up. CONCLUSIONS: Low-dose RTX infusions were sufficient to achieve undetectable CD19/20 cell counts and sustained clinical remission. In low and middle-income countries, the impact of low-dose RTX therapy represents a paradigm shift in decision-making for long-term treatment.

Evidence of neurophysiological improvement of early manifestations of small-fiber dysfunction after liver transplantation in a patient with familial amyloid neuropathy.

INTRODUCTION: Small fiber polyneuropathy (SFP) is a common heralding clinical manifestation of damage to the nervous system in patients with familial amyloidosis. The diagnosis of SFP is a significant factor in the decision to treat a previously asymptomatic gene carrier, as treatment would prevent irreversible nerve damage. This requires detection of the earliest but unequivocal signs of peripheral nerve involvement. CASE REPORT: We present the case of a young female who was diagnosed of SFP, supported by data from quantitative sensory testing. She had preserved sensory nerve action potentials in the distalmost nerves of her feet and recordable nociceptive evoked potentials. She was successfully transplanted the liver from a previously healthy donor, and recovered fully of her symptoms and signs. Improvement was documented with repeated psychophysical and electrodiagnostic testing in the course of 4 years after transplantation. SIGNIFICANCE: This case illustrates the utility of psychophysical testing to support the diagnosis of SFP.

Headache and Treatment of Unruptured Intracranial Aneurysms.

BACKGROUND AND PURPOSE: The relationship between unruptured intracranial aneurysms (UIAs) and chronic headache and the impact of aneurysm treatment on headache outcome are controversial. The aim of this study was to determine clinical features of a supposedly primary headache in patients with UIA. We also assessed changes in headache characteristics after UIA treatment. METHODS: We examined clinical and imaging data of patients in whom a UIA was diagnosed during diagnostic workup of a suspected primary headache. Medical records were reviewed and personal telephone follow-ups were performed after UIA treatment to assess changes in the frequency and intensity of the headache. RESULTS: Forty-two patients (76%) reported a substantial improvement in headache frequency and intensity after UIA treatment. Forty-five patients (81%) reported a decrease in headache frequency from a median of 8 days/month before treatment to 1 day/month after treatment (95% confidence interval [CI] 81-83, P < .001). The average intensity in an analog pain scale was 7.7 ± 1.6 before treatment and 5.6 ± 2.4 after treatment (P < .001). Higher headache frequency was associated with a greater odd of improvement after treatment (odds ratio 1.12, 95% CI 1.0-1.26, P = .03). No associations were found between the type of headache, type of treatment (endovascular versus surgical), number, size, or localization of the aneurysms and the response to treatment. CONCLUSIONS: The treatment of UIA had a robust beneficial effect on previous headache. Although a "placebo" effect of aneurysm treatment cannot be ruled out, these results suggest a potential association between UIA and certain chronic headaches usually considered to be primary.

Low familial risks for multiple sclerosis in Buenos Aires, Argentina.

BACKGROUND: Multiple sclerosis is a complex disease in which genetic susceptibility plays a role and familial occurrence has long been recognized. To date, no studies of familial occurrence have been conducted in Argentina, a country with low to intermediate prevalence. METHODS: As part of a cross-sectional study on multiple sclerosis in Buenos Aires, immediate and extended pedigree details were collected on 219 patients. Crude and age-adjusted recurrence risks for patient relatives were also assessed. RESULTS: Details on age or age of death and disease status were obtained for 4227 relatives. Ten percent of patients reported at least one relative with MS diagnosis, the highest risk (1.54%) was observed in daughters of patients who presented 92-times greater risk than the general population. Sibling recurrence risk ratio was similar to that reported in other locations. CONCLUSIONS: Relatives of patients with MS living in Argentina are at greater risk of developing the disease, although not as high as the risk reported for other geographic regions.

Autoimmune disease prevalence in a multiple sclerosis cohort in Argentina.

Background. Comorbid autoimmune diseases in MS patients have been studied extensively with controversial results. Moreover, no such data exists for Latin-American MS patients. Methods. We conducted a case-control study aimed to establish the prevalence of autoimmune disorders in a cohort of Argentinean MS patients. Results. There were no significant differences in autoimmune disease prevalence in MS patients with respect to controls. The presence of one or more autoimmune disorders did not increase risk of MS (OR 0.85, 95% CI 0.6-1.3). Discussion. Our results indicate absence of increased comorbid autoimmune disease prevalence in MS patients, as well as of increased risk of MS in patients suffering from other autoimmune disorders.