Successful Treatment with Rituximab in a Patient with Stiff-Person Syndrome Complicated by Dysthyroid Ophthalmopathy

Abstract

We report a patient with stiff-person syndrome and insulin-dependent diabetes mellitus with anti-glutamic acid decarboxylase (GAD)

Stiff-person syndrome (SPS) and anti-GAD-related CNS degenerations: Protean additions to the autoimmune central neuropathies

“…There is, however, little information on the extent to which rituximab penetrates the bloodebrain barrier or whether rituximab would be more efficacious given intrathecally. In fact, the literature reveals cases wherein rituximab did prove beneficial [65,66] but balanced by others showing no improvement despite B cell reduction in blood [67]. In a case of SPS, thyroid opthalmopathy and diabetes treatment with rituximab induced "obvious improvement" in SPS and thyroid opthalmopathy, and a decrease in levels of anti-GAD but with no amelioration of features of diabetes [66]; the authors speculated that different pathogenic mechanisms may operate in SPS and diabetes.…”

“…In fact, the literature reveals cases wherein rituximab did prove beneficial [65,66] but balanced by others showing no improvement despite B cell reduction in blood [67]. In a case of SPS, thyroid opthalmopathy and diabetes treatment with rituximab induced "obvious improvement" in SPS and thyroid opthalmopathy, and a decrease in levels of anti-GAD but with no amelioration of features of diabetes [66]; the authors speculated that different pathogenic mechanisms may operate in SPS and diabetes. Contrariwise, there is the report [67] on female monozygotic twins of whom each, remarkably, was afflicted with SPS, but rituximab had little effect either on symptoms, anti-GAD65 levels, enzyme inhibitory activity or epitope recognition by autoantibodies; in fact, both patients had an increase in numbers in blood of anti-GAD65 specific memory B cells during follow up.…”

Anti-B-Cell Therapies in Autoimmune Neurological Diseases: Rationale and Efficacy Trials

“…Rituximab effectively treated SPS, even in cases with complications, such as dysthyroid ophthalmopathy and respiratory failure [128][129][130]. In a most recent report of a case study, rituximab in combination with tizanidine improved SPS along with the immunomodulatory effect previously caused by benzodiazepines [131].…”

Transition of thyroid autoantibodies by rituximab treatment for thyroid MALT lymphoma

“…Recently, there have been reports about the effect of rituximab for autoimmune thyroid diseases such as Graves' disease [9][10][11][12]. B lymphocytes were completely absent from the thyroid gland of a Graves' disease patient after rituximab treatment [10], and both TSH receptor antibody and anti-thyroid peroxidase antibody (TPOAb) decreased after rituximab treatment for Graves' disease [11,12].…”

“…B lymphocytes were completely absent from the thyroid gland of a Graves' disease patient after rituximab treatment [10], and both TSH receptor antibody and anti-thyroid peroxidase antibody (TPOAb) decreased after rituximab treatment for Graves' disease [11,12]. However, it is unknown whether rituximab treatment influences thyroid function because of combination of anti-thyroid drug such as methimazole for Graves' disease.…”