Quantitative Assessment of Response to Long‐Term Treatment with Intravenous Immunoglobulin in Patients with Stiff Person Syndrome

BACKGROUND: Stiff person syndrome (SPS) is an autoimmune condition involving antibodies against several components of the inhibitory synapse in the spinal cord, with glutamic acid decarboxylase antibodies being the predominant immune marker. SPS affects approximately 1 patient per million population per year. The effect of intravenous immunoglobulin (IVIG) has been established, but studies on the long‐term efficacy of regular IVIG are limited. OBJECTIVES: To review clinical details and long‐term treatment response using a patient‐reported questionnaire in SPS and related syndromes. METHODS: Patients were identified from a tertiary neuroimmunology clinic based on classical clinical symptoms, autoimmune profiles, and neurophysiological changes (Dalakas criteria). They were followed up after treatment to assess the response to IVIG. RESULTS: A total of 23 patients fulfilled the selection criteria. Patients' demographic profiles and clinical presentations were akin to that reported in literature. There was significant improvement in the functional ability (assessed by the modified Rankin scale [mRS]) and quality of life (QoL) following treatment with IVIG within 4 to 10 weeks (pre‐mRS vs. post‐mRS, P < 0.0001; pre‐QoL vs. post‐QoL, P = 0.0003) and sustained after 5 years of treatment (pre‐mRS vs. present mRS, P = 0.0003; pre‐QoL vs. present QoL, P = 0.0002). CONCLUSIONS: This article describes one of the largest single‐center experiences of 23 patients with SPS and related syndromes and is the first to establish the long‐term efficacy of regular IVIG using a patient‐reported scoring system (Birmingham Response to Immunomodulatory Therapy [BRIT]). Consistent improvement in QoL and functional scores were seen over nearly 5 years after regular use of IVIG. It is recommended to use BRIT scores to assess the initial response as well as to monitor continued improvement to immunomodulation in SPS

Age at onset predicts outcome in aquaporin-4-IgG positive neuromyelitis optica spectrum disorder from a United Kingdom population.

BACKGROUND There are few studies exploring the prognostic factors in patients with aquaporin-4 (AQP4)-IgG positive neuromyelitis optica spectrum disorder (NMOSD). OBJECTIVE To assess the predictors of outcome in patients with AQP4-antibody positive NMOSD from a United Kingdom (UK) population. METHODS A retrospective study of 52 patients from 2 neuroscience centres in the UK Midlands. RESULTS The most common initial presentations were acute myelitis and optic neuritis, with 22/52 cases (42.3%) each. Relapsing course was seen in 32 patients (61.5%) with mean annualised relapse rate of 0.43 (standard deviation 0.45) and a mean interval time to first relapse of 31 months (range 2-108). The median Expanded Disability Status Scale (EDSS) score at the last follow up was 4 (range 1-9). Age at onset was an independent predictor of disability in the whole cohort of patients with NMOSD. For every 10-year increase in age at disease onset, the risk of developing an EDSS score of ≥4 increased by 34%. Patients who presented initially with a longitudinally extensive transverse myelitis (LETM) showed a higher risk to develop disability, compared to other clinical presentations (median time of 4 years versus 13 years). Late onset (LO-NMOSD) patients were likely to reach an EDSS score of 4 more quickly, compared to early onset (EO-NMOSD) (median time of 7 years versus 13 years). Higher median EDSS score at last follow up was observed in LO-NMOSD compared to EO-NMOSD (6 versus 2). CONCLUSION Increasing age at onset and LETM predict disability in AQP-4-IgG positive NMOSD patients

Disseminated strongyloidiasis: Breaking brain barriers

Strongyloides stercoralis (SS) is one of the most overlooked helminthic infections despite being highly endemic in tropical and subtropical areas. In immunocompromised patients, especially those on long-term steroids, infection can often escalate to fatal dissemination into major organs. We present a compendium of two immunocompromised patients, who were on high-dose steroids and presented with worsening neurological status. Cerebrospinal fluid analysis was notable for larvae of SS as diagnosed by direct visualization. A syndrome of SS hyperinfection with dissemination was made after stool, and sputum samples also revealed SS larvae. SS is an elusive disease and should be considered early on, especially in endemic regions like India. Early diagnosis and prompt initiation of antihelminthic therapy is indispensable for favorable outcomes