9 research outputs found
Susac syndrome:neurological update (clinical features, long-term observational follow-up and management of sixteen patients)
Quantitative Assessment of Response to LongâTerm 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 longâterm efficacy of regular IVIG are limited. OBJECTIVES: To review clinical details and longâterm treatment response using a patientâreported 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 10âweeks (preâmRS vs. postâmRS, Pâ<â0.0001; preâQoL vs. postâQoL, PÂ =Â 0.0003) and sustained after 5âyears of treatment (preâmRS vs. present mRS, PÂ =Â 0.0003; preâQoL vs. present QoL, PÂ =Â 0.0002). CONCLUSIONS: This article describes one of the largest singleâcenter experiences of 23 patients with SPS and related syndromes and is the first to establish the longâterm efficacy of regular IVIG using a patientâreported scoring system (Birmingham Response to Immunomodulatory Therapy [BRIT]). Consistent improvement in QoL and functional scores were seen over nearly 5âyears 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 31Â 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 â„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 4Â years versus 13Â 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 7Â years versus 13Â 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