Essential oil related seizures (EORS): A multi-center prospective study on essential oils and seizures in adults.

OBJECTIVE: Essential oils (EOs) like eucalyptus and camphor have pro-convulsant properties. These EOs are present in many over- the- counter balms and oils. The effect of exposure to these EOs and occurrence of seizure is not systematically studied. The aim of this study was to evaluate the relationship between essential oils and the first episode of seizure and breakthrough seizures in known epileptic patients. METHODS: This was a multi-center prospective study, conducted in four hospitals over four years. Every person presenting with the first episode of seizure or breakthrough seizure was asked about exposure to EOs, mode of exposure, time to onset of a seizure in relationship to exposure, duration of seizure, type of seizure, and antiepileptic drug therapy. RESULTS: During the four-year period there were 55 patients with essential oil-related seizure (EORS). 22(40 %) had essential oil-induced seizures (EOIS) and 33(60 %) had essential oil-provoked seizures (EOPS). The female: male ratio was 1:1.1, the age of the patients ranged from 8 months to 77 years. In the EOIS group, 95 % had generalized tonic-clonic seizures and 5% had focal impaired awareness seizures. In the EOPS group, 42.4 % had focal impaired awareness seizures, 27.3 % generalized tonic-clonic seizures, 15 % focal to bilateral tonic-clonic seizures, and 15 % focal aware motor seizures. EOs implicated were preparations containing eucalyptus and camphor. CONCLUSION: Exposure to essential oils of eucalyptus and camphor is an under-recognized cause of the first and breakthrough seizure. Identifying the true causative factor will prevent unnecessary antiepileptic drug therapy and future recurrence.

Efficacy and safety of rituximab in multiple sclerosis: Experience from a developing country.

BACKGROUND: Rituximab is increasingly being used in treatment of multiple Sclerosis (MS) in our centers due to its easy availability, efficacy and favorable side effect profile. Here we describe experience with rituximab over a period of 4 years from three MS centers from south India. METHODS: The data of MS patients who were treated with rituximab in three MS centers at Bangalore, India, from December 2015 to December 2019 were collected and evaluated with respect to relapse rate, EDSS score and adverse events. RESULTS: Over the four-year study period 118 MS patients were evaluated, 80 of whom were on rituximab. 58 (72%) had RRMS, 15 (19%) SPMS and 7 (9%) PPMS. Most patients (89%) received rituximab at a dose of 500 mg every 6-12 months. Nine patients (11%), all with progressive MS were on 1 gm to 2 gm every 6 months. Follow up ranged from 1 year to 3 years, with a median of 2 years. 56 (97%) RRMS patients had no relapses during follow up. EDSS score improved by a score of 0.5-2.0 in 68 (85%) patients, remained same in 10 (12.5%) and worsened in 2 patients (2.5%). Most patients (91%) tolerated rituximab infusions well. There were no opportunistic infections or neoplasms. CONCLUSION: Anti B cell therapy with rituximab appears effective, safe and affordable in the treatment of MS in developing countries like India with resource limited settings.

HIV infection associated neuromyelitis optica spectrum disorder: Clinical features, imaging findings, management and outcomes.

INTRODUCTION: HIV Infection associated NMOSD (HIV-NMOSD) is a recently recognized entity. Management of patients with HIV-NMOSD is a challenge. Here we report our own experience of HIV-NMOSD along with a complete review of all the cases of HIV-NMOSD reported in literature. OBJECTIVE: Describe the clinical features, radiological findings, treatment patterns and outcomes in patients with HIV-NMOSD. METHODS: The details of all cases of HIV- NMOSD were searched from our NMOSD registry. A literature search was also done using the terms NMO, NMOSD and HIV infection in PUBMED, Google Scholar and EMBASE. The details of all the reported cases and cases from our registry were collected and analyzed. RESULTS: Six cases of HIV-NMOSD were identified from the literature and one from our registry. There were four males and three females with age ranging from 8 years to 49 years. Duration of HIV infection ranged from newly detected to 15 years. Optic neuritis followed by myelitis was the commonest presentation, occurring in 5 out of 7 patients. 3 patients were anti-aquaporin 4 antibody positive while 3 were negative and in one anti- aquaporin 4 antibody assay was not done. All patients received immunomodulatory treatment. 5/7 patients had poor recovery from acute attacks but no patient had further relapses while on immunomodulatory treatment and antiretroviral therapy. CONCLUSION: HIV associated NMOSD is a recently recognized entity. A high index of suspicion is needed to diagnose these patients. In all patients with HIV infection presenting with optic neuritis or/and myelitis, anti aquaporin 4 antibody status should be checked and in all patients of NMOSD, HIV infection should be ruled out.

Expanding spectrum of RARS2 gene disorders: Myoclonic epilepsy, mental retardation, spasticity, and extrapyramidal features.

Pontocerebellar hypoplasia type 6 (PCH6) is an autosomal recessive mitochondrial disease, typically characterized by pontine atrophy, vermian hypoplasia, infantile encephalopathy, generalized hypotonia, and intractable seizures. The purpose of this study is to describe the seizures and other neurological manifestations of RARS2 gene mutations and to compare the clinical features with other causes of progressive myoclonic epilepsy. Detailed history, physical examination, and clinical and genetic work-up were performed in 2 siblings who presented with progressive myoclonic epilepsy. One sibling, a 20-year-old woman, and the other a 24-year-old man, had a homozygous missense variant (c.848T>A; p.Leu283Gln) in exon 10 of the RARS2 gene. The female patient had action and audiogenic myoclonic jerks, postural tremors, spastic dysarthria, and bradykinesia, and her male sibling had similar features with oculomotor apraxia. The RARS2 gene mutation can present with myoclonic epilepsy, mental retardation, and pyramidal and extrapyramidal features, and is an important differential for causes of progressive myoclonic epilepsy.