Observations on juvenile myoclonic epilepsy amongst ethnic Bengalees in West Bengal--an Eastern Indian State.

BACKGROUND: Juvenile myoclonic epilepsy (JME) is not too uncommonly encountered in Indian neurological practice. A number of reports from different parts of India have documented the clinical phenomenology and EEG characteristics of this genetically determined epileptic syndrome. However, no study has yet been reported from the Eastern part of India and none done so far in patients in a specific ethnic group. Furthermore therapy response and follow up data are not available in detail in the Indian studies. OBJECTIVE: To study disease expression, EEG characteristics and therapy response of JME patients in ethnic Bengalees in West Bengal, an Eastern Indian State, in a clinic based study. MATERIAL AND METHODS: 200 patients with JME attending the Neurology Department of the Institute have been followed up for 5 years and different parameters of disease expression as outlined above have been studied. RESULTS: Overall clinical disease expression has been found to be similar in this clinic based study in ethnic Bengalees as compared to other reports from India and elsewhere. About 16% of patients showed a relative resistance to Valproate therapy. Hundred percent of patients in whom therapy withdrawal was attempted, relapsed within<1-2 years. Amongst female patients (132), 16 developed features of polycystic ovarian syndrome while on Valproate therapy. In over half of them, the symptoms regressed after successful switch over from Valproate to Clobazam. 12/132 female patients became pregnant during follow up and while on Valproate; teratogenic effect was evident in only one such patient. CONCLUSIONS: Phenotypic variations in disease expression including therapy response have been noted within a single ethnic group of patients attending the clinic and might account for genetic heterogeneity noted in molecular genetic studies. JME cannot really be called a very 'benign' epileptic syndrome; recurrence after therapy withdrawal almost invariably occurs.     

Evaluation of socio-economic factors causing discontinuation of epilepsy treatment resulting in seizure recurrence: a study in an urban epilepsy clinic in India.

BACKGROUND AND PURPOSE: The prevalence rate of epilepsy in India ranges between 4.15 and 7.03 per 1000 population. In the developing countries, the major problems of epilepsy are lying in the treatment gap and discontinuation of treatment due to various adverse socio-economic factors. The objective of this study was to evaluate the rate of discontinuation of epilepsy treatment and its related socio-economic factors responsible for discontinuation. MATERIAL AND METHODS: Among 1450 patients with epilepsy who were recurrently followed up at an intervals of 2 months from 05 January to 06 January; 620 patients discontinued their treatment. Among them 88.7% patient had breakthrough seizures for more than in two occasions. Socio-economic factors in respect to the treatment were evaluated during the follow-up period vis-a-vis income and expenditure, unemployment status, negative attitude towards medical treatment, non-availability of drugs locally, co-morbid psychiatric and other illnesses, polytherapy and socialillusional thoughts about epilepsy. RESULTS: Discontinuation of epilepsy treatment was detected in 42.75% (n = 620) of total patients resulting in recurrence of seizures. Reasons for discontinuation were multiple in most of the cases. The discontinued group had an average annual cost of treatment and income of Rs. 5500 ($110) and Rs. 12,800 ($256), respectively, amounting to 40% of their total income being expended for the cost of the treatment, while in continued group annual cost of treatment and income were Rs. 4500 ($ = 90) and Rs. 24,400 ($ = 580) respectively amounting to only 18% of the total income (p < 0.001) for the cost of treatment. Among the discontinued group, 90% of the patients reported the cost factors, 29.09% due to the unemployment, 20% from the frustration and despair, 20.09% due to non-availability of medicines locally, 17.27% spiritual illusional thoughts about epilepsy, 10% for marital disharmony were the causes for discontinuation of treatment. In the discontinued group, 10% got polytherapy against 9.03% in the continued group (p > 0.01), co-morbid psychiatric illnesses were observed in 4.54% against 3.25% in the continued group (p > 0.10). CONCLUSION: The study showed a significant number of patients (42.75%) discontinued epilepsy treatment within 1 year due to poor knowledge regarding the problem of discontinuation, cost and income disparity, unemployment, spiritual illusional thoughts about epilepsy, frustration and mental impairment, lack of uniform availability of drugs in local market. To tide these shortcomings, uniform availability of cheaper antiepileptic drugs with adequate information and communication regarding the disease and upliftment of socio-economic status are to be ensured.     

Effect of selenomethionine on N-methylnitronitrosoguanidine-induced colonic aberrant crypt foci in rats.

An association between low selenium intake and the incidence or prevalence of cancers is well known. Selenium in the form of selenomethionine supplemented in drinking water has been found to be highly effective in reducing tumour incidence and preneoplastic foci during the development of hepatocarcinogenesis in rats in our previous studies. Here, an attempt has been made to investigate whether the dose and form of selenium found to be effective during hepatocarcinogenesis is equally effective in N-methylnitronitrosoguanidine-induced colorectal carcinogenesis in terms of antioxidant defence enzyme systems, DNA chain breaks and incidences of aberrant crypt foci. Treatment with selenomethionine either on initiation or on selection/promotion, or during the entire experiment showed that selenomethionine was most effective in regulating the cellular antioxidant defence systems, DNA chain break control and reducing aberrant crypt foci in the colorectal tissues of rats. Our results also confirm that selenium is particularly effective in limiting the action of the carcinogen during the initiation phase of this colorectal carcinogenesis, just as we found with hepatocarcinogenesis in our previous studies.     

Unexpected plasmablastic lymphoma in a young adult with unknown HIV infection: case report

BackgroundPlasmablastic lymphoma (PBL) is a rare, highly aggressive lymphoma of plasma cell differentiation. It commonly presents as an Epstein-Barr virus (EBV)+ oral lesion in an immunodeficient patient, predominately human immunodeficiency virus (HIV)+ patients. These aggressive lesions often demonstrate an immunoblastic or plasmablastic morphology with a typical immunohistochemical profile. The current case is unique due to the location at presentation, immunohistochemical features, and unknown presence of HIV infection in a young adult male.Case PresentationWe present an unexpected case of PBL found in a rare extra-oral location in a young adult male with undiagnosed HIV infection presenting as a perianal hemorrhoid mass/abscess. Swift treatment for HIV and the PBL resulted in complete remission and markedly improved CD4 counts.ConclusionsThis case highlights the importance of testing for HIV along with acquiring a thorough social/clinical history when a PBL is encountered. Although the overall prognosis of PBL is dismal with a median survival of about 6–11 months, a timely accurate diagnosis and prompt chemotherapy with an appropriate regimen along with antiretroviral therapy (ART) may still achieve a successful outcome with a relatively reasonable long-term remission like in our reported case.     

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

Purpose:To describe the clinic-radiological, pathological profile, and management outcomes of primary alveolar soft-part sarcoma (ASPS) of the orbit.Methods:A retrospective analysis of all histopathologically proven cases of orbital ASPS that presented between May 2016 and September 2019 was done. Data collected included demographics, clinical features, imaging characteristics, metastatic workup, management, and follow-up.Results:Five patients, of which four were males, presented to us during the study period. The mean age of presentation was 12.6 years (range 3–22 years). The most common presenting features were abaxial proptosis (n = 4) and diminished vision (n = 4). Imaging showed a well-defined orbital mass in all patients with internal flow voids in three. Preoperative percutaneous embolization with cyanoacrylate glue was done in these three patients owing to high vascularity. Four patients underwent complete tumor excision. One patient underwent exenteration. Histopathology showed polygonal tumor cells arranged in a pseudo-alveolar pattern and Periodic Acid-Schiff (PAS) positive crystals in the cytoplasm in all patients. One patient had systemic metastasis at presentation and developed a local recurrence after 3 months. No recurrence or metastasis was noted in the remaining four patients at a mean final follow-up of 11.2 months (range 5–15 months).Conclusion:ASPS is a rare orbital neoplasm that poses a diagnostic and therapeutic challenge. Imaging might show a soft-tissue tumor with high vascularity. Multidisciplinary management with interventional radiologists for preoperative embolization of vascular lesions helps minimize intraoperative bleeding and aids in complete tumor resection. A localized orbital disease carries a better prognosis.     

Chemistry and pharmacology of a tertiary alkaloid from Strychnos trinervis root bark

Bisnordihydrotoxiferine has been isolated as the major alkaloid from the root bark of Strychnos trinervis. This compound shows a wide antimicrobial spectrum against gram-positive, gram-negative and acid-fast microorganisms, filamentous and yeast-like fungi and also phytopathogenic microorganisms. Preliminary studies also reveal some cytotoxic activity in KB cells which was confirmed by tests against sarcoma 180 tumors.