A Case of Adulthood Joubert Syndrome

Abstract

Joubert Syndrome is a rare autosomal recessive disorder characterized by hypotonia, ataxia, breathing difficulties, developmental delay with hallmark molar tooth appearance in MRI. We report a rare case of adulthood Joubert Syndrome which is an unusual presentation. A 25 -years male presented to our outpatient department with developmental delay, dysphasia, ataxia, nystagmus, bilateral optic atrophy and hyperreflexia. MRI Brain showed classical ‘molar tooth’ appearance of cerebellar peduncles. Although an uncommon disorder, it is important to diagnose the condition early as physiotherapy and rehabilitation can be effective in coping with the symptoms causing developmental delay. Bangladesh Journal of Neuroscience 2018; Vol. 34 (1): 58-62

Combining species distribution models and big datasets may provide finer assessments of snakebite impacts

“…Unreliability of the current recording and reporting system used by health care facilities demand that snakebite is legislated to be a notifiable disease. Previously, a postal survey conducted in the year 1995 had suggested that 4.3-7 snakebite cases per 100,000 people per year might occur in Chattogram Division [40,41], which would correspond to 2,000 snakebite episodes per year among the 28.42 million population of this division during the study period. In the year 2010, Rahman et al [11] estimated a snakebite incidence of 397.8 (211.8-680.3, 95% CI) per 100,000 population-year in this division after conducting a nationwide community-based cluster survey in rural areas of Bangladesh.…”

“…Snakebite in Bangladesh has never been studied from an ecological perspective. Earlier studies were focused mostly on epidemiological analyses [11,40,41], clinical manifestations following the bites by different species and biological properties of their venoms [37,38,42], and socioeconomic loss caused by snakebite [43,44]. These studies showed that the study duration was significantly correlated with the number of admitted patients (r = 0.993, p<0.001) (Table 1).…”