The need for a population-based, dose optimization study for recombinant tissue plasminogen activator in acute ischemic stroke: A study from a tertiary care teaching hospital from South India.

CONTEXT: The guideline recommended dose of intravenous (i.v) recombinant tissue-type plasminogen activator (rt-PA) for acute ischemic stroke is 0.9 mg/kg in the European and American populations. In Asiatic population, some studies have shown that a lower dose of i.v rt-PA is equally efficacious. AIMS: To assess if there is a need for a dose optimization for i.v rt-PA study among Indians. SETTING AND DESIGN: A prospective, observational database of acute stroke cases that presented to a tertiary care institute over a period of 1 year was made. METHODS: The data procured using a prestructured elaborate pro forma. Based on the dose of rt-PA received, the individuals were divided into three groups; Group 1 (0.6-0.7 mg/kg), Group 2 (0.7-0.8 mg/kg), and Group 3 (0.8-0.9 mg/kg). Improvement was assessed in each group and between the thrombolysed and nonthrombolysed individuals. STATISTICAL ANALYSIS USED: The nonparametric Mann-Whitney U-test (Wilcoxon rank-sum test) was applied for assessing improvement of National Institutes of Health Stroke Scale score with significance level of α < 0.05 (P < 0.012) and compliance level at 95%. RESULTS: Between the thrombolysed (n = 46) and nonthrombolysed (n = 113) group, there was a statistically significant neurological improvement in the thrombolysed group. Clinical improvement was noted in 75%, 85.7%, and 66.7% of individuals receiving rt-PA in Groups 1, 2, and 3, respectively. Four out of the five who developed a clinically significant intracranial hemorrhage were thrombolysed at a dose of 0.8-0.9 mg/kg rt-PA (Group 3). CONCLUSION: There is a need for a properly randomized, dose optimization study of i.v rt-PA in the Indian subcontinent.

Factors delaying management of acute stroke: An Indian scenario.

BACKGROUND AND PURPOSE: The purpose of this study was to assess factors causing delay in treatment of acute stroke in a tertiary care institute in South India. METHODS: All clinically suspected cases of acute stroke presenting to the emergency department over a period of 1 year were prospectively followed up and data collected as per a preset pro forma. The various time intervals from stroke onset to definitive management and other pertinent data were collected. The time delays have been evaluated in the decision tree model: Chi-squared Automatic Interaction Detection. Significance was assessed at 5% level of significance (P < 0.05). RESULTS: The mean prehospital time delay for all clinically suspected stroke (n = 361) in our institute was 716 min and the median time 190 min. The mean total in-hospital delay was 94.17 ± 54.5 min and median time being 82 min. The onset of symptoms to first medical contact was the main interval that influenced the prehospital delay. Computed tomographic (CT) diagnosis to stroke unit admission influenced the in-hospital delay the most. CONCLUSIONS: Lack of awareness regarding stroke leads to delayed seeking of treatment for the same. The factors that contribute to the in-hospital delay included patient admission procedure delay, lack of staff to transport the patient, and the distance between the stroke unit and CT room. Educating the community with regard to "stroke" and implementation of a better pre- and in-hospital stroke care system is a need of the hour in the country.

Congenital myopathies: clinical and immunohistochemical study.

Congenital myopathies (CMs), a group of relatively non-progressive disorders presents with weakness and hypotonia of varying severity, morphologically recognized by specific structural abnormalities within the myofiber. This report presents the clinical and Histopathological features of 40 patients with CMs. Centronuclear myopathy was the commonest (40%) followed by congenital fiber type disproportion (37.5%). Other less common CMs included: myotubular myopathy (5%), nemaline myopathy (5%), central core disease (5%), multicore disease (2.5%) and congenital myopathy with tubular aggregate (5%). Immunolabeling to desmin corresponded to morphological changes within the myofibers while vimentin was negative in all the patients. There is no combined role of these proteins in the disease process.

Sarcoglycanopathy: clinical and histochemical characteristics in 66 patients.

BACKGROUND: Sarcoglycanopathies are a group or autosomal recessive muscular dystrophies designated as α, ß, γ, or δ sarcogycanopathy. MATERIALS AND METHODS: It is a retrospective analysis of case series. RESULTS: Sixty six patients immunohistochemically confirmed to have sarcoglycan deficiency were included in the analysis. The study period extended from 1997-2008. The male to female ratio was 1.5:1. Mean age at the onset of muscle complaints was 6.2±3.7 years (range 1-18). Mean age at evaluation was 10.0±4.8 years (range 3-31). Mean duration of illness was 47.02±44.80 months (range 3-325). Onset in the first decade was seen in 59 (89.4%) and 25 (42.4%) of these had onset before five years of age. The remaining seven (10.6%) had onset in second decade and none after 20 years of age. Consanguinity was present in 54 (81.8%). In 34 of 66 cases only a-SG was carried out and this had shown total absence of staining in all fibers. In the remaining 32 cases where the entire panel was performed, absence of all sarcoglycans was noted in 10 (15.1%), isolated α-SG deficiency in 7 (10.6%), isolated ß-SG deficiency in 6 (9.1%), and isolated γ-SG deficiency in 3 (4.5%). Combination deficiency was also observed: absence of α and ß (n=4), ß and γ (n=2), and α and γ (n=1). CONCLUSIONS: Our series was a large series and with predominantly pediatric age group. Sarcoglycanopathy should be particularly suspected in a child born to consanguineous parents and who presents with proximal muscle weakness and calf hypertrophy, elevated CK level, and myopathic pattern on EMG.

Congenital myopathy with tubular aggregates: report on two siblings from India.

The clinical features and morphological findings in 2 Indian siblings with tubular aggregates are described. The siblings aged 14 and 9 years, respectively, born of consanguineous marriage presented with early onset gradually progressive lower limb proximal muscle weakness associated with seizures and mental subnormality. Muscle biopsy in both revealed characteristic tubular aggregates in type II fibers, which were confirmed electron microscopically. To the best of our knowledge, association of seizures and mental subnormality in familial tubular aggregates has not been described. Muscle biopsy helped in establishing the diagnosis of this rare familial disorder.