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Byline: M. Gourie-Devi [INLINE:1] Identification of South Indian Paraplegia by Mani and colleagues,[1] as an important cause of non-compressive myelopathy, with an elegant description of the clinical profile and exclusion [...]

Byline: M. Gourie-Devi India is in an epidemiological transition, with the persistence of some communicable diseases, newly emerging infections, a remarkable increase in the incidence of non-communicable disorders, and the [...]

Byline: M. Netravathi, Nitish. Kamble, P. Satishchandra, M. Gourie-Devi, Pramod. Pal The Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka has a long tradition of [...]

Byline: M. Gourie-Devi Sir, I wish to bring to notice a typographical error in my publication in Neurology India.[sup][1] In [Table 5] (page 593) showing the prevalence of stroke in [...]

Byline: M. Gourie-Devi I am grateful to the Editor of Neurology India for asking me to contribute to the novel section of 'Timeless reverberations'. Through this narrative, I wish to [...]

Byline: M. Gourie-Devi Growth and development of neuroepidemiology in India during the last four decades has been documented highlighting the historical milestones. The prevalence rates of the spectrum of neurological [...]

Byline: M. Gourie-Devi Tuberculous meningitis (TBM) is a serious meningitic infection commonly found to occur in the developing countries endemic to tuberculosis. Based on the clinical features alone, the diagnosis [...]

Byline: M. Gourie-Devi Leprosy is a common cause of peripheral neuropathy in tropical countries including India[1] and neuropathy is observed across the spectrum of leprosy from polar tuberculoid (TT), borderline [...]

The article presents information about cranial neuropathy in patients diagnosed with leprosy. Affectation on cranial nerve is possible in lepers especially on facial, trigeminal and olfactory nerves. Cranial nerve paralysis occurs with polar tuberculoid, borderline and polar lepromatous types of peripheral neuropathy. Clinical manifestation like skin lesions can be observed in lepers during the treatment process of the disease.

Background: Stroke is a heterogeneous disorder comprising of clinical subtypes and many risk factors, also alluded to as cerebrovascular disorders (CVDs). Increase in the global burden of stroke in developed and developing countries has been alarming. To galvanize the efforts towards the prevention and treatment, there is a need for robust data on the burden of stroke., Objective: The aim of this study was to estimate the burden of stroke, a systematic review of community-based studies was conducted., Material and Methods: Systematic search of PubMed and Google Scholar for studies from January 1960 to December 2018 was done. The articles were screened and the data was retrieved and sorted into incidence, prevalence and mortality rates. Meta-analysis was done on Medcalc statistical software version 19.2.6., Results: Prevalence rate of stroke for total population inclusive of urban and rural population, varied from 44.54 to 150/100000.For the urban population prevalence rate was 45 to 487/100000 and 55 to 388.4/100000 for rural population. The incidence rate varied from 33 to 123/100000 in the urban population and in the rural population it was estimated to be 123.57/100000. The 30 days case fatality rate of stroke varied from 41.08% to 42.06% in urban population and 18% to 46.3%.in the rural population., Conclusions: Systematic review and meta-analysis reveal that the stroke burden in India is quite high., Competing Interests: None

Gerstmann-Sträussler-Scheinker (GSS) syndrome is a devastating hereditary prion disease, presenting in 4th-5th decade with progressive ataxia and dementia. Pathogenic variants in the PRNP gene lead to aggregation of misfolded prion protein which results in neurodegeneration and death within a few years of onset. A key feature of prion disorders is conversion of normal prion protein (PrPc) into its misfolded form (PrPSc). Genetic modifiers include methionine at position 129 in prion protein and octapeptide repeats. We present an Indian kindred with c. 305C > T, p.Pro102Leu mutation in PRNP gene causing GSS in multiple members and discuss the impact of the polymorphism at position 129 on the severity of illness., Competing Interests: None

Background: Amyotrophic lateral sclerosis (ALS) is a relatively rare neurological disorder affecting upper and lower motor neurons in the brain and spinal cord with survival for 3-5 years and rarely beyond 10 years. Sleep disturbances in ALS are underreported and undertreated and there is no related data from India. This study aimed to assess the frequency of sleep disorders in patients of ALS and their determinants., Methods: Patients with definite and probable ALS as per the El Escorial criteria were recruited from May 2014 to April 2016. Functional impairment, presence of sleep specific abnormalities and anxiety and depression were assessed using standardized questionnaires., Results: Forty patients with ALS (23 male; 17 female) with their median age at presentation being 58.5 years (range 44-75 years) and the median duration of illness being 18 months (range: 4-120 months) were includedin the study. Half of the patients had poor sleep quality, which was significantly worse across all components of Pittsburgh Sleep Quality Index (PSQI) compared to controls. Sleep disorders were observed in 70%, insomnia in 65%, sleep disordered breathing/hypoventilation in 52.5% and restless legs syndrome in 5% patients. Night time awakenings attributable to symptoms associated with ALS were noted in 85%, and anxiety and depression in 57.5% patients. Excessive daytime somnolence emerged as an independent predictor for the presence of sleep disorders in ALS patients on multivariate logistic regression [P = 0.043, odd's ratio (OR) 1.435; 95% confidence interval[CI] (1.011-2.036)]., Conclusion: This is the first study from India providing insight into the presence of sleep disorders in ALS. About half of the patients of ALS had a poor sleep quality and two-thirds suffered from sleep disturbances., Competing Interests: There are no conflicts of interest

Aims: In amyotrophic lateral sclerosis (ALS), death wish is expressed in a varying proportion of patients in different countries. In this first study from India, influence of belief system of religion/spirituality and attitude towards death, widely prevalent in the country, in decision making, was evaluated., Material and Methods: Twenty ALS patients were assessed using 'Wish-to-Die Questionnaire' (WDQ) developed to reflect seven domains, namely religion/spirituality, belief in karma, meaning of life, hope, family support, financial support and death wish. Functional impairment, depression, hopelessness and suicidal ideation were assessed by ALS Functional Rating Scale, Beck's Depression Inventory, Beck Hopelessness Scale and The Scale of Suicidal Ideation, respectively., Results: On WDQ, all the 20 patients had belief in religion/spirituality, had hope and family support. Nineteen patients (95%) believed in karma, 16 (80%) still found life meaningful and 15 (75%) had financial support. Six patients (30%) had mild to moderate depression; hopelessness was present in 6 (30%) and suicidal ideation was present in one (5%). The 5 (25%) patients who expressed death wish did not significantly differ from others in 6 domains (religion/spirituality, belief in karma, meaning of life, hope, family support, financial support) of WDQ. The main reason in 3 patients who expressed death wish was lack of financial support. The fourth patient could not find meaning of life after the onset of illness, and the fifth wished to end his life since he had satisfactorily fulfilled all his responsibilities., Conclusion: Smaller proportion of patients of ALS expressed death wish in India compared to the Western countries. This may be attributed to belief in religion/spirituality and karma, having meaning of life and family support. As this is the first report from India, useful information may be obtained if similar studies are done on a larger sample.

The characteristic features of Madras motor neuron disease (MMND) are onset in the young in the first two decades, sporadic occurrence, facial and bulbar paralysis, sensorineural hearing impairment, asymmetrical weakness of limbs and pyramidal signs with a slow progression. The majority of the cases reported are from South India. MMND variant has the additional features of optic atrophy and cerebellar signs. We are reporting a 48 year old female of MMND who had persistent fasciculations of chin, with electromyographic features of fasciculations and fibrillations in mentalis muscle. Chin fasciculations, a rare clinical feature, is now described for the first time in Madras motor neuron disease adding a new feature to the clinical constellation of symptoms.

Recognition of the magnitude of the burden and disability and mortality consequent to neurological disorders has led to global initiatives of declaring them as a "global epidemic", emphasizing public health approach and integration of neurology care with general health care. Epidemiological transition with increase in neurological disorders in India, the gross mismatch between the need and the available trained manpower and infrastructural facilities are posing challenges to health planners and policy makers for providing neurological care to the community. Alternative approaches of optimal utilization of rural, community health, satellite clinic and district models of the health care with close interaction with tertiary centres, nongovernmental agencies and private sector may facilitate achieving the goal of taking neurology care to the 'unreached'.

Centronuclear myopathy (CNM), an uncommon condition, is one of the congenital myopathies. It is believed to arise as a result of maturational arrest, with persistence of myotubes postnatally. However, denervation being the basic disease process and its possible influence on central nervous system causing defect in nuclear migration has also been postulated. Keeping in view these existing controversies, we have studied 17 cases of CNM (neonatal - 1, childhood - 13, adulthood - 3) during the last twelve and a half years. Diagnosis was based on histological and enzyme histochemical findings of muscle biopsy along with clinical data. Ultrastructural characterstics of muscle have been studied in 10 cases. The affected muscle fibres showed a central nucleus (40-99%) with perinuclear halo. Type I fibre predominance with hypoplasia was consistently seen. Fibre type disproportion was noticed in 7 cases. The neonatal form revealed dense oxidative enzyme reaction product in the centre. The morphological features of CNM were compared with foetal skeletal muscles obtained at gestational ages ranging from 9 weeks - 36 weeks (n = 18). In the severe neonatal form th myofibres resembled the foetal myotubes. In the less severe childhood and adult form of CNM, aberrant organization of cytoskeletal network might have played a pathogenetic role in causing the disease.

Spinal muscular atrophy constitutes one of the major disease entities amongst infantile neuro-muscular disorders. On the basis of morphological evidence, it had been suggested that the infantile spinal muscular atrophy (ISMA) is due to maturation arrest of the myofibres at 20 weeks gestation. Therefore, in the present study morphological features of skeletal muscles from patients with ISMA was compared with foetal muscle obtained at different gestational ages (9-36 weeks, n=18). Of the 35 cases ofISMA, 22 were diagnosed as having SMA-I and 13 cases an SMA-II, characterised histologically by fascicles composed of groups of small, normal, intermediate sized and hypertrophic fibres. The former ones belonged to both histochemical fibre types, while the hypertrophic fibres in 21/35 cases were type I in nature. Redundant basal lamina was a predominant finding at ultrastructural level. Mature myotubes, a feature seen during foetal muscle development was not noticed in any of the cases of ISMA. Our observations suggest denervation atrophy to be the basic pathogenic mechanism rather than arrest in maturation. This was further supported by the changes seen in the spinal cord specimen of a 20 day old infant from a case of SMA I which revealed marked fallout of motor neurons in the anterior horn, chromatolysis and gliosis. Thus ISMA is a dynamic and progressive neurogenic atrophy secondary to degeneration and loss of spinal motor neurons possibly resulting in lack of trophic factors.

Thirty five floppy children seen during two year period, were subjected to clinical examination, electroneuromyography and muscle biopsy. The muscle biopsy was sent for routine histology, histochemistry and electron microscopy. Using muscle pathology as the 'gold standard' for diagnosis, the aetiological entities were spinal muscular atrophy (16), congenital muscular dystrophy (6), mitochondrial myopathy (3), congenital fibre type disproportion (2), acid mutase deficiency (1) and benign congenital hypotonia (6). Mental subnormality, seizures, ptosis and ophthalmoplegia suggested mitochondrial disease (n=2). Macroglossia, hepatomegaly and cardiomegaly along with the dive bomber effect on electromyography were useful clues to the diagnosis of Pompe's disease (n=1). Positive decremental test established the diagnosis of congenital myasthenia in one patient. Contrary to most previously published reports, infantile onset of spinal muscular atrophy did not always spell a poor prognosis on follow up. 'Floppy infant syndrome' has varied etiology. Comprehensive evaluation including clinical, electrophysiological and detailed histological examination is necessary for proper diagnosis and prognosis of this heterogenous entity.