Shubhankar Mishra

Abstract

Background: The Idiopathic inflammatory demyelinating diseases of Central nervous system (IIDD) are common neurological disorders in neurology indoor. CNS demyelinations which affect brain, spinal cord and optic nerve are important neurological disorder in view of multi axial involvement and frequent relapse and causing significant morbidity. Materials and Methods : It is a retrospective study done in a tertiary care hospital in eastern India from Aug2018-jul2019 (one year). Patients presenting with paraplegia or paraparesis, quadriplegia or quadriparesis with MRI showing features of  demyelination were included in the study. Different criteria was taken for diagnosis to know the etiology of disease (mentioned in the introduction). Statistical Analysis Done Using SPSS Software Version-21. Result: Among all spinal cord demyelination longitudinally extended transverse myelitis (LETM) was more common than short segment demyelination. In our study >40yrs group is most commonly affected with more female preponderance. Serum AQP4-Ab was positive in 28% of LETM patients. EDSS was higher in LETM patients than non LETM patients. Conclusion: Among all spinal cord demyelination LETM is more common than short segment demyelination. Optic neuritis and multiphasic course was more associated in LETM with AQP4 positive NMOSD. Tuberculosis is a cause of LETM. Therefore, patients presenting with LETM require a thorough investigative workup to exclude treatable causes like infectious, inflammatory or metabolic. Key message: Spinal cord demyelination possess a wide range with multiple etiologies. Disease course, treatment and prognosis varies in different conditions. All spinal cord demyelination should be evaluated in detail to prevent frequent relapse and morbidity As spinal cord biopsy is an invasive procedure with significant morbidity MRI lesion location, length and enhancement pattern will help to narrow the possibilities. Keywords: LETM, NMOSD, AQ4AB, MOG AB, Demyelination.  

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Introduction: Friedreich Ataxia (FA) is an autosomal recessive inherited form of ataxia. Clinical presentation includes cerebellar and sensory ataxia, associated with pyramidal signs, an absence of tendon reflexes in the legs, deep sensory loss, and foot deformity. Aims and Scope: To know the hospital-based incidence, epidemiological profile of FA. To know the commonest mode of presentation. To watch for the complications. To observe its radiological features along with electrophysiological properties. Settings and Design: Prospective clinical study in the department of neurology of S.C.B Medical College, Cuttack, Odisha, India. The study was done for 2 complete years. Material and Methods: All patients who presented with clinical symptoms of FA with positive for allelic mutation were included in the study. The patients were evaluated in detail by neuroimaging and other tests. Brain and spinal cord MRI were done in all the patients followed by nerve conduction. Ethical approval was obtained from institutional ethical committee. Statistical analysis used: SPSS version 21.0 Results: Total numbers of patients were 12. Male to female ratio was 2:1. Most common presentation was unsteadiness of gait and other cerebellar signs. 25% patients (3/12) had ejection fraction reduced. Optic nerve atrophy was found in approximately 25% of individuals. Atrophy of the cervical spinal cord and cerebellum were observed in 58% (7/12) cases in MRI. Conclusions: FA is one of the commonest recessive ataxias of young people. It presents progressive ataxia and large fibre neuropathy with multisystem involvement. Usually it has very bad prognosis with cardiac complications. Key Messages: FA is one of the common cause of genetic ataxia. It is a multisystem disease with involvement of both central nervous system and peripheral nervous system. The knowledge of FA is necessary for early diagnosis and genetic counselling. Keywords: Ataxia, myeloneuropathy, friedreich’s, frataxin

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Objective and Design: Snakebite is a common hazard in Indian paediatric population. The objectives of our study was to find out the incidence of neurotoxic snakebite, to evaluate the clinical profile of neurotoxic snake bites along with outcome after treatment. Material and Methods: A hospital based study including all cases of neurotoxic snakebite below 14 years admitted to department of paediatrics, M.K.C.G M.C.H, Berhampur. Patients were diagnosed by clinical history along with signs and symptoms of envenomation and history of Snakebite during the study period from OCT 2014- SEP 2018. Management was done according to the WHO criteria. The various features and complications encountered during the case management were noted down in the format designed for study. Study Setting: Dept. of paediatrics M.K.C.G M.C.H, Berhampur, Ganjam Results: Total numbers of enrolled cases were 113. Out of them highest incidence of bites were seen in September (15.3%). Males and age group of 10-14 years were mostly attacked. Most frequent site of bite was leg. Most common identified snake was krait (6 0.7%). Commonest time of hospitalisation is 3-6 hrs after the bite. Ptosis was the most common manifestation. 30.3% of admitted children were having abnormal CRT whereas 7% were having abnormal BT/CT. 38.7% were treated with ASV and 22% by neostigmine with atropine. 23% patients were having complications with local gangrene being the most frequent one. 3.9% children died in my study most of them were in 10-14 age group. Maximum deaths are due to cobra bite. Conclusion: Early hospitalisation, abstinence from age old local practices, judicious use of ASV, aggressive management by neostigmine and atropine in diagnosed cases of neurotoxic snakebite and extensive treatment of severe complications by the WHO guidelines can lead to decreased mortality. Keeping the cost benefit ratio and burden of snakebite in mind the ASV should be used when indicated. Keywords: Snakebite, WHO, ASV, Cobra

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Introduction: Idiopathic inflammatory demyelinating disorders of the central nervous system (IIDCDs) represent a broad spectrum of central nervous system (CNS) disorders thought to be of autoimmune origin. Objective of the study was to diagnose multiple sclerosis by neurological examinations, to do neuroimaging and to see the most common area of involvement. Materials and Methods: Prospective clinical study in Department of Neurology, S.C.B. Medical College & Hospital, Cuttack from January 2017 to December 2018 (2 years). All cases with diagnosis of multiple sclerosis who fulfilled the recent mcdonald criteria were included in the study. The clinical and epidemiological data were recorded. Results: Incidence of MS among the IIDDs were 18(14.7%). Male: female ratio was 3.5:1. Periventricular involvement was most common site. FLAIR sequence of MRI was best tool for detection. Conclusion: Neuroimaging is best modality to diagnose MS. Periventricular lesions are most common. Keywords: MRI, MS, Periventricular, Mc Donald.

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