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Background:Acute hypokalemic paralysis, characterized by acute flaccid paralysis is primarily a calcium channelopathy, but secondary causes like renal tubular acidosis (RTA), thyrotoxic periodic paralysis (TPP), primary hyperaldosteronism, Gitelman’s syndrome are also frequent.Objective:To study the etiology, varied presentations, and outcome after therapy of patients with hypokalemic paralysis.Materials And Methods:All patients who presented with acute flaccid paralysis with hypokalemia from October 2009 to September 2011 were included in the study. A detailed physical examination and laboratory tests including serum electrolytes, serum creatine phosphokinase (CPK), urine analysis, arterial blood gas analysis, thyroid hormones estimation, and electrocardiogram were carried out. Patients were further investigated for any secondary causes and treated with potassium supplementation.Result:The study included 56 patients aged 15-92 years (mean 36.76 ± 13.72), including 15 female patients. Twenty-four patients had hypokalemic paralysis due to secondary cause, which included 4 with distal RTA, 4 with Gitelman syndrome, 3 with TPP, 2 each with hypothyroidism, gastroenteritis, and Liddle’s syndrome, 1 primary hyperaldosteronism, 3 with alcoholism, and 1 with dengue fever. Two female patients were antinuclear antibody-positive. Eleven patient had atypical presentation (neck muscle weakness in 4, bladder involvement in 3, 1 each with finger drop and foot drop, tetany in 1, and calf hypertrophy in 1), and 2 patient had respiratory paralysis. Five patients had positive family history of similar illness. All patients improved dramatically with potassium supplementation.Conclusion:A high percentage (42.9%) of secondary cause for hypokalemic paralysis warrants that the underlying cause must be adequately addressed to prevent the persistence or recurrence of paralysis.

Syphilis can affect any part of the neuraxis. A high index of clinical suspicion is required to diagnose NS and institute the treatment early, particularly in patients with promiscuous sexual behavior.

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Introduction:Major categories of endocrine myopathy include those associated with: Adrenal dysfunction (as in Cushing's disease or steroid myopathy); thyroid dysfunction (as in myxedema coma or thyrotoxic myopathy); vitamin D deficiency; parathyroid dysfunction; and pituitary dysfunction. Steroid myopathy is the most common endocrine myopathy.Objective:To study the etiology, varied presentations, and outcome after therapy of patients with endocrine myopathies.Materials and Methods:Myopathy was evaluated by the standard clinical procedures: Detailed clinical history, manual muscle strength testing, and creatine phosphokinase (CPK). Endocrine disorders were diagnosed as per clinical features and biochemical parameters. The treatment was given to patients as per underlying endocrine disease. Myopathy was assessed before and after treatment.Results:Out of the 37 patients who were diagnosed with endocrine myopathies, thyroid dysfunction was the most common cause (17 cases), followed by vitamin D deficiency in nine, adrenal dysfunction in six, parathyroid dysfunction in three, and pituitary dysfunction in two. Some patients had atypical presentation (repeated falls in one, tongue fasciculations in one, neck weakness in five, one with ptosis and facial weakness, asymmetrical onset in one, and calf hypertrophy in one. The serum creatine kinase (CK) concentration did not correlate with muscle weakness. Following the treatment regimen which was specific for a given myopathy, 26 patients recovered fully.Conclusion:We found varied clinical presentations of endocrine myopathies. All the patients with neuromuscular complaints should be investigated for endocrine causes because significant number of them recovers fully with specific treatment.

Background:The discovery of antibodies against aquaporin-4 and evolving concepts of noncompressive myelopathies in the 21st century have made a major impact on the etiological profile of these diseases, with few cases turning out to be idiopathic.Objective:To find causes of noncompressive myelopathy in a tertiary care hospital of Northeast India.Materials and Methods:An observational study was carried out in the Neurology Department of Gauhati Medical College, Guwahati, from September 2013 to February 2016. Patients of noncompressive myelopathies who underwent magnetic resonance imaging (MRI) of the spine were segregated into two categories: acute-to-subacute myelopathy (ASM) and chronic myelopathy (CM). In addition to routine blood tests, chest X-ray, urinalysis, and visual evoked potentials, investigations included MRI of the brain, cerebrospinal fluid analysis, and immunological, infectious, and metabolic profile based on the pattern of involvement.Results:The study had 151 patients (96 ASM and 55 CM) with a median age of 35 years and male: female ratio 1.4:1. The causes of ASM were neuromyelitis optica spectrum disorder (23), multiple sclerosis (MS) (8), systemic lupus erythematosus (1), Hashimoto's disease (1), postinfectious acute disseminated encephalomyelitis (6), postinfectious myelitis (8), infections (9), spinal cord infarct (5), and electrocution (1). The causes of CM were MS (1), probable or possible sarcoidosis (7), mixed connective tissue disease (1), Hashimoto's disease (2), infections (9), Vitamin B12 deficiency (4), folate deficiency (2), hepatic myelopathy (2), radiation (11), and paraneoplastic (1). No etiology could be found in 48 (31.8%) patients (34 ASM and 14 CM). In 21/96 (21.9%) patients of ASM, acute transverse myelitis was idiopathic based on current diagnostic criteria.Conclusion:Underlying etiology (demyelinating, autoimmune, infectious, vascular, metabolic disorder, or physical agent) was found in 68% patients of noncompressive myelopathy.

Introduction:Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs), anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs), accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India.Objectives:To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India.Material and Methods:Relevant investigations were done to rule out other causes of moyamoya syndrome.Results:We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA) in addition to stenosis of bilateral ICAs, ACAs and MCAs.Conclusion:Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.

Here we present the case of a 17-year-old girl belonging to an ethnic tribe (Bodo tribe) of Assam, presenting with bilateral cerebellar signs and with history suggestive of an autosomal dominant pattern of inheritance, who was found to have spinocerebellar ataxia 7 on genetic testing. This case throws light on the probability of more such cases in the multi-ethnic society of the North-Eastern Indian states, which are not studied or reported till date.