Metabolic encephalopathy (ME) represents a syndrome of temporary or permanent disturbance of brain functions that occurs in different diseases and varies in clinical presentation. It can be manifested in a range from very mild mental disorders to deep coma and death. Clinically, it is characterized by a variety of psychiatric and neurological symptoms and signs. The most common causes of ME are: hypoxia, ischemia, systemic diseases and toxic agents. ME is the most frequent in elderly people who have previously been exhausted by chronic illnesses and prolonged stay in bed. ME is a very common complication in patients treated in intensive care units. Treatment and prognosis of the disease are varied and depend on aetiology, as well as on the type and severity of clinical presentation. Mortality of patients with septic encephalopathy ranges from 16-65%, while the one-year survival of patients with encephalopathy and liver cirrhosis is less than 50%.

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Bangur Institute of Neurosciences is one of India's oldest teaching institutions in the field of neurosciences. It has contributed richly over four decades in training and research in Neurology and Neurosurgery. Situated in Kolkata, the City of Joy and the Cultural Capital of India, and run under the aegis of the Government of West Bengal's Ministry of Health and Family Welfare, it remains one of the highest-volume neurological and neurosurgical centers in the country. This is a humble attempt to illustrate the history of this Institute and to provide a vignette of the many illustrious neurologists and neurosurgeons who were intimately involved in the setting up practice of Clinical Neurosciences in Eastern India.

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Parkinson's disease is a common movement disorder seen in neurological practice, but the diagnosis and management is challenging. The diagnosis is clinical and sometimes difficult, considering a large number of motor and non-motor symptoms in PD patients. The medical management of PD patients is difficult, as choices of drugs are limited and levodopa is the mainstay of treatment. However, levodopa-induced dyskinesia (LID) is commonly seen in Parkinson's disease patients treated with levodopa. This side effect is usually encountered after a long duration of treatment, but occasionally, this may be seen even after a few days or months of treatment. Different types of surgical approaches, including unilateral pallidotomy and deep brain stimulation, have given very good results in PD patients, who cannot be managed by medications alone.

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In 1896, Joseph Babinski, a French neurologist, first described the best known neurologic eponym 'the Babinski sign'. This sign is characterised by dorsiflexion of the big toe and recruitment of the extensor hallucis longus muscle, on stimulating the sole of the foot. He has emphasised from the outset, the intimate relationship between this sign and the shortening movement in other leg muscles, which form the flexion synergy of the lower limb. The Babinski sign is not a new reflex, rather it is released as a result of breakdown of the harmonious integration of the flexion and extension components of the normal defence reflex mechanism, due to pyramidal tract dysfunction. A pathological Babinski sign should be clearly distinguished from upgoing toes that may not always be a part of the flexion synergy. This article reviews the Babinski sign in detail, focusing on the historical perspectives, role of pyramidal tract dysfunction and art of elicitation and interpretation. The significance of assessing this phenomenon in the entire leg, and the clinical clues that will help to dispel the myths regarding the Babinski sign, have been emphasised.

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Organophosphorous compounds, the anticholinesterases, produce significant morbidity and mortality in India. Although exact estimates are not available, hospital based statistics suggest that nearly half of the admissions to emergency with acute poisoning are due to organophosphates. Following accidental or suicidal exposure, these anticholinesterases lead to three well defined neurological syndromes i.e. initial life threatening acute cholinergic crisis which often requires management in intensive care unit, intermediate syndrome in which cranial nerve palsies, proximal muscle weakness and respiratory muscle weakness are common and patients often require respiratory support and delayed organophosphate induced polyneuropathy. In addition to these three classical neurological syndromes following acute exposure and in some following low dose chronic exposure, several neurobehavioural changes have been observed and these have been termed together as 'chronic organophosphate induced neuropsychiatric disorders' (COPIND). Organo-phosphate compounds produce significant pesticide related illness in developing countries. There is, thus, a need to determine exact extent of the problem and to develop appropriate strategies to manage these cases with available resources in these countries.

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The history of the prestigious Department of Neurosurgery, Gobind Ballabh Pant Institute of Medical Education Institute and Research, New Delhi, a leading tertiary care centre, is presented. Its eminent faculty and outstanding patient care attracts patients and students from all over the country.The patients opt for this institution to get a standard of care that may be comparable with the highest standards prevalent; and, the students achieve their goal of getting excellent education in Neurosurgery at par with the best institutes of the world. The department has, therefore, over the years, established its place in the country as a premier training facility and an epitome of medical excellence.

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Vertigo is a common symptom in everyday clinical practice. The treatment depends on the specific etiology. Vertigo may be secondary to inner ear pathology, or any existing brainstem or cerebellar lesion but may also be psychogenic. Central vertigo is a consequence of a central nervous system lesion. It is often associated with a focal neurological deficit. Peripheral vertigo is secondary to dysfunction of the peripheral vestibular system and is usually characterized by an acute vertigo with loss of balance, sensation of spinning in the space or around self, and is exaggerated with changes of the head and body position; no other neurological deficit is present. Some medications may also cause vertigo. Depending on the cause of the vertigo, drugs with different mechanisms of action, physical therapy, psychotherapy, as well as surgery may be used to combat this disabling malady. Symptomatic treatment has a particularly important role, regardless of the etiology of vertigo. We reviewed the current medications recommended for patients with vertigo, their mechanisms of action and their most frequent side effects.

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Head injury has been the leading cause of death and disability in people younger than 40 years and the incidence is rising continuously. Anticipation of the pathological consequences of post-traumatic subarachnoid hemorrhage (tSAH) and an outcome-oriented management are very important in these cases. To encounter the complications pertaining to traumatic brain injury (TBI) and tSAH, various classifications have been proposed and goal-oriented screening strategies have been offered. The role of serial computed tomography (CT) scans, perfusion studies, transcranial Doppler, magnetic resonance imaging (MRI), and angiographic studies as diagnostic tools, has been described. Recently, MRI fluid-attenuated inversion recovery (FLAIR), gradient reversal echo (GRE), and susceptibility weighted imaging (SWI) have emerged as excellent complimentary MRI sequences, and the authors of this article have evaluated their role in the diagnosis and prognostication of patients with tSAH. Numerous studies have been conducted on the various complications associated with tSAH such as vasospasm, hydrocephalus, and electrolyte disturbances and their management. This article discusses these aspects of tSAH and their management nuances.

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Airplane travel headache is rare and has recently been described as a new form of headache associated with a specific situation. Of the 1,208 patients with primary headaches attending a tertiary care neurology hospital, two (0.16%) patients satisfied the criteria for headache related to airplane travel. Both the patients fulfilled the proposed diagnostic criteria for airplane travel headache. This unique headache had a mean duration of 24 minutes, localized to the medial supraorbital region described as having an intense jabbing or stabbing character that occurred exclusively and maximally during aircraft landing or take-off, following which pain intensity subsided . This rare headache felt on aircraft descent is probably due to the squeeze effect on the frontal sinus wall, when air trapped inside it contracts producing a negative pressure leading to mucosal edema, transudation and intense pain. Use of nasal decongestants either alone or in combination with naproxen sodium prior to ascent and descent abated the headache episodes. Awareness about this unique entity is essential to provide proper treatment and avoid patient suffering.

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Botulinum toxins are, as a group, among the most potent neuromuscular toxins known, yet they are clinically useful in the management of conditions associated with muscular and glandular over-activity. Botulinum toxins act by preventing release of acetylcholine into the neuromuscular junction. While botulinum toxin type A is commonly available, different manufacturers produce specific products, which are not directly interchangeable and should not be considered as generically equivalent formulations. Type B is also available in the market. Each formulation of botulinum toxin is unique with distinct dosing, efficacy and safety profiles for each use to which it is applied. Botulinum toxin type A is the treatment of choice based on its depth of evidence in dystonias and most other conditions. Botulinum toxin type A is established as useful in the management of spasticity, tremors, headache prophylaxis and several other neurological conditions. Active research is underway to determine the parameters for which the type B toxin can be used in these conditions, as covered in this review. Botulinum toxin use has spread to several fields of medicine.

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Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease of the central nervous system. The true incidence of the disease in India is undetermined and is likely to be more frequent than reported, as the common antecedent events, exanthematous fevers and Semple antirabies vaccination, which predispose to ADEM, are still prevalent. The existing evidence suggests that ADEM results from a transient autoimmune response towards myelin or other self-antigens, possibly via molecular mimicry, or by non-specific activation of auto-reactive T cell clones. ADEM is a monophasic illness with favourable long-term outcome. Involvement of neuroaxis is variable and can be diffuse or multifocal and site restricted. Magnetic resonance imaging (MRI) is highly sensitive in detecting white matter lesions and the lesions described are rather extensive and subcortical in location. Involvement of the deep gray matter, particularly basal ganglia, is more frequent. Oligoclonal bands in CSF are usually absent. No therapy has been established by controlled trials in ADEM. Use of high-dose methylprednisolone, plasma exchange, and IVIG are based on the analogy of the pathogenesis of ADEM with that of multiple sclerosis (MS). Differentiation of ADEM from the first attack of MS is important from prognostic as well as therapeutic point of view. However, in the absence of biological marker, at times differentiation of ADEM from the initial presentation of MS may not be possible even by combination of clinical, CSF analysis, and MRI. This differentiation is more relevant to India where the incidence of MS is low.

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The Department of Neurological Sciences at Christian Medical College (CMC), Vellore was the first department to start neurosurgical and neurological services in India. Jacob Chandy started the department in 1949 against several odds. He started a formal training program in neurosurgery in 1958, for the first time in India, and went on to qualify several neurosurgeons, who in turn pioneered neurosurgical departments all over India. After 1970, K V Mathai and Jacob Abraham guided the department through some difficult times when there was a severe shortage of personnel and no faculty in the neurology section. Through their commitment and hard work, they continued not only the neurosurgery service but also looked after patients with neurological disorders. Mathew J Chandy, son of Jacob Chandy, joined them in 1980 and introduced micro-neurosurgery and several other neurosurgical techniques. Training of residents in micro-neurosurgery began in the early 1980s. The last quarter of a century has been a period of rapid progress for neurosurgery at CMC. There has been an exponential rise in the number of surgeries, number of residents and number of publications. Research has always been an integral part of the activities of the department and several high impact articles have been published by the faculty and residents. The neurosurgical faculty at CMC has also contributed significantly to organized neurosurgery in India and internationally, with five of them serving as President of the Neurological Society of India, a society which had Jacob Chandy as its founder President. With this heritage, the neurosurgery section at CMC, Vellore is likely to continue to provide high quality ethical neurosurgical care to patients from all over India and overseas.

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Background: Autism spectrum disorder (ASD) is a developmental disability and is of public health importance. It affects not only the child and the family. It also has direct and indirect cost implications on the nation that are incurred in providing health care, support for education, and rehabilitative services. There is a lack of evidence-based estimate of the population prevalence of ASD in India. Therefore, this systematic review was aimed at determining the prevalence of ASD in the Indian population. Materials and Methods: We conducted a systematic review and meta-analysis of the published studies evaluating the prevalence of ASD in the community setting. A search within the published literature was conducted from different databases (PubMed, OvidSP, and EMBASE). The analysis of data was done using STATA MP12 (StataCorp, College Station, TX, USA). Results: Four studies were included in this systematic review. Of the four included studies, one had studied both urban and rural populations, and the other three had studied the urban populations only. The study from the rural setting showed a pooled percentage prevalence of 0.11 [95% confidence interval (CI) 0.01–0.20] in children aged 1-18 years; and, four studies conducted in the urban setting showed a pooled percentage prevalence of 0.09 (95% CI 0.02–0.16) in children aged 0-15 years. Conclusion: The scarcity of high-quality population-based epidemiological studies on ASD in India highlights an urgent need to study the burden of ASD in India. The proper acquisition of data related to the prevailing burden of ASD in India would lead to a better development of rehabilitative services in our country.

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Duchenne muscular dystrophy (DMD), an X-linked disorder, is the most common muscular dystrophy in children, presenting in early childhood and characterized by proximal muscle weakness and calf hypertrophy in affected boys. Patients usually become wheelchair-bound by the age of 12 years, and die of cardiorespiratory complications in their late teens to early twenties. Advances in the management of DMD, including treatment with corticosteroids and the use of intermittent positive pressure ventilation have provided improvements in function, ambulation, quality of life and life expectancy, although novel therapies still aim to provide a cure for this devastating disorder. The clinical features, investigations, and management of DMD are reviewed, as well as the latest in some of the novel therapies.

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History helps us to become better students, judge wisely, understand change, and most importantly, it tells us who we are. It helps us to understand what happened, why it happened and what its ramifications are. Winston Churchill once said: “Study history, study history. In history lie all the secrets of statecraft.” Here, we take this opportunity to pay our gratitude to our esteemed teachers who worked relentlessly for uplifting of the department of Neurology, PGIMER, Chandigarh; and, narrate chronicles of all those people who made this department reach the heights where it stands today.

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We report a family of three siblings with Childhood Ataxia with Cerebral Hypomyelination. All the siblings presented with early onset cerebellar ataxia beginning around five years of age with mild mental retardation. MRI showed diffuse white matter signal changes in all three patients with cerebellar atrophy while the spectroscopy was abnormal only in the eldest who was the most severely affected. The cases are reported for their rarity as well as for an opportunity of observing this uncommon disease in its stages of evolution in three siblings.

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The Kluver-Bucy syndrome (KBS) is a neurobehavioral syndrome and can be seen in association with a variety of neurological disorders. Case records of 6 patients with KBS seen during a period of 5 years in a university hospital were reviewed. During the study period 6 patients with KBS, aged between 4 and 14 years, were seen. Hyperorality, hypersexuality, and abnormal behavior were the most common manifestations. Of the 6 patients, 5 had recurrent unprovoked seizures. The associated neurological disorders included anoxia-ischemic encephalopthy (2), herpes simplex encephalitis (1), neurocysticercosis (NCC) (1), traumatic brain injury with gliosis (1 case) and tuberculous meningitis (1 case). Prognosis was poor in all the patients except in the patient with NCC.

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Nerve root cysts of the sacral region are generally asymptomatic but are known to cause neurogenic claudication. In this paper we present an elderly lady who presented with claudication, whose MR imaging showed a sacral cyst. She underwent lumbo-sacral laminectomy, partial excision of the cyst wall with plication. A review of the possible pathophysiology of such a lesion is discussed.

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Parkinsonism plus syndrome is a group of heterogeneous degenerative neurological disorders, which differ from the classical idiopathic Parkinson’s disease in certain associated clinical features, poor response to levodopa, distinctive pathological characteristics and poor prognosis. Associated clinical features include symmetrical onset, infrequent or atypical tremor, prominent rigidity in axial musculature, bradykinesia, early postural instability, supranuclear gaze palsy, early autonomic failure, pyramidal affection, cerebellar involvement, alien limb phenomenon, apraxia and significant early cognitive dysfunction in some cases. Progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and dementia with Lewy body disease (DLB) are commoner disorders. Less frequent disorders are cortico-basal ganglionic degeneration (CBGD), frontotemporal dementia with chromosome 17 (FTDP-17), Pick’s disease, parkinsonian-dementia complex of Guam, Pallidonigral degeneration, Wilson’s disease and a rigid variant of Huntington’s disease. During the last 3 decades, major progress has been made in understanding PSP, CBGD and FTDP-17, which are tau disorders. MSA and DLB together with idiopathic Parkinson’s disease are called a-synucleinopathies. Recent studies show that the diagnosis of these Parkinsonism plus syndromes improves when strict diagnostic criteria are used. However, unusual presentations may pose a diagnostic challenge. The shortcomings of the current studies demand the need for further research to identify biologic markers that may allow earlier diagnosis, and understanding of the factors leading to a-synuclein or tau aggregation. Identification of therapeutic strategies that may prevent the aggregation of these proteins and rescue dysfunctional cells has been stressed. This review focuses on the advances in the clinical, neuroimaging, pathologic, genetic and management aspects of these disorders.

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We present an interesting case of sacral perineural cyst which caused chronic perineal pain. Perineural cyst is relatively rare, especially the sacral region. Chronic perineural pain is an often encountered problem that is difficult to evaluate and sacral perineural cyst may be the etiology of chronic perineal pain in many instances.

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The present report highlights an unusual presentation of vitamin B12 deficiency— recurrent seizures in a 26-year-old man. His symptoms responded to parenteral vitamin B12 therapy. The relevant literature is reviewed.

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Vertebral artery dissection is an important cause of brain stem stroke, especially in the young. Dissections of carotid and vertebral arteries in neck account for about 20% of strokes in young compared with 2.5% in the elderly. Three patients of vertebral artery dissection related to indirect neck trauma are described. The first patient developed the symptoms while dancing, the second after a trivial fall and the third while he was on a dental chair. None of them had a direct severe neck trauma or concomitant risk factor like hypertension, connective tissue disease or migraine. Clinical symptomatology was similar in all the patients and included occipito-nuchal pain, headache and brain stem dysfunction chiefly in the posterior inferior cerebellar artery (PICA) territory. One of the patients also had associated ischaemic myelopathy. MRA and DSA confirmed dissection in all with a predominant steno-occlusive picture. Cases of so called trivial neck movement/torsion related dissection have been described previously but have not received any major importance. Usually classified as 'spontaneous' or 'traumatic', there is a possible ambiguity in literature about appropriate terminology. We emphasise that a history of such subtle precipitating events be taken while diagnosing young patients with brain stem strokes, to recognise this clinical entity. Although mechanisms are not absolutely clear, yet there seems to be an important relationship between arterial dissection and neck movements or minor trauma.

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Thirteen consecutive cases with symptomatic vertebral hemangiomas, managed during a five year period from January 1995 at the Christian Medical College and Hospital, Vellore, were analysed. Twelve patients had lesions in the thoracic and 1 in the sacral region. Eight patients had multiple level involvement. Seven patients had laminectomy and soft tissue component excision, of which one had intraoperative injection of absolute alcohol and one had postoperative radiotherapy. One patient had vertebrectomy and stabilization with preoperative embolization. One patient underwent only endovascular embolization. However, the focus of this communication is on 4 patients who underwent a CT guided percutaneous transpedicular injection of absolute alcohol into the affected vertebral body. In the surgical group, 6 patients had cavernous type and 2 patients had mixed type of hemangiomas. Ten patients improved on the Ranawat grade by the time of discharge. On the MRC grade, 11 patients had improved, one was grade 5 pre and postoperative, while one did not improve. Several options are available for the management of symptomatic vertebral hemangiomas and multiple modalities may have to be used for a single patient. CT guided percutaneous transpedicular injection of absolute alcohol shows promising results. However, long term follow up is mandatory.

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COVID-19, in most patients, presents with mild flu-like illness. Elderly patients with comorbidities, like hypertension, diabetes, or lung and cardiac disease, are more likely to have severe disease and deaths. Neurological complications are frequently reported in severely or critically ill patients with comorbidities. In COVID-19, both central and peripheral nervous systems can be affected. The SARS-CoV-2 virus causes the disease COVID-19 and has the potential to invade the brain. The SARS-CoV-2 virus enters the brain either via a hematogenous route or olfactory system. Angiotensin-converting enzyme two receptors, present on endothelial cells of cerebral vessels, are a possible viral entry point. The most severe neurological manifestations, altered sensorium (agitation, delirium, and coma), are because of hypoxic and metabolic abnormalities. Characteristic cytokine storm incites severe metabolic changes and multiple organ failure. Profound coagulopathies may manifest with ischemic or hemorrhagic stroke. Rarely, SARS-CoV-2 virus encephalitis or pictures like acute disseminated encephalomyelitis or acute necrotizing encephalopathy have been reported. Nonspecific headache is a commonly experienced neurological symptom. A new type of headache “personal protection equipment-related headache” has been described. Complete or partial anosmia and ageusia are common peripheral nervous system manifestations. Recently, many cases of Guillain-Barré syndrome in COVID-19 patients have been observed, and a postinfectious immune-mediated inflammatory process was held responsible for this. Guillain-Barré syndrome does respond to intravenous immunoglobulin. Myalgia/fatigue is also common, and elevated creatine kinase levels indicate muscle injury. Most of the reports about neurological complications are currently from China. COVID-19 pandemic is spreading to other parts of the world; the spectrum of neurological complications is likely to widen further.

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Hydrocephalus is one of the commonest complications of tuberculous meningitis (TBM) occurring in up to 85% of children with the disease. It is more severe in children than in adults. It could be either of the communicating type or the obstructive type with the former being more frequently seen. The Vellore grading system for clinical grading of patients with TBM and hydrocephalus with grade I being the best grade and grade IV being the worst grade has been validated by several authors. The management of hydrocephalus can include medical therapy with dehydrating agents and steroids for patients in good grades and those with communicating hydrocephalus. However, surgery is required for patients with obstructive hydrocephalus and those in poor grades. Surgery can involve either a ventriculo-peritoneal shunt or endoscopic third ventriculostomy (ETV). Complications of shunt surgery in patients with TBM and hydrocephalus are high with frequent shunt obstructions and shunt infections requiring repeated revisions. ETV has variable success in these patients and is generally not advisable in patients in the acute stages of the disease. Mortality on long-term follow up has been reported to vary from 10.5% to 57.1% in those with altered sensorium prior to surgery and 0 to 12.5% in patients with normal sensorium. Surgery for patients in Vellore grade IV is usually associated with a poor outcome and high mortality and therefore, its utility in these patients is debatable

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