It is a great pleasure to be asked to honour the memory of Dr. Baldev Singh by reviewing the field of autoantibodies in myasthenia gravis and other neurotransmission disorders. The neuromuscular junction (NMJ) is the site of a number of different autoimmune and genetic disorders, and it is also the target of many neurotoxins from venomous snakes, spiders, scorpions and other species. The molecular organization of the NMJ is graphically represented in [Figure 1A], where different ion channels, receptors and other proteins are shown. Four of the ion channels or receptors are directly involved in autoimmune diseases. This brief review will not only concentrate on these conditions but also illustrate how their study is helping us to understand the etiology of rare but treatable neurological syndromes of the central nervous system.

The global workspace of consciousness was proposed in its elementary framework by Baars, in 1982. Since the time of inception, there have been many speculations and modifications of this theory, but the central theme has remained the same, which refers to the global availability of information in the brain. However, the present understanding about the origin of this global workspace or its mechanism of operation is still deficient. One of the less-studied candidates for this global workspace is the electromagnetic field of the brain. The present work is a brief review of the theoretical underpinnings of the Global workspace model, in terms of its theoretical framework and neuroimaging evidences. Subsequently, we turn towards another broad group of theories of consciousness, in the form of electromagnetic field theories. We then proceed to highlight some electromagnetic correlates derived from these theories for this global access phenomenon.

Background: The aim of this paper is to trace the nucleotide alterations within the D-loop region of the mitochondrial DNA, affecting both the mtDNA ability to replicate and the transcription activity of the coding genes located in the H and L threads, in Caucasian patients with an ischemic and hemorrhagic brain strokes. Materials and Methods: The DNA from the peripheral blood of 85 patients with recent sustained ischemic and primary hemorrhagic brain stroke was analysed. The control group consisted of 24 volunteers. The genetic studies were conducted by the PCR method, with the application of the primers for the tRNA-treonine. Results: In the blood samples examined, 3-striatal mtDNA patterns were detected. Pattern-1 is characterised by the C16126T substitution, pattern-2 by the G16319A substitution, and pattern-3 by the C16242T substitution. The frequency of occurrence for the particular mtDNA-1, -2,and -3 patterns, established for the group with an ischemic stroke (77.3, 15.2, and 7.6%), the group with a hemorrhagic stroke (0, 73.7, and 26.3%), and the control group (75, 0, and 25%), differs significantly. Discussion: The exchange of the nucleotides within the D-loop region may affect both the mtDNA replication ability and the transcription activity of the coding genes located in the H and L threads. A hypothesis might be made. The G16319A mutation may result in the formation of lesions within the vascular wall. These lesions have a tendency to form microaneurysms or other defects, which, in turn, will decrease the strength of the vascular wall, making it more susceptible to ruptures. Conclusion: The G16319A substitution may be considered a factor that increases the risk of a hemorrhagic brain stroke.

Objective: The aim of this study is to present a comprehensive profile of clinical and psychosocial characteristics of children with psychogenic nonepileptic seizures and to assess the short-term outcome of these patients. Materials and Methods: The subjects were consecutive cases of children with a diagnosis of nonepileptic seizures (N=17, mean age = 10.7 years, S.D. = 1.26) and two groups of control groups matched on age and sex: true seizure group and healthy controls. All the children were recruited from the out-patient services of the Department of Pediatrics of a tertiary care teaching hospital in North India. Detailed history taking and clinical examination was done in the case of every child. A standard 18 channel EEG was done in all the children and a video EEG was done in 12 cases of children with nonepileptic seizures. The Childhood Psychopathology Measurement Schedule (CPMS) and Life Events Scale for Indian Children (LESIC) were used to measure the children's emotional and behavioral functioning at home, and the number of life events and the stress associated with these events in the preceding year and the year before that. Short-term outcome was examined three to six months after the diagnosis of nonepileptic seizures was made. Results: Unresponsiveness without marked motor manifestations was the most common "ictal" characteristic of the nonepileptic seizures. Pelvic thrusting, upper and lower limb movements, head movements, and vocalization were observed in less than one-third of the patients. Increased psychosocial stress and significantly higher number of life events in the preceding year were found to characterize children with nonepileptic seizures, as compared to the two control groups. The nonepileptic seizures and true seizures groups had a higher proportion of children with psychopathology scores in the clinically significant maladjustment range, as compared to those in the healthy control group. A majority of the patients (82.4%) either recovered completely or had more than 50% reduction in the frequency of their symptoms, after three to six months of initiation of therapy. Conclusions: Psychosocial stress is common among children with nonepileptic seizures. Confirmatory diagnosis by video EEG, along with prompt psychosocial intervention, often results in a favorable outcome for most children with nonepileptic seizures.

Context: Headache patients commonly report sleep disruption and sleep disorders. Available literature suggests that the sleep pattern of headache sufferers is different from the control group. Patients in these studies were recruited from headache clinics; they did not include tension type headache. Aims: The aim of this study is to find out whether primary headaches affect sleep patterns. Settings and Design: Community based cross sectional study Materials and Methods: This study was conducted in three high schools. Children in the 12-19 age group were allowed to participate. They were given a questionnaire in the presence of at least one of the authors, who assisted them in filling it. They were asked to provide responses based on most severe recurrent headache that they had experienced rather than the more frequent ones. The questionnaire included questions regarding demographic data and the characteristics of headache according to International Classification of Headache Disorders-2 criteria. Part B of the questionnaire contained questions regarding sleep habits. The children were asked to provide data regarding sleep habits on a normal school day. Diagnosis was based upon the information contained in the questionnaire. A telephonic interview was also done, where the information provided was found inadequate. Statistical Analysis Used: Analysis was done with the help of SPSS v. 11.0., descriptive analysis, Chi square, and one way ANOVA with post hoc analysis. Kruskall-Wallis tests were run. Results: A total of 1862 subjects were included in the study. Migraineurs and tension type headache sufferers comprised 35.7% and 13.4% of the group respectively. Migraineurs had the highest prevalence of nocturnal awakenings ( P < 0.001), abnormal movements ( P =0.001) and breathing problems during sleep ( P < 0.001). Approximately half the migraineurs felt sleepy during the day ( P < 0.001) and spent around 1.17 hours in sleep during the day ( P = 0.007). Similarly, values for frequency of nocturnal awakenings per week ( P < 0.001), wake time after sleep onset and offset ( P < 0.001 and 0.002 respectively) were the maximum in migraineurs. Only 32.8% migraineurs reported refreshing sleep ( P < 0.001). Post hoc analysis revealed that migraineurs were different from the other two groups on most of the parameters. Conclusions: Sleep disruption is more common in migraineurs than those in the tension type headache sufferers and the control group.

Background: Atherosclerotic carotid artery disease poses a grave threat to cerebral circulation, leading to a stroke with its devastating sequelae, if left untreated. Carotid endarterectomy has a proven track record with compelling evidence in stroke prevention. Objectives: a)To confirm that carotid endarterectomy (CEA) is safe and effective in preventing stroke at both short and long term. b) to demonstrate long term patency of internal carotid artery when arteriotomy repair is performed using autologous saphenous vein patch. Materials and Methods: During ten years, from September 1997 to February 2008, thirty nine patients who underwent consecutive carotid endarterectomy at our institute, form the basis of this report. Their age ranged from thirty to seventy eight years, with a mean age of 56. There were four women in this cohort. Thirty seven patients were symptomatic with >70% stenosis and two were asymptomatic with >80% stenosis, incidentally detected. Imaging included Duplex scan and MRA for carotid territory and brain, and non-invasive cardiac assessment. Co-morbidities included smoking, hypertension, diabetes, and coronary artery disease. Carotid Endarterectomy was performed under general anaesthesia, using carotid shunt and vein patch arteriotomy repair. Results: All the patients made satisfactory recovery, without major adverse cerebral events in this series. Morbidities included Transient Ischemic Attack (TIA) in two, needing only medications in one, and carotid stenting in the other. Minor morbidities included neck hematoma in two and transient hypoglossal paresis in three patients. Yearly follow-up included duplex scan assessment for all the patients. Two patients died of contralateral stroke, two of myocardial events and two were lost to follow up. Thirty three patients are well and free of the disease during the follow up of three to 120 months. Conclusion: Carotid endarterectomy provided near total freedom from adverse cerebral events and its catastrophic sequelae, leading to excellent outcome, both short as well as long term.

Cirrhosis presenting as Parkinsonism is a distinct subset of acquired chronic hepatocerebral degeneration. The entity is not rare, and unless suspected, cirrhosis can easily be overlooked. We report our experience with three such patients. They presented to us, over a period of two years, with symmetrical Parkinsonism and were later diagnosed to have cirrhosis with portal hypertension. All patients had minimal or absent tremors. Reversal of serum albumin to globulin ratio and evidence of cirrhosis on abdominal ultrasound were consistent. All three patients had the characteristic MRI abnormality of symmetrical T1 hyperintensity in basal ganglia and anterior midbrain. They improved to variable extents after treatment for cirrhosis, along with dopa agonists. We stress the importance of recognizing this syndrome and briefly review the relevant literature.

Fabry's disease, an X linked recessive disorder caused by the deficiency of a-galactosidase A (a-gal A), leads to progressive accumulation of glycosphingolipids. We report this rare disease in a 19-year-old boy who presented with angiokeratomas, paresthesia and corneal opacities, and nerve biopsy revealed by electron microscopy lamellated inclusions in the smooth muscle, perineurial and endothelial cells characteristic of Fabry's disease.

Giant cell arteritis (GCA) is a common disease of the geriatric age group in the western world, with a prevalence of 0.2% in the fifty plus age group. It is an important cause of morbidity, with irreversible visual loss being the most ominous complication. This diagnosis is an important consideration in all cases of new onset headache in elderly subjects. Reports of giant cell arteritis are few and far between in the Indian subcontinent. In this report, we describe the clinical details of four cases of giant cell arteritis, detected at Guwahati, Assam.The four patients were in the 70-82 age group. Sex distribution was equal. All of them had polymyalgia rheumatica (PMR), with one case displaying an initial presentation as only PMR. Cardinal manifestation was a severe headache, frequently accompanied by scalp allodynia and abnormalities of the superficial temporal artery (STA) on examination. STA biopsy yielded histopathological confirmation in three patients. Permanent visual loss was noted in one patient. These cases highlight the importance of assessing the possibility of giant cell arteritis through appropriate clinical history, estimation of acute phase reactants and the judicious use of superficial temporal artery biopsy, to clinch the diagnosis.

We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient.

An 11-year-old girl manifested with photophobia, ptosis, external ophthalmoplegia, hypotonia, weakness of proximal limb muscles, hyporeflexia, and generalized seizures (six months). Her elder sister had had uncontrolled seizures and photophobia and died at seven years of age. In the patient, serum lactate was high (55 mg/dl). Muscle biopsy revealed characteristic ragged red and ragged blue fibers, diagnostic of mitochondrial cytopathy. Sequencing of the complete mitochondrial genome of the DNA obtained from the muscle biopsy of the patient did not show any characteristic mutation. Four months later, the girl was admitted with a one-week history of epilepsia partialis continua (EPC). EEG revealed Periodic Lateralized Epileptiform Discharges (PLEDs), once in 2-4 seconds, over the right temporo-occipital leads. MRI revealed signal change of right motor cortex, which had restricted diffusion. MR spectroscopy (MRS) from this region revealed lactate peak. EPC remained refractory to multiple anti-epileptic drugs, immuno-modulators, coenzyme-Q, and carnitine. This thought provoking report expands the spectrum of mitochondrial cytopathies.