Glossopharyngeal neuralgia is a relatively rare condition characterized by severe, paroxysmal episodes of pain localized to the external ear canal, the base of the tongue, the tonsil or the area beneath the angle of the jaw. This pain is many a times confused with Trigeminal Neuralgia and mistreated. There are various diagnostic and management dilemmas which are herein addressed in this review.

We report a case of alien hand sign in a male with stroke and briefly discuss the pathogenesis of this rare condition symptom.

Brachial plexus injury can occur as a result of trauma, inflammation or malignancies, and associated complications. The current topic is concerned with various forms of brachial plexopathy, its clinical features, pathophysiology, imaging findings, and management. Idiopathic brachial neuritis (IBN), often preceded with antecedent events such as infection, commonly present with abruptonset painful asymmetric upper limb weakness with associated wasting around the shoulder girdle and arm muscles. Idiopathic hypertrophic brachial neuritis, a rare condition, is usually painless to begin with, unlike IBN. Hereditary neuralgic amyotrophy is an autosomal-dominant disorder characterized by repeated episodes of paralysis and sensory disturbances in an affected limb, which is preceded by severe pain. While the frequency of the episodes tends to decrease with age, affected individuals suffer from residual deficits. Neurogenic thoracic outlet syndrome affects the lower trunk of the brachial plexus. It is diagnosed on the basis of electrophysiology and is amenable to surgical intervention. Cancer-related brachial plexopathy may occur secondary to metastatic infiltration or radiation therapy. Traumatic brachial plexus injury is commonly encountered in neurology, orthopedic, and plastic surgery set-ups. Trauma may be a direct blow or traction or stretch injury. The prognosis depends on the extent and site of injury as well as the surgical expertise.

Electrodiagnosis (EDX) is a useful test to accurately localize the site, determine the extent, identify the predominant pathophysiology, and objectively quantify the severity of brachial plexopathies. It can also be used to examine muscles not easily assessed clinically and recognize minimal defects. Post-operatively and on follow up studies, it is important for early detection of re-innervation. It can be used intra-operatively to assess conduction across a neuroma, which would help the surgeon to decide further course of action. Localization of the site of the lesion can be very challenging as there may be multiple sites of involvement and hence the electroneuromyographic evaluation must be adequate. The unaffected limb also needs to be examined for comparison. The final impression must be co-related with the type and severity of injury.

Adult post traumatic Brachial plexus injury is unfortunately a rather common injury in young adults. In India the most common scenario is of a young man injured in a motorcycle accident. Exact incidence figures are not available but of the injuries presenting to us about 90% invole the above combination This article reviews peer-reviewed publications including clinical papers, review articles and Meta analysis of the subject. In addition, the authors' experience of several hundred cases over the last 15 years has been added and has influenced the ultimate text. Results have been discussed and analysed to get an idea of factors influencing final recovery. It appears that time from injury and number of roots involved are most crucial.

This review is on ultrastructure and subcellular physiology at normal and abnormal neuromuscular junctions. The clinical and electrophysiological findings in myasthenia gravis, Lambert-Eaton myasthenic syndrome (LEMS), congenital myasthenic syndromes, and botulinum intoxication are discussed. Single fiber electromyography (SFEMG) helps to explain the basis of testing neuromuscular junction function by repetitive nerve stimulation (RNS). SFEMG requires skill and patience and its availability is limited to a few centers. For RNS supramaximal stimulation is essential and so is display of the whole waveform of each muscle response at maximum amplitude. The amplitudes of the negative phase of the first and fourth responses are measured from baseline to negative peak, and the percent change of the fourth response compared with the first represents the decrement or increment. A decrement greater than 10% is accepted as abnormal and smooth progression of response amplitude train and reproducibility form the crux. In suspected LEMS the effect of fast rates of stimulation should be determined after RNS response to slow rates of stimulation. Caution is required to avoid misinterpretation of potentiation and pseudofacilitation.

Objective: To observe the urodynamic profile of the patients following non-traumatic myelopathies (NTMs) with neurogenic bladder. Setting: Neurological rehabilitation department of university tertiary research hospital. Materials and Methods: Seventy-nine patients (44 men) with monophasic NTM, with the age range 8-65 years (31.0 ± 16.0 years), were admitted for inpatients' rehabilitation. Length of stay in rehabilitation ranged from 6 to 120 days (32.0 ± 24.8 days). Fifty-six patients (70.9%) had spinal lesion above D10, 17 had lesion between D10 and L2 (21.5%), and 6 (7.6%) had cauda equina syndrome. All patients had neurogenic bladder with urinary complaints. Urodynamic study (UDS) was performed in all patients. Results: UDS showed 71.4% patients (40/56) had neurogenic detrusor overactivity (NDO) with or without sphincter dyssynergy (DSD) with lesion above D10; only 52.9% patients (9/17) had NDO with or without DSD detrusor with lesion between D10 and L2; and majority (5/6 patients) had underactive detrusor in the cauda equina group. Bladder management was based on the UDS findings. No significant correlation was found (P > 0.05) between detrusor behavior and the level, severity (ASIA Impairment Scale) of spinal injury, or gender using chi-square test. Conclusions: Neurogenic bladder following NTM was observed in all patients. UDS suggested predominantly NDO in lesions above D10 and mixed pattern in between D10 and L2 lesions. No significant correlation was found between detrusor behavior and the level or severity of NTM in the study.

Objective: Musculoskeletal pain commonly occurs in the elderly, many of whom are also prone to suffer from strokes. We studied whether short-term use (≤ 4 weeks) of cyclooxygenase-2 (COX-2) inhibitors for musculoskeletal pain in stroke patients helped them to participate in their therapies and was safe and efficacious. Materials and Methods: Three hundred and three patients admitted consecutively with first ischemic stroke were studied. Two cohorts were defined, based on whether patients with acute stroke had sufficient musculoskeletal pain that warranted oral COX-2 inhibitors (COX-2 group) or not (case-matched controls). Primary efficacy measures were change in Fugl-Meyer (F-M) pain score and change in total functional independence measure (TFIM) scores on discharge from hospital. Safety was judged by the incidence of vascular episodes during the study period. Results: From the original 303 patients, 64 patients in the COX-2 group were matched with 64 patients in the non-COX-2 group. The groups were matched for age (±5 years), gender, and admission TFIM score (± 5 points). Baseline characteristics between the 2 groups were similar. The primary and secondary outcome measures were similar between the 2 groups, except for ambulation endurance, which favored the non-COX-2 group (P < 0.03). Greater change in the pain score (less pain) was found in the COX-2 group; this effect was strongest in patients who were independent prior to their stroke (on post hoc analysis). There were too few adverse events in either group of any significance. Conclusions: The short-term use of COX-2 inhibitors reduced musculoskeletal pain in acute stroke patients, improved functional motor outcome, and were found to be safe.

Introduction: Lesions of the brain, recognized as unidentified bright objects (UBOs), are commonly observed as areas of increased T2-weighted signal intensity on magnetic resonance imaging (MRI) in children with neurofibromatosis type 1 (NF1). Identification of these lesions is not currently encompassed in the National Institute of Health (NIH) diagnostic criteria for NF1. Objective: We aimed to determine the prevalence of UBOs in children with NF1 and identify areas of the brain that are commonly affected by these lesions, allowing us to evaluate whether UBOs should be included in the diagnostic criteria for the diagnosis of NF1. Materials and Methods: We reviewed the cranial MRI scans of 22 children who had been diagnosed with sporadic or familial NF1 in accordance with the criteria established by NIH. UBOs were present in 81% of the children with NF1. Results: These lesions have a predilection for specific areas of the brain, including the globus pallidus (72%), cerebellum (66%), brainstem (27%) and cerebral hemispheres (16%). The prevalence of UBOs identified varied significantly with age and sex; they were infrequent in children less than 4 years of age but were common in those aged between 4 and 12 years of age. UBOs were more commonly seen in males (66.6%) compared with females (33.3%). Repeat MRI scan on a subset of these patients with UBOs did not show any significant changes despite a worsening in clinical symptoms. Conclusion and Discussion: We have shown that UBOs are a common finding in children with NF1, and are most prevalent between the ages of 4 and 12 years. Many sites of the brain are affected by these lesions, most notably the globus pallidus and the cerebellum. Further research must be conducted to elucidate the significance of UBOs in patients with NF1 and whether these lesions have any utility in the clinical detection of NF1.

Background: Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder involving degeneration of anterior horn cells of spinal cord, resulting in progressive muscle weakness and atrophy. Aims: The purpose of our study was to determine the frequency of SMN and NAIP deletions in Tunisian SMA patients. Materials and Methods: Polymerase chain reaction (PCR) combined with restriction fragment length polymorphism (RFLP) was used to detect the deletion of exon 7 and exon 8 of SMN1 gene, as well as multiplex PCR for exon 5 and 13 of NAIP gene. Results: Fifteen (45.4%) out of 33 SMA patients were homozygously deleted for exons 7 and/or 8 of SMN1. Homozygous deletion of NAIP gene was observed in 20% (3 / 15) of patients. Conclusions: The molecular diagnosis system based on PCR-RFLP analysis can conveniently be applied in the clinical testing, genetic counseling, prenatal diagnosis, and pre-implantation genetic diagnosis of SMA.

Background: On the basis of clinical experience, it seems that sleep disturbances are common in children with cerebral palsy (CP); however, there is a lack of research and objective data to support this observation. Aim of Work: Our aim was to assess sleep of children with cerebral palsy, using validated sleep questionnaire. Subjects and Methods: one hundred children with diagnosis of CP were investigated via sleep questionnaires, with their ages from 2-12 years. The 100 children with CP were divided into two groups, pre-school group (52 children had a mean age 2.35 ± 1.04 years) and school ages group (48 children had a mean age 10.21 ± 3.75 years). Results: We found high incidence of sleep problem in both pre-school and school age groups. We found that pre-school children have more prevalence of early insomnia (46.2%, P value 0.028) and sleep bruxism (50%, P value 0.000), while school group suffer more sleep disordered breathing (SDB) (50%, P value 0.001), more nightmares (50%, P value 0.001), more sleep talking (12.5% P value 0.049), and more excessive daytime sleepiness (EDS) (62.5%, P value 0.001). Conclusion: Results of our study indicate that CP children have high incidence of sleep problem in both pre-school and school age groups.

Objectives: Electrocardiographic (ECG) changes are reported frequently after acute strokes. It seems that cardiovascular effects of strokes are modulated by concomitant or pre-existent cardiac diseases, and are also related to the type of cerebrovascular disease and its localization. We aimed to determine the pattern of ECG changes associated with pathophysiologic categories of acute stroke among patients with/without cardiovascular disease and to determine if specific ECG changes are related to the location of the lesion. Materials and Methods : The electrocardiographic records of 361 patients with acute stroke were studied to assess the relative frequencies of ECG abnormalities among the pathophysiologic categories of stroke. Results: In the present study, the most common ECG abnormalities associated with stroke were T-wave abnormalities, prolonged QTc interval and arrhythmias, which were respectively found in 39.9%, 32.4%, and 27.1% of the stroke patients and 28.9%, 30.7%, and 16.2 of the patients with no primary cardiac disease. We observed that other ECG changes comprising pathologic Q- wave, ST-segment depression, ST-segment elevation, and prominent U wave may also occur in selected or non-selected stroke patients; thereby simulate an acute myocardial injury. We observed an increased number of patients with abnormal T-wave and posterior fossa bleedings and more rhythm disturbances for ischemic lesions, localized in the anterior fossa. Conclusion: Ischemia-like ECG changes and arrhythmias are frequently seen in stroke patients, even in those with no history or signs of primary heart disease, which support a central nervous system origin of these ECG abnormalities. Further study is necessary to better define the brain-heart interaction.

Alzheimer's disease is a common kind of dementia. This disorder can be detected in all countries around the world. This neurological disorder affects millions of population and becomes an important concern in modern neurology. There are many researches on the pathogenesis of Alzheimer's disease. Although it has been determined for a long time, there is no clear-cut that this is a case with genetic disorder or not. A physiological genomics is a new application that is useful for track function to genes within the human genome and can be applied for answering the problem of underlying pathobiology of complex diseases. The physiogenomics can be helpful for study of systemic approach on the pathophysiology, and genomics might provide useful information to better understand the pathogenesis of Alzheimer's disease. The present advent in genomics technique makes it possible to trace for the underlying genomics of disease. In this work, physiological genomics analysis for Alzheimer's disease was performed. The standard published technique is used for assessment. According to this work, there are 20 identified physiogenomics relationship on several chromosomes. Considering the results, the HADH2 gene on chromosome X, APBA1 gene on chromosome 9, AGER gene on chromosome 6, GSK3B gene on chromosome 3, CDKHR1 gene on chromosome 17, APPBP1 gene on chromosome 16, APBA2 gene on chromosome 15, GAL gene on chromosome 11, and APLP2 gene on chromosome 11 have the highest physiogenomics score (9.26) while the CASP3 gene on chromosome 4 and the SNCA gene on chromosome 4 have the lowest physiogenomics score (7.44). The results from this study confirm that Alzheimer's disease has a polygenomic origin.

Background: Near death experiences (NDE) are receiving increasing attention by the scientific community because not only do they provide a glimpse of the complexity of the mind-brain interactions in 'near-death' circumstances but also because they have significant and long lasting effects on various psychological aspects of the survivors. The over-all incidence-reports of NDEs in literature have varied widely from a modest Figure of 10% to around 35%, even up to an incredible Figure of 72% in persons who have faced close brush with death. Somewhat similar to this range of difference in incidences are the differences prevalent in the opinions that theorists and researchers harbor around the world for explaining this phenomena. None the less, objective evidences have supported physiological theories the most. A wide range of physiological processes have been targeted for explaining NDEs. These include cerebral anoxia, chemical alterations like hypercapnia, presence of endorphins, ketamine, and serotonin, or abnormal activity of the temporal lobe or the limbic system. In spite of the fact that the physiological theories of NDEs have revolved around the derangements in brain, no study till date has taken up the task of evaluating the experiences of near-death in patients where specific injury has been to brain. Most of them have evaluated NDEs in cardiac-arrest patients. Post-traumatic coma is one such state regarding which the literature seriously lacks any information related to NDEs. Patients recollecting any memory of their post-traumatic coma are valuable assets for NDE researchers and needs special attention. Materials and Methods: Our present study was aimed at collecting this valuable information from survivors of severe head injury after a prolonged coma. The study was conducted in the head injury department of Guangdong 999 Brain hospital, Guangzhou, China. Patients included in the study were the ones Recovered from the posttraumatic coma following a severe head injury. A total of 86 patients were chosen. Near death experience scale (NDES) score of 7 or more was used as the criteria of screening NDE experiences. After identifying such individuals, the Prakash-modification of the Interpretative Phenomenological Analysis (IPA) was used to interview and record the data for qualitative analysis. Results: We found that contrary to earlier incidence reports, NDEs in post head injury patients were markedly low. Only 3 out of 86 of the patients recruited had a clear and confident experience of NDE. We conducted a qualitative study to explore further into these experiences. IPA of these 3 patients revealed four master themes: 1. Unique light visions 2. Intense feelings of astonishment, pleasure, and fear 3. The sense of helplessness 4. Supernatural but rationality of experience. Conclusion: NDE is uncommon in head-injury cases as compared to other near-death conditions. But the persons experiencing it have immense impacts on their belief systems and emotions. This experience should be further explored by studies of larger samples.

Background: Effectiveness of intermittent short course chemotherapy for tuberculous meningitis (TBM) has not been well studied. There are scarce reported studies on this issue in the world literature. Neurologists all over India are reluctant to accept Directly Observed Treatment Short course (DOTS) for TBM since its introduction in India. Aim: We did a prospective study to assess effectiveness of Revised National TB Control Program (RNTCP-DOTS) regimes among TBM patients. Materials and Methods: In this study we include the TBM patients admitted from September 2008 to March 2011. All were referred to RNTCP for treatment. Diagnostic Algorithm as per RNTCP guidelines was strictly followed and treatment outcome and follow-up status were recorded. We exclude HIV and pediatric age group. Results: A total of 42 cases registered for DOTS regimen were included in the study, of which 35 completed the treatment (83%). All the patients were started with DOTS but finally 78% received actual DOTS. All patients were given 9 months intermitted regimen as per RNTCP guidelines. Seven patients died during the treatment (16%). Conclusion: We found intermitted short course chemotherapy was effective in TBM.

Headache is the most common symptom of cerebral venous thrombosis (CVT); however, the detailed underlying mechanisms and characteristics of headache in CVT have not been well described. Here, we report two cases of CVT whose primary and lasting presentation was orthostatic headache, suggestive of decreased intracranial pressure. Contrary to our expectations, the headaches were associated with elevated cerebrospinal fluid (CSF) pressure. Magnetic resonance imaging and magnetic resonance venography showed characteristic voiding defects consistent with CVT. We suggest that orthostatic headache can be developed in a condition of decreased intracranial CSF volume in both intracranial hypotensive and intracranial hypertensive states. In these cases, orthostatic headache in CVT might be caused by decreased intracranial CSF volume that leads to the inferior displacement of the brain and traction on pain-sensitive intracranial vessels, despite increased CSF pressure on measurement. CVT should be considered in the differential diagnosis when a patient complains of orthostatic headache.

Racemose cysticercosis is a less frequent presentation of neurocysticercosis (NCC). It's presentation and management is quite different from cerebral parenchymal NCC. Diagnosis of racemose cysticercosis is based on the combination of clinical, epidemiologic, radiographic, and immunologic information. Compared with cysticercus cellulose, which most commonly presents as seizures, racemose NCC due to its extraaxial location presents with raised intracranial pressure and meningitis, and frequently requires neurosurgical intervention. Dementia as a sole presenting feature of NCC is rare. We report a case of racemose NCC with dementia as the presenting manifestation. The outcome of dementia patients with NCC seems favorable in most cases therefore a high index of suspicion for NCC should be kept especially in endemic areas.

Etiologic diagnosis of seizure requires proper consideration of apparently unrelated clinical features of the patient. Here, we report the case of a patient of status epilepticus with moderate-to-severe bilateral sensorineural deafness. Investigations showed extensive intracranial calcification, hypoparathyroidism and unilateral renal agenesis. The features were consistent with Barakat syndrome, a rare developmental disorder associated with mutations in the GATA3 gene. To the best of our knowledge, this is the first reported case of Barakat syndrome from India.

The aim of this study is to report a case of spontaneous intracranial hypotension complicated by bilateral subdural hemorrhage that resolved with conservative management. A young male presented with severe orthostatic headache associated with dizziness, neck pain and diplopia. Brain imaging revealed characteristic pachymeningeal enhancement and bilateral subdural hemorrhage. Radionuclide cisternography confirmed the Cerebrospinal fluid leak at the cervical 5 and cervical 6 vertebral level. He had clinical and radiological resolution with bed rest, hydration and analgesics and has remained symptom free since then. Spontaneous intracranial hypotension may be complicated by bilateral subdural hemorrhage. A conservative treatment approach is a viable option, as it may help improve the clinical and radiological outcome, especially when interventional facilities are not available.

Cerebral amyloid angiopathy (CAA) usually manifests as cerebral hemorrhage, especially as nontraumatic hemorrhages in normotensive elderly patients. Other manifestations are subarachnoid (SAH), subdural, intraventricular hemorrhage (IVH) and superficial hemosiderosis. A 52-year-old hypertensive woman presented with recurrent neurological deficits over a period of 2 years. Her serial brain magnetic resonance imaging and computed tomography scans showed recurrent SAH hemorrhage, and also intracerebral, IVH and spinal hemorrhage, with superficial siderosis. Cerebral angiograms were normal. Right frontal lobe biopsy showed features of CAA. CAA can present with unexplained recurrent SAH hemorrhage, and may be the initial and prominent finding in the course of disease in addition to superficial cortical siderosis and intracerebal and spinal hemorrhages.

Acute ischemic stroke may be the first clinical manifestation of the underlying cardioembolic source. We are reporting a 28-year-old man presenting with acute posterior circulation infarct due to underlying bicuspid aortic valve disease with vegetation detected by transesophageal echocardiography in the absence of clinical features of heart disease and infective endocarditis. The case report highlights the importance of routine evaluation of cardioembolic sources in all cases of ischemic stroke.

Perioperative stroke can occur following 0.2-0.3% of general surgical and orthopedic procedures. We are reporting a patient who developed multiple strokes in the immediate postoperative period following total knee replacement, where etiological workup revealed multiple pulmonary arteriovenous fistulae (PAVF). The significance of PAVF with paradoxical embolism in perioperative settings has rarely been reported in the literature.

Osmotic demyelination syndrome resulting from postpartum hypernatremia is a recently described entity wherein young women present with hypernatremic encephalopathy and white matter hyperintensities along with quadriparesis from rhabdomyolysis. It is an acute monophasic condition with acute hypernatremia occurring during puerperium with good recovery in majority of the patients with treatment. To the best of our knowledge, recurrent postpartum hypernatremia with encephalopathy, osmotic demyelination, and rhabdomyolysis has not been described. We present a young lady who had two episodes of reversible postpartum hypernatremic encephalopathy with rhabdomyolysis. Cerebral magnetic resonance imaging (MRI) before treatment revealed osmotic demyelination on both occasions. During first admission MRI revealed hyperintensities in internal capsule and corpus callosum, and at second admission revealed more extensive white matter hyperintensity, which simulated the 'wine glass' appearance.

We report about a young male who presented with generalized muscle stiffness, involving the limb, facial, and paraspinal muscles. The stiffness was severe enough to restrict all his daily activities, progressively increased with movements and also produced recurrent falls. This clinical picture resembled one of stiff person syndrome. As he had hypertrophy of calf muscles and generalized muscle tautness he was evaluated for other disorders which can resemble stiff person syndrome. Investigations revealed severe hypothyroidism with thyroid antibodies being elevated. This case is reported to highlight the fact that myopathy as a presenting manifestation of hypothyroidism can simulate stiff person syndrome. It is essential to identify the condition early as it recovers fully with treatment. Our patient responded well to thyroid replacement therapy and was able to lead a normal life.

Optic nerve tuberculomas are rarely reported and their natural history, prognosis, and duration of required treatment remain unclear. A 40-year-old immunocompetent male presented with complete loss of vision in his right eye, which had evolved over 6 weeks. He had optic atrophy on examination. Initial imaging showed right optic nerve swelling and thickening suggesting an infiltrative inflammatory optic neuropathy (infectious or noninfectious). Serial imaging revealed appearance of ring enhancement with a necrotic centre. Biopsy and culture of the coexistent parietal lobe lesion revealed Mycobacterium tuberculosis. Persistent optic nerve granuloma with evidence of radiological improvement was noted at 18 months follow-up with antituberculous therapy (ATT). Visual recovery could not be achieved. The salient features in this case include the clinical presentation initially mimicking an infiltrative or compressive optic neuropathy, rapid radiological evolution into a tuberculoma, subtle paradoxical radiological worsening after initiation of ATT and persistence of granuloma on follow up scan. The challenges involved in early diagnosis and during the treatment course will be discussed.

Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous syndrome characterized by a classical triad of facial port wine nevus, ipsilateral leptomeningeal angiomatosis (LAM) and glaucoma. The incidence of SWS is 1/50,000 live births, although it is more often underreported. The incidence of SWS without facial nevus is not known, although very few patients without facial nevus have been reported. In these patients, the diagnosis of SWS is made by the findings of computed tomography, magnetic resonance imaging, and histopathology. Here, we report three patients with SWS from our cohort of 28 patients with SWS without facial nevus and discuss their clinical profile and outcome.

Although the clinical features in some patients with cerebrovascular ischemia may be ill defined, majority of the patients present with focal neurological deficits caused by an arterial occlusion, and the clinical presentations are usually referable to the involved arterial territory. Therefore, vascular imaging constitutes an important component of the diagnostic workup. Cervical duplex ultrasonography of carotid and vertebral arteries is employed to evaluate the extracranial vasculature while transcranial Doppler provides important information about intracranial hemodynamic changes in cerebrovascular ischemia. These two components of cerebrovascular ultrasonography are fast and reproducible, and can be performed at the bedside. They provide real-time information about the status of cervico-cranial arterial patency and various hemodynamic alterations, including collateral flow. The information obtained from cerebrovascular ultrasonography is useful for diagnostic as well as prognostic purposes. Furthermore, it can be used to monitor cerebral blood flow for extended periods and aid in decision making for various interventions. The hemodynamic information obtained from cerebrovascular ultrasonography helps in determining the underlying mechanisms of brain ischemia, and is complementary to the clinical examination and other imaging modalities.We describe the technique of performing cervical duplex sonography, diagnostic criteria for arterial stenosis, characterizing plaque morphology, measuring intima-media thickness and various pitfalls while performing the test.