Cerebrospinal fluid (CSF) pressure changes can manifest as either intracranial hypertension or hypotension. The idiopathic forms are largely under or misdiagnosed. Spontaneous intracranial hypotension occurs due to reduced CSF pressure usually as a result of a spontaneous dural tear. Idiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial tension without hydrocephalus or mass lesions and with normal CSF composition. Neuroimaging plays an important role in excluding secondary causes of raised intracranial tension. As the clinical presentation is varied, imaging may also help the clinician in arriving at the diagnosis of IIH with the help of a few specific signs. In this review, we attempt to compile the salient magnetic resonance imaging findings in these two conditions. Careful observation of these findings may help in early accurate diagnosis and to provide appropriate early treatment.

Background: Idiopathic inflammatory myopathies are a heterogeneous group of acquired muscle disorders with considerable overlap in the histological features, making histological diagnosis difficult at times. Aims: To determine the immunohistochemical profile of clinically suspected cases of inflammatory myopathies, using monoclonal antibodies to HLA-1 and membrane attack complex (MAC), and to correlate the clinical, serological, and electromyographic profile and the histopathological picture, with the immunohistochemical profile. Settings and Design: This was a retrospective study analyzing the clinical and histopathological features in muscle of clinically suspected cases of inflammatory myopathy and correlating it to their HLA-1 and MAC immunostaining profiles. Material and Methods: The study subjects included 33 cases with suspected inflammatory myopathy and 59 with non-inflammatory muscle disease, as controls. Clinical data, electromyographic findings, serological profile, and details of therapy were obtained from patient records. Statistical Analysis: Student 'T' test, Pearson's Chi square test, and Kappa statistics were used appropriately. Results: Although HLA-1 and MAC immunostaining did not help to differentiate the individual subtypes of inflammatory myopathy, when either HLA-1 or MAC was positive, inflammatory myopathy could be ruled in with 86.5% certainty and when both HLA-1 and MAC were negative, it could be ruled out with 95% certainty. Conclusions: A combination of clinical presentation, serological profile, electromyographic and histopathological features, together with the immunoprofile for HLA-1 and MAC, contribute toward making a diagnosis of inflammatory myopathy.

Background and Objectives: Evidence from experimental and genetic studies suggest the existence of a potential link between the polymorphisms of human leukocyte antigen class II gene (HLA-DR) and ischemic stroke. This study addressed the association of HLA-DR gene with atherosclerotic cerebral infarction (ACI) in a North Chinese Han population. Material and Methods: The genotyping of HLA-DRB1 was determined by standard techniques based on polymerase chain reaction and sequence-specific oligonucleotides hybridization in a gene chip. Results: The relative risk (RR) of HLA-DRB1 * 04 and HLA-DRB1 * 03 (17) in patients with ACI and their first-degree relatives were significantly higher than those in the control group (RR=2.56 and 18.77, respectively; P <0.05). In contrast, the RR of HLA-DRB1 * 12 was dramatically reduced in patients with ACI in relation to healthy controls (RR=0.17; P <0.01). Conclusions: These data indicate that the polymorphisms in HLA-DRB1 may influence the risk of ACI in the North Han population of China. Further studies are necessary to validate the observation in larger samples.

Background: Familial hypokalemic periodic paralysis is an autosomal-dominant disorder characterized by episodic attacks of muscle weakness with hypokalemia. The combination of sarcolemmal depolarization and hypokalemia has been attributed to abnormalities of the potassium conductance governing the membrane potential; however, the molecular mechanism that causes hypokalemia has not yet been determined. Aim: To test the hypothesis that the expression patterns of delayed rectifier potassium channel genes in the skeletal muscle cells of patients with familial hypokalemic periodic paralysis differ from those in normal cells. Material and Methods: We examined both mRNA and protein levels of two major delayed rectifier potassium channel genes KCNQ3 and KCNQ5 in the skeletal muscle cells from three patients with familial hypokalemic periodic paralysis and three healthy controls. Results: When normal cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the KCNQ3 protein level significantly increased in the membrane fraction but decreased in the cytosolic fraction, whereas the opposite was true in patient cells. Conclusion: Abnormal subcellular distribution of the KCNQ3 protein was observed in patient cells. Our results suggest that the altered expression of KCNQ3 in patient cells exposed to high extracellular potassium levels could possibly hinder normal function of the channel protein. These findings may provide an important clue to understanding the molecular mechanism of familial hypokalemic periodic paralysis.

Aims and Objectives: To report hormonal outcome after surgery in a special subgroup of prolactinomas in whom medical therapy is not effective or not indicated. Patients and Methods: All patients who underwent surgery for prolactinomas, between December 2002 and December 2009, were analyzed retrospectively. The study group consisted of patients who were either intolerant or noncompliant to dopamine agonist (DA) therapy or those in whom medical treatment could not be given due to various reasons. The surgical results were assessed according to whether patients had microadenoma (<1 cm diameter), macroadenoma (>1 cm diameter), or giant prolactinoma (>4 cm diameter). Initial and final hormonal remission was defined as normalization of serum prolactin levels (<25 ng/ml) at 7 days after surgery and at last follow up, respectively. Results: Of the 172 patients, 133 (77%) were operated by primary transsphenoidal approach and 25 (14.5%) were operated transcranially and 14 patients required reoperation, either transsphenoidally (6 patients) or transcranially (8 patients). Overall, at last follow-up, hormonal remission could be achieved in 44% patients (83% microadenomas, 48% macroadenomas, and 16% of giant adenomas). There were three deaths (1.7%), 12 patients (7%) had single/multiple postoperative treatable complications. 59% of those patients, who did not achieve remission after surgery, finally attained normoprolactinemia with either DA therapy or radiotherapy, at last follow-up. Conclusions: Medical treatment of prolactinomas with DA should be considered as treatment of choice for these tumors. However, there is a major subgroup of patients who respond better to surgery rather than protracted medical therapy.

Background: Radial artery grafts (RAG) have been used in the treatment of complex vascular lesions, but not for symptomatic cavernous carotid aneurysms in elderly patients. Aim: To investigate the safety, usefulness, and perioperative complications of RAGs for symptomatic cavernous carotid aneurysms in elderly patients. Material and Methods: Of the 74 consecutive patients, in whom RAGs were used, we retrospectively investigated the postoperative outcomes and complications in eight elderly patients aged over 70 years with symptomatic internal carotid artery aneurysms in cavernous sinus. Results: Postoperative complications included one case of cerebral infarction, one case of symptomatic seizures, and one case of delayed cranial nerve palsy. Outcome: Seven patients had an mRS score of 0, and one patient had a score of 2. Postoperatively there was improvement in cranial nerve palsy in seven patients and the patients who had symptoms for one year and eight months had residual deficits. Although it cannot be stated that the frequency of perioperative complications was low, the final outcomes were favorable. Conclusion: Even in carotid disease that is difficult to treat among the elderly, RAG would appear to be a useful and safe treatment.

Background: Colloid cysts are not common brain lesions and account for 0.2-2.0% of all brain lesions. Transcallosal, transforaminal approach is a safe route and the most direct path to excise third ventricular colloid cyst, without dependence on hydrocephalus. Aim: To assess the surgical outcome of patients with colloid cysts of the anterior third ventricle treated by the transcallosal, transforaminal approach. Patients and Methods: Seventy-eight patients operated by the above approach over a period of 20 years were analyzed. A pre- and postoperative neurological assessment was done in all the patients. Neuro-cognitive evaluation of corpus callosum function was done in the last 20 patients. Computer tomography scan of the brain was done in all patients pre- and postoperatively. Results: Clinical features of raised intracranial pressure without localizing signs were the commonest presenting feature in 52 (66.7%) patients. Hydrocephalus was present in 65 (83.3%) patients. All patients underwent the transcallosal, transforaminal approach, and total excision of the lesion was achieved in 77 patients and subtotal in 1. Four patients required a postoperative shunt for acute hydrocephalus. There was no incidence of postoperative disconnection syndrome. In two patients, there was recurrence of the lesion after 2 and 6 years, respectively. Two patients died in the postoperative period. Conclusion: Colloid cyst is surgically curable. Early detection and total excision of the lesion can be a permanent cure with low mortality and minimum morbidity, when compared to the natural history of the disease. The limited anterior callosotomy does not result in disconnection syndromes or behavioral disturbance.

Background: The development of self-expanding stents dedicated to intracranial use has significantly widened the applicability of endovascular therapy to many intracranial aneurysms Objective: To report the angiographic and clinical outcomes of wide-necked intracranial aneurysms treated using the Enterprise stent. Material and Methods: Forty-six patients with 50 wide-necked and fusiform aneurysms (19 aneurysms with dissections), admitted between June 2009 and December 2010, were treated using Enterprise stents. Demographic information, procedural data, and technical complication were reviewed. Angiographic and clinical outcomes were evaluated at a 9.1-month follow-up. Results: Stent deployment was successful in all the 50 lesions, and additional coil embolization was performed in 48. Complete or near-complete occlusion immediately after stent deployment was achieved in 44 aneurysms, whereas, no immediate coil embolization was chosen in two cases. There was one (2%) procedure-related complication, which resulted in death. At mean 9.1-month follow-up, the result was good (GOS ≤2) in 45 patients. Angiographic recurrences arose in two cases, one of which was re-treated completely. Conclusions: The Enterprise stent is very useful for endovascular embolization of wide-necked intracranial aneurysms, because it is easy to navigate and place precisely. The overall morbidity and mortality rates are low.

First created in 2006 from adult somatic cells by a simple molecular genetic trick, induced pluripotent stem cells (iPS) system is the latest platform in stem cell research. Induced pluripotent stem cells are produced by nuclear reprogramming technology and they resemble embryonic stem cells (ES) in key elements; they possess the potentiality to differentiate into any type of cell in the body. More importantly, the iPS platform has distinct advantage over ES system in the sense that iPS-derived cells are autologous and therefore the iPS-derived transplantation does not require immunosuppressive therapy. In addition, iPS research obviates the political and ethical quandary associated with embryo destruction and ES research. This remarkable discovery of cellular plasticity has important medical implications. This brief review summarizes currently available stem cell platforms, with emphasis on cellular reprogramming and iPS technology and its application in disease modeling and cell replacement therapy in neurodegenerative diseases.

Pharmacological or neurosurgical therapies currently in practice to treat the damage in various neurodegenerative disorders are not efficient in preventing progression or cure of these progressive neurodegenerative processes. Recently, a new approach, cell therapy using stem cell, is being evaluated. However, the use of this therapy in the treatment of these neurological diseases is highly restricted, mainly owing to several technical difficulties and limitations. The strategy of isolation and characterization of neural stem cells from various sources will probably provide a major impetus and open up an interesting, novel therapeutic modality for several neurodegenerative disorders. The high regenerative potential of damaged neural tissues suggests that various embryonic/adult sources serve as a proxy for neural stem cells for cell-based therapy.

Background: Olfactory ensheathing cells (OEC) are considered to be the most suitable cells for transplantation therapy in the central nervous system (CNS) because of their unique ability to help axonal regrowth and remyelination in the CNS. However, there are conflicting reports about the success rates with OEC. Aim: This study was undertaken to evaluate the therapeutic effect of OEC in rat models using different cell dosages. Material and Methods: OECs harvested from the olfactory mucosa of adult white Albino rats were cultured. Spinal cord injury (SCI) was inflicted at the lower thoracic segment in a control and test group of rats. Two weeks later, OECs were delivered in and around the injured spinal cord segment of the test group of the rats. The outcome in terms of locomotor recovery of limb muscles was assessed on a standard rating scale and by recording the motor-evoked potentials from the muscles during transcranial electrical stimulation. Finally, the animals were sacrificed to assess the structural repair by light microscopy. Statistical Analysis: Wilcoxon signed rank test and Mann-Whitney U-test were used to compare the data in the control and the test group of animals. A P value of <0.05 was considered significant. Results: The study showed a moderate but significant recovery of the injured rats after OEC transplantation (P=0.005). Conclusion: Transplantation of OECs along with olfactory nerve fibroblasts improved the motor recovery in rat models with SCI.

Background: The effect of intralesional mesenchymal stromal cell (MSC) transplant in the subacute phase of brain injury has not been studied. Aim: To evaluate the role of intralesional transplant of mouse MSC following coldinduced cerebral cortical injury in mouse in improving neurological function. Material and Methods: Twelve mice (Swiss albino strain) received an intralesional injection of 2×10⁶ mouse MSCs labelled with Bromodeoxyuridine (BrdU) and suspended in phosphate-buffered saline (PBS), 72 h after cerebral cryoinjury. Six mice received intralesional injection of PBS and served as controls. Neurological severity score (NSS) and rotarod treadmill test were used to perform serial assessments. Results: The mean NSS in the control group (n=5) on the first posttrauma day was 9.3±1.2 and it improved to 14.2±1.3 on day 28. The mean NSS in the MSC group (n=11) was 10.7±1.8 on the first posttrauma day and it improved to 16±1.1 on day 28 posttransplant. This difference was not found to be statistically significant when subgroup analysis of animals, where the assessments were blinded, was performed. There was no significant difference in the rotarod treadmill scores between the control and the MSC group at any time point. Few BrdU-positive cells could be identified in the periphery of the contusion up to day 10 posttransplant. Conclusions: Transplanted MSCs were shown to survive for at least 10 days after intralesional transplant in the cryoinjury model of the mouse cerebral cortex but the functional recovery observed in the experimental group was not statistically different from the controls.

Background and Aims: Studies have shown that administration of NEP1-40, a Nogo-66 receptor antagonist peptide, improves locomotor recovery in rats. We hypothesize that combining NEP1-40 with another promising therapy, neural stem cell transplantation, might further improve the degree of locomotor recovery. In the present study, we examined whether NEP1-40 combined with bone marrow stromal cells-derived neurospheres (BMSC-NSs) transplantation would produce synergistic effects on recovery. Material and Methods: Adult Sprague-Dawley rats were subjected to spinal cord injury (SCI) at the T10 vertebral level. Immediately after injury, rats were administrated NEP1-40 intrathecally for 4 weeks. BrdU-labeled BMSC-NSs (2×10⁵ ) were transplanted into the injured site 7 days after SCI. Locomotor recovery was assessed for 10 weeks with BBB scoring. Animals were perfused transcardially 10 weeks after contusion, and histological examinations were performed. Results: The combined therapy group showed statistically better locomotor recovery than the control group at 7 weeks of contusion. Neither of the two single-agent treatments improved locomotor function. The average area of the cystic cavity was significantly smaller in the combined therapy group than in the control group. Fluorescence microscopic analysis showed that NEP1-40 dramatically inhibited the formation of glial scar and promoted the axons penetration into the scar barrier. Conclusion: This study revealed that BMSC-NSs and NEP 1-40 exhibit synergistic effects on recovery in rat SCI. This may represent a potential new strategy for the treatment of SCI.

We describe clinical, biochemical, radiological profile, and treatment outcome in 97 patients with idiopathic hypoparathyroidism seen over a period of 18 years. Of the 97 patients, 78 (80%) had idiopathic hypoparathyroidism and 19 (20%) had pseudohypoparathyroidism. The mean age±standard deviation (SD) at presentation was 28.7±14.1 years. There were 52 males, the mean lag time from first reported symptom to diagnosis was 5.9±5.2 years and the mean (±SD) follow-up was 1.8±0.4 years. The most common presenting manifestation was carpopedal spasm in 68 (70%) patients, followed by paresthesia and seizures in 52 (54%) patients. The mean (±SD) serum calcium and inorganic phosphate concentrations were 6.1±1.5 mg/dl and 6.3±1.5 mg/dl, respectively. The most common imaging abnormality noted was basal ganglia calcification followed by cerebral cortex and cerebellum calcification. More than one-third of patients were on various antiepileptic drugs including phenytoin. In addition to oral calcium and active vitamin D (calcitriol), twenty-six patients (27%) also required hydrochlorothiazide. The important finding in our study was long lag time from the first reported symptom to diagnosis. Phenytoin was the drug in almost one- third of our patients with seizures. Practicing clinicians should have high index of suspicion of diagnosis hypoparathyroidism in the appropriate clinical states to avoid the morbidity associated with hypoparathyroidism. Phenytoin should be avoided in patients with hypoparathyroidism and seizures.

There is a relatively high incidence of screw misplacement during spinal instrumentation due to distortion of normal anatomy following spinal trauma. The O-arm^® is the next-generation spinal navigation tool that provides intraoperative 3-D imaging for complex spine surgeries. In this prospective study over 1-month period, 25 patients (mean age 29.16 years (range 7-58 years), 22 (88%) males) with spinal injury who underwent spinal instrumentation under O-arm^® guidance were included. Fall from height (64%) was the most common etiology seen in 16 patients. The majority (68%) had dorsolumbar fractures. Spinal canal compromise was seen in 21 patients (84%). Ten patients (40%) had American Spinal Injury Association (ASIA) grade A injuries, two patients (8%) had grade B, five patients (20%) had grade C, four patients (16%) each had grade D, and grade E injuries. A total of 140 screws were inserted under O-arm guidance. Of these, 113 (81%) were dorsolumbar pedicle screws, 2 were odontoid screws, 12 were anterior cervical screws, and 12 screws (48%) were lateral mass screws. Mean duration of surgery was 4.5 h with a mean blood loss of 674 mL. The mean postoperative stay was 6.3 days. None of the patients had screw malplacement ort canal breach. No patient deteriorated in ASIA grade postoperatively. The system was rated as excellent for ease of use by all faculty using the system. Accurate screw placement provides better patient safety and reduces the in hospital stay thereby leading early patient mobilization and may reduce the cost incurred in patient management.

Herpes simplex virus (HSV) encephalitis complicated by cerebral hematoma is extremely rare. We report a 54-year-old man with hepes simplex encephalitis complicated by intracerebral hematoma and review the literature and describe the characteristics of HSV encephalitis complicated by intracerebral hematoma

Balo's concentric sclerosis (BCS) is a rare inflammatory demyelinating disease of central nervous system, pathologically chracterized by alternate bands of demyelination and preserved myelin tissue. Before the era of magnetic resonance imaging (MRI), most cases of BCS were diagnosed on postmortem examination. MRI allows for noninvasive diagnosis by demonstrating characteristic changes which closely parallels the histopathological features of BCS. We report a case of 26-year-old female with BCS involving bilateral thalami, with typical MRI appearance.

Functional hemispherectomy is an accepted treatment in hemispherical intractable epilepsy syndromes. We report a patient who had functional hemispherectomy for intractable seizures secondary to right hemispheric cortical dysplasia. Preoperatively, the patient had mild left hemiparesis and functional magnetic resonance imaging (fMRI) showed bilateral motor function lateralization to normal left hemisphere. The patient remains seizure free at 1-year follow-up, with no deterioration of motor power on left side. This report reviews physiology of neural plasticity for motor function lateralization and also reliability of fMRI in determining the functional shift.

Clinico-radiological features of two patients with cerebrospinal fluid polymerase chain reaction-positive Epstein Barr virus (EBV) encephalitis have been reported. Both the patients presented with fever and altered sensorium, one had visual hallucination, decerebration followed by visual loss and the other had downward ocular deviation and orofacial and upper limb choreiform movement. Magnetic resonance imaging (MRI) revealed parieto-occipital involvement in both the patients. Follow-up MRI at one month was normal in one and revealed regression of lesion in the other. Both the patients, however, had severe neurologic sequelae at 18 months' follow-up. EBV encephalitis may have diverse clinical presentation with characteristic parieto-occipital involvement.

Endolymphatic sac tumors are rare, histologically non-aggressive lesions, but often become locally invasive and lead to erosion of the petrous bone. We report a rare case with multiple endolymphatic sac tumors located at bilateral cerebellopontine angles and vermis of the cerebellum. One of the lesions was surgically resected and others were treated using gamma knife radiosurgery. Local recurrence and metastasis occurred, which was treated with gamma knife radiosurgery. With an appropriate prescription dose and isodose, gamma knife radiosurgery may have an important role in the treatment of endolymphatic sac tumor.

Hemangioblastoma is a benign tumor of the cerebellum, and treatment involves surgical excision, both as the initial treatment and also in case of recurrence. Recurrence of hemangioblastoma can be local due to incomplete resection or can be distant and separate from the tumor resection region. Local recurrence can largely be avoided by verifying for any residual tumor intraoperatively before closure. In this study, we used intraoperative fluorescent diagnosis using 5-aminolevulinic acid (5-ALA) to verify the presence of a residual tumor during surgical resection. Nine patients with hemangioblastoma were given 1 g of 5-ALA orally before surgery, and a laser beam of 405 nm was focused on the tumor during resective surgery. Fluorescence of protoporphyrin IX (PPIX) was observed in the core of tumor in all the cases. Fluorescence of PPIX was observed in the peritumoral cyst wall in two patients after tumor resection, and in both of them fluorescent parts of PPIX were resected and histological examination showed tumor cells. Usually, there are no tumor cells in the peritumoral cyst of a hemangioblastoma, yet hemangioblastomas may sometimes recur from an unresected cyst wall. It is thus necessary to excise an infiltrating cyst of tumor cells to prevent recurrence. Intraoperative fluorescent diagnosis using 5-ALA is a useful method to discern whether tumor cells are present in the peritumoral cyst wall of a hemangioblastoma.

Tension pneumocephalus complicating ventriculoperitoneal shunt is extremely rare. We report an elderly male who developed delayed tension pneumocephalus 12 months after ventriculoperitoneal shunt for hydrocephalus complicating aneurysmal subarachnoid hemorrhage. Fine-cut reformatted computer tomography scan revealed a large pneumatocele on the petrous apex associated with tegmen tympani defect. The shunt valve pressure was temporarily raised from 120 mm H ₂ O to 200 mm H ₂ O, and the patient underwent successful subtemporal extradural repair of the bony defect in the temporal bone. Although extremely rare, otogenic tension pneumocephalus is a potentially life-threatening condition, and urgent surgical repair of the bony defect in the temporal bone reduces the risk of both the morbidity and mortality.

Infections of central nervous system are rare complications of endovascular procedures. Review of literature revealed only four reported cases of brain abscesses after embolization of intracranial arteriovenous malformations (AVMs). We report two new cases of delayed brain abscess after embolization of AVM. In one of the patients, it was due to an unusual organism, Burkholderia caeci.

We report a rare case of chronic encapsulated intracerebral hematoma (CEIH) after radiosurgery for a cerebral arteriovenous malformation (AVM). A 49-year-old male underwent transarterial embolization and surgical excision of a cerebral AVM presenting with cerebral hemorrhage in the left temporal lobe. Stereotactic radiosurgery (SRS) was done after 12 months. Magnetic resonance imaging (MRI) 14 months after SRS showed a small-enhancing lesion close to the left lateral ventricle and marked white matter edema. At that time a diagnosis of radiation-induced necrosis was made and steroids administered. At 43 months after SRS, MRI showed a small-enhancing mass close to the lateral ventricle with a hematoma cavity. Surgical excision was performed and histological examination revealed that the capsule consisted of an outer collagenous layer and an inner granulated layer with deposits of hemosiderin, which was compatible with CEIH. CEIH should be considered after SRS for AVM.