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Endoscopic third ventriculostomy (ETV) has been in vogue for the past two decades, as a tool in the armamentarium of the neurosurgeon, for the management of hydrocephalus. Its utility has been proven consistently in congenital / acquired aqueductal stenosis, although the outcomes in communicating hydrocephalus as well as hydrocephalus secondary to other etiologies have not been as impressive. It is a relatively safe procedure with the appropriate selection of patients with a low rate of permanent morbidity. This review aims to define the current indications, management outcomes, and complications of ETV.

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Background: The technique of multiplex ligation-dependent probe amplification (MLPA) assay is an advanced technique to identify deletions and duplications of all the 79 exons of DMD gene in patients with Duchenne/Becker muscular dystrophy (DMD/BMD) and female carriers. Aim: To use MLPA assay to detect deletions which remained unidentified on multiplex polymerase chain reaction (mPCR) analysis, scanning 32 exons of the "hot spot" region. Besides knowing the deletions and/or duplications, MLPA was also used to determine the carrier status of the females at risk. Materials and Methods: Twenty male patients showing no deletions on mPCR and 10 suspected carrier females were studied by MLPA assay using P-034 and P-035, probe sets (MRC Holland) covering all the 79 exons followed by capillary electrophoresis on sequencing system. Results: On MLPA analysis, nine patients showed deletions of exons other than 32 exons screened by mPCR represented by absence of peak. Value of peak areas were double or more in four patients indicating duplications of exons. Carrier status was confirmed in 50% of females at risk. Conclusion: Combining the two techniques, mPCR followed by MLPA assay, has enabled more accurate detection and extent of deletions and duplications which otherwise would have remained unidentified, thereby increasing the mutation pick up rate. These findings have also allowed prediction of expected phenotype. Determining carrier status has a considerable significance in estimating the risk in future pregnancies and prenatal testing options to limit the birth of affected individuals.

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Background and Objective: Duchenne and Becker muscular dystrophies are X-linked diseases caused by mutations in the dystrophin gene, which affect approximately 1 in 3,500 and 1 in 18,000 boys, respectively. The aim of this work was to develop a method to assist the diagnosis and classification of the disease. Materials and Methods: A large data set of dystrophin mutations was detected in 167 Chinese patients by multiplex ligation-dependent probe amplification and sequencing. Muscle biopsy, immunohistochemistry and STR analysis were also carried out in the patients and carriers. Results: One hundred and three deletions, 23 duplications and two-point mutations. The deletion of one or more exons was detected in 103 (61.7%) patients. The region spanning exons 44-55 was the most frequent deletion. The duplication was identified in 23 (13.8%) patients, which was more common than previously reported. Most duplications were found in exons 2-18. Six out of the 45 muscle biopsies analyzed showed the presence of other muscle diseases. Conclusions: This study may be important to enable comparisons of mutation type and the most appropriate analytical approach for samples from different geographical areas and ethnicities.

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Corticobasal degeneration and Parkinson's disease are pathologically distinct disorders with unique histological and biochemical features of a tauopathy and a-synucleinopathy respectively. We report the first case of co-occurrence of these pathologies in the same patient. Convergence of such distinctly separate neuropathology in the same brain highlights the need for extensive brain banking and further research in supporting the hypothesis that tauopathies and a-synucleinopathies might share common pathogenic mechanisms.

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McArdle's disease (myophosphorylase deficiency), an uncommon autosomal recessive metabolic disorder, is characterized clinically by exercise intolerance beginning in childhood, myalgia, cramps, exercise-induced rhabdomyolysis, "second wind" phenomenon, elevated Creatine Kinase (CK) levels at rest, and previous episodes of raised CK levels following exercise. Several mutations in the PYGM gene and geographic variations have been described. We report three biopsy confirmed cases of McArdle's disease.

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Objective: Despite dramatic advances in all medical era, cerebral vasospasm is still the major complication in patients with subarachnoid hemorrhage (SAH). The purpose of this study was to assess the influence of intraarterial (IA) nimodipine in the treatment of symptomatic vasospasm and in preventing neurological disabilities. Materials and Methods: We retrospectively reviewed 10 patients of SAH who received IA nimodipine in 15 procedures. The decision to perform angiography and endovascular treatment was based on the neurological examination, brain computed tomography (CT) and CT-angiography. The procedure reports, anesthesia records, neurological examination before and after the procedure, brain imaging and short- and long-term outcome were studied. Results: The average dose of nimodipine was 2 mg. The median change in mean arterial pressure at 10 min was -10 mmHg. No significant change of heart rate was observed at 10 min. There was radiological improvement in 80% of the procedures. Neurological improvement was noted after eight out of 12 procedures when nimodipine was used as the sole treatment and after 10 out of 15, overall. Six patients clinically improved after the treatment and had good outcome. In one patient, an embolus caused fatal anterior and middle cerebral arteries infarction. There was no other neurological deficit or radiological abnormality due to the nimodipine treatment itself. Conclusion: Low-dose IA nimodipine is a valid adjunct for the endovascular treatment of cerebral vasospasm. Beneficial effects are achieved in some patients, prompting a prospective control study.

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Background: Endoscopic third ventriculostomy (ETV) has replaced shunt surgery for several indications. Failure of ETV secondary to restenosis can result in recurrence of symptoms of raised intracranial pressure. Objective: To analyze the rates of restenosis due to ostomy closure and factors resulting in failures and to assess the role of re-ETV in such cases. Materials and Methods: Re-ETV was performed after counselling and obtaining informed consent. The technique of re-ETV was essentially the same as in primary ETV. Video analysis of primary ETV was performed before selecting a patient for re-ETV. Factors analyzed included age, gender, etiology of hydrocephalus, cerebrospinal fluid (CSF) findings, presence of shunt tube and adequacy of ETV and bleeding at the time of ETV. Results: Thirty-two patients underwent re-ETV. The mean interval between the first ETV and re-ETV was 1.4 years (3 days to 2.9 years). Overall failure of ETV due to restenosis was 8.78%. The technical success rate of performing re-ETV was 93.2%. The overall clinical recovery following surgery was observed in 89% of the patients, three from early and 25 from delayed ETV failures. The radiological recovery was seen in 20 (63%) patients. The good flow of CSF via the re-ETV site was documented with cine mode magnetic resonance imaging (MRI) in seven patients. Unlike primary ETV, the success of re-ETV in children aged less than 2 years was 90% (P < 0.005). There were 56.25% failure of ETV in patients with previous infection or foreign body within the ventricle (P < 0.001). While the chances of restenosis were high in the procedure with some infections, the outcome was equally better. Gender of the patients and CSF findings had no influence on ostomy closure. Conclusions: re-ETV can be considered in carefully selected patients of failed ETV. It is more useful in delayed ETV failures and can be offered before a patient is advised VP shunt.

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Background: Endoscopic third ventriculostomy (ETV) is increasingly being used as an alternative treatment in tubercular meningitis (TBM) hydrocephalus. This study is aimed to evaluate the role of ETV in TBM hydrocephalus. Materials and Methods: This is a prospective study of 59 patients with TBM and obstructive hydrocephalus. The diagnosis was confirmed by a computed tomography scan and/or magnetic resonance imaging scan preoperatively. The procedure was performed using the standard technique or water jet dissection. Results: Three (5.1%) patients had blocked stoma, 31 (53%) had associated malnutrition, and 13 (22%) had complex hydrocephalus. Clinical improvement was seen in 34 (58%) after ETV and in 47 (80%) patients after ETV with lumber peritoneal shunt. Thirteen patients with patent stoma and complex hydrocephalus did not improve after ETV alone; an additional lumber peritoneal shunt was required. Clinical outcome was significantly better in good grade. Early recovery was observed in 81%. Results of ETV were better in patients without cisternal exudates, good nutritional status, thin and identifiable floor of third ventricle compared to cases with cisternal exudates, malnourished, thick and unidentifiable floor respectively, although the difference was statistically insignificant. There was no operative death. Three patients with normal ICP did not show any improvement. The radiological recovery after 3 weeks of surgery was 52%; follow-up ranged between 7 and 54 months. Six patients developed CSF leak. Conclusion: Endoscopic third ventriculostomy was safe and effective in TBM hydrocephalus. Complex hydrocephalus and associated cerebral infarcts were the major causes of failure to improve. Good results were observed in better grades.

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Congenital myopathies (CMs), a group of relatively non-progressive disorders presents with weakness and hypotonia of varying severity, morphologically recognized by specific structural abnormalities within the myofiber. This report presents the clinical and Histopathological features of 40 patients with CMs. Centronuclear myopathy was the commonest (40%) followed by congenital fiber type disproportion (37.5%). Other less common CMs included: myotubular myopathy (5%), nemaline myopathy (5%), central core disease (5%), multicore disease (2.5%) and congenital myopathy with tubular aggregate (5%). Immunolabeling to desmin corresponded to morphological changes within the myofibers while vimentin was negative in all the patients. There is no combined role of these proteins in the disease process.

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Postpartum cerebral angiopathy (PPCA) is a rare form of reversible cerebral vasoconstriction syndrome. A 39-year-old female presented with a 5×6 cm left frontal intracerebral hemorrhage (ICH) and following an emergent neurosurgical evacuation of ICH, she developed a contralateral ICH and intraventricular hemorrhage. Her postoperative course was highlighted by the finding of diffuse bilateral segmental vasospasm on angiography and trans-cranial Doppler, left anterior cerebral artery region infarct and intractable elevated intracranial pressure (ICP). Medical management including osmotic therapy, nimodipine, corticosteroids, and phenytoin was unable to control ICP. Intractable elevated ICP led to evacuation of the right-sided ICH. After 3.5 years, the patient appears to be cognitively intact but continues to have right lower extremity weakness and is wheelchair-bound. Our case suggests that PPCA can rarely manifest as a life-threatening emergency for which neurosurgical intervention may be life-saving.

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Background: Surgical procedures for correction of craniosynostosis are often performed in pediatric patients who have a small blood volume; it represents major surgery. Literature is scarce on factors affecting blood loss, intensive care unit (ICU) and hospital stay in these patients. Objectives: To identify the factors which directly affect the outcome of craniosynostosis surgery. Materials and Methods: A detailed review of records pertaining to preanesthetic evaluation, associated anomalies, intraoperative course, and postoperative follow-up was done for patients who underwent craniosynostosis surgery between June 2000 and June 2010. The correlation between different variables was evaluated using Spearman's rank correlation. Results: During the study period 95 patients (mean age 29 months, range: 3 months-13 years) underwent corrective surgery for craniosynostosis. Hospital stay was found to be significantly associated with type of surgery and postoperative complications (P<0.001) Factors such as number of associated medical conditions, number of postoperative complications, type of induction of anesthesia, duration of surgery, type of recovery affected the ICU stay in these patients (P = 0.01). Conclusion: The outcome of patients undergoing craniosynostosis in terms of ICU and hospital stay is affected by the number of medical and postoperative conditions, type of anesthesia induction, duration of surgery and type of recovery.

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Objective: Neuroendoscopy has become an integral part of neurosurgery, in particular in the ventricular system. Obstructive hydrocephalus secondary to intraventricular hemorrhage (IVH) is a good indication for neuroendoscopic surgery. We evaluated its efficacy and limitations in these patients. Materials and Methods: During a 5-year period, 13 patients with obstructive hydrocephalus secondary to hypertensive IVH were studied. Patients with IVH with no evidence of obstructive hydrocephalus or with a large parenchymal hematoma or IVH of vascular origin were excluded. Rigid endoscope was used to evacuate hematoma in lateral ventricles and third ventricle in all patients. Glasgow Coma Scale (GCS), Graeb score and ventriculo-cranial ratio were evaluated before and after endoscopic intervention and Glasgow Outcome Scale (GOS) was appraised at 1 month and 12 months, postoperatively. Results: Of the 13 patients, eight (61.5%) patients had thalamus hemorrhage. A successful endoscopic removal of intraventricular hematoma was achieved in all patients. Complications observed included, fornix contusion (1) and meningitis (1) and there was no procedure-related mortality. The mean Graeb score reduced from 8.69 ± 1.89 to 6.00 ± 2.68 (P=0.001) and ventriculo-cranial ratio decreased from 0.41 ±0.05 to 0.39 ± 0.05 (P=0.085) following the procedure. The mean GOS scores at 1 month and 12 months both were 2.7, but a bipolar distribution was observed at 12 months. The Graeb score changed significantly with positive correlation to GCS score change (=0.565 and P<0.05). Conclusions: Endoscopic management of severe IVH with obstructive hydrocephalus allows effective reduction of the amount of ventricular blood and improves level of consciousness. Future refinement in instrumentation and discreet case selection may make this method more applicable and effective.

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Objective: To analyze the value of readout-segmented echo-planar imaging (rs-EPI) with parallel imaging and a two-dimensional (2D) navigator-based reacquisition technique in the detection of brain lesions at the skull base. Materials and Methods: A total of 54 patients (male 37, female 17) with suspected skull-base intracranial lesions underwent magnetic resonance imaging (MRI), including pre-T1 weighted imaging, T2-weighted imaging, Fluid Attenuated Inversion Recovery (FLAIR), standard single shot echo-planar imaging diffusion weighted imaging (ss-EPI DWI) and rs-EPI DWI, post-contrast T1-weighted. The total number of lesions and the number of lesions at different sites on all MRI sequences were used as reference measures. Then differences in detecting lesions and image quality between standard ss-EPI DWI and rs-EPI DWI were analyzed. Results: There was a significant difference in the total number of lesions detected by rs-EPI DWI and standard ss-EPI DWI (P = 0.01). But this difference was mainly due to an improved ability of rs-EPI DWI to detect lesions located in the anterior cranial fossa, compared to ss-EPI DWI (P=0.02); the ability of ss-EPI and rs-EPI DWI to detect lesions in the middle cranial fossa and posterior cranial fossa was not significantly different (P = 0.471, P = 0.486, respectively). For image quality, rs-EPI images were significantly better than standard ss-EPI DWI images (P<0.001). Conclusion: The rs-EPI DWI technique is a useful tool for the detection and evaluation of lesions located at the skull base.

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Context: Although most of the meningiomas are benign, some of them are associated with a less favorable clinical outcome. The major prognostic question regarding meningiomas is the prediction of recurrence which is largely dependent on the histopathological type, grading, proliferation indices and progesterone receptor status. Aims: Our study was done to assess the expression of Ki-67 and progesterone receptor (PR) in different histological types, grades, recurrent and non-recurrent meningiomas. Materials and Methods: A total of 60 cases were collected over a period of 2.5 years. Hematoxylin and eosin staining was done for histological typing and grading of the tumors. Immunohistochemical staining was done for Ki-67 and PR using standard immunoperoxidase technique and Ki-67 Labeling Index (LI) and PR score was calculated. Results: Mean Ki-67 LI was significantly higher in males versus females, in Grade II versus Grade I, in recurrent versus non-recurrent and in PR-negative versus PR-positive meningiomas. Moreover, considering Grade I and Grade II-meningiomas separately, mean Ki-67 LI was significantly higher in recurrent cases. PR positivity was significantly higher in females versus males, in Grade I versus Grade II and in non-recurrent versus recurrent meningiomas. Correlation between Ki-67 LI and PR expression was also evaluated and a strong inverse correlation was found between Ki-67 LI and PR expression. A strong negative correlation was also established between the values of Ki-67 LI and recurrence-free survival. Conclusion: Our results establish that the immunodetection of Ki-67 LI and PR expression in meningiomas provides a practical tool for assessing the biological behavior of meningiomas.

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Objective: The clinical and angiographic characteristics of vertebral artery (VA) aneurysm were evaluated to demonstrate the safety and efficacy of endovascular techniques of VA aneurysms. Materials and Methods: Case records of 38 consecutive patients with 40 VA aneurysms admitted during a 2-year period were reviewed. The data analyzed included age, sex, size of aneurysm, ruptured or unruptured, endovascular techniques, angiographic results after embolization, duration of follow-up, angiographic follow-up results and Glasgow Outcome Score at follow-up. Results: Of the 38 patients, 33 patients had 35 dissecting aneurysms and five patients had five saccular aneurysms. Seventeen (42.5%) aneurysms were ruptured. Of the 34 patients treated with endovascular techniques, immediate post-procedural angiograms showed complete and subtotal occlusion (>90%) of 27 (67.5%) aneurysms and incomplete and no occlusion of 13 (32.5%) aneurysms, including four conservatively treated aneurysms. A clinical improvement or stable outcome was achieved in all the patients (100%) during a mean 12.1-month follow-up. There was no complication related to endovascular treatment and no rebleeding during the follow-up period. Angiographic follow-up (mean of 7.2 months, range 1-18 months) was available in all the patients. Complete and subtotal occlusion was observed in 31 (81.6%) patients, including one spontaneous thrombosis of a conservatively treated VA dissecting aneurysm. Recanalization in two patients (5.9%) at 6 and 9 months did not require retreatment. Conclusion: This series demonstrates the safety and efficacy of multimodality of endovascular techniques for VA aneurysms.

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Background: Neuroendoscopic procedures are now being performed more frequently, and with advancement in technology, complications related to the procedure and equipments have also minimized or changed. We report our experience with 223 patients who underwent intracranial neuroendoscopic procedures. Materials and Methods: The rates of various perioperative complications, both surgical and anesthesia related, during intracranial neuroendoscopic surgeries were studied. Data collected included demographics, patient's medical history and any associated comorbid conditions, diagnosis, procedure performed, anesthetic management, intraoperative and postoperative complications and outcomes. Results: Of the 223 patients studied, 119 were pediatric (age <14 years) and 104 were adults. Hypothermia (25.1%) and cardiovascular complications (such as tachycardia 18.8%, bradycardia 11.3%, hypertension 16.1%, and hypotension 16.6%) were the commonly observed complications during intraoperative period both in pediatric and adult patients. At the end of the procedure, delayed arousal was observed in 17 patients and 19 patients required postoperative ventilatory support. Postoperative frequent complications included: fever (34.1%), tachycardia (32.7%), nausea and vomiting (18.8%). Potentially fatal complications such as intraoperative hemorrhage, air embolism, etc. were rare. Most of the complications were transient and self-limiting. Conclusion: Although endoscopic procedures are considered minimally invasive, at times may lead to life-threatening complications and one should be aware of them.

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Treatment of Type 1 carotid-cavernous fistula (CCF) is complex and endovascular stent grafting is proving to be an excellent technique not only in successful treatment of fistula but also preserving patency of parent artery. We describe our initial experience in the use of covered coronary stent grafts in the treatment of three patients with Type 1 post-traumatic CCF. All patients were successfully treated with placement of stent grafts. Immediate closure of fistula was achieved in all the three patients. One patient developed partial in-stent thrombosis. In this patient antiplatelet therapy had to be stopped as he developed a small intracerebral hematoma post procedure. Subsequently, he was restarted on antiplatelets and recovered completely. Except for this no other complication was observed. Covered stent grafts may be the procedure of choice for treatment of post-traumatic Type 1 CCF especially in young patients with favorable anatomy.

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The purpose of this study was to investigate the characteristics of six patients with dural arteriovenous fistula (DAVF) with drainage directly into the perimedullary venous system. In five patients with subarachnoid hemorrhage (SAH), cerebral angiography revealed a DAVF with spinal venous drainage located at the petrosal sinus in one, at the tentorium in one, and at the craniocervical junction in four. In the patient with myelopathy, angiographic exploration began with a spinal angiogram. Bilateral vertebral angiography initially failed to demonstrate the fistula, and a tentorial DAVF was established with carotid artery angiography. Patients had no myelopathy when the venous drainage was limited to the cervical cord; myelopathy was present when the venous drainage descended toward the conus medullaris. Diagnosis of a DAVF presenting with myelopathy is more challenging than of those presenting with SAH.

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Objective: To discuss the role of FLOW 800 innovative software for analytical color visualization and objective evaluation of fluorescence videos obtained by microscope-integrated intraoperative indocyanine green (ICG) fluorescence angiography in arteriovenous malformations (AVM) surgery. Materials and Methods: Microscope-integrated intraoperative FLOW 800 was used and evaluated in three consecutive AVM surgeries over a period of two months. The role of FLOW 800 to distinguish feeding arteries from arterialized veins and other arteries was evaluated. Its advantages and limitations over conventional intraoperative ICG angiography were evaluated. Results: This software was found to be useful in identifying arterial feeders, arterialized veins and other arteries in all the three patients and it gives additional information on the status of AVM before and after clipping suspected feeders which is sometimes difficult to interpret on conventional ICG angiography. Conclusion: Flow 800 is a reliable and useful addition to microscope-integrated color ICG video angiography. Although its role is limited in deep-seated AVMs, if properly dissected and exposed it can give useful information which can be easily interpretable and reproducible.

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