Purpose: To evaluate the MR findings in clinically suspected cases of Hirayama disease. Materials and Methods: The pre and post contrast neutral and flexion position cervical MR images of eight patients of clinically suspected Hirayama disease were evaluated for the following findings: localized lower cervical cord atrophy, asymmetric cord flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component with flow voids. The distribution of the above features in our patient population was noted and correlated with their clinical presentation and electromyography findings. Observations: Although lower cervical cord atrophy was noted in all eight cases of suspected Hirayama disease, the rest of the findings were variably distributed with asymmetric cord flattening, abnormal cervical curvature, anterior shifting of the posterior wall of the cervical dural canal and enhancing epidural component seen in six out of eight (75%) cases. An additional finding of thoracic extension of the enhancing epidural component was also noted in five out of eight cases. Conclusion: Dynamic post contrast MRI evaluation of cervicothoracic spine is an accurate method for the diagnosis of Hirayama disease.

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Vasospasm, so commonly described after aneurysmal rupture, is very rare after surgery for brain tumors. Its occurrence after transsphenoidal surgery is extremely uncommon with only three cases reported as per the authors' review of the literature. The authors report a case of pituitary macroadenoma in a 34-year-old female who underwent transsphenoidal tumor decompression and developed angiographically documented vasospasm during the course of her illness. The rarity of the occurrence of vasospasm after transsphenoidal surgery makes its management very challenging, frequently leading to a delay in diagnosis and a poor outcome. The key to successful management is a high index of suspicion and early proactive management.

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Context: Glomus jugulare (GJ) tumors are paragangliomas found in the region of the jugular foramen. Surgery with/without embolization and conventional radiotherapy has been the traditional management option. Aim: To analyze the efficacy of gamma knife radiosurgery (GKS) as a primary or an adjunctive form of therapy. Settings and Design: A retrospective analysis of patients who received GKS at a tertiary neurosurgical center was performed. Materials and Methods: Of the 1601 patients who underwent GKS from 1997 to 2006, 24 patients with GJ underwent 25 procedures. Results: The average age of the cohort was 46.6 years (range, 22-76 years) and the male to female ratio was 1:2. The most common neurological deficit was IX, X, XI cranial nerve paresis (15/24). Fifteen patients received primary GKS. Mean tumor size was 8.7 cc (range 1.1-17.2 cc). The coverage achieved was 93.1% (range 90-97%) using a mean tumor margin dose of 16.4 Gy (range 12-25 Gy) at a mean isodose of 49.5% (range 45-50%). Thirteen patients (six primary and seven secondary) were available for follow-up at a median interval of 24 months (range seven to 48 months). The average tumor size was 7.9 cc (range 1.1-17.2 cc). Using a mean tumor margin dose of 16.3 Gy (range 12-20 Gy) 93.6% coverage (range 91-97%) was achieved. Six patients improved clinically. A single patient developed transient trigeminal neuralgia. Magnetic resonance imaging follow-up was available for 10 patients; seven recorded a decrease in size. There was no tumor progression. Conclusions: Gamma knife radiosurgery is a safe and effective primary and secondary modality of treatment for GJ.

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Aims: To investigate the characteristics and surgical treatment of traumatic intracranial pseudoaneurysms. Materials and Methods: Twelve patients with traumatic intracranial pseudoaneurysms were operated on in our hospital between 2000 and 2006. Their clinical characteristics, radiological features and surgical techniques were analyzed retrospectively. Four traumatic cavernous segment pseudoaneurysms underwent trapping of the internal carotid artery and others underwent "neck reinforcement and clipping" or "crevasse clipping". Results: Nine patients were excellent or good and two patients were poor when they discharged. One patient died of postoperative cerebral infarction. Nine patients underwent follow-up (three months to seven years) and rebleeding was not seen in them. Conclusions: The surgical treatment of traumatic intracranial pseudoaneurysms is risky and difficult and individualized surgical option is necessary. Understanding the compensation of intracranial blood circulation, preoperative "Matas test" if it is necessary, perioperative hemodynamics testing and the application of revascularization techniques, will help reduce surgical risk and achieve a good surgical outcome.

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Asleep-awake-asleep technique of anesthesia is used during awake craniotomy with or without securing airway. We assessed this technique using laryngeal mask airway (LMA) in two patients. Patients underwent awake craniotomy for epilepsy surgery and the removal of a frontotemporal glioma. After anesthesia induction, airway was secured using LMA. Anesthesia was maintained using oxygen, nitrous oxide and sevoflurane, supplemented with an infusion of propofol and remifentanil. Twenty minutes before corticography, anesthesia was discontinued and LMA removed. Both patients were awake and cooperative during the neurological assessment and surgery on eloquent areas. The LMA was reinserted before the closure of the dura and remained in place until the end of surgery. Both patients had no recall of events under anesthesia, although experienced mild pain and discomfort during awake phase of surgery. Both expressed complete satisfaction over the anesthetic management. Asleep-awake-asleep technique using LMA offers airway protection. The painful aspect of surgery can be performed under anesthesia, hence minimizing the duration of stress and pain. Patients remained awake and cooperative throughout the time of neurological testing.

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Background: Repeat gamma knife radiosurgery (GKRS) is considered to be an effective treatment for refractory or recurrent trigeminal neuralgia (TN). Aims: The purpose of this report was to demonstrate the relationship between the outcome of repeat GKRS and prior operative procedures on patients with recurrent or refractory TN. Materials and Methods: A retrospective analysis was performed on 34 patients with refractory or recurrent TN who had undergone repeat GKRS; 21 patients had undergone other types of procedures, 11 of whom had undergone more than three such procedures prior to radiosurgery. The maximum dose of the repeat procedure was between 60 and 75 Gy. The mean follow-up time was 21.6 months. Statistical Analysis Used: The log-rank test and Fisher's exact test were used to analyze the data. Results: Excellent pain relief was achieved in 14 patients (41.2%) after repeat GKRS, while a successful outcome occurred in 29 of 34 patients (85.3%). Better pain relief occurred in the patients who did not have a prior procedure or who had undergone fewer than three prior procedures ( P = 0.042). Twenty-four of 25 patients (96.0%) who had recurrent pain had a successful operation and five of nine patients (55.6%) who did not have significant relief of pain after the first procedure had a successful operation. The difference was statistically significant ( P < 0.01). Only four patients had mild complications. Conclusion: It is more likely to relieve pain in patients with recurrent or refractory TN who did not have a prior procedure or who had fewer than three procedures before undergoing their first GKRS. Moreover, it seems that patients who had a good response following the initial GKRS had better results after a repeat procedure.

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Recognition of the magnitude of the burden and disability and mortality consequent to neurological disorders has led to global initiatives of declaring them as a "global epidemic", emphasizing public health approach and integration of neurology care with general health care. Epidemiological transition with increase in neurological disorders in India, the gross mismatch between the need and the available trained manpower and infrastructural facilities are posing challenges to health planners and policy makers for providing neurological care to the community. Alternative approaches of optimal utilization of rural, community health, satellite clinic and district models of the health care with close interaction with tertiary centres, nongovernmental agencies and private sector may facilitate achieving the goal of taking neurology care to the 'unreached'.

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Background: Treatment of brain abscess is still a subject of controversy. Simple therapeutic approaches like twist drill/burr hole aspiration with or without insertion of a drain are also quite effective. There are reports of encouraging results following endoscopic treatment. We are reporting our results of endoscopic approach on 24 patients. Materials and Methods: This is a prospective study on 24 patients of brain abscesses treated between January 2004 and January 2007. All the cases except those with small abscesses (less than 1.0 cm in diameter) and multiloculated abscesses were included. Gabb 6-degree rigid endoscope was used. Repeat CT scan was done in all cases within 7 and 30 days after surgery. Ten patients (42%) had small residual abscess on 7 ^th post-operative day's CT scan, while 30 ^th post-operative day's CT scan did not show any significant lesion in all the cases. Results: There were 23 patients of chronic otitis media and one of congenital cyanotic heart disease. Glasgow coma score (GCS) was 3 in one patient, 13 in two cases, 14-15 in 21 cases. There were 14 cerebellar, 8 temporal and 1 frontal and thalamic abscess each. All the patients recovered completely except one who died (GCS 3). There was no procedure-related complication. Hospital stay ranged from 7 to 12 days with an average of 8.2 days. Follow-up ranged between 6 and 42 months. Conclusion: Endoscopic aspiration of brain abscess appears to be a safe and effective alternative method of treatment. There is direct visualization of abscess cavity, completeness of aspiration can be assessed, and perioperative bleeding can be controlled.

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Context: Acute oxidative stress following a traumatic head injury (HI) has been implicated in inducing severe secondary brain damage and influencing the clinical outcome of HI patients. Aims: This study was performed to evaluate and compare the oxidative changes in patients with varying severity of HI in the early posttraumatic period using erythrocyte indicators. Settings and Design: Head injury patients were divided into two groups based on their Glasgow Coma Scale (GCS) scores recorded at admission to the hospital on the day of trauma itself. Accordingly, the study included 30 severe HI (SHI, GCS scores 8 or less) and 25 Mild HI (MHI, GCS scores more than 8) patients. Thirty age and sex-matched healthy individuals were included in this comparative study as controls. Materials and Methods: Blood samples were obtained from controls and HI patients (within 24 h of trauma onset). Erythrocyte oxidative changes were studied by estimating thiobarbituric acid reactive substances (TBARS), glutathione (GSH), superoxide dismutase (SOD) and glutathione reductase (GR). Results: Erythrocyte TBARS levels were significantly higher and GSH levels were significantly lower in SHI and MHI patients as compared to controls. The SOD activity was significantly increased only in SHI patients and remained unchanged in MHI patients as compared to controls. As compared to MHI patients, erythrocyte TBARS levels were significantly higher, GSH levels were significantly lower and SOD activity was markedly elevated in SHI patients. Erythrocyte GR activity did not show significant changes in both groups of patients as compared to controls. Conclusion: Oxidative stress is evident in both SHI and MHI patients in the early posttraumatic period as reflected by their erythrocyte indicators, but the severity of oxidative stress has varied relatively with the severity of head injury. The present findings provide indications that early oxidative changes could influence the neurological recovery of HI patients.

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A 20-year-old male had torticollis and short neck since birth. He presented with symptom of progressive quadriparesis over a two-year period. Investigations revealed basilar invagination with marked rotation in the craniovertebral region and relatively large C3-4 region osteophytes. Serial MRI over two years showed persistent signal opposite C3-4 disc space suggestive of cord compression. Although the cord was humped over the odontoid process, there was no clear radiological evidence that the cord was compromised at this level. During surgery, instability was identified only at the craniovertebral region and not at the level of C3-4. Distraction of the lateral masses of atlas and axis and fixation using interarticular spacers and bone graft and direct screw implantation in the lateral mass of the atlas and pars of the axis resulted in reduction of the basilar invagination and of atlantoaxial dislocation. The patient had marked clinical recovery, despite the fact that no direct procedure was done for C3-4 disc decompression. The case suggests that C3-4 disc changes could be secondary to primary instability at the craniovertebral junction.

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Cyclopentolate is a synthetic anti-cholinergic agent widely used in ophthalmology clinics. It can cause cardiovascular side-effects such as hypertension, ventricular arrhythmias and tachycardias. A 55-year-old male lost his consciousness after topical cyclopentolate hydrocloride (1%) administration for routine fundoscopic examination in another center. An urgent cranial magnetic resonance imaging examination revealed a midbrain hemorrhage and he was transferred to our hospital. The Glasgow Coma Scale score was at 4 points at admission. The patient was transferred to the intensive care unit and mechanically ventilated. Despite vigorous medical treatment, spontaneous respiration and brainstem reflexes disappeared 12 h after his administration. A control cranial computerized tomography showed enlargement and opening of the hemorrhage into the ventricular system. The patient died on the 12th day of his administration. Systemic side-effects of topical ocular cyclopentolate administration and prevention methods were discussed with regard to the current literature.

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Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is included among trigeminal autonomic cephalalgias in the International Classification of Headache Disorders-2. Available literature suggests that it responds to anticonvulsants, particularly lamotrigine. However, management of partial responders is difficult and antiepileptic duo-therapy may be an answer to it. Nonetheless, to our knowledge, anticonvulsant combination has never been tried in partial responders to monotherapy. We are presenting a case of SUNCT that had overlapping symptoms with Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic features and responded well only to the combination of lamotrigine with carbamazepine. However, lamotrigine had to be stopped as patient developed leucopenia and it resulted in partial recurrence of symptoms.

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Tuberous sclerosis complex (TSC) is an inherited genetic disorder commonly associated with neuropsychiatric complications like epilepsy, mental retardation, autism and other behavioral problems and constitutes about 1-4% of the autistic population. Mental retardation and seizures, particularly infantile spasms are significant risk factors for the development of autism. Patients of TSC with autism are more likely to have temporal tubers than those cases without autism. We describe clinical and neuroimaging features of two such cases of tuberous sclerosis with autism.

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Young adults with stroke frequently do not have any of the traditional risk factors associated with stroke, prompting a search for other mechanical and hypercoagulable causes. The authors report a young man presenting with stroke and subsequently diagnosed with a carotid dissection. Recurrent strokes while on heparin prompted a search for a second etiology and the patient was found to have antiphospholipid antibody syndrome. Although these conditions may be coincidental, we propose that their interaction was significant in this patient's presentation. Other reports of this association will also be reviewed.

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Chondromyxoid fibroma is a rare benign bone tumor representing less than 0.5% of all bone tumors. It commonly involves the long tubular bones. Involvement of the spine is rare. A 35-year-old man presented with history of neck pain, restriction of neck movements, pain and numbness along the medial aspect of the left forearm and weakness with wasting of the left hand. A presumptive diagnosis of a bony tumor such as an aneurysmal bone cyst or a giant cell tumor involving the seventh vertebral body was made on plain X-rays, MRI and bone scan. He underwent C7 central corpectomy, incomplete intralesional curettage with iliac bone grafting and C6 to T1 interspinous wiring. The histological diagnosis was chondromyxoid fibroma. On eight years' follow-up, CT scan showed no progression of the tumor with good alignment and fusion of the graft at the site of the corpectomy. The authors conclude that corpectomy and iliac bone grafting for chondromyxoid fibroma has a good outcome on long-term follow-up.

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Background: There is paucity of studies evaluating the role of asymmetry index (AI) on single photon emission computed tomography (SPECT) studies in patients with intracerebral hemorrhage (ICH). Aim: To evaluate cerebellar perfusion in ICH employing SPECT study and correlate with clinical and CT scan findings. Setting and Design: Tertiary care teaching hospital. Materials and Methods: A total of 29 patients with ICH were subjected to neurological examination including Glasgow Coma Scale (GCS) and Canadian Neurological Stroke Scale (CNS). Clinical features of raised intracranial pressure and herniation were noted. On CT scan, ICH location, volume, ventricular extension and midline (ML) shift were noted. On SPECT, cerebral and cerebellar perfusion was measured semiquantitatively and AI calculated. Outcome was defined at 3 months into poor and good. Results: Fourteen patients had putaminal and 15 thalamic hemorrhages. Their mean age was 59 years. The mean GCS score was 10 and CNS score 2.8. Hematoma was large in five, medium in 16 and small in eight patients. ML shift was present in 15 and hematoma extended to ventricule in 16 patients. On SPECT, cerebellar AI significantly related to ML shift but not with size of hematoma. AI was low in patients with ML shift. Outcome was related to GCS score, ML shift, size of hematoma and cerebellar AI. Conclusion: In acute stage of ICH, cerebellar AI is lower in patients with more severe stroke having ML shift.

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Background: The autonomic nervous system in Behcet's patients may be affected due to various reasons. This entity may be detected with the measurement of the electrodermal activities, heart rate variability and pupillometric methods. Habituation is one of the implicit forms of learning and memory and the loss of habituation can reveal pathological changes in the synaptic regions. Aim: To determine whether there is a functional decrease in the synaptic effectiveness (habituation) of the pathways to sympathetic neurons that had been repeatedly activated in Behcet's. Materials and Methods: Twelve patients with Behcet's disease and 12 healthy controls were included in the study. Sympathetic skin potential (SSP) records were taken at normal room temperature in a quiet place within a Faraday cage. Sixteen square wave single shock impulses (duration: 1200 ms, strength: 5 mA) were applied on each case. Results: After the 1 ^st stimulus, the SSP amplitudes were lower in the patients compared to the controls ( P <0.001, t value = 7.69). There was no significant differences among the SSP amplitudes after the 13 ^th impulse in the patients ( P >0.05). Whereas there was no significant differences among the SSP amplitudes after the 9 ^th impulse in the controls ( P >0.05). The habituation rate of the SSP after consecutive impulses was slowest in the patients compared to controls ( P <0.001, t value = 12.39). Conclusions: There is a delayed habituation in Behcet's disease and that may due to pathologic changes with vasculitis through their peripheral nerves.

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Background: Gustatory dysfunction associated with vestibular schwannomas (VS) is a poorly represented clinical presentation. Materials and Methods: One hundred and forty-nine cases operated from 1997 to 2005 where at least six-month follow-up was available were included. All patients were tested for taste sensations using four modalities of standard taste solutions. Apart from the taste sensations, any altered or abnormal taste perceptions were recorded both in the preoperative and postoperative period. Results: After applying the exclusion criteria, the taste dysfunction was studied in 142 patients. The evidence of decreased taste sensation was found in 58 (40.8%) patients prior to surgery. Preoperatively, taste disturbance was found in 29 (37.2%) giant, 28 (45.9%) large and one (33.3%) medium-sized tumors, respectively. There were no significant age or sex-related differences. The postoperative taste disturbances were found in 65 (45.8%) patients. Among patients with anatomically preserved facial nerve, postoperative taste disturbances were found in 55 (42.3%) patients whereas nine (6.9%) patients reported improvement in taste sensations. Conclusions: Taste dysfunction is common following vestibular schwannoma surgery. Patient counseling prior to surgery is necessary to avoid any distress caused by taste dysfunction. Taste dysfunction should be included in the facial nerve functional grading system while assessing outcome.

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Background: The success of Duchenne muscular dystrophy gene therapy requires promising tools for gene delivery and mini-gene cassettes that can express therapeutic levels of a functional protein. Aims: To explore the expression feasibility of truncated dystrophin cDNAs mediated by a lentiviral vector derived from feline immunodeficiency virus. Materials and Methods: Three truncated dystrophin cDNAs were constructed by PCR cloning, then these cDNAs were inserted into lentiviral vectors. Recombinant lentiviruses were generated by transient transfection of lentiviral vector constructs into 293Ad ⁵⁺ cells. Cultured myoblasts were then infected with recombinant lentiviruses. Expression of truncated dystrophin cDNAs was detected by Western blot analysis. Results: Mediated by lentiviral vectors, three cDNAs constructed by PCR cloning expressed relative truncated dystrophins in cultured myoblasts. Conclusions: Truncated dystrophin cDNAs can express themselves successfully mediated by feline immunodeficiency virus vectors. It offers the possibility of an approach utilizing truncated dystrophin cDNAs and lentiviral vectors toward gene therapy of Duchenne muscular dystrophy.

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We present a 43-year-old female patient who had recurrent headache for one year. An intracranial bony lesion surrounded by a cyst in the anterior cranial fossa was found on imaging. Postoperative histological examination confirmed the diagnosis of osteoma and mucocele.

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A 72-year-old male was admitted with subarachnoid hemorrhage associated with a ruptured cerebral aneurysm. The aneurysm was treated with clipping soon after radiological examination. Eight weeks after the treatment, the patient suffered from secondary hydrocephalus resulting from blockage of the subarachnoid space due to subarachnoid granulation. Previous pathological examination revealed the granulation was associated with hemosiderin deposition. We investigated subarachnoid hemosiderin deposition in this patient using T2*-weighted (T2*-w) magnetic resonance image (MRI), a sensitive method for hemosiderin detection. computed tomography (CT) cisternography demonstrated that cerebrospinal fluid (CSF) flow was disturbed adjacent to sites of subarachnoid hemosiderin deposition on T2*-w MRI. Placement of a ventriculo-peritoneal shunt contributed to neurological improvement. In this case, T2*-w MRI was an effective means of diagnosing the location of disturbed CSF flow associated with subarachnoid hemosiderin deposition.

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