Vein of Galen malformations are unique congenital malformations of the cerebral vasculature that result in persistence and ‘aneurysmal’ dilatation of the venous structures. The varied clinical presentations and their distinctive and complex angioarchitecture make it important for the caring physician to understand their embryological and pathophysiological aspects. Management of these lesions – both in the neonatal period and at the time of definitive intervention, is challenging. Considering the rarity of these lesions, there are very few studies that have been able to compare the results of different techniques in the management. Continuing developments in the diagnostic as well as interventional aspects during the last two decades have radically changed the management of these lesions. Antenatal diagnosis and referral to a center with facilities for advanced neonatal cardiac care as well as for interventional neuroradiological therapy can go a long way in improving the prognosis in these children.

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The present report highlights an unusual presentation of vitamin B12 deficiency— recurrent seizures in a 26-year-old man. His symptoms responded to parenteral vitamin B12 therapy. The relevant literature is reviewed.

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Background: Lateral cutaneous femoral nerve (LCFN) injury or Meralgia paraesthetica (MP) results in restriction of activity. Compression of the nerve by disc hernia, retroperitoneal tumors, and external pressure around the anterior superior iliac spine is common. However, it is not commonly observed after lumbar spinal surgery in prone position. Study design: In this prospective study of 110 patients who underwent elective lumbar spinal surgery, managed from January 2002 to June 2002, the incidence, possible risk factors, etiopathogenesis and management of MP were analyzed. Results: There were 66 males and 44 females. The age of the patients ranged from 15 to 81 years (mean 46.9 yrs.). Thirteen patients (12%) suffered from MP. It is more common in thinner individuals due to pressure injury to the nerve at its exit point. Ninety-two per cent of the patients were asymptomatic at follow-up after 6 months. In 7 out of 13 patients, patchy sensory loss on clinical examination was seen at 6 months. Conclusion: MP after posterior lumbar spinal surgery is uncommon. Smaller bolsters may avoid some of the vulnerable pressure points, as the surface area available is relatively smaller. The posts of the Hall-Relton frame over the anterior superior iliac crest should be adequately padded. The condition is usually self-limiting. Surgical division or decompression of the LCFN is reserved for persistent or severe MP.

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Background: Several prognostic factors have been described but there are few studies evaluating the prognostic importance of seizure in patients with glioblastoma multiforme (GBM). Aims: To evaluate the prognostic importance of seizure at the time of the diagnosis of glioblastoma multiforme (GBM) and compare it with other known prognostic factors. Settings and Design: Between January 1994 and December 2000, 81 patients underwent irradiation for intracranial GBM at our institution. The criteria for inclusion in this study were biopsy-proven GBM, being treated for primary disease. Seventy-six patients were retrospectively evaluated and the remaining five patients could not be enrolled due to lack of details. Material and Methods: The prognostic importance of age, sex, performance status, a history of seizure at diagnosis, extent of surgery, radiotherapy field and dose were studied. Statistical analysis: The Kaplan-Meier method, the Log rank test, the Cox proportional hazard model and the Mann-Whitney U test were used for statistical analysis. Results: Survival at first and second years was 19.74% and 4.81%, respectively. Univariate analysis revealed age, performance status, history of seizure, and radiotherapy dose as significant prognostic factors and with multivariate analysis age, history of seizure and radiotherapy dose were positive prognostic factors. Conclusion: This study concluded that in GBM, history of seizure prior to diagnosis of GBM was a positive prognostic factor.

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The results of the International Subarachnoid Aneurysm Trial (ISAT) drew attention from both scientific and lay press, impacting the management of aneurysm patients significantly. In this review, the ISAT report was analyzed critically and the available literature was scrutinized stratifying the common criticisms as to the weak aspects of this study. The aim of ISAT was to compare the safety and efficacy of endovascular coiling with neurosurgical clipping for aneurysms, which were suitable for both treatments. The results showed a 22.5% relative and 6.9% absolute risk reduction at one year in the disability outcome of patients who were treated with coiling. However, long-term risk of re-bleeding from the treated aneurysms and the risk of repeat procedures was higher in this group also. Lack of angiographic data following the initial treatment and long-term follow-up represents one of the main flaws of this study. The outcome assessment scale, biases regarding patient selection and center participation criteria were further issues of criticism. The results of ISAT are not sufficient to provide a definitive answer as to the superiority of endovascular treatment over microsurgery, although coiling appears to produce less peri-procedural morbidity in a selected group of patients. An optimum outcome assessment should include a universally accepted scale and a detailed long-term angiographic outcome.

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From 1996-2002 we treated 5 consecutive cases of pial fistula. There were 3 patients with a single hole-single channel pial fistula and two patients had a complex pial fistula. Three patients presented with intracerebral hematoma and had a focal neurological deficit. One patient presented with history of seizures and 1 patient had headache. The results of the treatment were analyzed both clinically and angiographically. The follow-up period ranged from 6 months to 6 years. All fistulas were treated with concentrated glue. The glue cast included the distal part of the feeding artery, A-V connection and the proximal part of the vein. Post-embolisation angiography showed complete occlusion of two single-hole fistulas and one complex pial A-V fistula and near total occlusion of one single-hole and one complex pial A-V fistula. Four patients had excellent clinical outcome. One patient with single-hole fistula had a hemorrhagic venous infarct resulting in transient hemiparesis.

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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is an inherited arterial disease, commonly overlooked or misdiagnosed. We report a case of CADASIL in a 51 years old woman who presented with progressive subcortical dementia, recurrent ischemic events and seizures in the absence of known vascular risk factors of five years’ duration. Her mother had a history of similar illness. Magnetic resonance imaging (MRI) of brain revealed subcortical and deep white matter hyperintense lesions within the cerebral white matter on T2-weighted images. DNA mutation of Notch 3 gene confirmed the diagnosis of CADASIL.

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Background: Neurological soft signs (NSSs) are defined as abnormal motor or sensory findings, including involuntary movements, a variety of dispraxia, difficulties in performing rapid alternating movements, difficulties in two-point discrimination, and graphesthesia in a person without a neurological disorder which can be determined as its focus. Aims: to investigate the relationship of NSSs with obsessive-compulsive disorder (OCD). Settings and Design: This study was designed in the Psychiatry Polyclinic of Ondokuz Mayis University Hospital. After signing an informed consent form, all the subjects were divided into 2 groups: (1) the patient group and (2) the control group. Material and Methods: Thirty consecutive patients presenting with DSM-IV OCD were included in this study. The control group consisted of 30 healthy subjects without a psychiatric/neurological disorder. All subjects underwent a physical and neurological examination for soft signs (PANESS). Statistical analysis used: The Mann-Whitney U test was used for statistical analysis of data. Results: It was seen that graphesthesia, two-point discrimination, and total PANESS scores were significantly higher in the group with OCD than the control group. In other NSSs, there was no significant difference between the patient and control groups. Conclusions: Unlike some studies, in the present study, the difference between the groups in graphesthesia compared to other NSSs was significant. The results of this preliminary study suggest that there is a relationship between NSSs and OCD. We think that NSSs may point to a structural brain abnormality in patients with OCD.

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Acute Disseminated Encephalomyelitis (ADEM) is an acute or subacute condition characterized by widespread multifocal neurological deficits of variable severity. We report a case of a 45-year-old man with ADEM following Anti-Rabies vaccine (ARV).

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Aims and Objectives: A clinico-pathological study of cerebral aneurysms was undertaken to understand the histopathogical nature of the lesions and to ascertain possible etiological risk factors. Material and Methods: Of the 255 cases of cerebral aneurysms operated upon at our Institute during the two-year period between Jan 1999 to Dec 2000, a detailed study was conducted on 57 cases where the aneurysm sac could be excised and subjected to histopathological examination. Aneurysm sacs were fixed in 10% buffered formaldehyde and processed through graded alcohol. Paraffin-embedded sections were examined, using hematoxylin and eosin, Verhoeff van Gieson’s and toluidine blue staining techniques. Histopathological observations were analyzed and correlated with clinical features. Results: The ages of the patients ranged from 17-65 years and there were 33 males and 24 females. Twenty-six patients were chronic smokers and 19 patients had hypertension. There were 54 saccular and 3 fusiform aneurysms, predominantly involving the anterior circulation (52 cases) than posterior circulation (5 cases). In 35 cases, histopathological studies demonstrated mucoid deposits between hyperplastic cellular elements in the true and false aneurysm wall and/or parent artery or vasavasora. The changes were associated with dystrophic changes in the internal elastic lamina. Conclusion: Besides significant risk factors like smoking and hypertension, such mucoid vasculopathic changes may have predisposed vessels to structural weakness and aneurysm formation in our patients.

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Aims and objectives: To assess the efficacy and results of expansive laminoplasty in advanced (Nurick’s Grade III or greater) cervical myelopathy. Materials and Methods: We reviewed data in 24 patients who underwent cervical laminoplasty from January 1999 to December 2002. Nuricks grading was used for quantifying the neurological deficits and outcome analysis was done using Odom’s criteria. A modified Hirabayashi’s open door laminoplasty was done using Titanium miniplates and screws in 22 patients, autologous bone in one and hydroxyapatite spacer in one patient. Observations: There were 3 females and 21 males with a mean age of 56 years (range 39-72 years). Four patients presented in Nuricks Grade III, 15 in Grade IV and five in Grade V. MR imaging showed MSCS in 21 cases, OPLL in nine cases and ligamentum flavum hypertrophy in nine cases with cord signal changes being present in 19 cases. Results: All patients with duration of symptoms less than three years, and 50 % with duration ranging from three to six years had improvement by at least one Nurick’s grade following surgery. Eighty-seven per cent Grade IV patients (ambulatory with support) improved to Grade III (ambulatory without support) following laminoplasty. Using Odom’s criteria, 23 patients (95.8%) had a good to fair outcome. Conclusions: Cord decompression with expansive laminoplasty using titanium miniplate fixation may improve the neurological outcome even in patients presenting late, and improvement by even one grade may have major ‘quality of life’ benefits for these patients.

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An unusual disorder of cerebellar development, rhombencephalosynapsis is a unique entity which presents with cerebellar fusion and absence of cerebellar vermis on imaging studies, often associated with supratentorial findings. No specific clinical syndrome has been described in these patients so far, and most cases are found in infancy and childhood. MRI and its multiplanar capabilities and high spatial and contrast resolution increased its recognition. Two cases are reported, with emphasis on imaging findings.

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The pathophysiological mechanisms and the salient features of normal perfusion pressure breakthrough (NPPB) are discussed on the basis of an operated case of arteriovenous malformation.

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We describe functional imaging findings using MRI, 1H-Magnetic resonance spectroscopy and positron emission tomography in a case of radiation-induced medulloblastoma following radiotherapy for pineal gland tumor. MRS showed a prominent choline peak; FDG, 11C-Met and 11C-Choline PET showed a minimal glucose, increased methionine and choline uptake.

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Background: Cerebral perfusion pressure management (CPPM) is an accepted modality of treatment of severe diffuse head injury (SDHI). However, CPPM has the potential to cause transcapillary exudation in the presence of a disrupted blood brain barrier and can lead to further increase of intracranial pressure (ICP) and worsening of compliance. Aims: This study attempts to evaluate the effect of both transient and prolonged changes in cerebral perfusion pressure (CPP) on ICP and cerebral compliance as measured by the Pressure Volume Index (PVI), and to correlate changes in PVI with outcome at 12 months using the Glasgow Outcome Score. Settings and Design: Prospective study in a neurosurgical ICU. Material and Methods: Twenty-seven SDHI patients managed using standard protocol to maintain CPP above 70 mmHg. Mean arterial pressure (MAP), ICP and CPP were monitored every half-hour. Daily monitoring of the PVI and ICP was done before, and after the induced elevation of MAP using IV Dopamine infusion. The relationship between CPP, MAP, ICP, PVI and outcome was evaluated. Statistical analysis used: The paired and independent samples T-test, and the Pearson correlation coefficient. Results: CPPM rarely leads to progressive rise in ICP. Maintaining CPP above 70mmHg does not influence ICP or PVI. Transient elevations in CPP above 70mmHg may produce a small rise in ICP. Trend of change in PVI influenced outcome despite similar ICP and CPP. Conclusion: Elevating the CPP above 70mmHg does not either reduce the ICP or worsen the compliance. Monitoring changes in compliance should form an integral part of CPPM.

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The fragile X syndrome is the most frequent cause of inherited mental retardation. It is caused by a dynamic mutation: the progressive expansion of polymorphic (CGG)n trinucleotide repeats located in the promoter region of the FMRI gene at Xq27.3. The cloning of the FMRI gene and the elucidation of the molecular basis of the fragile X syndrome is of great importance for the diagnosis and understanding of this unusual type of mutation. Although extensively studied, the mechanism behind the transition from stable normal (CGG)n alleles to the carrier state (an unstable premutation) and from premutation to mutation is partially understood. The clinical diagnosis of fragile X mental retardation (FXMR) is not possible as dysmorphic features are subtle. Molecular diagnosis by Southern Blot is the confirmatory test that makes carrier detection and prenatal diagnosis possible. As the risk of recurrence of FXMR is high in the family and carrier relatives, an identification of fragile X positive children, and offering carrier detection and prenatal diagnosis to the families is very important. It is possible by screening mentally retarded children and adults even if there is no family history of mental retardation or typical behavioral or physical features associated with the fragile X phenotype. In this review we have discussed the method for the diagnosis and counseling of the families. The complexities due to premutation and the variable severity of manifestations in carrier females need to be understood while counseling fragile X families.

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Three members of an Indian family with facio scapulohumeral dystrophy (FSHD linked to chromosome 4q35 with short EcoR1 segment of 23 Kb are reported where two male adults had schizophrenia. One family member developed isolated facial weakness with mild mental retardation. This genetically proven FSHD family is reported because of its uncommon associations.

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Satoyoshi syndrome (Komuragaeri disease) is a rare disorder of presumed autoimmune etiology, characterized by painful muscle spasms, alopecia, diarrhea, endocrinopathy with amenorrhoea and secondary skeletal abnormalities. Most of the previous reports are of the Japanese people. We report the first case from India.

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Isolated intracranial hypertension is a common manifestation of intracranial sino-venous thrombosis (ISVT). Markedly elevated intracranial tension presents with unusual features including cranial neuropathies and radiculopathy. We report two cases with ISVT, which presented with headache, papilledema, progressive visual loss, complete ophthalmoplegia and flaccid areflexic quadriparesis along with a normal sensorium. Magnetic resonance imaging (MRI) of the brain and cervical spinal cord showed no lesions that could account for the neurological deficits. Markedly elevated lumbar CSF pressure was noted in both cases. Nerve conduction study favored radiculopathy in one case and was normal in the other. Raised intracranial pressure was found to be the sole cause for the clinical manifestations. Visual impairment persisted in one patient despite lumbo-peritoneal shunting while the other died of septicemia. To our knowledge there are no previous reports of a syndrome comprising blindness, ophthalmoplegia and flaccid quadriplegia due to intracranial hypertension in ISVT.

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Background and Aims: We have utilized lift-up laminoplasty to treat patients with myelopathy caused by ossification of the posterior longitudinal ligament (OPLL) of the cervical spine. The preliminary surgical outcome with computer-assisted morphological assessment is presented. Material and Methods: The surgical technique of lift-up laminoplasty includes standard posterior exposure of the cervical spine, en-bloc laminectomy, and expansion of the cervical canal by lift-up of the laminae with custom-designed hydroxyapatite laminar spacers and stabilization of the laminae using titanium miniplates. From 1998 to 2003, 10 consecutive patients with cervical myelopathy secondary to OPLL have been treated with this method and comprehensively evaluated. Care was taken to tailor the treatment to individual patients by using different sizes of spacers to adjust the degree of expansion depending on the amount of stenosis of the cervical spine. The degree of expansion of the cervical canal was altered by design, based on the preoperative imaging simulation. Results: Preliminary surgical outcome, evaluated at 6 months after surgery, revealed a significant improvement of neurological function. Image analysis revealed that the cervical canals were significantly expanded, with a mean reduction of 13.1% in the stenosis ratio. Lift-up laminoplasty was effective in the treatment of patients with myelopathy secondary to cervical OPLL, and the amount of expansion could be individually adjusted at the discretion of the surgeon. Conclusion: Although analysis with a larger population and a longer follow-up period needs to be undertaken, our method of lift-up laminoplasty appears to be a viable choice among standard posterior cervical approaches for cervical OPLL.

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Sporadic paroxysmal kinesigenic dyskinesia (PKD) secondary to thyrotoxicosis is an extremely rare entity. A 36-year-old female presented with the features of PKD. Her investigations revealed thyrotoxicosis. Her dyskinesia did not respond to carbamazepine but remitted with the anti-thyroid drug, neomercazole. Perhaps hyperthyroidism-related PKD is a result of a metabolic disturbance of the basal ganglia circuits rather than a permanent and irreversible change.

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A senile Chinese female patient with alien hand syndrome of vascular etiology is reported. This case exhibited contradictive movement, left-lateral paresis and disorder of color discrimination, which might be a new subtype of the alien limb syndrome.

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A 27-year-old man suffered a relatively minor trauma. He developed signs of raised intracranial pressure three days after injury. Investigations revealed superior sagittal sinus and torcular thrombosis.

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We report a case of severe cervical spondylosis and atlantoaxial dislocation (AAD) in association with idiopathic cervical dystonia (ICD) in a middle-aged male. To our knowledge, this is the first case of ICD reported in association with AAD.

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Recurrent chronic subdural hematomas (CSDH) can be a therapeutic challenge. We report the use of reservoir shunts for continuous irrigation and drainage of the subdural space for a prolonged period. This system appears to be more useful than an external drainage system.

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Background: Regional differences in vascular response to anesthetic agents in brain areas with and without tumors have not been investigated till now. The existence of such differences may influence the regional distribution of cerebral blood flow under anesthesia. Material and Methods: In 8 patients with frontotemporal gliomas, middle cerebral artery blood flow velocity (Vmca), pulsatality index (PI) and resistance index (RI) were determined in the normal and pathological cerebral hemispheres before and after stable nitrous oxide-halothane anesthesia. Results: During halothane anesthesia, there was an increase in Vmca both in the normal cerebral hemisphere (69 ± 11 to 100 ± 38 cm/s; P = 0.05) and the hemisphere with tumor (65 ± 17 to 83 ± 28 cm/s; P = 0.04). A significant decrease in the pulsatality index and the resistance index was also observed in both the hemispheres (P < 0.05). The percentage changes of Vmca,, PI and RI in both the hemispheres after anesthesia were not significantly different. Conclusions: In patients with cerebral tumors, nitrous oxide-halothane anesthesia causes comparable changes of cerebral blood flow velocities in the hemisphere with tumor and the normal hemisphere.

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Aiming to achieve better results in microlumbar discectomy and foraminotomy, a double-hook retractor has been designed to retract lumbar paraspinal muscles away from the spinous process. A double-hook retractor obviates the limitations of single-hook systems.

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