Moyamoya is probably an inherited vasocclusive disease first described in Japan and now reported from all over the world. Genetic linkage studies and study of the factors possibly involved in its pathogenesis have shed new light on this disease. There is some suggestion that the pathogenesis may vary between races. A number of revascularization procedures have been devised. There is still controversy regarding the timing and the type of surgery. Functional MRI and PET scanning may provide more objective criterion on the outcome of these procedures. Neuropsychological studies may also be of use in judging the benefits of surgery. In this review, some of the recent advances in the study of the genetics and pathogenesis of the disease are reviewed and the clinical manifestation and the role of surgery are discussed. Relative lack of literature from outside Japan and the Korean peninsula has hampered the understanding of the disease in other countries.

In order to become cost-effective, epilepsy surgery centers in developing countries will have to achieve excellent results by selecting candidates destined to have a seizure-free outcome using locally available limited technology and expertise, without compromising on patient safety. Our experience illustrates that this goal can be accomplished by selecting patients, whose epileptogenic zone can be unquestionably established, based on history, magnetic resonance imaging, and interictal and ictal scalp electroencephalogram findings. Patients with mesial temporal lobe epilepsy, and those with circumscribed potentially epileptogenic lesions belong to this category. A stepwise approach by reserving more difficult to treat patients to later date as experience develops, or by referring them to a better-equipped center, will help each center to understand its capabilities and limitations and to move forward. It would be essential to work with and educate the local public and professionals, if the epilepsy surgery program in a developing region were to have a lasting impact, It is encouraging to note that, despite major challenges, in the last one-decade, several epilepsy centers in the developing world have not only successfully implemented epilepsy surgery programs, but also have produced results comparable to that from developed countries at a fractional cost.

Serial roentgenograms of 40 patients who had 70 cervical intervertebral spaces grafted with ethylene oxide sterilized cadaver bone and 28 patients who received 44 iliac crest auto grafts for anterior cervical spine fusion, were studied. The radiological evaluation was made on the basis of settlement of intervertebral spaces, fusion rate, delayed union, non-union, graft collapse and extrusion of the graft. Indigenous methodologies were designed for the assessment of settlement of grafted intervertebral spaces in percentage. Disc space settlement was more common in autografts (93% cases) than in allografts (80% cases). The average percentage of settlement of intervertebral disc space (S%) was 22 in autografts and 28 in allografts during the first four months. By the end of eight months, allograft disc spaces settle more. No significant difference was noted in fusion rate at the end of one year viz. allografts (90% cases) and autografts (93% cases). Autograft and allograft (ethylene oxide sterilized cadaver bone) are equally useful in anterior cervical spine fusions.

An experimental study was designed to compare the histological analysis of nerve anastomosis with 10-0 microsurgical sutures and fibrin adhesive. Wistar albino rats' sciatic nerves were transected and repaired either with fibrin adhesive-Beriplast P (M/s Centeon-Cadila Health Care) or with 10-0 monofilament microsutures. Histological assessment was performed at 10, 20, 30, 60 and 90 days after surgery. Functional recovery of the sciatic nerves started at two months and was near normal by three months. Separation of the stumps did not occur in any of the glued nerves. Histological evaluation showed no appreciable difference in the outcome of nerve regeneration after microsurgical repair using sutures or fibrin tissue adhesive. However, inflammation and granuloma formation were appreciated at the suture site, which presented a focal hindrance to myelin and axonal regeneration. Fibrin glueing is attractive for clinical purposes, since it is simpler and less time consuming than suturing.

Twenty five patients with sarcoglycanopathies were studied prospectively. 21 of them had mild phenotype. Muscle involvement was more pronounced in adductor and flexor groups of muscles of the limbs, hip adductor muscles being the weakest. The selective and differential weakness between weak hip adductors and stronger hip abductors resulted in the hip abduction sign in 64% of cases. Distal muscle involvement in lower limbs was seen in 92% of cases, but was mild and late in the course of the disease. 44% patients had winging of scapulae. Immunocytochemistry showed multiple sarcoglycan deficiencies in 84% patients. Primary beta and delta sarcoglycanopathy was seen in the remaining 16% cases. Secondary dystrophin reduction was seen in 44% patients and correlated with beta sarcoglycan deficiency but not with functional disability.

Several clinical signs have been described for lateralization and localization of seizure focus in complex partial seizures; however, the specificity of each of these has been widely debated upon. The present study was done to evaluate 'dystonic unilateral limb posturing' and 'versive head movements' for lateralization and localization of epileptic foci in patients with intractable partial complex seizures, being investigated with long term Video-EEG monitoring. Fifteen patients with 46 seizures, studied with long term Video EEG, had either one or both of these signs. The video recordings of the clinical behaviour were noted and later compared with the corresponding EEG. Unilateral dystonic limb posturing and versive head movements had good lateralising value in complex partial seizures, suggestive of temporal lobe origin, contralateral to the seizure focus, with a specificity of 87.5% and 86% respectively. In addition, turning of the whole body to one side was associated with a contralateral seizure focus in 100% cases. Appendicular automatisms were found to be of no lateralising significance, as they occurred on either side with equal frequency. These motor phenomena should, therefore, be enquired about in detail, during history taking for lateralization or localization of seizure focus.

Retrospective analysis of 27 patients of thalamic glioma including adults and children treated over a period of 7 years from 1991-1997 was done. The study group included 19 males and 8 females; 9 patients were less than 15 years and 18 patients more than 15 years of age at the time of diagnosis. The commonest symptoms were headache and vomiting. 12 patients underwent VP shunt as an initial procedure and 7 underwent total or partial surgical resection. Confirmed histopathological examination was possible in 16 patients; while 12 had low grade astrocytoma, 4 cases had high grade histology. All patients were treated with radiotherapy to a total dose of 50-60 Gy in 25-30 fractions. Median follow up was 9.63 months. The disease free survival in these patients was 28% at 2 years. Prognostic factors which included age, sex, duration of symptoms, surgical procedures, histology and radiotherapy dose were evaluated for significance. A subtotal resection conferred a better prognosis.

The exact diagnosis of demyelinating diseases is an enigma even in the best neurological centres. In the present study, the potential role of differential CSF proteins has been critically evaluated in differentiating multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). Cellulose acetate electrophoresis was carried out on CSF and serum samples of 14 MS patients, 23 ADEM patients and 30 controls. There was no statistically significant difference between serum electrophoresis of controls and MS patients. However, in case of CSF electrophoresis there was a statistically significant decrease in beta-1 fraction in 92.2% of MS patients (p=0.01). A comparison between serum electrophoresis of controls and ADEM patients indicated a statistically significant decrease in serum albumin in 87% patients and an increase of alpha-2 globulin in 73.9%. There was no statistically significant difference between CSF electrophoresis of controls and ADEM patients except for the prealbumin fraction which was raised in 60.9% of patients. No statistically significant difference was seen between the serum electrophoresis of ADEM and MS patients. However, on comparing CSF electrophoresis, it was seen that beta-1 fraction was significantly higher in ADEM patients (p<0.05). The predictive value of beta-1 fraction in differentiating MS and ADEM was then evaluated. The negative predictive value was 100% indicating that all samples with a beta-1 fraction of>6.5% cannot be diagnosed as MS. The significant decrease in beta-1 fraction in MS patients may prove to be an early indicator in differentiating between MS and ADEM patients.

Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.

Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. The diagnosis of idiopathic hypertrophic cranial pachymeningitis was based on neuroimaging findings of thickened enhancing dura, exclusion of known causes and histopathologic findings compatible with nonspecific inflammation in the meningeal biopsies. Corticosteroid therapy was effective in all cases in inducing a complete or partial remission of the neurologic symptoms and signs. We describe the clinical, radiological and pathological features of idiopathic hypertrophic cranial pachymeningitis and discuss the relationship of this entity with other inflammatory fibrosclerotic disorders to explain the pathogenesis. A high index of suspicion, prompt confirmation of the diagnosis by meningeal biopsy, and early institution and long-term maintenance of steroid therapy may help to prevent irreversible neurologic sequelae, especially blindness.

Therapeutic drug monitoring (TDM) of carbamazepine (CBZ) in saliva is an attractive alternative, because its collection is painless, non-invasive and simpler than drawing blood. Salivary drug levels, also closely reflect the free drug concentration. The aim of the study was to evaluate the suitability of saliva in routine TDM of CBZ in adult epileptic patients. Blood and saliva samples were taken simultaneously at 0 hours and 24 hours of CBZ dosing from 31 epileptic patients, receiving CBZ monotherapy for three or more months. Levels of CBZ in both these fluids were measured by high performance liquid chromatography. Strong and highly significant correlation was found between serum and salivary CBZ concentration (r = 0.659, p<0.001). Estimation of CBZ level in saliva is thus a practicable, valid and convenient method of TDM in epileptic patients.

Routine use of steroids in the treatment of bacterial meningitis remains controversial. A prospective placebo controlled double blind study of dexamethasone was carried out in 40 patients (age>10 years) of acute bacterial meningitis. The patients were randomly assigned to receive either placebo (n=20) or dexamethasone (n=20) in addition to injection ceftriaxone 100 mg/kg/day (maximum 4 gm/day) for 14 days. Dexamethasone sodium phosphate was given in dose of 0.6 mg/kg/day in 4 divided doses, for first 4 days of therapy. First dose of dexamethasone was given 15 minutes prior to first dose of ceftriaxone. Baseline demographics, clinical and laboratory features of the two groups were similar. Clinical improvement of signs of meningeal irritation was rapid in dexamethasone group than in the placebo group, but no significant difference was observed regarding resolution of fever, headache and vomiting. Secondary fever (mean+/-SD 15.00), gastrointestinal tract bleeding (mean+/-SD 15.00) and psychiatric manifestations (mean+/-SD 10.00) were more common in dexamethasone group. Neurological complications and hearing loss were more common and severe in placebo group as compared to the dexamethasone group (p<0.05). It is concluded that dexamethasone may be beneficial in some aspects of bacterial meningitis, in adults. A study with a larger number of cases in each group is recommended.

Asymmetrical tone and weakness in antagonistic muscles often result in misalignment of joint, contractures and deformities. Traditional static splints used to prevent these complications are expensive, have to be custom made and cannot be used when deformities are marked. Authors describe fabrication and use of pneumatic splints, which are economic, safe and easy to apply. These splints have wide application in the management of common medical problems in neurological rehabilitation like hypotension, edema, pain, spasticity and early deformities.

The authors present two patients, with poorly controlled temporal lobe epilepsy, who received transplants of omental tissue on the anterior perforated space and left temporal lobe. At present, 26 months after the operation, the first patient has improved about 85 percent; whereas the second patient has complete control of seizures nine months after the operation. These clinical results indicate that epileptic seizures can be reduced or aborted with this new surgical modality (reconstructive technique).

The concept of primitive neuroectodermal tumors (PNETs) has been evolving for many years, as has been its nomenclature. A 5 year old boy presented with pain in lower cervicodorsal region and left leg. Preoperative MRI of the spine and paravertebral region revealed a hyperintense lobulated lesion extending from D1-D4 with a large intraspinal and thoracic component. A total removal of tumor was achieved via a dorsal laminectomy and right posterolateral thoracotomy. The pathological findings were consistent with PNET. Post operative neurological examination had been unremarkable. Six months follow up scan showed no recurrence. A review of the literature shows that only 18 cases of primary intraspinal PNETs have been reported to date and the present case is exclusive, in which the tumor was thoracic, extradural in location and the child is alive at 8 months of follow up, with no evidence of tumor recurrence/metastasis. Primary intraspinal PNETs are rare tumors and carry a poor prognosis. Newer modalities of treatment should be tried to improve survival.

Developmental stenosis without any significant spondylotic changes frequently occurs at C3 vertebra or below, and typically extends to C6-C7. However, high cervical focal canal stenosis is unusual. A case of cervical canal segmental stenosis at C2-3 level in addition to a developmental stenosis of the lumbar region, in a 45 year old male, has been presented in this article. The dynamics of the spinal canal in relation to the likely pathology of such conditions are reviewed. We speculate that focal segmental stenosis in the high cervical region may be due to a possible premature fusion of the neurocentral synchondrosis of the cartilage, or due to an abnormal rotary biomechanics which can result in facetal hypertrophy.

Germs cell tumors of extrapineal region are extremely uncommon. The clinicopathological features of germinoma in the basal ganglia, in a 21 year old male are presented in this report. Post-operative radiotherapy is advocated, in view of the good radiosensitivity of these tumors.

Familial trigeminal neuralgia is infrequent. A report of a couple and their son being afflicted by this malady is presented. The clinical features, radiological findings and surgical management are discussed and literature reviewed.

Dissemination of primary cutaneous nocardiosis is a rare event. An interesting case of a 20 year old female labourer with progressive weakness in both the lower limbs and large multiple subcutaneous abscesses over the back, since 4 years, is presented. MRI showed an epidural abscess compressing the cord. Histopathology of skin lesions suggested a chronic suppurative lesion. Microbiological tests on the aspirate from the skin lesion identified the causative organism as Nocardia brasiliensis.

A case of cervico thoracic junctional area spinal tuberculosis presenting as painful radiculitis of the upper extremity is reported. The predominant symptom of radicular pain and muscle weakness in the hand, along with a claw deformity, led to considerable delay in diagnosis. The presence of advanced bone destruction with severe instability was demonstrated on the MRI scan done later. Surgical management by radical anterior debridement and fusion, along with chemotherapy, led to resolution of the upper extremity symptoms. The brachial plexus radiculopathy secondary to tuberculosis has not been reported. The absence of myelopathic signs even in the presence of advanced bone destruction, thecal compression and instability is uncommon in adults.

A case of limb hypertrophy secondary to syringomyelia is presented.

A case of secondary dural arteriovenous fistula presenting as infantile stroke, in a fifteen month old boy, is reported. The initial impression on CT scan in this case was misleading, due to the atypical appearance of the pathological periventricular blood vessels, interpreted as periventricular calcification.

Association of stiff person syndrome, an immune related disorder of anterior horn cells and myasthenia gravis an endplate disorder with similar pathogenesis, is rare. This communication documents this association in the Indian literature for the first time.

Multiple cerebral infarcts, bilateral optic neuropathy with limb ischemia, following scorpion bite is documented. Vasospasm and autonomic storm due to envenomation is a plausible explanation for this symptom complex.

Emotional facial palsy (EFP) commonly results from anterolateral thalamic or striatocapsular infarcts. Its occurrence in brainstem lesions is uncommon, with previously reported cases being restricted to superior cerebellar artery infarction (3 cases). We report an unusual case of EFP ipsilateral to an anterior inferior cerebellar artery infarction, which opens new insights into the facial corticobulbar tract pathway.