Glioneuronal migration disorders of the brain evolve primarily due to aberration in neuronal migration, maturation and programming in the development of various topographic zones in the brain, following pathological alterations in glial and neuronal interactions. These are broadly referred as cortical dysplastic conditions. While these dysplastic conditions involving cerebral cortex present as drug resistant seizure disorder, those involving cerebellum present as mass lesions or slowly progressing vertigo.We report 17 cases, representing the histological spectrum of dysplastic, glioneuronal migration disorders which include, hemimegalencephaly (1), tuberous sclerosis (4), Sturge Weber Syndrome with focal dysplasia (1), Dysembryoplastic neuroepithelial tumor (7) and Lhermitte Ductos disease of cerebellum (2). The dysplastic neurons in varied stages of maturation showed neuronal cytoskeletal pathology similar to that in neuro degenerative diseases, especially when associated with cytomegaly. Similarly, cells exhibiting dual expression of glial and neuronal markers were noted in the cerebral dysplastic lesions. The dysplastic glial elements probably form the subependymal giant cell astrocytomas. Dysplastic neuronal elements form the nidus for DNT. When localized, surgical resection ameliorate the symptoms in many of these condition. Study of these conditions provide better insight into glioneuronal interaction and maturation of the brain.

The hypothalamus produces digoxin, an endogenous membrane Na+-K+ ATPase inhibitor and regulator of neurotransmission. Digoxin being a steroidal glycoside, is synthesised by the isoprenoid pathway. In view of the reports of elevated digoxin levels in metabolic syndrome X with high body mass index, the isoprenoid pathway mediated biochemical cascade was assessed in individuals with high and low body mass index. It was also assessed in individuals with differing hemispheric dominance to find out the relationship between digoxin status, body mass index and hemispheric dominance. The isoprenoid pathway metabolites, tryptophan / tyrosine catabolic patterns and membrane composition were assessed. In individuals with high body mass index an upregulated isoprenoid pathway with increased HMG CoA reductase activity, serum digoxin and dolichol levels and low ubiquinone levels were observed. The RBC membrane Na+-K+ ATPase activity and serum magnesium levels were decreased. The tyrosine catabolites (dopamine, morphine, epinephrine and norepinephrine) were reduced and the tryptophan catabolites (serotonin, quinolinic acid, strychnine and nicotine) were increased. There was an increase in membrane cholesterol : phospholipid ratio and a reduction in membrane glycoconjugates in individuals with high body mass index. The reverse patterns were seen in individuals with low body mass index. The patterns in individuals with high body mass index and low body mass index correlated with right hemispheric dominance and left hemispheric dominance respectively. Hemispheric dominance and digoxin status regulates the differential metabolic pattern observed in individuals with high and low body mass index.

The frequency of complications resulting from angiograms reported in the literature vary between 0.2-5 percent. This study was planned to determine the changes in cerebral blood flow velocity before and after angiography, using transcranial doppler in patients of subarachnoid hemorrhage (SAH) undergoing angiographies. Thirty patients with subarachnoid hemorrhage underwent transcranial doppler ultrasonography immediately before and after angiography. Nonionic water-soluble agents were used during the angiograms. The mean flow velocity (MFV) and pulsatility index (PI) at the M1 segment of both middle cerebral arteries was simultaneously measured. When the patients (11 male, 19 female, mean age+SD; 52.45+12.06) were compared according to changes in MFV and PI, pre and post-angiography, there was no statistical difference in MFV (p=0.51 and p=0.99, left and right side respectively), and in PI (p=0.48 and p=0.66) pre and post angiography. Although angiogram can be used to detect vasospasm in SAH, it can also be cause of vasospasm, partially due to the effect of the contrast agent on the cerebral arteries. This study proposes that the angiographic method is still safe and TCD can be used to follow up any possible changes in diameter of cerebral arteries before and after angiography.

Oligodendrogliomas are rare glial tumors with a long natural history. Radical surgery, postoperative radiotherapy, chemotherapy and observation have been suggested as part of primary management. This series reports the presentation and outcome in 34 patients with histologically verified pure oligodendrogliomas treated between 1983 and 1993. All patients were treated at the time of initial diagnosis with surgery, with or without additional radiotherapy. The overall survival and progression free survival rates at five years were 69.8% and 55% respectively. There was no tumor or treatment related factor influencing survival significantly.

84 patients of juvenile myoclonic epilepsy (JME) of Janz were studied. Diagnosis was confirmed using clinical and electro-encephalographic (EEG) criterias. 58 (78%) patients of JME were referred as 'refractory or uncontrolled seizures'. Ignoring myoclonic episodes and non-use of activation procedures in EEG were important reasons for diagnostic delay. Sodium valproate (VPA) or clonazepam are the drugs of choice while phenobarbitone (PB), carbamazepine (CZ), and phenytoin (PHT) are ineffective. Clinical spectrum of JME is slightly different in India. Family history of epilepsy or JME is not forthcoming and there is gross delay in the diagnosis. Other differences include age of presentation and mild cognitive impairment. All juvenile patients of generalized epilepsy, not responding to more commonly used CZ, PB and PHT should be strongly suspected for JME by carefully searching for myoclonus.

Acute amebic meningoencephalitis caused by free-living amebae naegleria fowleri is extremely rare and uniformly fatal with only seven survivals reported till date. An interesting case of naegleria meningitis diagnosed by wet mount cytology of cerebrospinal fluid (CSF) and treated with amphoterecin B, rifampicin and ornidazole with complete recovery is presented. In cases of suspected pyogenic meningitis, if CSF staining, antigen detection or culture is negative for bacteria, a wet mount cytology of CSF for naegleria is suggested. Early treatment with amphoterecin B and rifampicin may improve survival.

Tardive dystonia is an uncommon form of chronic dystonia, which usually develops on exposure to neuroleptics. Tardive dystonia (Tdt) following lithium therapy has not been previously reported. The case of 38 year old man with bipolar affective disorder who developed tardive dystonia while on maintenance lithium treatment is described. Presentation of Tdt in this patient was fairly characteristic although there was no suggestion of recent neuroleptic exposure. Tdt known to have poor treatment response, responded very well to clozapine, a novel anti-psychotic, in this case. To conclude, Tdt may develop on exposure to drugs other than neuroleptics. An adequate trial to clozapine can prove to be a useful treatment option.

Endolymphatic sac tumors (ELST) are rare papillary tumors of the temporal bone. Previously named as aggressive papillary middle ear tumors, they have recently been shown to arise from the endolymphatic sac. They are a rare in cerebello-pontine angle (CPA). We present a case of an ELST who presented as a CPA tumor with hydrocephalus. He underwent a ventriculo-peritoneal shunt initially. On exploration of the CP angle, the tumor was found to be extremely vascular. He was re-explored following embolization, and a subtotal excision of the tumor was done. Extensive petrous bone infiltration and vascularity of the tumor makes total excision almost impossible with high risk of cranial nerve deficits, excessive blood loss and CSF leak. This tumor should be considered in the differential diagnosis of vascular CPA tumors which erode the petrous temporal bone. The relevant literature is reviewed.

Suprasellar dermoid cysts are uncommon intracranial lesions. CT and MRI findings in a rare case of asymptomatic rupture of suprasellar dermoid cyst with subarachnoid dissemination is described.

A 45 year old lady presented with history of recent surgery for uterovaginal prolapse and retained vaginal tampons following which she developed chronic meningitis due to acanthameba infection. Patient responded to a regimen containing albendazole. She was left with hydrocephalus as a sequelae.

Sensory phenomena in tic disorders such as Tourette's syndrome are known but are substantiated by only a handful of studies. In this preliminary report, we studied premonitory urge, a type of sensory phenomenon in three patients of tic disorders. Bereitschaftspotential, a movement-related cognitive potential indicative of motor preparation, was assessed in these patients. As bereitschaftspotential was observed in all our cases prior to occurrence of tics, it is speculated that tics are not entirely involuntary but are quasi-volitional in nature. Bereitschaftspotential may thus represent a neurophysiological marker of premonitory urge in tic disorders. Implications of exploring the voluntary nature of tics are discussed.

A clinicopathological study of 41 cases of pituitary apoplexy in a series of 324 surgically treated pituitary adenomas is presented. In 23 patients, the predominant operative finding was hemorrhage with or without necrosis. However, there were 15 (37.7%) cases where pale, necrotic tissue with no evidence of hemorrhage was found at surgery. Pale, necrotic material was particularly found when there was a long interval between the acute clinical event and surgery. It is concluded that the pale, necrotic debris represents one stage in the resorption process of blood after hemorrhagic necrosis of pituitary adenomas. This entity needs to be kept in mind especially since the material closely resemble the pultaceous material seen in craniopharyngiomas and epidermoid cysts.

Five cases of spinal intramedullary tuberculomas (IMT) and one case of spinal intramedullary tuberculous abscess (ITA) are presented. Gd enhanced MRI revealed ring enhancing lesion with central hypodensity, suggesting granulomatous pathology. Surgical excision of the intramedullary lesions was carried out in four cases, while two patients received presumptive anti-tuberculous chemotherapy only. Repeat MRI after completion of anti-tuberculous therapy showed total resolution of the lesion. In other cases following surgical excision, the patients improved significantly. The management of these rare lesions is discussed and the literature reviewed.

A 14 year old girl presented with a 6-month history of headaches and visual blurring. CT showed a right tentorial enhancing lesion. Angiogram showed no enhancement. Histopathological examination of the excised material was consistent with tuberculoma. The presentation of such unusual intracranial tuberculoma is reported and the literature reviewed on the subject.

Limb girdle myasthenic syndromes are rare genetic disorders described under the broad heterogeneous group known as congenital myasthenic syndromes and present with mixed features of myasthenia and myopathy. The familial limb girdle myasthenia has been described as one with selective weakness of pectoral and pelvic girdles, showing a positive response to edrophonium chloride. A report of two sisters affected by this disorder is presented.

A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.

Placement of lumboperitoneal (LP) shunt as a surgical treatment for benign intracranial hypertension (BIH) is generally a safe procedure, with complications like mechanical failure, overdrainage and infections. Subarachnoid hemorrhage and intracerebral hematoma were seen after lumboperitoneal shunt in a patient having BIH. These complications were the cause of the patient's deterioration. After removal of the hematoma and performing a decompressive procedure, patient's neurological condition improved. The clinical features, investigations and clinical course are described and the literature reviewed.

Aneurysmal bone cyst in temporal region is rare. It is a benign condition and may extend intracranially. Total surgical removal is recommended, whenever possible in one stage or in multiple stages. Prognosis is excellent and total removal will effect a cure. An unusual case of aneurysmal bone cyst of temporal region is reported.

We present an interesting case of sacral perineural cyst which caused chronic perineal pain. Perineural cyst is relatively rare, especially the sacral region. Chronic perineural pain is an often encountered problem that is difficult to evaluate and sacral perineural cyst may be the etiology of chronic perineal pain in many instances.

Diffuse neurofibroma is an uncommon but distinct variety of neurofibroma, usually affecting trunk, head and neck regions of adolescents and young adults. The clinical features, gross macroscopic and histopathological findings are enunciated and the criteria for instituting the preferred modality of treatment for such lesions has been reviewed, stressing upon the need to exclude the neurofibromatoses preoperatively.

Levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH) and creatine kinase (CK) were estimated in serum and cerebrospinal fluid of 25 patients of stroke, and were correlated with severity of disease. 21 (84%) patients had ischemic stroke and four (16%) had hemorrhagic stroke. Serum and CSF AST levels were significantly elevated in the study group. The rise in CSF AST was more in the hemorrhagic subtype than in the ischemic subtype. Serum ALT and CSF LDH levels were also significantly elevated in patients with ischemic stroke. None of the enzyme levels were related to the severity of disease as assessed by the Glasgow coma scale.

A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.

A 43 year old man with thirty years history of recurrent hemiplegic migraine, consistently occurring on one side (left sided paresthesia and weakness of less than forty five minutes followed by right sided headache) and lately increasing to once in three days was investigated. CT head and carotid angiogram showed gyral calcification with prominent medullary and subependymal veins confirming the diagnosis of leptomeningeal angiomatosis. Management and followup is discussed.

A case of benign epilepsy with centro-temporal spikes (BECT) is reported, in whom tapping of fingures activated typical spikes.

Over the last decade, various studies have been reported to evaluate the circadian pattern of cardiovascular and cerebro-vascular diseases. The data from Indian population is lacking. We undertook this prospective observational study to evaluate the circadian variation in disorders like cerebro-vascular accidents and transient ischemic attacks. Total of 146 patients (events) were studied. Only 10 patients had TIA's. 55% had hemorrhage and 45% had infarction. The 24 hours period was divided into 6 equal portions of 4 hours each. The maximum events were seen between 4 am to 8 am and 12 noon to 4 pm (23.28%) each. Minimum events were seen between 12 midnight to 4 am 14/146 - 9.58%). The circadian variation in occurrence of cerebro-vascular disorders was present with two equal peaks.

Papilledema and raised intracranial pressure have been reported in association with Guillain-Barre syndrome. Papilledema is usually asympotomatic or associated with mild visual field defects, without any visual loss. The cerebrospinal fluid protein is usually reported to be high. A case of a 35 year old lady is reported, who presented with headache, diplopia and progressive visual loss in both eyes and limb weakness with hyporeflexia. Optic fundus examination showed bilateral papilledema. She had features of pseudotumor cerebri. Nerve conduction studies were suggestive of polyradiculopathy. The unusual things in this case, were the profound visual loss normal cerebrospinal fluid profiles and the presentation of papilledema before the limb weakness.

Intradural extramedullary epidermoid cysts are rare tumors especially those not associated with spinal dysraphism. We report 3 cases of spinal intradural extramedullary epidermoid cysts. In all the cases, the lesion was situated at dorsal level. The clinical features, MRI characteristics and surgical treatment of such rare intradural extramedullary benign tumors are discussed and relevant literature is reviewed.

Plethsymographic recordings are used to evaluate changes in peripheral vascular tonus. Twenty-six volunteers (15 men, 11 women) with a mean age 31 years were included in this study. Plethsymographic recordings were done both at baseline and +40C temperatures bilaterally. Significant reductions in systemic blood flow were noted with different maneuvers. Reactive hyperemia occurring after ischemia was local, however local reduction in blood flow after cold test was systemic. As the local temperature increased, the blood flow parameters normalized, too. In normal controls these values normalized in 20 minutes. The changes in vasomotor tonus due to cold test demostrated a parallel pattern.

Chondroblastoma is a rare tumor of the skull. Temporal bone is the commonest site of involvement in the skull. We present a thirty one year old man who presented with painless swelling over the left temporal bone, which was near totally excised after preoperative embolization. Management of this unusual tumor and its complications are discussed.

DMD and BMD are X-lined recessive disorders. RAP-PCR was utilized to investigate differentially expressed gene transcripts in lymphocytes from DMD, BMD and normal individuals as possible diagnostic parameter. A 1583 bp transcript was found to be expressed in both DMD and BMD patients which was unrelated to the known dystrophin gene. This may prove helpful in determining the carrier status of DMD/BMD.