Dopaminerigic replacement therapy with levodopa/carbidopa is still the cornerstone for the treatment of Parkinson’s disease (PD). However, the medical management of PD is complicated by the appearance of disabling motor response fluctuations, levodopa-induced dyskinesias and psychosis. Since the early 1990s, surgical therapies have made a rapid reentry into the therapeutic armamentarium for PD and deep brain stimulation (DBS) of the globus pallidus interna or subthalamic nuclei is currently the most promising of such interventions. Recognition of the physiological changes in basal ganglia circuits in animal models of PD has provided the much-needed theoretic basis for targeting these areas. DBS of these areas has proven to be a safe procedure and effective against all the major motor symptoms of PD. Though not curative it can substantially reduce motor response fluctuations, levodopa-induced dyskinesias, and improve the quality of life of these patients. DBS is an expensive treatment and hardware-related complications are not rare. The results of the procedure are dependent on careful patient selection and the experience of the performing team. An update on the principles, methods and results of such procedures is essential to raise the awareness of this new therapeutic modality and to provide guidelines to the referring physicians.

The present review traces the origin of Friedreich’s Ataxia (FA) from the time of Nikolaus Friedreich in the mid-nineteenth century. The early hesitation on the part of the neurological community in accepting FA as a distinct entity, rather than a variant form of tabes dorsalis and multiple sclerosis, has been highlighted. Research within the last 6-7 years, has firmly established FA as a trinucleotide repeat disorder, the location of the offending gene, and the disease-related gene product, frataxin. Frataxin is now thought to interfere with the mitochondrial oxidative process and enhance iron accumulation. However, whether this iron accumulation is a primary causative event for symptom production is not clear and iron chelators are unlikely to be helpful in therapy. Of great promise is the use of free radical scavengers and antioxidants. One such agent idebenone, a short chain analogue of co-enzyme Q10, may have a future.

Parkinsonism plus syndrome is a group of heterogeneous degenerative neurological disorders, which differ from the classical idiopathic Parkinson’s disease in certain associated clinical features, poor response to levodopa, distinctive pathological characteristics and poor prognosis. Associated clinical features include symmetrical onset, infrequent or atypical tremor, prominent rigidity in axial musculature, bradykinesia, early postural instability, supranuclear gaze palsy, early autonomic failure, pyramidal affection, cerebellar involvement, alien limb phenomenon, apraxia and significant early cognitive dysfunction in some cases. Progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and dementia with Lewy body disease (DLB) are commoner disorders. Less frequent disorders are cortico-basal ganglionic degeneration (CBGD), frontotemporal dementia with chromosome 17 (FTDP-17), Pick’s disease, parkinsonian-dementia complex of Guam, Pallidonigral degeneration, Wilson’s disease and a rigid variant of Huntington’s disease. During the last 3 decades, major progress has been made in understanding PSP, CBGD and FTDP-17, which are tau disorders. MSA and DLB together with idiopathic Parkinson’s disease are called a-synucleinopathies. Recent studies show that the diagnosis of these Parkinsonism plus syndromes improves when strict diagnostic criteria are used. However, unusual presentations may pose a diagnostic challenge. The shortcomings of the current studies demand the need for further research to identify biologic markers that may allow earlier diagnosis, and understanding of the factors leading to a-synuclein or tau aggregation. Identification of therapeutic strategies that may prevent the aggregation of these proteins and rescue dysfunctional cells has been stressed. This review focuses on the advances in the clinical, neuroimaging, pathologic, genetic and management aspects of these disorders.

Background: Corpus Callosum (CC) abnormalities have been described in schizophrenia. Review of CC measurement methods in schizophrenia reveals inconsistency and lack of neuroanatomical basis. Aims: The aims of the study are to describe a new CC measurement method with valid a neuroanatomical and cytoarchitectural basis and to demonstrate inter-rater reliability for the same. Settings and Design: The study was performed in the National Institute of Mental Health & Neurosciences, Bangalore, India. Ours is a cross-sectional study in which both the first author and senior neuroradiologist were blind to clinical details. Material and Methods: We report a reliable, semi-automated CC measuring technique with a neuroanatomical and cytoarchitectural basis tested in a group of 16 never-treated schizophrenia patients using 1-mm thick, objectively defined midsagittal MRI section. Measurement on coded slices using scion image software ensured elimination of rater bias. Statistical Analysis Used: The statistical analysis used for assessing inter-rater reliability is intraclass correlation coefficient analysis. Results: The intraclass correlation coefficients for the CC measurements were as follows: CC Area = 0.93; Anterior CC area = 0.84; Area of body of the CC = 0.83; Area of CC isthmus = 0.65; Area of CC splenium = 0.88; maximum antero-posterior distance of CC = 0.96. Conclusion: Measurements showed good inter-rater reliability. The methodology demonstrated in our study is simple, relevant, reliable and can be used for future schizophrenia research.

Background and Aims: Foramen magnum tumors, especially those located ventrally, are surgically challenging. We studied clinical and surgical details of extramedullary tumors located in the region of the foramen magnum. Material and Methods: A series of 30 cases of extramedullary tumors at the foramen magnum, operated over a period of 8 years is presented. Results: There were 10 neurofibromas, 8 meningiomas, 6 chordomas, 2 tuberculomas and 4 miscellaneous tumors. The clinical profile consisted of sensory symptoms in all the patients, quadriparesis in 24, lower cranial nerve affection in 8 and sphincter disturbances in 6 patients. The surgical approaches consisted of an extreme lateral approach in 10, a posterior or posterolateral approach in 18 and a per-oral route in 2 cases. Total excision of the tumor was performed in 24 and a subtotal excision of the tumor was done in 6 cases. Two patients died, one due to meningitis and the other due to chest infection. Eight other patients had complications like CSF leak, meningitis, pseudomeningocele, laryngeal edema, and transient worsening of neurological deficits. Conclusions: Foramen magnum tumors have long been regarded as difficult lesions both in terms of diagnosis and management. However, with the availability of MR imaging, newer surgical techniques and skull basal exposures, the excision of these lesions is becoming easier and safer.

A prospective study was carried out to evaluate the efficacy of intraventricular sodium nitroprousside (SNP) in the reversal of refractory vasospasm secondary to aneurysmal subarachnoid hemorrhage (SAH). Ten patients of aneurysmal SAH with symptomatic vasospasm, corroborated on Transcranial Doppler (TCD) and/or angiography, were included in the study. The mean age distribution of the patients was 50.8 years (range 33-65 years) with an equal number of males and females. Once vasospasm was refractory even after 12 hours of SAH therapy, intraventricular SNP was instilled in an escalating dose and the reversal of vasospasm was monitored on TCD and/or angiography. All patients showed improvement in TCD velocity on day 0 through day 3. Partial to complete reversal of vasospasm was demonstrated on angiography in all the patients, though not in all the vessels. Two patients who had weakness of limbs due to vasospasm improved following intraventricular SNP therapy. Vomiting was the commonest adverse effect (7/10). Three patients had mild fluctuation in blood pressure. The overall outcome was good in 6 out of 10 patients. The study suggests that intraventricular SNP therapy is effective in reversing the changes even in established cases of SAH-induced vasospasm.

Guillain-Barré syndrome (GBS) is the commonest peripheral neuropathy causing ventilatory failure, and 10-30% patients may require respiratory support. Records of 11 adult patients of GBS in respiratory failure, admitted to the Respiratory Intensive Care Unit (RICU) of our institute for mechanical ventilation over a four-year period, were studied. Six patients received intravenous immunoglobulin. The median duration of mechanical ventilation was 38 days. Seven patients underwent tracheostomy. Four patients were ventilated for less than 2 weeks and 3 for more than 2 months each. Seven developed ventilator-associated pneumonia and/or sepsis. Three patients died in, and two shortly after discharge from RICU; all had systemic problems or complications of hospitalization.

Objective: We conducted this study to demonstrate the value of non-invasive three-dimensional CT angiography (3D CTA) in the detection of a cerebral aneurysm. Material and Methods: A helical CT acquisition was obtained using non-ionic contrast media in 50 patients with 1 mm per second table speed, 1 mm collimation, and pitch 1:1. Axial source images were transferred into a workstation console (Advantage windows GE) and CTA was obtained using MIP (Maximum Intensity Projection) reconstruction. Fourteen patients underwent conventional digital subtraction angiography (DSA) after 3D CTA prior to surgery. Results: A total of 39 aneurysms were detected in 50 patients. All patients with an aneurysm were operated and the presence of aneurysms was confirmed. 3D CTA detected 38 of these 39 aneurysms. In 37 aneurysms, the origin of the aneurysm and aneurysm neck was adequately visualized. 3D CTA was found to be 100% specific and 97.2% sensitive in picking up cerebral aneurysms. 3D CTA was 97% specific in demonstrating the exact origin and showing the neck of the aneurysm. Conclusions: CT angiography is a simple, quick, and non-invasive technique and can replace DSA in most patients with acute subarachnoid hemorrhage aneurysm for the detection and characterization of aneurysms of the circle of Willis. The relation of the aneurysm with bony landmarks was clearly depicted.

Low-molecular-weight-heparin (LMWH) has been widely used in the treatment of acute ischemic stroke but controlled trials are few. In this study, 40 patients with acute ischemic stroke of less than 24 hours duration were randomized to receive either aspirin (325 mg/day) alone or aspirin (325 mg/day) plus subcutaneous nadroparin 4100 units/day. At the end of 4 weeks, the morbidity and mortality were significantly less in the nadroparin group as compared to the aspirin group. There was no increased risk of clinically significant intracranial hemorrhage in either group. The combination of aspirin and LMWH deserves to be tested in larger studies.

Low-molecular-weight-heparin (LMWH) has been widely used in the treatment of acute ischemic stroke but controlled trials are few. In this study, 40 patients with acute ischemic stroke of less than 24 hours duration were randomized to receive either aspirin (325 mg/day) alone or aspirin (325 mg/day) plus subcutaneous nadroparin 4100 units/day. At the end of 4 weeks, the morbidity and mortality were significantly less in the nadroparin group as compared to the aspirin group. There was no increased risk of clinically significant intracranial hemorrhage in either group. The combination of aspirin and LMWH deserves to be tested in larger studies.

A quantal perceptive model of brain function has been postulated by several groups. Reiki-like healing practices in seizure disorder (ILAE classification – II E – generalized seizures – tonic clonic), involving transfer of life force or low level of electromagnetic force (EMF) from the healer to the recipient patient, may act via quantal perceptive mechanisms. Increased synthesis of an endogenous membrane Na+-K+ ATPase inhibitor digoxin and a related tyrosine / tryptophan transport defect has been demonstrated in refractory seizure disorder (ILAE classification – II E – generalized seizures – tonic clonic). Reiki-like healing practices in refractory epilepsy results in a reduction in seizure frequency. Reiki-like healing practices produce membrane stabilization and stimulation of membrane Na+-K+ ATPase activity by quantal perception of low levels of EMF. The consequent intracellular hypermagnesemia inhibits HMG CoA reductase activity and digoxin synthesis resulting in the alteration of the neutral amino acid transport (tryptophan / tyrosine) defect. A hypothalamic digoxin-mediated quantal perception model of brain function is proposed. The phenomena of biological transmutation and consequent hypermagnesemia occurring in the resultant neuronal quantal state is also discussed.

Sixty cases of missile injuries (59 males, average age 25 years) were studied over a period of one year. Forty-three patients had suffered splinter injuries, 12 had gunshot wounds and 5 had suffered injuries from improvised explosive devices. The Glasgow coma scale was <5 in 8 patients, 5-8 in 14, 8-12 in 30 and 13-15 in 8 patients. Extensive comminution of skull bones was found in 10 patients. Thirty-five patients had penetration of the skull and the rest had orbito-cranial or facio-cranial wounds. CT scan revealed small hemorrhagic contusion with in-driven bones without mass effect in 15, contusion with mass effect in 36 cases, cortical contusions without in-driven bones (tangential injuries) in 3, distant intracranial contusions in 4, intraventricular hemorrhages in 5, multilobar injuries in 14, and unilobar injury in 40. Fifty-two patients were operated upon at our center, of which 30 were operated within 24 hrs, 10 between 24 to 48 hrs, and 12 between 48 to 72 hrs. Six patients were treated conservatively and 2 underwent only a simple closure of scalp wound. Craniectomy was done in 10 and craniotomy in 42 patients. Two patients developed wound sepsis, one had aspiration pneumonia, one had septicemia and one had deep vein thrombosis while one had post-traumatic hydrocephalus. On follow-up at 6 months, the outcome as per the Glasgow outcome scale was as follows: Good outcome in 42, moderate disability in 7, severe disability in 6 and death of 5 patients. Retained bone fragments were found in 36.3 % on follow-up CT scan but no one had brain abscess.

Plasma levels of vitamins A, E and C were analyzed in 102 patients with different types of brain tumors. A follow-up study was done with 27 postoperative patients. On comparison with plasma from normal individuals, vitamin A and E were decreased, but the decrease was statistically insignificant. Vitamin C levels remained in the normal range. In a comparative study of preoperative and postoperative cases, plasma vitamin A levels in postoperative glioma patients were significantly higher than those in the pre-operative state. There was no significant difference in the plasma level of vitamins C and E. The results of the present study suggest that the plasma antioxidant vitamins are not altered effectively in brain tumor cases.

The dystrophin gene was analyzed in 8 Duchenne muscular dystrophy (DMD) and 10 Becker muscular dystrophy (BMD) unrelated families (22 subjects: 18 index cases and 4 sibs) for the presence of deletions by multiplex polymerase chain reaction (mPCR; 27 exons) and Southern hybridization using 8 cDMD probes. Deletions were identified in 5 DMD and 7 BMD patients (6 index cases and 1 sib). The concordance between the clinical phenotype and “reading frame hypothesis” was observed in 11/12 patients (92%). The female relatives of DMD/BMD patients with identifiable deletions were examined by quantitative mPCR. Carriers were identified in 7 families. We also describe a variation in the HindIII pattern with cDNA probe 8 and 11-14. Molecular characterization of the dystrophin gene in this study has been helpful in advising the patients concerning the inheritance of the condition, and carrier diagnosis of female relatives, and should also prove useful for prenatal diagnosis.

Seventy cases of primary degenerative cerebellar ataxias in ethnic Bengalees from southern West Bengal, India, were studied by the authors. Of these, 50 cases were of the familial type (hereditary ataxias) encountered in 23 families and the remaining 20 were of sporadic onset. 18 cases (from 11 families) were of ‘probable’ autosomal recessive (AR) inheritance, 12 cases (8 families) had Friedreich’s type ataxia (FA), 4 cases (2 families) had FA type ataxia with retained reflexes and in 2 cases (1 family) the exact phenotypic characterization could not be made. AR inheritance in these cases seemed most likely in view of the occurrence in a single generation with unaffected parents and history of consanguinity in many of the families studied. Genotypic confirmation of FA type ataxia and its variants could not be done in any case due to the non-availability of technology for studying the FA locus but some common dominant ataxia genotypes could be excluded. Thirty-two cases (from 12 families) with autosomal dominant ataxias (ADCA) were studied. Genotype analysis revealed 4 families with SCA2 genotype, 5 families with SCA3 and 3 families where genotypic characterization could not be made (phenotypically 2 were of ADCA I and 1 of ADCA II). No clear preponderance of one particular genotype of SCA over another could be demonstrated in our ethnic Bengalee patients. We also noted significant intra and inter-family variations in phenotypes within the same genotypic form as well as overlapping of clinical signs between different genotypes. Slow saccades and peripheral neuropathy were not seen consistently in our ethnic Bengalee subjects with SCA2 genotype. Similarly, extrapyramidal features, ophthalmoplegias and distal amyotrophy were seen in some but not all families with the SCA3 genotype. Phenotypic expression appeared to be an inconsistent marker of the SCA genotype in our patients. Of the 20 sporadic cases with cerebellar ataxia, genotype analysis revealed 2 cases with SCA1 and 1 with SCA2. Some of the sporadic ataxia cases had extracerebellar involvement and may warrant classification as Multiple System Atrophy. In all the 3 subjects with genotype characterization, phenotype correlation was lacking. The clinical pattern of hereditary ataxias in ethnic Bengalees seems to be somewhat different from that seen in Western India. The need for clinical and genetic studies of ataxias in different specific ethnic populations of India has been stressed.

Background and Aims: Computerized electroencephalographic topography (CET) (brain mapping) is a non-invasive, spatially oriented procedure for depicting amplitude and frequency patterns by two-dimensional color-coded frequency and amplitude display of the electroencephalograph (EEG). Material and Methods: We prospectively studied the relationship between CT scan lesions, conventional EEG and brain mapping in 40 patients with ischemic stroke within 1 week of onset. EEG and brain mapping was done within 24 hours of doing CT scan. Results: There were 32 males and 8 females and the mean age was 56 years (range 27-75 years). CET/EEG was normal in 2 patients with capsular infarct. Brain mapping showed abnormalities (most commonly low-frequency high-amplitude changes) in 95% of patients, whereas EEG abnormalities were seen in only 40%. Brain mapping abnormalities were appropriate to the side of the CT scan lesion in 20 (52.63%) patients and were present bilaterally n 18 (47.37%) patients. Brain mapping abnormalities were ipsilateral to the side of the CT lesion when the lesion was < 2 cm in diameter and they were bi-hemispherical when the CT lesion was > 2 cm in diameter. Conclusions: There was no correlation between motor deficits and brain map abnormalities.

The incidence of primary central nervous system lymphoma (PCNSL), previously a rare tumor, has increased significantly over the past few decades, partly due to the AIDS epidemic but also in immunocompetent individuals. Studies from Western countries have shown a consistent association of tumors occurring in immunocompromised individuals with the Epstein-Barr virus (EBV) suggesting an important role for the virus in the pathogenesis of these tumors, but an infrequent association of the virus with PCNSL in the immunocompetent host has also been noted. We studied 11 patients with PCNSL who had no evidence of an immunocompromised state. All the tumors were aggressive B cell lymphomas. EBV association was studied using EBER in-situ hybridization. 10 out of the 11 tumors were negative for EBV, indicating that tumors in immunocompetent individuals in developing countries are also infrequently EBV associated and that a different pathogenetic mechanism is operative in the evolution of these tumors.

One thousand nine hundred and forty newly recruited entrants for training as pilots (Group A) underwent photic stimulation during EEG recording during the entrance examination to the flying stream. One hundred and sixty individuals (Group B) working on radars for prolonged periods were interviewed for eliciting complaints referable to photosensitivity and were subjected to EEG. EEGs in respect to 1000 cases (Group C) of known epilepsy were examined for the incidence of a photosensitive response. The study has revealed that 14 cases (0.72%) in group A had an abnormal response to photic stimulation out of which one case developed seizure during EEG recording. One case (0.62%) in group B and 30 cases (3%) in group C were detected to have photosensitivity. The maximum response was seen at 20 Hz stimulation. The prevalence of photosensitivity and its manifestations in these groups indicates that this condition is not uncommon.

A patient with small cell lung cancer presented with a rare presentation of an acute onset pancerebellar dysfunction. His clinical condition markedly improved following the surgical removal of the tumor and chemo- and radiotherapy.

Lipidized glioblastoma multiformis (LGB) and pleomorphic xanthoastrocytoma (PXA) are often supratentorial in location and occur in the second to fourth decade. This report presents two young patients, one having LGB and the other having PXA in the cerebellum. Histological differentiation between LGB and PXA is discussed.

Glial heterotopia or the occurrence of isolated non-teratomatous extracranial glial tissue is rare. We report a neonate with extensive extranasal glial heterotopia involving the left buccopharyngeal region, palate and base of the skull and presenting with respiratory distress and a bleeding oral mass. A staged operative approach was adopted to excise the lesion. The literature on the subject is briefly reviewed.

A rare case of the complete absence of the posterior elements of C2 is reported. The patient presented with neck pain without any neurological deficits and radiology revealed a mobile, partially reducible dislocation of the C2 over C3 vertebra. A posterior fusion utilizing a contour rod, sublaminar wire fixation, and onlay bone grafts between the occiput and the C3 vertebra was performed for spinal stability.

Dopa-responsive dystonias are rare. We report a 14-year-old male who was diagnosed as a case of limb girdle dystrophy and had features suggestive of dopa-responsive dystonia.

Akathisia, an involuntary movement disorder resulting from exposure to antipsychotics, is characterized by subjective restlessness and a strong desire to move about. The diagnosis is often complicated by the overlapping symptoms of pseudoakathisia, chronic akathisia and tardive dyskinesia. This report deals with a patient with schizophrenia who developed akathisia after exposure to antipsychotics. Later, she developed movements that were more like pseudoakathisia and tardive dyskinesia rather than acute akathisia. On failure of anti-akathisia medication, she was treated with a behavioral regime to which her akathisia responded. This behavioral regime used the technique of distraction as a primary tool. This case report highlights the diagnostic difficulties in akathisia and the application of behavioral treatment for akathisia that is non-responsive to anti-akathisia medication.

We report two siblings with Allgrove’s syndrome and extrapyramidal features. Though various neurological abnormalities have been described in this disorder, we report the first patient of Allgrove’s syndrome associated with dystonia and chorea.

Giant cell reparative granuloma is an infrequent non-tumoral lesion affecting the maxillary and mandibular bones and only rarely, the cranial bones. We report a case of giant cell reparative granuloma of the base of the skull in a 12-year-old female who presented with a parapharyngeal mass. Computerized Tomography (CT) showed the intracranial extension and destruction of surrounding bones.

The simultaneous occurrence of multiple meningiomas in different neuraxial compartments is rare. A 35-year-old female patient had multiple meningiomas involving both the supratentorial compartments and in the upper dorsal spine. The patient underwent excision of 2 cranial and 2 spinal meningiomas, and has remained asymptomatic over a follow-up period of 18 months.

The presence of frontally-dominant alpha pattern in the EEG is common in patients with coma due to trauma, toxic-metabolic causes and following cardiorespiratory arrest. Diffuse beta activity following resuscitation after a cardiac arrest is not well recognized. We report a case of coma in a 3-year-old girl who had a cardiac arrest from which she was revived. Initial EEG showed diffuse beta activity, which later evolved to predominantly alpha activity. The possible mechanisms involved in the generation of such rhythms are discussed. Transition of EEG activity from faster to slower frequencies is suggested as an adverse prognostic factor in post-cardiorespiratory arrest coma.

Intra-abdominal migration of the catheter and formation of a cerebrospinal fluid pseudocyst are both rare complications of a ventriculoperitoneal shunt. Traditionally, each condition is treated by a formal laparotomy. Laparoscopic management of the complications in two patients is described.

The association of internuclear ophthalmoplegia (INO) with torsional nystagmus is rare. We report a case of a 72-year-old male who developed brainstem stroke and was found to have left INO with torsional nystagmus. An MRI correlation in this case has been described.

Rhabdoid tumors of the central nervous system are uncommon tumors. About 188 cases have been reported in the literature so far. In this report, we describe a case of a rhabdoid tumor of the thalamus in a 35-year-old male patient. Light microscopic and immunohistochemical features are discussed and the relevant literature reviewed.

We report the characteristic neuropathological features of a rare case of cerebellar liponeurocytoma in a 62–year-old female. The tumor has a low proliferative potential and carries a favorable prognosis.

A case of left cerebral hemispheric anaplastic astrocytoma with metastasis to the cerebrospinal fluid in a 50-day-old male child diagnosed on cytology and later confirmed by histological examination is reported.

Temporal lobe epilepsy (TLE) associated with mesial temporal sclerosis (MTS), mesial TLE (MTLE), is the commonest medically refractory adult epilepsy syndrome. Corpora amylacea (CoA) have been shown to be a marker of MTS. We compared 9 patients with MTS who had dense deposition of CoA in their hippocampi with 25 patients with MTS who did not have CoA. The patients with CoA were significantly older and they showed a trend towards having a significantly longer duration of epilepsy. The postoperative seizure outcome at 2 years was not different in the 2 groups. Our results could indicate the progressive nature of the pathology of MTS, probably indicating excitotoxic damage due to recurrent seizures, but they need to be verified by clinicopathological correlation among a larger number of patients with MTLE.

A rare case of a lobar holoprosencephaly with cyclopia, associated with non-nervous system anomalies is being reported.