Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease of the central nervous system. The true incidence of the disease in India is undetermined and is likely to be more frequent than reported, as the common antecedent events, exanthematous fevers and Semple antirabies vaccination, which predispose to ADEM, are still prevalent. The existing evidence suggests that ADEM results from a transient autoimmune response towards myelin or other self-antigens, possibly via molecular mimicry, or by non-specific activation of auto-reactive T cell clones. ADEM is a monophasic illness with favourable long-term outcome. Involvement of neuroaxis is variable and can be diffuse or multifocal and site restricted. Magnetic resonance imaging (MRI) is highly sensitive in detecting white matter lesions and the lesions described are rather extensive and subcortical in location. Involvement of the deep gray matter, particularly basal ganglia, is more frequent. Oligoclonal bands in CSF are usually absent. No therapy has been established by controlled trials in ADEM. Use of high-dose methylprednisolone, plasma exchange, and IVIG are based on the analogy of the pathogenesis of ADEM with that of multiple sclerosis (MS). Differentiation of ADEM from the first attack of MS is important from prognostic as well as therapeutic point of view. However, in the absence of biological marker, at times differentiation of ADEM from the initial presentation of MS may not be possible even by combination of clinical, CSF analysis, and MRI. This differentiation is more relevant to India where the incidence of MS is low.

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Thirteen consecutive cases with symptomatic vertebral hemangiomas, managed during a five year period from January 1995 at the Christian Medical College and Hospital, Vellore, were analysed. Twelve patients had lesions in the thoracic and 1 in the sacral region. Eight patients had multiple level involvement. Seven patients had laminectomy and soft tissue component excision, of which one had intraoperative injection of absolute alcohol and one had postoperative radiotherapy. One patient had vertebrectomy and stabilization with preoperative embolization. One patient underwent only endovascular embolization. However, the focus of this communication is on 4 patients who underwent a CT guided percutaneous transpedicular injection of absolute alcohol into the affected vertebral body. In the surgical group, 6 patients had cavernous type and 2 patients had mixed type of hemangiomas. Ten patients improved on the Ranawat grade by the time of discharge. On the MRC grade, 11 patients had improved, one was grade 5 pre and postoperative, while one did not improve. Several options are available for the management of symptomatic vertebral hemangiomas and multiple modalities may have to be used for a single patient. CT guided percutaneous transpedicular injection of absolute alcohol shows promising results. However, long term follow up is mandatory.

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Neuronavigation provides intraoperative orientation to the surgeon, helps in planning a precise surgical approach to the targetted lesion and defines the surrounding neurovascular structures. Incorporation of the functional data provided by functional MRI and magnetoencephalography (MEG) with neuronavigation helps to avoid the eloquent areas of the brain during surgery. An intraoperative MRI enables radical resection of the lesions, the possibility of immediate control for tumor remnants and updates of neuronavigation with intraoperative images to compensate for brain shift. In this study, the experience of 432 patients undergoing neuronavigation assisted neurosurgical interventions using either the pointer-based or microscope-based navigational systems at the University of Erlangen-Nuremberg, Germany is presented. The procedures included stereotactic biopsy (n=53), stereotactic cyst puncture/ventricular drainage (n=15), eloquent cortex/tumor localization to facilitate tumor resection, assessment of neurovascular structures in the vicinity of tumors of the sellar-suprasellar regions, skull base, posterior fossa and ventricular region (n=252), and, surgery for epilepsy (n=9). Functional brain mapping using fMRI and MEG and their integration with neuronavigation was carried out in 24 and 128 patients respectively. The simultaneous use of intraoperative MRI to look for the remaining tumor was done in 159 patients and the update of navigational data was carried out in 17 patients. The mean system accuracy obtained by using both the fiducial registration as well as anatomical landmark-surface fitting computer algorithm was 1.81 mm. This study reviews the relative merits and demerits of the pointer and microscope based navigational systems and also highlights the role of functional brain mapping and intraoperative MRI, when integrated with neuronavigation, in the surgical decision-making to offer the chances of more radical resections with minimal morbidity.

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Wasp stings generally cause local reactions like pain, wheal, flare, edema and swelling, which are generally self-limiting. Multiple stings can lead to vomiting, diarrhea, generalized edema, dyspnea, hypotension, collapse, renal failure or death. Unusually, they may cause serum sickness, vasculitis, neuritis or encephalitis. We report a case of a 40 year old male who developed focal neurological deficit 10 hours following a wasp sting, which was confirmed to be ponto-cerebellar infarction on MRI scan, and recovered within five days.

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A 56 year old man presented with acute onset posterior column and lateral spinothalamic tract dysfunction over a period of 15 days. MRI showed diffuse hyperintensity on T2WI involving the posterior columns. A diagnosis of subacute combined degeneration (SCD) of the spinal cord was considered and confirmed by laboratory findings. The patient showed complete recovery on B12 therapy. MRI lesion also compeletely resolved.

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Ossified ligamentum flavum is increasingly appreciated as an important cause of thoracic myeloradiculopathy. Fifteen patients with age ranging from 30-61 years were studied. Fourteen presented with spastic paraparesis, and radiculopathy was the only complaint in one patient. Routine skiagrams and myelograms showed non-specific changes. Baseline CT and CT myelogram, however, documented the ossification of ligamentum flavum comprehensively. MRI was done in three patients. Multiple levels of the disease were seen in two cases. Four patients had ossified posterior longitudinal ligament. Thickened ligamentum flavum should be considered as an important cause of thoracic cord compression.

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Magnetic resonance images (MRI) in a patient with primary lateral sclerosis is described. T2WI, MRI of brain showed characteristic hyperintensities of the corticospinal tracts bilaterally, extending from the internal capsule to the brain stem - producing a 'wine glass' appearance on coronal sections, which has not been described so far to the best of our knowledge. Differential diagnosis of this interesting 'wine glass' appearance on MRI along with a review of the MR-signal abnormalities in motor neuron disease is discussed.

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The cerebellopontine angle (CPA) is a rare site for the growth of choroid plexus papilloma (CPP). The clinicoradiological diagnosis of this tumor in the CPA is difficult because of the nonspecific clinical presentation and radiological features. Five cases of choriod plexus papilloma (3 males, 2 females) operated upon at this center are reviewed. All the cases were operated upon by retromastoid suboccipital craniectomy. As they all presented with a typical CPA syndrome without any distinctive radiological feature, a clinicoradiological diagnosis of CPP could not be reached in any of these. The diagnosis of CPP could only be suspected at the operation table and established on histopathological examination. Two patients developed recurrences at the primary site following surgery. One patient developed recurrence twice despite gross total excision of tumor in each sitting. Subsequently, this patient remained symptom free for a follow-up period of 1 year. Another patient developed recurrence 2 years following surgery, but he died due to septicemia and aspiration pneumonitis. Therefore definitive surgery could not be performed. Radiotherapy was offered to one of the patients having residual mass post operatively, to render her symptom free for a 4 year follow-up. The remaining two patients have also showed progressive improvement in their symptoms following surgery for 4 years on follow-up. Hydrocephalus was a common feature in all the cases preoperatively, but only one required shunt CSF diversion, because of rapid deterioration in visual equity. In all other cases, hydrocephalus was managed conservatively. Surgery remains the main modality of treatment for CPP, both for primary and recurrent tumors, but radiotherapy may have a role in cases of recurrence, which are quite frequent.

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MRI, done later in life, in two patients with infantile hemiplegia syndrome showed significant volume loss in the cerebellar hemisphere contralateral to the side of the affected cerebrum. The cerebellar volume loss seemed to correlate with the degree of volume loss in the contralateral cerebral hemisphere. These observations provide morphological evidence of the phenomenon of crossed cerebral-cerebellar diaschisis (CCD). Functional neuroimaging studies in support of the concept of CCD has been critically reviewed.

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A 53 year old male underwent total excision of a large sphenoidal wing meningioma. Patient was treated with cephalosporins and phenytoin for postoperative meningitis. Three weeks after surgery, a follow up lumbar puncture was done. The patient became paraplegic over a few hours. Imaging of the dorsolumbar spine and other investigations demonstrated a large intraspinal hematoma caused by thrombocytopenia which was probably drug induced. After normalising the platelet count surgical evacuation of the spinal subarachnoid hematoma was done. Relevant literature is reviewed.

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Rett's syndrome (RS), a pervasive neurodevelopmental disorder, is being increasingly recognized all over the world, but there is paucity of reports from India. Due to its manifestations at any stage of development, it is important to arrive at a correct diagnosis. Two cases are presented, highlighting the need to recognize this relatively uncommon disorder and to differentiate it from various other neurodegenerative disorders.

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The dorso lumbar segment of spine (D10 to L2) is an unstable zone between fixed dorsal and mobile lumbar spine. A combined anterior and posterior approach with short segment stabilization was found most appropriate. Thirty cases were treated over a period of 4 years and 6 months. There were 26 male and 4 female patients with mean age of 32.6 years. L1 vertebra was fractured in 17 cases, D1 in 8 cases, D11 in 4 cases and D10 in one case. 14 cases had total neurological deficit, 9 cases had partial and 7 had no neurological deficit. We have used three column classification of Denis to assess the cases. Seven patients returned to regular physical work, 5 had restricted physical work, 5 remained in full time light job and 9 patients were unable to return to original job but did some work. Most had flaccid paraplegia but 4 patients were completely disabled due to spastic paraplegia. Neurological recovery occurred in all the patients with partial paralysis, and appeared to be dependent on initial kyphosis. The overall recovery rate varied from 50% to 90%. There is no correlation between canal compromise and severity of injury. Neurological injury occured at the time of trauma, rather than as a result of pressure of fragment in the canal. No strong conclusion could be drawn to say that the results of surgery were superior to non-operative treatment.

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531 epileptic patients, who had achieved remission mostly for 2 years or more were studied. The mean follow up period was 5 years. Recurrence was noted in 103 patients (19%) after gradual withdrawal of AED, over a period of 3-4 months. 424 patients (81%) did not have recurrence. The recurrence rate was influenced adversely by factors like adolescent age and later onset seizures, pre-treatment duration of symptoms more than 3 years, pre-treatment precipitating factors like emotional stress, lack of sleep and meals (however, number in each group is small), positive family history of epilepsy, focal neurodeficit, absence and myoclonic plus grandmal type of clinical seizures, paroxysmal generalized spike and wave discharges and generalized short polyspike and wave discharges in the pretreatment EEG, atrophic changes on CT brain scan (in small numbers), head trauma at birth or later and hereditary factors as etiology of epilepsy, and more than 30 number of seizures before achieving the remission. Factors like, sex, frequency of seizures, period of remission i.e. two years or more and number of drugs used to achieve remission, did not have any significant adverse effect. However, in the last parameter 95% remission was achieved by one or a combination of two drugs (72% and 23% respectively).

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Two brothers (3 and 2 year old) with characteristic findings of atypical benign partial epilepsy of childhood (pseudo-Lennox syndrome) are reported, to emphasize the presence of a possibility of a genetic basis of this disorder and the importance of intravenous immune globulin (IVIG), vigabatrin (VGB) and lamotrigine (LTG) therapy. Sleep EEGs of both the patients showed typical features of Lennox-Gastaut syndrome. On follow-up, the convulsions were found to be resistant to numerous antiepileptic agents in one patient while they were easily controlled with LTG monotherapy in the other patient. In the elder brother, who was diagnosed as intractable epilepsy, the convulsions disappeared with IVIG and VGB. During the long term follow-up, they were seizure free for five and two years respectively, and their mental motor development was excellent.

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Miller fisher syndrome (MFS) is a variant of Guillain-Barre syndrome characterized by the triad of ophthalmoplegia, ataxia and areflexia. Recurrences are exceptional with MFS. A case with two episodes of MFS within four years is reported. He presented with findings of ophthalmoplegia, ataxia, areflexia, and oropharyngeal weakness and mild distal sensory impairment during both episodes. Electrophysiological findings showed reduced compound muscle action potentials and sensory nerve action potentials with no evidence of conduction blocks. Nerve biopsy showed segmental demyelination. MRI of brain was normal. He responded well to immunoglobulins during both episodes suggesting that immunomodulating drugs have a role in the treatment of MFS.

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Facial myokymia is a rare presenting feature of a vestibular schwannoma. We present a 48 year old woman with a large right vestibular schwannoma, who presented with facial myokymia. It is postulated that facial myokymia might be due to a defect in the motor axons of the 7th nerve or due to brain stem compression by the tumor.

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Chemical asymmetries in normal human brain were studied using the non-invasive technique of volume localized proton magnetic resonance spectroscopy (MRS). The technique of STEAM was used to acquire water-suppressed proton spectra from 8 ml voxels placed in bilaterally symmetrical positions in the two hemispheres of the brain. One hundred and sixty eight right-handed male volunteers were studied for six different regions in the brain (n=28, for each region). Parietal, occipital, temporal, frontal, thalamus and cerebellum regions were studied. The focus was on metabolites such as N-acetyl aspartate (NAA), creatine/phosphocreatine (Cr/PCr) and choline (Cho) containing compounds. Ratios of the peak areas were calculated for them. Quantitation of the metabolites were carried for data on 18 volunteers. Significant interhemispheric differences in the distribution of metabolites were observed for all the regions studied. There were statistically significant differences on right and left side for the metabolite ratios in all the regions studied. The study has shown the existence of significant lateralization in the distribution of proton MR visible metabolites for all the regions studied.

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Six patients received mechanical ventilation for 8 episodes of myasthenic crisis in a two year period. Median duration of ventilation was 8 days. Only one patient required ventilation for more than three weeks and underwent tracheostomy. Atelectasis and pneumonia were the common ventilator associated complications observed, and these responded to therapy. Two patients underwent thymectomy after initial stabilization and while being ventilated. All patients recovered completely.

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Intracranial metastases of central nervous system are relatively common in patients with systemic cancer. Computed tomography (CT) scans of 60 patients of intracranial metastatic disease of unknown primaries, at the time of surgery, were retrospectively analyzed. These patients primarily presented with neurological dysfunction. They were operated upon for various reasons and histopathological diagnosis was obtained. There were 39 male and 21 female patients, with age range of 18 to 74 years. The common clinical symptoms were raised intracranial pressure without lateralization, acute onset hemiplegia and seizures. Multiple cranial nerve palsies were observed in 4 patients. Histopathologically the intracranial lesions consisted of metastatic adenocarcinoma (32 cases) or metastatic squamous cell carcinoma (28 cases). Among cases of adenocarcinoma, CT revealed solitary lesions in 17 and multiple lesions in 13 cases. Two had extraaxial deposition in the region of petrous apex. Out of squamous cell carcinomas, 17 lesions were solitary, while 10 were multiple and one had extraaxial deposition in the region of petrous apex. This study is unique as it consisted of CT features of intracranial metastases of unknown primary malignant disease elsewhere in the body.

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Atretic parietal cephalocele is a congenital anomaly of childhood with poor prognostic value. The authors report an adult with associated brain malformations (interhemispheric cyst, partial corpus callosum agenesis) - but, interestingly, without any clinical symptoms related to the entity.

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Three patients of delayed extradural hematoma (EDH) were seen in the last one year among forty eight consecutively treated cases of EDH. All the three hematomas were evacuated. Awareness of this entity and a high degree of vigilance are strongly recommended to detect such cases. Repeat CT should always be done, especially after decompression by either surgical or medical means, recovery from shock or whenever there is evidence of even minimal bleeding under a skull fracture on initial CT scan.

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Physicians are faced with the task of predicting the immediate and long term outcome in stroke patients. It is also important to efficiently and optimally utilize resources. We used APACHE III scoring system or predicting in hospital outcome in patients with stroke. We found it to be sensitive (>90%) and resonably specific (73%) in predicting short term, in-hospital mortality, in our study group.

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The present study was addressed to find out the expression of Bcl2 proto-oncogene in tumor tissues derived from 25 patients with primary central nervous system tumors. Brain parenchyma in 8 cases, with deeply located tumor, was also examined for Bcl2 expression which served as control. Both benign and malignant tumors (confirmed by histopathological examination) expressed Bcl2 gene product. Tumors exhibited 2-6 fold increase in Bcl2 expression as compared to the normal parenchyma adjacent to some of these tumors studied. However, no correlation was found between the histopathological types of tumor, glial fibrillary acidic protein positivity and degree of Bcl2 expression. Based on this study, we propose that the overexpression of Bcl2 gene product found in primary CNS tumors may be an important molecular event which is known to make the various types of tumor resistant to chemotherapy or radiotherapy.

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Epidural abscess is a relatively uncommon disorder. Although the surgical management is the mainstay of treatment, there are case reports of it being managed conservatively in selected patients. We report a patient who presented with quadreparesis due to epidural abscess and had infective endocarditis due to Staphylococcus aureus septicemia. Both epidural abscess and infective endocarditis were managed conservatively with intravenous antibiotics given for four weeks, with complete recovery of patient.

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Japanese encephalitis (JE) is an encephalomyelitis involving cortex, subcortex, brainstem and spinal cord. There is paucity of studies on the neurophysiological evaluation in JE. This study aims at comprehensive evaluation of EEG, sensory and motor evoked potentials, nerve conduction and electromyography; and correlate these with clinical findings. Sixty five patients with JE diagnosed on the basis of clinical, radiological and virological criteria were subjected to a detailed clinical evaluation during the acute stage of illness. Cranial CT scan or MRI was carried out in all the patients. All the patients underwent 10 or 18 channel EEG, motor and sensory evoked potentials to both upper and lower limbs bilaterally as well as peroneal and sural nerve conductions and concentric needle EMG. Outcome, was defined at the end of 3 months into poor, partial and complete recovery. The patient's age ranged between 2-65 years. There were 40 males and 25 female patients. Fifteen patients were less than 12 years of age. History of seizure was present in 31 patients. Quadriplegia was seen in 39 and hemiplegia in 8 patients. Muscle wasting was present in 16 patients and tendon reflexes were reduced in 12 and of mixed pattern in 14 patients. Cranial MRI revealed thalamic lesion in 38, basal ganglia in 21, substantia nigra in 30, pons in 5, cerebellum in 3 and cerebral cortex in 7 patients out of 57 patients. EEG revealed nonspecific theta to delta slowing in 45, alpha pattern coma in 5 and epileptiform discharges in 8 patients. EMG revealed fibrillations in 23 patients. Motor evoked potentials were abnormal in 34 out of 46 patients and revealed patchy and focal abnormalities comprising of unrecordable, prolonged and normal pattern. Somatosensory evoked potentials were abnormal in 8 patients only. At 3 month, 26 patients had complete, 13 partial and 15 had poor outcome. Eight patients died in acute stage and 3 were lost to followup. MEP correlated with weakness and 3 month outcome whereas EEG, SEP and EMG did not have any correlation. MEP changes were more frequent in JE and had prognostic significance.

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Two male siblings aged 8 and 11 years, born of non-consanguineous parents, presented with classical triad of congenital ichthyosis, mental retardation and spastic diplegia. They had no eye, hair, nails and teeth changes. MRI showed diffuse dysmyelination.

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52 patients (25 males and 27 females) suffering from refrectory partial seizures, of not more than two years duration and on carbamazepine monotherapy were enrolled in this study. Patients were randomly put on gabapentin (19 males and 8 females) or lamotrigine (6 males and 19 females) as add on therapy. The efficacy of the drugs was assessed by the seizure frequency, pattern of seizures and seizure free interval. The safety was evaluated from the biochemical investigations and the adverse effects observed or reported by the patients during the course of the study. The average frequency of basal partial seizures was 6.26+3.86 and 5.04+2.47 which decreased significantly (p<. 001) after 12 weeks of add on therapy to 1.75+2.16. and 1.68+2.94 in the GBP and LTG group respectively. However, there was no significant difference between the two drugs after 12 weeks of add on therapy. The PCB (primary change in basal seizure frequency) values decreased to -72+34.92 and -76.22+29.68 in the GBP and LTG group respectively. The difference in these two groups was not significant. The responder rate was 77.7% and 92% respectively in GBP and LTG group respectively. GBP was found to be more effective in partial seizures with secondarily generalization while LTG was effective in all subtypes of partial seizures. The abnormal scalp EEG was recorded in 33.3% (9 of 27 patients) in GBP group and 40 %( 10 of 25 patients) in LTG group and it did not revert to normal in 33.3% and 40% of patients in either of groups (GBP/LTG). Minor side effects which were self limiting were noticed in 80% in groups I and 74% were groups II.

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Metastasis of intraventricular meningiomas through CSF pathways is a rarity and only 4 cases have been reported in world literature describing meningiomas which were intraventricular and malignant. Here we report a case of benign intraventricular meningioma which had spread through CSF pathways, the recurrences as well as the primary tumor being benign in nature.

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Ninety white hybrid rabbits, each weighing 2.5 to 3.5 kg, were used for this experimental model. Thirty rabbits were used for control, and sixty other rabbits were investigated for the response of host to the dural graft. In all animals, a dural defect, 1 x 1 cm in size, was created on the left parietal area following craniotomy. In the control group the excised free dural piece was then sutured again to the area from which it had been excised before. The dural defect was closed with dehydrated human dura mater (DHD) in the half of the rabbits in the group of study, and with autogenous fascia lata (AFL) in the other half. After operation, animals in each group were then subjected to one of five different groups comprising of 3,14,30,60 and 90 days follow-up periods. At the end of follow-up periods, histological, parameters such as cellular inflammatory response, development of fibrous tissue, capsulation, and calcification were examined in specimens obtained from the animals. There was no significant difference between AFL and DHD grafts. In conclusion, it seems that DHD is suitable as an ideal dural graft, because the immune response of host to DHD was almost similar to AFL.

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A family with coexistence of hypotension, recurrent respiratory infection, motor tics, obsessive compulsive disorder, major depressive disorder, early onset osteoporosis, low body mass index, bulimia nervosa and healthy aging with longevity is described. The family members had hyposexual behavior, less tendency for spirituality, had no insomnia but a tendency towards increased somnolence, no addictive behaviour, had more bonding and affectionate behavior and were less creative with an average IQ. There was no vascular thrombosis, systemic neoplasm and neuronal degeneration in the index family. All members of the family were left hemispheric dominant. The level of serum digoxin, HMG CoA reductase activity and dolichol was found to be decreased in all with a corresponding increase in RBC Na(+)-K(+) ATPase activity and serum ubiquinone magnesium level. There was increase in tyrosine catabolites and a reduction in tryptophan catabolites in serum. Total and individual glycosaminoglycan fractions, carbohydrate residues of glycoproteins, glycolipids, activity of GAG degrading enzymes and glycohydrolases were decreased in serum. The concentration of RBC membrane total GAG and carbohydrate residues of glycoproteins increased while cholesterol : phospholipid ratio of membrane decreased. The activity of free radical scavenging enzymes were increased while the concentration of free radicals decreased significantly. The same biochemical patterns were observed in left hemispheric dominance as opposed to right hemispheric dominance. The significance of these findings in the pathogenesis of these disorders is discussed.

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A family of six members is reported, who accidently consumed arsenic, during preparation of indigenous medicine in their home, and developed arsenic poisoning.

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