Background: Astrocytoma is the most common neuroepithelial neoplasm, and its grading greatly affects treatment and prognosis. Objective: According to relevant factors of astrocytoma, this study developed a support vector machine (SVM) model to predict the astrocytoma grades and compared the SVM prediction with the clinician's diagnostic performance. Patients and Methods: Patients were recruited from a cohort of astrocytoma patients in our hospital between January 2008 and April 2009. Among all astrocytoma patients, nine had grade I, 25 had grade II, 12 had grade III, and 60 had grade IV astrocytoma. An SVM model was constructed using radial basis kernel. The SVM model was trained with nine magnetic resonance (MR) features and one clinical parameter by fivefold cross-validation and differentiated astrocytomas of grades I-IV at two levels, respectively. The clinician also predicted the grade of astrocytoma. According to the two prediction methods above, the areas under receiving operating characteristics (ROC) curves to discriminate low- and high-grade groups, accuracies of high-grade grouping, overall accuracy, and overall kappa values were compared. Results: For SVM, the overall accuracy was 0.821 and the overall kappa value was 0.679; for clinicians, the overall accuracy was 0.651 and the overall kappa value was 0.466. The diagnostic performance of SVM is significantly better than clinician performance, with the exception of the low-grade group. Conclusions: The SVM model can provide useful information to help clinicians improve diagnostic performance when predicting astrocytoma grade based on MR images.

Background: Sarcoglycanopathies are a group or autosomal recessive muscular dystrophies designated as α, β, γ, or δ sarcogycanopathy. Materials and Methods: It is a retrospective analysis of case series. Results: Sixty six patients immunohistochemically confirmed to have sarcoglycan deficiency were included in the analysis. The study period extended from 1997-2008. The male to female ratio was 1.5:1. Mean age at the onset of muscle complaints was 6.2±3.7 years (range 1-18). Mean age at evaluation was 10.0±4.8 years (range 3-31). Mean duration of illness was 47.02±44.80 months (range 3-325). Onset in the first decade was seen in 59 (89.4%) and 25 (42.4%) of these had onset before five years of age. The remaining seven (10.6%) had onset in second decade and none after 20 years of age. Consanguinity was present in 54 (81.8%). In 34 of 66 cases only a-SG was carried out and this had shown total absence of staining in all fibers. In the remaining 32 cases where the entire panel was performed, absence of all sarcoglycans was noted in 10 (15.1%), isolated a-SG deficiency in 7 (10.6%), isolated b-SG deficiency in 6 (9.1%), and isolated g-SG deficiency in 3 (4.5%). Combination deficiency was also observed: absence of α and β (n=4), β and γ (n=2), and α and γ (n=1). Conclusions: Our series was a large series and with predominantly pediatric age group. Sarcoglycanopathy should be particularly suspected in a child born to consanguineous parents and who presents with proximal muscle weakness and calf hypertrophy, elevated CK level, and myopathic pattern on EMG.

Background and Aim: To determine the quality of life and its relationship with socio-demographic and medical factors in patients with stroke. Setting and Design: Seventy patients with stroke seen in the neurology clinic on a follow-up visit; at least a period of 3 months of follow-up was included. Materials and Methods: Data were collected using (a) a questionnaire to determine patients' socio-demographic factors, (b) Short Form-36 (SF-36) and (c) Perceived Social Support from the Family Scale. Results: The mean score of global quality of life in patients was 37.08±17.03, and the mean score on Perceived Social Support from the Family Scale was 11.21±7.12. The mean score of global quality of life varied significantly according to age, gender, education, profession, place of residence, comorbidities, affected brain area, disease duration and educational level of the caregiver. Conclusion: These results showed a low mean quality-of-life score in patients with stroke. There is a positive and significant correlation between scores on the Perceived Social Support from the Family Scale and various aspects of quality of life, such as functional status, well-being, general health perception and global quality of life.

Background: The Mini Mental State Examination (MMSE) is widely used for dementia screening but has several shortcomings such as prominent ceiling effects, inadequate sensitivity to mild cognitive impairment, and uneven sampling of the major cognitive domains. Aims: In this study, we pilot a new dementia screening test - the Concise Cognitive Test (CONCOG) - designed to overcome the above short comings and describe the reliability measures and age, education, and gender effects. The CONCOG has a total score of 30, and has subtests for orientation, naming, registration, free recall and recognition of four words, semantic verbal fluency and copying. Patients and Methods: Participants were screened to exclude those with any neurological or psychiatric disease, simultaneously administered the CONCOG, and a Hybrid Mini Mental State Examination (HMMSE) adapted from Folstein's MMSE and Ganguli's Hindi Mental State Examination. Results: The study sample had 204 subjects over the age of 60 years with a mean of 73 years and education level of 8 (4.5) years. Internal consistency for the CONCOG (Cronbach's alpha) was 0.74, inter-rater reliability (Kendall's tau-b) was 0.9, and the one-month test-retest reliability (Kendall's tau-b) was 0.7. Age and education level, but not gender, significantly influenced performance on both scales. Although the influence of age on the two scales was to a similar degree, the HMMSE was more affected by education than the CONCOG. Of 204 subjects, only 12 (5.7%) subjects obtained the maximum score on the CONCOG compared with 30 (14.1%) subjects on the HMMSE. The CONCOG took less than 10 minutes to complete in this sample. Age and education stratified norms are presented for the CONCOG. Conclusions: The CONCOG is a reliable cognitive screening measure. It has negligible ceiling effects, is less influenced by education compared with the HMMSE, and offers subscale scores for the major cognitive domains.

Background: Systematic studies on neuropsychological profile in patients with Wilson's disease (WD) are far and few. Aim: To examine the profile of cognitive deficits and their magnetic resonance imaging (MRI) findings in patients with WD. Patients and Methods: Twelve confirmed patients of WD (age at onset and evaluation, 13.7±11.2 and 21.7±5.3 years, respectively; M-F ratio, 7:5) on de-coppering therapy constituted the study sample. Battery of neuropsychological tests measuring mental speed, motor speed, sustained attention, focused attention, verbal category fluency, verbal working memory, response inhibition, planning, concept formation, set-shifting ability, verbal and visual learning and memory were administered. Phenotypic details and observations on MRI of brain carried out within six months of neuropsychological assessment were documented. Results: Neuropsychological assessment elicited cognitive deficits in multiple domains in all but one patient, who had normal MRI. Percentage of patients in the deficit range in various domains included: motor speed: 73%; verbal working memory, sustained and focused attention: 50%; verbal learning: 42%; visuo-constructive ability, verbal memory, mental speed: 33%-34%; verbal fluency, set-shifting ability, visual memory, verbal memory: 25%-27%; and verbal recognition: 17%. MRI was normal in three patients, and revealed variable abnormalities in the remaining: cerebral atrophy in 3; brainstem atrophy in 2; signal changes in basal ganglia in 9; and brainstem signal changes in 5. None had subcortical white matter changes. Two patients with normal MRI showed cognitive deficits. Conclusion: This study provides insight into the complex cognitive and brain changes observed on MRI in WD. Use of advanced MRI techniques in a larger cohort may improve understanding regarding functional and structural brain changes observed in similar disorders.

Tuberculous meningitis (TBM) is a serious meningitic infection commonly found to occur in the developing countries endemic to tuberculosis. Based on the clinical features alone, the diagnosis of TBM can neither be made nor excluded with certainty. Unfortunately there is still no single diagnostic method that is both sufficiently rapid and sensitive. Most factors found to correlate with poor outcome can be directly traced to the stage of the disease at the time of diagnosis. The only way to reduce the mortality and morbidity is by early diagnosis and timely recognition of complications and institution of the appropriate treatment strategies.

Tuberculous meningitis (TBM) is a serious meningitic infection commonly found to occur in the developing countries endemic to tuberculosis. Based on the clinical features alone, the diagnosis of TBM can neither be made nor excluded with certainty. Unfortunately there is still no single diagnostic method that is both sufficiently rapid and sensitive. Most factors found to correlate with poor outcome can be directly traced to the stage of the disease at the time of diagnosis. The only way to reduce the mortality and morbidity is by early diagnosis and timely recognition of complications and institution of the appropriate treatment strategies.

Background: The duration of anti-tuberculous therapy in tuberculous meningitis is controversial. There is variation in recommendations by different societies and expert groups on this issue. Objective: To determine the strength of evidence for short-term therapy in tuberculous meningitis through review of literature and critical appraisal. Materials and Methods: Cochrane CENTRAL (Issue 4, 2010), TRIP database, and PubMed (from 1966 to present) were searched for relevant papers with keywords 'meningeal tuberculosis' and tuberculous meningitis' combined with 'chemotherapy'. A critical appraisal of a systematic review was done using standard criteria. Results: A total of 10 relevant papers were identified. All papers were included in a systematic review. The systematic review did not specify study design of studies to be included, had only case series but no randomised controlled trial, and unclear definition of endpoints. Conclusions: The evidence base for short-term therapy for tuberculous meningitis is weak. There is a need to conduct a randomised controlled trial with non-inferiority hypothesis of adequate sample size with well-defined end points and adequate follow-up. This is a challenge as well as opportunity for Indian neurologists.

Background: Rapid and specific diagnosis of tubercular meningitis (TBM) is of utmost importance. Aim: To evaluate polymerase chain reaction (PCR) using protein b primers directed against M. tuberculosis for the diagnosis of tuberculous meningitis. Materials and Methods: PCR using protein b primers was performed in ten patients with confirmed TBM (culture positive), 60 patients with clinically suspected TBM and 40 patients with no TBM (control group). Results: Protein b PCR had a sensitivity of 90% and a specificity of 100% in patients with confirmed TBM. In 60 clinically diagnosed TBM patients, protein b PCR was positive in 49 (81.7%) patients. The overall sensitivity of microscopy, culture and PCR using protein b primers was 1.4%, 14.3%, and 82.8% and specificity was 100%, 100%, and 100% respectively. Conclusion: Protein b PCR is valuable in rapid diagnosis of TBM.

Arachnoiditis involving optic nerve and the optic chiasm can occur as a complication of tuberculous meningitis (TBM). This study evaluates the clinical features, cerebrospinal fluid (CSF) and laboratory parameters and imaging findings of optochiasmatic arachnoiditis (OCA) and also tried to identify any factors which can predict this complication in patients with TBM. Patients admitted with TBM in the neurology wards of a tertiary care teaching hospital over a period of 6 years formed the material for this study. Student's "t" test and univariate analysis were done to identify any predictors for this complication and the variables found to be significant were further analyzed by multivariate logistic regression analysis. One hundred sixty-three patients with TBM, admitted over a 6-year period, were studied. Twenty-three (14%) patients developed OCA. Eighteen out of 23 (78%) developed this complication while on antituberculous treatment (ATT) and 5/23 (22%) were newly diagnosed cases of TBM. Of those already on treatment, 12/23 (52%) were receiving only ATT, the remaining 6/23 (26%) had received steroids along with ATT in varying doses and duration. The average period from diagnosis of TBM to visual symptoms was 6.4 months. On the multivariate logistic regression analysis, female sex (P < 0.037), age less than 27 (P < 0.008) years and protein content in the CSF > 260 mg% (P < 0.021) were the factors predisposing toward this complication. At 6 months follow-up, on treatment with steroids and ATT, 17% had improvement and no further deterioration was noted in visual acuity in 52%. OCA can develop even while on treatment with ATT. Young women with a high CSF protein content seem to be more prone for this complication.

"Telestroke" is emerging as a potential timesaving, efficient means for evaluating patients experiencing acute ischemic stroke. It provides an opportunity for administration of thrombolytic drugs within the short therapeutic time window associated with AIS. We describe our experiences of the feasibility and safety of remote radiology interpretation with telephone consultation. Thammasat Stroke Center employs a computed tomography-digital imaging and communication in medicine (CT-DICOM) image transfer by PACS (SYNAPSE-Fujifilm), providing a real-time CT image transferred directly to the stroke consultants. The patient data are communicated by traditional telephone conversation. Here, we assessed patients who received intravenous rt-PA treatment for ASI between October 2007 and January 2009. A total of 458 patients with AIS and transient ischemic attack (TIA) were admitted to a stroke unit during the study period. One hundred patients received intravenous rt-PA (21%). Median NIHSS before thrombolysis was 15 (3-34). Mean door-to-needle time was 54 minutes (15-125). Mean onset-to-treatment time OTT was 160 minutes (60-270). There were 13 asymptomatic intracerebral hemorrhages and two (one fatal) symptomatic intracerebral hemorrhages. At 3 months, 42 patients had achieved excellent recovery (mRS, 0-1) and 14 had died. Administration of rt-PA for AIS with remote radiology interpretation with telephone consultation was feasible and safe, and the system was well received. Further studies are needed to determine the benefit of this method as compared to the conventional telephone consultation alone.

Myotonia congenita (MC) is a genetic disease characterized by mutations in the muscle chloride channel gene (CLCN1). To date, approximately 130 different mutations on the CLCN1 gene have been identified. However, most of the studies have focused on Caucasians, and reports on CLCN1 mutations in Chinese population are rare. This study investigated the mutation of CLCN1 in two Chinese families with MC. Direct sequencing of the CLCN1 gene revealed a heterozygous mutation (892G>A, resulting in A298T) in one family and a compound heterozygous mutations (782A>G, resulting in Y261C; 1679T>C, resulting in M560T) in the other family, None of the 100 normal controls had these mutations. Our findings add more to the available information on the CLCN1 mutation spectrum, and provide a valuable reference for studying the mutation types and inheritance pattern of CLCN1 in the Chinese population.

Tubular aggregates (TAs) are inclusions described in skeletal muscle in a variety of disorders. In a retrospective analysis, TAs were found in 18 (0.24%) cases and involved a spectrum of clinical phenotypes. Ultrastructurally, four distinct types of aggregates were noted. There was no correlation between the clinical phenotypes, duration of illness and types of TAs.

Head injuries caused by television (TV) sets falling on small children are becoming frequent in India with increasing sales of TV sets. This report describes television tip-over injuries in eight children aged 14 months to 6 years. Symptoms and findings were varied, from only swelling of the eyes to coma. Head computerized tomography scan findings were also diverse and included fractures (most common), intracranial hematomas and infarcts. Six of the eight children were successfully managed conservatively, one patient died on the third postoperative day, and one patient refused admission. The anatomical proportions and biomechanics of spine and body of small children make them more vulnerable to these crushing types of head injuries. As there is significant morbidity and mortality associated with these injuries, there is a need for increased awareness about these injuries among health personnel, general public, teachers, government officials and TV-manufacturing companies.

Coagulation abnormalities are common in patients with head injuries. However, the effect of brain injury on fibrinogen levels has not been well studied prospectively to assess coagulation abnormalities in patients with moderate and severe head injuries and correlate these abnormalities with the neurologic outcome. Consecutive patients with moderate (Glasgow Comma Scale (GCS),9-12) and severe (GCS≤8) head injuries were the subjects of this pilot study, All patients had coagulation parameters, including plasma fibrinogen levels measured. Clinical and computed tomography (CT) scan findings and immediate clinical outcome were analyzed. Of the 100 patients enrolled, only seven (7%) patients had hypofibrinogenemia (fibrinogen ≤200 mg/dL). The head injury was moderate in two patients and severe in five patients. Fibrinogen levels showed a progressively increasing trend in four patients (three with severe head injuries and one with moderate head injury). CT scan revealed subdural hematoma in five patients; extradural hematoma in one; and subarachnoid hemorrhage in another patient. Of the seven patients, two patients died during hospital. Large-scale prospective studies are needed to assess the fibrinogen level in patients with head injury and its impact on outcome.

We report the first case of a pilomotor seizure detected by electroencephalography /functional magnetic resonance imaging (EEG/fMRI). An adult woman presented with history of bouts of gooseflesh feeling and poilomotor activity in the left leg following viral encephalitis. 24-hour video-EEG and simultaneous EEG during fMRI revealed ictal discharges in the right parietal and temporal lobes. Associated blood oxygen level- dependent (BOLD) activations were found mainly in the right parietal region. The result represents a different generator of pilomotor seizure compared to prior reports. We suggests that the feeling of gooseflesh could be the core ictal symptom and a direct pathway from the sensory cortex to the lower autonomic system may exist bypassing the classic cerebral autonomic center.

D-penicillamine induced status dystonicus is a unique but serious drug related complication in a subset of patients with Wilson disease. Patho-physiological basis of its occurrence is not known. It often responds poorly to anti dystonia medications. We present three patients with Wilson disease who developed severe paroxysmal dystonic spells after receiving D-penicillamine treatment. All three patients responded well to gabapentin after failing to respond to other anti dystonia drugs.

Localized intervertebral disc and ligamentous ossification may precipitate neurological deficits at any time during the natural course of ankylosing spondylitis (AS). We report 2 patients with AS with "moustache' sign (localized intervertebral disc fibrosis and panligamentous ossification) and neurological deficits. One patient had syndesmophytosis (with paraparesis secondary to thoracic spinal canal stenosis), and the other had "bamboo spine" above and below the involved level. A laminectomy in the former relieved paraparesis, and posterior element excision in both the patients relieved focal tenderness and pain; and both of them could lie supine following surgery.

Germinoma occurring in the medulla oblongata is extremely rare. We report a case of primary intracranial germinoma arising in the medulla oblongata of a 24-year-old postpartum female who presented with progressive weakness of upper and lower limbs, seventh nerve palsy, and decreased palatal movements. Her MR imaging showed a heterointense mass lesion in the posterior portion of upper medulla, the histology of which was reported as germinoma. Germ cell tumors should be considered in the differential diagnosis of tumors occurring in the brain stem.

Pleomorphic xanthochromic astrocytoma primarily of the spinal cord is a rare entity. The case is possibly the fifth such report. Complete surgical excision is the essential requirement for good survival. In the absence of any clearly laid down protocols of adjuvant treatment, anecdotal reports support treatment with chemotherapy alone or both chemotherapy and radiotherapy.

Enterogenous cyst is extremely rare in the supratentorial compartment. Two adult patients with histologically variefied supratentorial entrogenous cyst are described. Light microscopy and immunohistochemistry examination revealed the endodermal origin of the cyst. Enterogenous cyst should be considered in the differential diagnosis of non-enhancing cyst in the supratentorial compartment. Total excision of the cyst wall should be done wherever feasible. Preventing spillage of the cyst contents during surgery is mandatory to avoid postoperative intractable seizures and craniospinal dissemination.

Pituitary carcinomas are rare adenohypophyseal tumors with cerebrospinal or extracranial metastasis. None of the histologic findings distinguish pituitary adenoma from carcinoma. We describe clinico-pathological and immunohistological features of malignant prolactinoma. The patient initially presented with a prolactin-secreting pituitary adenoma. The tumor showed aggressive clinical course presenting with repeated recurrences and eventually metastasized to multiple bones. MIB-1 and p53 labeling indices were also compared in primary adenoma, recurrent invasive adenoma and metastatic tumor.

Central nervous system primary malignant melanoma accounts for approximately 1% of all melanomas. Primary spinal melanomas are even more unusual. We report a patient with primary spinal melanoma of the cervical leptomeninges. The histology of the tumor showed tumor cells arranged in sheets, ill-defined fascicles and nests and displayed a moderate grade of cellular and nuclear pleomorphism and mitoses with abundant pigment in the cytoplasm. The tumor cells were immunoreactive for HMB-45, and for S-100.