In 1896 Joseph Franηois Felix Babinski described for the first time the phenomenon of the toes; nevertheless in this first paper he simply described extension of all toes with pricking of the sole of the foot. It was not until the second paper of 1898 that he specifically described the extension of the hallux with strong tactile stimulation (stroking) of the lateral border of the sole. Babinski probably discovered his sign by a combination of chance observation and careful re-observation and replication. He also had in mind practical applications of the sign, particularly in the differential diagnosis with hysteria and in medico-legal areas. Several of the observations and physiopathological mechanisms proposed by Babinski are still valid today, e.g., he realized since 1896 that the reflex was part of the flexor reflex synergy and observed that several patients during the first hours of an acute cerebral or spinal insult had absent extensor responses. He also found that most patients with the abnormal reflex had weakness of dorsiflexion of the toes and ankles and observed a lack of correlation between hyperactive myotatic reflexes and the presence of an upgoing hallux. He discovered that not all patients with hemiplegia or paraplegia had the sign but thought erroneously that some normal subjects could have an upgoing toe. Between 1896 and 1903 Babinski continued to think on the sign that bears his name and enrich its semiological and physiopathological value.

Context : In previous studies, patients with migraine and tension-type headaches have shown asymmetries at the central nervous system level. Aims: Hence we would like to figure out whether the lateral cerebral dominance might be more pronounced in the line bisection performance with these patients. Settings and Design: Patients were enrolled in a specialized headache clinic and healthy volunteers from a community as controls. Materials and Methods: The visual line bisection is used to test the unilateral neglect of subjects. Altogether, we studied 28 patients with chronic tension-type, 16 frequent episodic tension-type headache, 31 migraine patients without aura between attacks and 146 healthy volunteers. Statistical Analysis: One-way ANOVA was applied to the mean Index and Net of line bisection errors and the Spearman rank order to the relationship between the Index, Net, subject's age and time since onset of head pain. Results and Conclusions: As reflected by group means of Index of line bisection errors, healthy subjects and migraine patients bisected slightly rightward. Conversely, both forms of tension-type headache sufferers bisected significantly leftward compared to the healthy subjects as well as the migraine sufferers. The study indicates relatively strong right or weak left hemisphere activation or both in the two forms of tension-type headaches, confirming the central nervous system alterations in such patients.

Background: Though large epidemiological studies have not established associations between blood lipids and ischemic stroke, increasing evidences have suggested that lipid-modifying agents may reduce cerebrovascular events. Aims: To determine whether blood lipids are risk factors for ischemic stroke among hypertensive rural adults in China. Settings and Design: A cross-sectional survey was conducted during 2004-2006, which underwent cluster multistage sampling to a hypertensive resident group in the countryside of China. Materials and Methods: A total of 6,412 individuals (2,805 men, 3,607 women) with age ≥ 35 years were included. At baseline, lifestyle and other factors were obtained and blood lipids were assessed at a central study laboratory. Ischemic stroke was defined according to the criteria established by the National Survey of Stroke and all cases were further classified into lacunar infarction and other ischemic strokes. Statistical Analysis: Univariable and multivariable logistic regression were used. Results: In the univariable logistic regression model, LDL cholesterol (LDLc) in men and total cholesterol (TC), LDLc and TC-to-HDL cholesterol (TC: HDLc ratio) in women were risk factors for other ischemic strokes, with OR 1.42 (95% CI , 1.16-1.75), 1.31 (95% CI , 1.11-1.55), 1.47 (95% CI , 1.16-1.88) and 1.67 (95% CI , 1.28-2.14), respectively. After adjusting for independent variables, an increase in non-HDL cholesterol (non-HDLc) was associated with a significant increased risk of other ischemic strokes in women, with adjusted OR 1.45 (95% CI, 1.08-1.93). Conclusions: LDLc was the common risk factor for ischemic stroke in men and women, whereas Non-HDLc, TC and TC: HDLc ratio levels were related to ischemic stroke as risk factors only in women.

Background: Outcome following epilepsy surgery has traditionally been measured in terms of relief of seizures. However, changes in health-related quality of life (HRQOL) after surgery for intractable epilepsy are also important to document. There are no studies on the Indian population which assess the outcome of epilepsy surgery in terms of HRQOL. Materials and Methods: We conducted a prospective study on the patients undergoing epilepsy surgery for intractable seizures, between February 2004 and May 2006 at our center. All patients cleared for epilepsy surgery by the epilepsy surgery team were taken up for study. All patients < 15 years age and mentally retarded or with progressive neurological diseases were excluded. Demographic profile, seizure characteristics and seizure outcome using Engel grading was assessed. Health-related quality of life was assessed using QOLIE-31 questionnaire before surgery and six months after surgery. Results: Thirty-six patients satisfying the inclusion/exclusion criteria were included in the analysis. Twenty-nine of these (Group 1) had good seizure outcome (Engel 1 and 2), while seven patients (Group 2) had poor seizure outcome (Engel 3 and 4) at six months. Overall, 77% of all study patients were completely seizure-free at follow-up. There was no baseline difference in the seven domains of QOLIE-31 between the two groups. There was very significant improvement ( P value >0.005 using paired sample T test) in all the domains of QOLIE-31 in the good outcome group after surgery. Health-related quality of life improvement was seen in all the domains in the poor outcome group also, however, it was statistically significant only for the following parameters: seizure worry, overall QOL, emotional wellbeing, energy fatigue and social functioning domains. Improvement in seizure worry, overall QOL, emotional wellbeing and social functioning was significantly more in Group 1 as compared to Group 2. Conclusion: Complete seizure-free state after surgery is associated with very significant improvement in HRQOL parameters. Several, but not all parameters of HRQOL as assessed by QOLIE-31, improved after surgery even in the poor seizure outcome group. The improvement in domains of seizure worry, overall QOL, emotional wellbeing and social functioning is significantly more in those patients in whom complete seizure-free state is achieved.

Background: Head injury contributes significantly to mortality and morbidity in India. Evaluation of the available trauma care facilities may help improve outcome. Aim: To evaluate the factors influencing the mortality of patients with head injury who had intensive care management and evolve strategies to improve outcome. Setting and Design: Retrospective study in a tertiary hospital where intracranial pressure monitoring (ICPM) is not routinely practiced. Materials and Methods: All patients with head injury managed in the intensive care unit in a two-year period were included. The factors evaluated were age, vital signs, Glasgow Coma scale score (GCS) at admission, pupillary light reflex (PR), oculocephalic reflex (OCR), hemodynamic stability, computerized tomography (CT) findings, diabetes mellitus, anemia, infections and abnormalities of serum sodium. Results: We analyzed 208 patients (202 without ICPM). In-hospital mortality was 64 (31%). Only 24 (11.5%) patients were admitted within one hour of injury, while one-third arrived after six hours. The clinical factors (at admission) that influenced mortality included age, GCS, PR, OCR and diastolic blood pressure (DBP). Effacement of the basal cisterns in the initial and repeat CT scans, hyperglycemia, hemodynamic instability and serum sodium imbalances were associated with higher mortality. The independent predictors of mortality by logistic regression were initial GCS, DBP, hemodynamic instability and effacement of cisterns on repeat CT. Conclusions: Mortality following head injury is high. Pre-hospital emergency medical services are disorganized. The key to reducing mortality within the limitations of our current trauma system is maintenance of DBP>70 mmHg and SBP >90 mmHg from the time of first contact.

Background: Ventriculostomy is a common neuroendoscopic operation but one with disastrous complications in rare cases. Aims: The aim of this study was to perform an intravital analysis of the configuration at the floor of the third ventricle as a possible basis for selection of the ventriculostomy site. Materials and Methods: The study population consisted of 32 patients who underwent ventriculostomy for the treatment of hydrocephalus. Perforation of the floor of the third ventricle was carried out on an individual basis following evaluation of the anatomic situation. Video material and magnetic resonance images (MRI) were analyzed. Results: A classification system including three major groups was developed using the inner distance of the mamillary bodies as the key criterion. It was defined as narrow for values between 0 and 1 mm (observed range: 0-0.5 mm), medium for values between 1.1 and 3.4 mm (range 1.1-3.4 mm) and large for values greater than 3.4 mm (range: 3.8-6.9 mm). Statistical analysis of MR and video measurements revealed a good correlation. The ventriculostomy site was rostral of the mamillary bodies in 23 of the patients (n=27) and sligthly occipital in four. The ventriculostomy site was located more to the left in 22 patients and more to the right in five. Conclusion: As a conclusion the ventriculostomy site has to be chosen in each case following a careful review of all available information. A classification system for the anatomical variations as well as the exact size and site of ventriculostomy should be introduced.

Background: The occipitocervical contoured rod (CR) stabilization for use in craniovertebral junction (CVJ) pathologies is an effective and economical technique of posterior fusion (PF). Aims: The various indications for CR in CVJ pathologies are discussed. Settings and Design: Retrospective analysis. Materials and Methods: Fifty-four patients (mean age: 31.02 ± 13.44 years; male: female ratio=5.75:1) who underwent CR stabilization are included. The majority had congenital atlantoaxial dislocation (AAD; n=50); two had CVJ tuberculosis; one each had rheumatoid arthritis and C2-3 listhesis, respectively. The indications for CR fusion in congenital AAD were associated Chiari 1 malformation (C1M) (n=29); occipitalized C1 arch and/or malformed or deficient C1 or C2 posterior elements (n=9); hypermobile AAD (n=2); and, rotatory AAD (n=3). Contoured rod as a revision procedure was also performed in seven patients. Most patients were in poor grade (18 in Grade III [partial dependence for daily needs] and 15 in Grade IV [total dependence]); 15 patients were in Grade II [independent except for minor deficits] and six in Grade I [no weakness except hyperreflexia or neck pain]. Results: Twenty-four patients improved, 18 stabilized and six deteriorated at a mean follow-up (FU) of 17.78 ± 19.75 (2-84) months. Six patients were lost to FU. In 37 patients with a FU of at least three months, stability and bony union could be assessed. Thirty-one of them achieved a bony fusion/ stable construct. Conclusions: Contoured rod is especially useful for PF in cases of congenital AAD with coexisting CIM, cervical scoliosis, sub-axial instability and/or asymmetrical facet joints. In acquired pathologies with three-column instability, inclusion of joints one level above the affected one by using CR, especially enhances stability.

Background and Aims: To translate and test the reliability and validity of the Multiple Sclerosis Quality of Life Questionnaire (MSQoL-54) in Iranian MS patients. Setting and Design: Using a standard "forward-backward" translation, cognitive debriefing and cultural adaptation procedure, the English version of the MSQoL-54 was translated to Persian which is the Iranian official language. Materials and Methods: The subjects were multiple sclerosis (MS) patients referred to Motaharri clinic, Shiraz, South of Iran. Demographic data were recorded. Epidemiological data concerning MS type, duration of the disease, Functional System Score (FSS) and Expanded Disability Status Scale (EDSS) of patients were also provided by a qualified neurologist. Statistical Analysis: The reliability of the questionnaire was assessed by Cronbach's alpha coefficient. Construct validity was assessed through factor analysis. Factor analysis was performed to determine that the Persian version is a two-dimensional measure including physical and mental parameters. Results: Multiple sclerosis patients (female:106 (75.2%), male:35 (24.8%)), with a mean±SD age of 32.2±9.8 years were enrolled in the study. Cronbach's α was 0.962. There were no significant differences between each item and the mean of physical and mental scores of MSQoL-54, regarding sex, marital status and education. There was a negative significant correlation between EDSS and physical health, role limitation due to physical problems, pain, energy, health perception, social function, cognitive function, health distress, overall Quality of Life. The scaling success rates were 100%, demonstrating convergent validity of each scale. Factor analysis confirmed the construct validity of the questionnaire. Conclusions: The Persian version of the MSQoL-54 questionnaire has a good structural characteristic, it is a reliable and valid instrument and can be used for measuring the effect of MS on the Quality of Life.

Context: Awake craniotomy is increasingly performed the world over. We share our experience of performing craniotomy awake with our anesthetic protocol. Aims: To evaluate and analyze the anesthesia records of the patients who underwent awake craniotomy at our institution. Settings and Design: University teaching hospital, Retrospective study. Materials and Methods: We reviewed records of the 42 consecutive patients who underwent awake craniotomy under conscious sedation using Fentanyl and Propofol infusion until December 2005. The drugs were titrated (Bispectral monitoring was used in 16 patients) to facilitate intermittent intraoperative neurological testing. All patients received scalp blocks with a mixture of bupivacaine and lignocaine with adrenaline. Haloperidol and ondansetron were administered in all patients at induction of anesthesia. Results: All patients completed the procedure. One patient each needed endotracheal intubation and LMA for airway control during closure, while another required CPAP perioperatively because of desaturation to <80%. There was significantly decreased use of anesthetics ( P <0.001) and a trend towards reduction in complications (e.g. respiratory depression and deep sedation) ( P >0.05) with the use of BIS as compared to without BIS. Intraoperative complications were hypertension (19%), tight brain (14.2%), focal seizure (9.5%) respiratory depression (7.1%), deep sedation (7.1%), tachycardia (7.1%) and bradycardia. Two patients desaturated to <95%. 23.8% patients developed transient neurological deficits. The most frequent postoperative complications were PONV (19%) and seizures (16.6%). Conclusions: With the use of advanced monitoring and newer anesthetics, awake craniotomy is a relatively safe procedure with an accepted rate of complications.

Syndrome of spontaneous cerebrospinal fluid hypovolemia (SCH) is a rare cause of new onset headache. We report six cases of SCH presenting with new onset headache. All six cases were females. Acute onset orthostatic headache and neck pain were the chief characteristics of SCH in our cases. The MRI brain showed pachymeningeal gadolinium enhancement in all patients. Spinal extradural CSF collection was demonstrable on MRI in three cases. All cases improved with conservative therapy. High index of clinical suspicion and contrast enhanced MRI brain is the key to accurate diagnosis in the majority of cases.

Abnormal magnetic resonance spectroscopic (MRS) signals in the basal ganglia may be one of the characteristics in mitochondrial disease. We report MRS study in a family with myoclonic epilepsy with ragged-red fibers (MERRF). Their MRS studies over the basal ganglia revealed decreased N-acetylaspartate/creatine ratio and increased choline/creatine ratio in the four symptomatic members, but normal in the two asymptomatic members. However, negative MRI study was found in all members of this family. Our report suggests that the increased choline/creatine ratio in basal ganglia MRS may be one of the early information to suspect MERRF disease.

A 50-year-old female presented with complaints of progressive ataxia. Investigations showed a large intradural arachnoid cyst located anterior to the brainstem. Following marsupialization of the cyst she improved remarkably in her symptoms. The symptoms recurred nine months later and investigations revealed recurrence of the cyst. The cyst was evacuated again and two Teflon sponge sheets were placed such that they traversed the length of the cyst cavity and extended into the cisterna magna. At follow-up after 25 months, there has been no recurrence of symptoms or the cyst. The role and advantages of Teflon sponge in such cases is evaluated.

Worsening of ocular symptoms in cavernous sinus dural arteriovenous fistulae (CSDAVF) is rarely due to superior ophthalmic vein (SOV) thrombosis after palliative transarterial embolization and may resolve spontaneously. We present a unique case of a 38-year-old female whose ocular symptoms worsened three days after palliative embolization. Repeat angiography revealed incomplete thrombus of SOV and severe orbital venous congestion. Complete occlusion of the CSDAVF was achieved by urgent transvenous embolization through the SOV and the patient's ocular symptoms and visual impairment recovered. In such situations repeat angiogram may be needed in evaluating the subtle angiographic changes and curative embolization may be necessary.

Volatile substance abuse is the intentional inhalation of volatile solvents, aerosols, gases or nitrates for the purpose of intoxication. This practice is more common among young people, due, in part, to the low cost and ready availability of these inhalants. In this report, we present the case of a 22-year-old male with a seven-year history of chronic paint sniffing. The patient presented with vigorous postural and kinetic tremor in both hands. A neurological examination revealed a bilateral, non-fatiguing geotropic positional nystagmus and a mild ataxia together with dysdiadochokinesis. He also had a mild chronic encephalopathy. Following treatment with clonazepam, the tremors subsided, but were not completely controlled.

We report a case of a traumatic cavernous fistula supplied by a persistent primitive trigeminal artery. The process of treatment was unique in this case. Fistula was subcompletely occluded by coiling from primitive trigeminal artery. Residual fistula was helped to form thrombosis by compression of the carotid artery with hand in the procedure. Long-term follow-up was satisfactory. Traumatic cavernous fistula supplied by a persistent primitive trigeminal artery could be treated by embolization and temporal compression of the parent artery might be useful for residual minimal fistula.

A 37-year-old gentleman presented with macrocephaly since early childhood and progressive impairment of motor and cognitive functions. Magnetic resonance imaging revealed extensive white matter involvement and frontotemporal subcortical cysts. Absent ankle jerk and abnormal nerve conduction study raised a possibility of associated peripheral neuropathy. Sural nerve biopsy was suggestive of dysmyelinating neuropathy. This report serves to expand the clinical spectrum of this rare leukodystrophy.

This 61-year-old man presented with weakness and sensory disturbance in the legs. There was a spinal dural arteriovenous fistula (SDAVF) fed by the left sixth intercostal artery with dorsal perimedullary drainage. Surgical division of the perimedullary drainage led to rapid neurological improvement. However, on the second postoperative day he experienced transient deterioration of second neuron function in the left upper lumbar segment resulting in motor weakness of the proximal leg muscles, absence of the patellar deep tendon reflex and thigh pain. No radiological findings explaining this deterioration were obtained. He was treated conservatively and all segmental symptoms and signs subsided by the fifth postoperative day. Although the precise mechanisms underlying the dramatic but often reversible deterioration after radical SDAVF treatment remain to be determined, we postulate that this was attributable to postoperative segmental venous hemodynamic changes based on the neurological changes.

Chordoid glioma is a rare low-grade tumor located in the third ventricle-hypothalamic region. Since its first report, 37 cases have been described in the literature. We report on an additional case that we considered significant because of its incidental detection and its uneventful surgical removal.

The authors report an Indian adult female patient with a history of generalized tonic clonic seizures who developed severe features of phenytoin (DPH) toxicity on therapeutic dosage of this antiepileptic drug. Administration of 300mg/day of DPH in this patient resulted in toxic symptoms associated with an excessive serum DPH concentration of 33μg/ml. The PCR-RFLP analysis revealed a homozygosity involving CYP2C9*3*3. This mutation results in a marked decrease in the enzymatic activity (CYP2C9) and leads to a decreased clearance of the drug which can lead to severe acute and chronic toxicity. On switching the antiepileptic therapy from DPH to sodium valproate, there was reversal of both.

The co-existence of cerebral aneurysms and pituitary adenomas is rare. Here, we report a patient with a coexisting anterior communicating artery aneurysm and a pituitary adenoma and review the available literature concerning this phenomenon. There is a debate in the literature regarding any causal relationship between aneurysms and pituitary tumors, although there are many reports of aneurysms caused by trauma or radiation following treatment of pituitary tumors. These simultaneous lesions are best diagnosed with magnetic resonance imaging with magnetic resonance angiography. Craniotomy for simultaneous aneurysm clipping and resection of the pituitary tumor is the best treatment option.

Acute intermittent porphyria presenting with short duration of gastrointestinal symptoms followed by rapidly progressive fulminant neurological syndrome during first attack is relatively uncommon. It is a neurological emergency and mimics many other psychiatric and medical disorders and can be fatal if it remains undiagnosed and untreated. Further, specific treatment in the form of Heme arginate is not universally available and very costly, so high clinical suspicion and early diagnosis and management of acute attack and prevention of further attacks are very important. We report a series of six cases who presented with convulsion and/or polyneuropathy early in the course of disease to highlight this fact.