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Cervical spondylotic myelopathy (CSM) is uncommon at the C3-4 level. Fourteen patients with C3-4 CSM were treated over a period of 3 years. The radiological factors contributing to CSM at [...]

Accepted on 03.05.2001. Code Number: ni03019 A 21-year-old male presented with sudden onset of right-sided third nerve paresis. Angiogram showed a fenestrated posterior communication artery on the right side and [...]

Byline: S. Pandey, S. Jain, R. Chatterjee Sir, Lithium toxicity is known for its diverse clinical manifestations ranging from mild tremor to severe coma and death. We report two cases [...]

Byline: S. Jain, G. Swami, A. Arya, B. Chowdhry Sir, A 4-year-old boy presented with muscle and bone pain starting from both the lower limbs and later on affecting both [...]

Byline: A. Srivastava, M. Tripathi, S. Gaikwad, M. Padma, S. Jain The association of internuclear ophthalmoplegia (INO) with torsional nystagmus is rare. We report a case of a 72-year-old male [...]

Byline: S. Jain, D. Bhandarkar, R. Shah, U. Vengsarkar Intra-abdominal migration of the catheter and formation of a cerebrospinal fluid pseudocyst are both rare complications of a ventriculoperitoneal shunt. Traditionally, [...]

Background: There has been sparse description of cortical dysplasias (CDs) causing intractable epilepsy from India. Aim: Clinical retrospective study of CDs causing intractable epilepsy that underwent surgery. Materials and Methods: Fifty-seven cases of CDs reviewed (1995 till July 2006) are presented. All patients had intractable epilepsy, and underwent a complete epilepsy surgery workup (inter ictal electroencephalography (EEG), video EEG, MRI as per epilepsy protocol, SPECT {interictal, ictal with subtraction and co-registration when required}, and PET when necessary). Surgical treatment included a wide exposure of the pathology with a detailed electrocorticography under optimal anesthetic conditions. Mapping of the sensori-motor area was performed where indicated. Procedures included resection either alone or combined with multiple subpial transactions when extending into the eloquent areas. Results: Our study had 28 (49.12%) cases of isolated focal CDs, and 29 (50.67%) with dual pathology. Average age at the time of onset of seizures in our series was 7.04 years (three months to 24 years), and average age at the time of surgery was 10.97 years (eight months to 45 years). Among coexistent pathologies, one had associated MTS, 16 had coexistent gangliogliomas and 12 (dysembryonic neuroepithelial tumor) DNTs. At an average follow-up of 3.035 years (range 5-10 years), three patients were lost to follow-up. Fifty-one per cent (29/57) patients had a good outcome (Engel Grade I) and 26%(15/57) had a Grade II outcome. Conclusion: Cortical dysplasias have a good outcome if evaluated and managed with concordant electrical and imaging modalities. [ABSTRACT FROM AUTHOR]

Neuronal migration disorders are an important differential diagnosis to be considered in the evaluation of intractable epilepsy. Though the underlying causative factors which govern their development are many and varied, genetic factors have been found to be contributory in a few forms of these disorders. An X-linked association with lissencephaly has recently been discovered and there are a few families described till now with this entity. [ABSTRACT FROM AUTHOR]

Infantile central palsy (CP) is caused due to damage to the immature developing brain usually before birth, leading to altered topography and biochemical milieu. CP is a life-limiting disorder, which causes changes in sensory, motor, cognitive, and behavioral functioning. Understanding its pathophysiology is complex, and current therapeutic modalities, oral medication, surgical treatment, physical therapy, and rehabilitation provide minimal relief. As the brain is plastic, it has an inherent capacity to adapt to altered activity; thus, non-invasive brain stimulation (NIBS) strategies, like repetitive transcranial magnetic stimulation, which can modulate the neuronal activity and its function, may lead to recovery in CP patients. Further, in recent years, nanomedicine has shown a promising approach in pre-clinical studies for the treatment of central nervous system disorder because it can cross the blood-brain barrier, improve penetration, and provide sustained release of the drug. The review focuses on the principles and mechanisms of various NIBS techniques used in CP. We have also contemplated the effect of rehabilitation and nanomedicine in CP children, which will definitely lead to advancing our diagnostic as well as therapeutic abilities, in a vulnerable group of little ones., (Copyright © 2024 Copyright: © 2024 Neurology India, Neurological Society of India.)

Background: Huntington's Disease (HD) is an autosomal dominant, progressive neuropsychiatric illness caused by CAG repeat expansion. The high penetrance of the mutation and limited treatment options make it challenging for patients and caretakers. Proper counseling enables families to cope better and make informed life choices., Objective: To explore some complex issues in genetic counseling and testing (GCAT) in HD., Materials and Methods: Vignettes of patients who underwent genetic testing along with pre and post-test counseling at our GCAT clinic., Results: Case 1: Diagnosis of juvenile HD meant that the healthy parent was an obligate carrier of the mutation. Case 2: Consanguinity resulted in a dense prevalence of HD and >50% risk for the progeny. Case 3: Predictive testing in youth with healthy parents but affected uncles and aunts revealed a HD expansion., Conclusions: HD can present with complex inheritance patterns and proper counseling is necessary for better outcomes., Competing Interests: None

Background: Microvascular decompression (MVD) of the trigeminal nerve is a well-accepted nondestructive procedure for trigeminal neuralgia. Usually, Teflon (PTFE) puff or felt graft techniques, which are most commonly used, are associated with arachnoiditis and recurrence among other complications. We use the "sleeve graft" technique using PTFE to separate the neurovascular conflict and here we describe our experience with the same in 376 cases., Objectives: To study the outcomes in 376 patients treated with sleeve graft technique for trigeminal neuralgia., Materials and Methods: For a period of 18 years, from 2002 to 2020, all cases of medically refractory trigeminal neuralgia were subjected to the "sleeve graft" technique for MVD. Pre- and post-operatively, pain score was given according to Barrow Neurological Institute pain intensity score. Cases were observed for any complications and pain relief in short and long-term follow-up., Results: In total, 376 cases of refractory primary trigeminal neuralgia cases, among which 198 patients underwent MVD with no prior intervention, 158 underwent MVD following percutaneous ablative procedure, 13 were "Revision MVD" previously done at other centers, and four were post gamma knife failure. There was no incidence of arachnoiditis or recurrence of symptoms. Further, 368 (97.8%) patients had complete recovery from symptoms while eight (2.2%) had partial recovery after 5 years of follow-up. Complications included hearing loss (n = 1), temporary hypoesthesia (n = 45), and permanent hypoesthesia (n = 7)., Conclusion: "PTFE Sleeve Graft" technique to remove the neurovascular conflict in micro vascular decompression (MVD) for trigeminal neuralgia is a safe and effective technique that yields better results., Competing Interests: None

Background: An increased incidence of systemic macrothrombotic phenomena such as strokes has been observed in moderate and severe COVID. However, strokes have also been increasingly observed in mild COVID, post COVID, or without obvious COVID illness., Objective: To share our experience with a specific stroke type noted during the COVID pandemic period., Materials and Methods: A single-center observational study was conducted in Western India from January to December 2020, and data regarding stroke patients admitted under Neurology services were noted. Clinical, laboratory, and radiological characteristics of strokes and subtypes were documented., Results: A total of 238 stroke patients were admitted in 2020, 76.5% during the COVID pandemic period. Among 153 ischemic strokes, 16.3% and 56.2% had large vessel occlusion (LVO) in pre-COVID and COVID pandemic period, respectively. Of all ischemic strokes, 20.9% (18 patients) and 12% (3 patients) had free floating thrombus (FFT) in the COVID versus pre-COVID period, respectively. Only 44.4% of all FFT patients could be proven SARS-CoV-2 RT-PCR positive while 50% were COVID suspect with surrogate markers of heightened inflammation at time of stroke. All patients were given anticoagulation and average mRS at discharge was 3.1 (range: 1-6) and 1.84 (range: 0-4) at 3-month follow-up in survivors., Conclusions: This study highlights the presence of FFT causing LVO as a new stroke subtype during the COVID-19 pandemic. With renewed and steeper spike in COVID-19 cases, especially new variants, the resurgence of this stroke subtype needs to be actively explored early in the course of illness to reduce morbidity and mortality., Competing Interests: None

Competing Interests: None

Hydrocephalus is a common clinical problem encountered in neurosurgical practice. With greater subspecialisation, pediatric neurosurgery has emerged as a special discipline in several countries. However, in the developing world, which inhabits a large pediatric population, a limited number of neurosurgeons manage all types of hydrocephalus across all ages. There are some essential differences in pediatric and adult hydrocephalus. The spectrum of hydrocephalus of dysgenetic origin in a neonate and that of normal pressure hydrocephalus of the old age has a completely different strategy of management. Endoscopic third ventriculostomy outcomes are known to be closely associated with age at presentation and surgery. Efficacy of alternative pathways of CSF absorption also differs according to age. Managing this disease in various age groups is challenging because of these differences in etiopathology, tempo of the disease, modalities of investigations and various treatment protocols as well as prognosis., Competing Interests: None

Introduction: Autologous Stem Cell Transplant (ASCT) provides long periods of progression-free-survival in multiple sclerosis (MS). This is an observational study to demonstrate the safety of ASCT in MS patients at a transplant center in North India using a lymphoablative regimen., Materials and Methods: MS patients > 18 years referred by a neurologist or who came of their own volition were evaluated. Kurtzke Expanded Disability Status Scale (EDSS) score was calculated and those with a score of >7 were excluded. Informed written consent was taken. Mobilization was done with G-CSF with prednisolone to prevent disease flare-up. A minimum of 2 × 10 6 CD34 cells/kg was collected. Conditioning regimen consisted of rabbit ATG and cyclophosphamide. Rituximab 375 mg/m 2 was given to prevent EBV reactivation and disease relapse. Antibiotic prophylaxis was given with levofloxacin, fluconazole, and valacyclovir. Any persistent change in EDSS scores ≥0.5 was considered significant., Results: Twenty patients were included. Seven patients had positive urine cultures prior to transplant and were treated before starting any chemotherapy. Majority patients were women (13/20). All patients developed febrile neutropenia, which was managed as per department policy. There was no mortality. Subjective symptoms improved in all patients. EDSS score improved in 6/19 patients (5/6 with RRMS) with no disease progression in any patient at a median follow-up duration of 242 days., Conclusion: ASCT can be done safely for patients with relatively high EDSS scores with additional precautions for screening for infections. RRMS patients with the active disease show most improvement. SPMS patients may not show significant improvement in the short term., Competing Interests: None

Background: Hemifacial spasm (HFS) is a hyperkinetic movement disorder of adults. The condition is usually primary, but may be secondary to facial nerve damage, and responds well to botulinum toxin therapy., Aim: To study the demographic and clinical features of primary and secondary HFS and assess its response to botulinum toxin therapy., Materials and Methods: A retrospective study of 40 patients of primary (33) and secondary (7) HFS, who attended our botulinum toxin clinic over the last 18 months, was conducted. We collected the data including the demographic profile, clinical features of primary and secondary HFS, severity measured by Likert scale, etiology, details of magnetic resonance imaging (MRI) of the brain, and a detailed account of the botulinum toxin injection., Results: Thirty-three out of the 40 patients had primary HFS. The mean age at presentation was 48.4 ± 12.6 years whereas the mean duration of symptoms was 57.4 ± 39.3 (6-144) months. The onset of spasm on the upper part of face was seen in 31 patients (77.5%). An ipsilateral ear click was found in 17 (42.5%) patients. Mild spasms were seen in 18 patients, whereas 12 patients had severe spasms. The severity of spasm was lower in patients with secondary HFS. Five out of 22 magnetic resonance imaging (MRI) results showed an abnormality. The mean dosage of botulinum toxin was 21.86 ± 4.19 units in primary spasm and 17 ± 4.16 units in secondary spasm (P =0.018). The mean duration of response was 112.6 ± 55.7 days (median: 90 days; range: 30-240 days) in primary spasm and 135 ± 63.6 days (median: 135 days; range: 90-180 days) in secondary spasm (P = 0.29)., Conclusion: Ipsilateral auditory clicks are fairly common in primary and secondary HFS. The patients with secondary HFS have a milder intensity of disease and they may have the onset of spasm from the upper part of face. Both primary as well as secondary HFS patients respond well to botulinum toxin injection; however, the adverse effects are more likely to occur in secondary HFS, and therefore, these patients require lower dosages of toxin., Competing Interests: There are no conflicts of interest

An early description of paralysis confined to an upper limb can be found in the casebook of Dr. Charles I Smith, who was a doctor in Bangalore in the 19th century. The historical and clinical aspects, as well as the current importance of this entity are described briefly.

Aims: Intracranial aneurysms (IAs) express a variety of differentially expressed genes when compared to the normal artery. The aim of this study was to evaluate the expression level of a few genes in the aneurysm wall and to correlate them with various clinicoradiological factors., Materials and Methods: The mRNA level of collagen 1A2 (COL1A2), tissue inhibitor of metalloproteinase 4 (TIMP4), and cathepsin B (CTSB) genes were studied in 23 aneurysmal walls and 19 superficial temporal arteries harvested from 23 patients undergoing clipping of IAs, by real-time polymerase chain reaction method., Results: The mean fold change of COL1A2 gene between the aneurysm sample and the superficial temporal artery (STA) sample was 2.46 ± 0.12, that of TIMP4 gene was 0.31 ± 0, and that of CTSB gene was 31.47 ± 39.01. There was a positive correlation of TIMP4 expression level with maximum diameter of aneurysm (P = 0.008) and fundus of aneurysm (P = 0.012). The mean fold change of CTSB of patients who had preoperative hydrocephalus in the computed tomogram (CT) scan of the head at admission was 56.16 and that of the patients who did not have hydrocephalus was 13.51 (P = 0.008). The mean fold change of CTSB of patients who developed fresh postoperative deficits or worsening of the preexisting deficits was 23.64 and that of the patients who did not develop was 42.22 (P = 0.039)., Conclusions: COL1A2 gene and CTSB genes were overexpressed, and TIMP4 gene was underexpressed in the aneurysmal sac compared to STA and their expression levels were associated with a few clinicoradiological factors.

Background: Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy that affects young boys and the dystrophin gene on the X chromosome has been found to be associated with the disorder., Materials and Methods: In this prospective study, 112 clinically diagnosed DMD patients had muscle biopsy and were tested for exon deletions. Genotyping was also carried out at STR44, STR45, STR49 and STR 50 markers in 15 families., Results: Of the 112 clinically suspected DMD patients, the diagnosis of DMD was confirmed by histopathology and/or genetics in 101 patients. The mean age of onset was 3.1+/-1.44 years (1-6 years) and the mean age at presentation was 8.0+/-3.1 years (1.1-18.0 years). Delayed motor milestones were present in 63 (62.3%) patients. The mean creatine kinase value was 11822.64+/-8206.90 U/L (1240-57,700). Eighty-four patients had muscle biopsy and immunohistochemistry was done in 60 muscle samples, all of which demonstrated absence of dystrophin staining. Of the 60 dystrophin-negative cases, 73% showed deletion of at least one exon. Single exon deletion was found in 20.4%. Distal hotspot Exons 45, 47, 49 and 50 were the commonly deleted xenons and the deletion rates were 36%, 35%, 33.7% and 38.5% respectively., Conclusions: In this study population in south India the deletion rate was 73% and were more frequent in the distal end exon. With the availability of genetic analysis, the first investigation of choice in DMD should be genetic studies and muscle biopsy should be considered only if the genetic tests are negative or not available.

Objective: Genetic counseling for individuals undergoing presymptomatic testing is lacking in India although testing is easily available. This has an impact on family members of Huntington's disease (HD), an autosomal dominant disease, wherein the age at onset of symptoms varies., Aim: We examine if attitudes differ towards presymptomatic testing for HD amongst HD family members, physicians and laypersons., Materials and Methods: A modified questionnaire enquiring about opinions on various personal, family, social and future health care with regards to presymptomatic testing of HD was designed. A physician explained briefly about HD and presymptomatic testing of HD and recorded responses of unaffected family members of HD (n=25) and laypersons (n=50). Medical doctors (n=50) answered the questionnaire based on their knowledge of HD., Results: HD family members, Medical doctors and laypersons were similar in their opinion to undergo the testing. Majority (60%) of HD family members did not wish to communicate test results with their friends when compared to the other two groups. Medical doctors and HD family members were more concerned about certainty of developing disease when the test results are positive. Majority (80%) of Medical doctors and less than half in the other groups felt that their decision to have a child would strongly depend on test results. Large proportion (80%) of HD family members did not wish to report their test results to their employers., Conclusions: Individuals with knowledge about HD and the test differ in their decision of sharing test results and reproductive choices.

Background: Surgical management of intractable epilepsies is currently an established mode of therapy in various clinical settings., Aims: To retrospectively evaluate the neuropathological findings in both temporal and extratemporal lobe resections in such patients., Materials and Methods: The study included resected specimens from patients with intractable epilepsy managed at a tertiary care hospital of India, during a 10-year period (1995-2004)., Results: A total of 153 patients, with mean age of 19.4 years and male predominance (73.2%) were included in the study. Overall, there was a predilection for the temporal lobe (73.2%), while 41cases were extratemporal in location. On histopathology, mesial temporal sclerosis (MTS) (24.8%) was the commonest lesion, followed by tumors (19.6%) and isolated focal cortical dysplasia (FCD - 15.11%). Other less common findings included Rasmussen encephalitis, non-specific gliosis and vascular malformations. In addition, 20.9% (32 cases) had dual lesions, majority of which included FCD with ganglioglioma (15 cases) or with dysembryoplastic neuroepithelial tumor (12 cases). In the temporal lobe, neoplasms and dual lesions formed the majority (apart from MTS), unlike dual lesions followed by neoplasms and FCD, in the extratemporal location., Conclusion: This series demonstrates that most patients with chronic intractable epilepsy have significant histopathological findings and highlights the neuropathological spectrum of such lesions, in the Indian context. This was similar to that reported from the West, but different from the single Indian series available in the literature. Further, the overall profile of temporal lobe lesions was not different from the extratemporal ones.

Background: Rasmussen encephalitis (RE) is a chronic epileptic disorder of unknown etiology, and is clinically characterized by progressive neurological deterioration, focal seizures often progressing to intractable epilepsy, cognitive decline and hemispheric atrophy., Aims: We report the spectrum of neuropathological changes seen in RE, and discuss the evolutionary mechanisms of this disease., Materials and Methods: Surgically obtained specimens from RE patients diagnosed during 2002-2004 at this institute were evaluated for the presence and extent of different histopathological features of RE. The H&E and immunohistochemistry stained slides were also evaluated for the type and distribution pattern of inflammatory infiltrates, along with a semiquantitative evaluation for the severity of inflammation., Results: Four cases of RE were admitted during the study period, all of which presented with partial seizures with successive deterioration to intractable epilepsy. The age at onset varied between 5 and 10 years (mean 7.8 years), with three male and one female patients. Subsequently, all four patients underwent hemispherotomy. Histopathological features of perivascular lymphocytic infiltrate, neuronal loss, microglial nodules, and reactive astrocytosis, with or without evidence of neuronophagia confirmed a diagnosis of RE. These cases also had varying degrees of T-cell-rich (CD3-positive) inflammatory infiltrates and CD68-immunopositive microglial proliferation. It was observed that the severity of inflammation had a trend to inversely correlate with the duration of symptoms., Conclusion: It is proposed that an accurate evaluation and histopathological grading of these lesions may possibly have a role in patient prognostication.

Introduction: Obstructive sleep apnoea (OSA), is characterized by loud snoring and excessive daytime sleepiness. Though the gold standard for diagnosis is overnight polysomnography (PSG), sleep questionnaires have also been used to diagnose this with good predictive value., Material and Methods: A pre-designed proforma with clinical details, symptom-specific questions for diagnosis of OSA, and Epworth Sleepiness Scale (ESS) was administered to 20 patients presenting to the Sleep Disorder Clinic of our hospital and to 40 age and sex-matched relatives (control group). The students 't-test' and chi-square were used as the statistical tests., Results: There were 20 patients with a mean age of 41+/- 8 years, and 40 controls with a mean age of 41 +/- 6 years (P=>0.05). Seven had family history of snoring in the study group and 3 in the control group (P=0.02). Four had met with road traffic accidents in the study group and none in the control group (P=0.001). The body mass index (BMI) was 29.9 (SD 4.4) in the study group and 24.5 (SD3.5) in the controls (P=0.001). The mean ESS was 13.3 +/- 6 in the patients and 4.2+ 4 in the controls (P=0.001). A larger number of patients with OSA had hypertension: 5/20 vs. 3/40 (P=0.01)., Conclusion: Patients with OSA had significantly higher BMI and ESS score, and were more likely to have hypertension and road traffic accidents. Increased awareness of this entity is essential.

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

Identification of the epileptic focus is the most important requirement for a successful surgical outcome in intractable epilepsy. Patients with normal MRI on high-resolution imaging pose a significant challenge in this regard. We tried to identify the epileptic focus using interictal / ictal SPECT and ictal EEG patterns in 14 patients with normal MRI. Rhythmic ictal onset activity helped to identify a single focus in 10 patients (71.43%). Inter ictal SPECT detected a focus corresponding to ictal EEG in 6 out of 10 patients (60%) in whom the test was performed. Ictal SPECT done in 5 patients correctly identified the focus in all patients (100%). Our data indicate that it is possible to obtain localization in patients with normal MRI using a combination of ictal EEG patterns and an ictal SPECT study.

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.

The concept of primitive neuroectodermal tumors (PNETs) has been evolving for many years, as has been its nomenclature. A 5 year old boy presented with pain in lower cervicodorsal region and left leg. Preoperative MRI of the spine and paravertebral region revealed a hyperintense lobulated lesion extending from D1-D4 with a large intraspinal and thoracic component. A total removal of tumor was achieved via a dorsal laminectomy and right posterolateral thoracotomy. The pathological findings were consistent with PNET. Post operative neurological examination had been unremarkable. Six months follow up scan showed no recurrence. A review of the literature shows that only 18 cases of primary intraspinal PNETs have been reported to date and the present case is exclusive, in which the tumor was thoracic, extradural in location and the child is alive at 8 months of follow up, with no evidence of tumor recurrence/metastasis. Primary intraspinal PNETs are rare tumors and carry a poor prognosis. Newer modalities of treatment should be tried to improve survival.

Several clinical signs have been described for lateralization and localization of seizure focus in complex partial seizures; however, the specificity of each of these has been widely debated upon. The present study was done to evaluate 'dystonic unilateral limb posturing' and 'versive head movements' for lateralization and localization of epileptic foci in patients with intractable partial complex seizures, being investigated with long term Video-EEG monitoring. Fifteen patients with 46 seizures, studied with long term Video EEG, had either one or both of these signs. The video recordings of the clinical behaviour were noted and later compared with the corresponding EEG. Unilateral dystonic limb posturing and versive head movements had good lateralising value in complex partial seizures, suggestive of temporal lobe origin, contralateral to the seizure focus, with a specificity of 87.5% and 86% respectively. In addition, turning of the whole body to one side was associated with a contralateral seizure focus in 100% cases. Appendicular automatisms were found to be of no lateralising significance, as they occurred on either side with equal frequency. These motor phenomena should, therefore, be enquired about in detail, during history taking for lateralization or localization of seizure focus.

The present study attempts to find a correlation between low serum B-12 levels and dementias. A total of 178 patients of dementia were seen at the cognitive disorder clinic from 1996 to 1998. Serum B-12 levels were studied in 100 patients. 15 patients (39.5%) of Alzheimer's dementia had reduced B-12 levels, while only 5 patients (13.9%) with vascular dementias and 3 patients (11.5%) with other types of dementias had reduced levels. The incidence of low serum B-12 was statistically significant in the Alzheimer's group when compared with the other groups, individually as well as combined together (p<0.05). The aetiopathogenesis and significance of these findings is discussed and the literature is reviewed.

A 46 year old man with trigeminal schwannoma displayed symptoms of ataxia with pathological laughter and crying. The tumour developed in the cerebellopontine angle, compressing the pontomesencephalic structures backward, extending in the posterior parasellar region and Meckel's cave. No recurrence of laughter and crying attacks were noted after total removal of the tumour. Theories of mechanism of pathological laughter and crying reported in the literature are reviewed.

A prospective study was done to evaluate the role of short-term Video EEG (VEEG) recording in assessing the nature of clinical behaviour and in classification of seizures in children with frequent intractable seizure episodes. Forty five children upto 12 years of age with frequent intractable seizure episodes (> or =3/week) were included in the study. VEEG was done on an outpatient basis until an event was recorded or for a minimum period of 6 hours. The events were detected in 78% of cases. The seizures were classified in all children with recorded events and seizure classification was changed in 22%. Anti-epileptic drugs could be stopped or reduced in 11%. Short term VEEG monitoring was useful in characterising events and in classification of seizures in children with frequent, intractable seizure episodes. Use of this investigation as a screening procedure for diagnosis in children with frequent episodes is stressed, thereby reducing the cost associated with prolonged VEEG recordings and disability associated with misdiagnosis of epilepsy.

Forty adult patients (age> 14 years) of generalised convulsive status epilepticus were studied prospectively. All the patients were subjected to detailed history, neurological examination and baseline investigations including haemogram and metabolicprofile. CT scan was carried out in 26 patients. All the patients were treated with intravenous diazepam and phenytoin. The age range of the patients was 14-71 years with a mean of 32.75 ? 15.78. There were 24 males and 16 females. Twenty six patients had generalised tonic clonic seizures while 14 had partial seizures with secondary generalisation. Symptomatic epilepsy was the commonest type of epilepsy and was seen in 25 patients. The diagnosis of cryptogenic and idiopathic epilepsies was made in 11 and 4 patients respectively. Drug withdrawl precipitated status epilepticus in 5 patients and systemic infection in one. The mean frequency of seizures was 4.70??1.83/hour, mean duration was 1.78???l.0l/minutes and cumulative convulsive time 25.27??21.50 minutes. Twelve patients had focal neurologic deficits. Papilloedema was seen in four cases only. CT scan was abnormal in 18 of 26 patients. All patients except one showed immediate response to therapy. Seven patients had recurrence of seizures after initial response to diazepam and phenytoin. Low serum level of the drug at 12 hours had a significant correlation with recurrence of seizures. Ataxia was the most common side effect of phenytoin therapy. Serious side effects e.g. hypotension and respiratory depression were seen in 4 and 2 patients respectively. Two patients expired during 48 hours follow up.

This multicentre, randomized, double-blind, cross over, placebo controlled study evaluated the efficacy and tolerability of oral Sumatriptan (100 mg) in 100 migraineurs. 59 patients completed the study. The results indicate that by 4 hours post-dose62 of patients treated with Sumatriptan achieved relief of headache, compared with 10 of patients treated with placebo. The results show that oral Sumatriptan is an effective drug for treatment of acute migraine in Indian patients, though smaller dosage may be more beneficial.

A case of facioscapulohumeral dystrophy (FSH) with several unusual feature is being presented. It's early onset in infancy as against commonly occurring onset in second decade, the relentless progressive course without abortive or apparent arrest phase as is often seen, the calf hypertrophy, marked skeletal changes and associated retinal changes seen in Coat's disease are the noteworthy features of this unusual case.

A female patient with Duchenne muscular dystrophy (DMD) with no prior family history of DMD is described. She presented with proximal muscle weakness, enlarged calf muscles and an elevated serum creatine kinase (CK). Histological examination of skeletal muscle revealed myopathic changes and immunoperoxidase examination for dystrophin in muscle biopsy demonstrated a mosaic pattern. Immunostaining of a muscle biopsy with anti-dystrophic serum proved to be valuable in the diagnosis of DMD in a symptomatic female carrier.

A manifesting female carrier of Duchenne muscular dystrophy (DMD) with no prior family history of DMD is described. She presented with proximal muscle weakness, enlarged calf muscles and an elevated serum creatine phosphokinase (CPK). Histological myopathic changes and immunoperoxidase examination for dystrophy in muscle biopsy with antidystrophin serum proved to be valuable in the diagnosis of DMD in a manifesting female carrier.