Sangeeta Ravat

Childhood absence epilepsy with tonic-clonic seizures and electroencephalogram 3-4-Hz spike and multispike-slow wave complexes: linkage to chromosome 8q24.

Childhood absence epilepsy (CAE), a common form of idiopathic generalized epilepsy, accounts for 5%-15% of childhood epilepsies. To map the chromosomal locus of persisting CAE, we studied the clinical and electroencephalographic traits of 78 members of a five-generation family from Bombay, India. The model-free affected-pedigree member method was used during initial screening with chromosome 6p, 8q, and 1p microsatellites, and only individuals with absence seizures and/or electroencephalogram 3-4-Hz spike- and multispike-slow wave complexes were considered to be affected. Significant P values of .00000-.02 for several markers on 8q were obtained. Two-point linkage analysis, assuming autosomal dominant inheritance with 50% penetrance, yielded a maximum LOD score (Zmax) of 3.6 for D8S502. No other locus in the genome achieved a significant Zmax. For five smaller multiplex families, summed Zmax was 2.4 for D8S537 and 1.7 for D8S1761. Haplotypes composed of the same 8q24 microsatellites segregated with affected members of the large family from India and with all five smaller families. Recombinations positioned the CAE gene in a 3.2-cM interval.

Is psychiatric assessment essential for better epilepsy surgery outcomes

Epilepsy surgery is one of the most accepted and beneficial treatment for resistant epilepsies. However there is some variability in the comprehensive epilepsy care programs offered globally. Many centers do not do a psychiatric assessment unless required. It is now evident from a large body of research that epilepsy is associated with psychiatric morbidity which is also seen in patients considered for epilepsy surgery. There is also evidence to state that the risk for worsening or de novo psychiatric disorders is often seen post surgery. This calls for a comprehensive psychiatric assessment of all patients enrolled for the epilepsy surgery program to be evaluated pre and post surgically to minimize the risk of post surgical psychological disturbances and/or poor quality of life. Efficacious treatment of psychiatric disorders in those having psychiatric morbidity contributes to improved patient wellbeing, seizure freedom and better quality of life. Hence there is a need for most centers globally to include regular psychiatric assessment of epilepsy surgery patients as a protocol.

The effect of phenobarbitone on cognition in adult patients with new onset epilepsy: A multi-centric prospective study from India

OBJECTIVE In view of the conflicting results of cognitive and behavioral consequences of PB, the present study was planned to analyze its efficacy, serial neuropsychological functions and its impact on psychosocial functioning in adults with epilepsy while on phenobarbitone (PB). METHODOLOGY This prospective multi-centric study carried out across 4 centers in India included 75 adult patients of ≥18 years (M:F=52:23; age: 27.3 ± 8.5 years) with epilepsy who were prescribed phenobarbitone and underwent serial standardized neuropsychological assessment (NIMHANS battery for adults) at baseline, 1 month, 3 months, 6 months and 12 months. The demographic, seizure details and outcome measures were recorded. RESULTS Of the 75 patients, 63 had completed clinical and neuropsychological assessment, i.e. visit 1 (baseline), visit 4 (6 months) and visit 5 (12 months). There was no deterioration rather an improvement during the follow visits in all the neuropsychological functions. The results indicate that 16 neuropsychological variables changed significantly, viz. mental speed (p<0.001), sustained attention (p<0.001), focused attention (p<0.002), planning (p<0.001), concept formation (p<0.05), set shifting (p<0.001), verbal learning (p<0.0001), verbal memory (p<0.0001), visual memory (p<0.0001) and intelligence (p<0.001). The scales measuring the outcome of psychosocial functioning significantly changed during follow up included happiness (p<0.002), Impact of Epilepsy on patients life (p<0.02), A-B Neuropsychological Assessment (p<0.015), HADS anxiety (p<0.001) and emotional disorder (p<0.006). There was a significant reduction in seizure severity as measured by Liverpool Seizure Severity Scale (p<0.002) and seizure freedom was maintained. CONCLUSIONS This study demonstrated that phenobarbitone is effective, well tolerated AED and do not have cognitive impairment over one year. There was variable but distinct improvement in cognition and psychosocial functioning, and effective seizure control could be one of the factor for it.

Subacute sclerosing panencephalitis presenting as schizophreniform disorder

Subacute sclerosing panencephalitis (SSPE) is a slow progressive and mostly fatal neurological disorder caused by the measles virus typically affecting children and young adults. SSPE is characterised by progressive mental deterioration, recurrent myoclonic jerks and distinctive periodic complexes on the electroencephalogram (EEG). Diagnostic laboratory findings include immunoglobulin G (IgG) measles antibodies in the cerebrospinal fluid (CSF) (1). In developed countries where there is high population coverage for the measles vaccination, very low rates of SSPE have been reported. However, in developing countries such as India or Papua New Guinea where the vaccination coverage is inadequate, there is a high prevalence of SSPE (2,3). Behavioural abnormalities are the common early clinical presentations of patients with SSPE before the distinctive neurological features emerge. Sometimes, adolescents may present to psychiatrists with overt psychotic features. The case described below is such a presentation. A 15 year-old tenth-grade student was brought by his parents to a board-certified psychiatrist with paranoid and selfreferential delusions of several months duration. There was no family history of psychosis or any precipitating life events prior to the onset of psychotic symptoms. Based on a history and mental status examination, he was diagnosed with firstepisode schizophreniform disorder (International Classification of Diseases (ICD) F20.8). It was unclear whether this patient was tested for known causes of psychotic symptomsat this age, suchas illicit druguse, or autoimmune disorders such as central nervous system lupus, Huntington’s disorder or Wilson’s disease etc. Nonetheless, he was treated with a first-generation antipsychotic medication, the duration of which was not clear. He then started to experience recurrent falls for which he was referred to the Department of Neurology. At this point, positive psychotic symptomswere no longer evident,butwhatwas evident instead was apathy andmostly negative symptoms. In reviewing his history, it was noted that he had suffered from measles exanthemata at 8 months of age and that he had not received the measles vaccine. On neurological examination, he had bilateral upper and lower extremity cogwheel rigidity and axial myoclonic jerks with delayed relaxation. It was surmised that these axial myoclonic jerks were the cause of the patient’s falls. A tentative diagnosis of SSPE, stage 2, wasmade based on a history of measles in infancy, altered behaviour, recurrent falls, myoclonic jerks and extra pyramidal involvement (4). On investigation, the patient had the following findings: the EEG showed distinctive Raeder-Mecker complexes (Fig. 1) consisting of periodic high-amplitude slowwave complexes, which were synchronous with themyoclonic jerks. The CSF analysis was positive for IgG measles antibodies with a titre of 3.65 relative fluorescence values (RFV) units. These findings confirmed a diagnosis of SSPE, and the antipsychotic medicationwas stopped. The parents of the patient were advised about the option of starting isoprinosine and intraventricular alpha-interferon treatment, but they were not able afford this treatment. He received sodium valproate for the myoclonic jerks. Following discharge from the hospital, the patient could not be contacted for follow-up. SSPE has been reported from all parts of the world, but in the developed countries, it is considered a rare disease with lesser than 10 cases per year reported in the United States (5). However, in developing countries like India, SSPE continues to be highly prevalent with rates as high as 21 per million population (6). The most effective way to reduce the incidence of SSPE is to reduce the cases of measles in the community. Since the introduction and broad use of themeasles vaccine, there has been a decrease in the incidence of measles and consequently of SSPE inmost of the developed world (7). The World Health Organisation indicates that if 95% of the susceptible population is immunised, then it is possible to eliminate measles in the community (8). In countries such as the United States, Measles, Mumps and Rubella vaccine coverage has remained consistent, ranging from 91 to 93% since 2001 (9). However, in developing countries like India, the coverage of measles vaccine is still around 65% as per a survey performed in 2001 (10). The behavioural symptoms of SSPE usually start in the teenage years, and this coincides with the first-episode presentations of either a schizophreniform disorder or a manic episode associated with bipolar disorder. As behavioural abnormalities are a common first presentation of SSPE, psychiatrists working in developing countries should consider SSPE in the differential diagnosis of first episodes of psychoses. There have been case reports of SSPE patients presenting with psychotic features. Jahnel reported a case of 19 year-old man who initially presented with hallucinations and negative symptoms and was diagnosed with schizophrenia. Later, when the full-blown neurological symptoms set in, the patient was diagnosed to have SSPE. The author noted that there were only four case reports in the literature about patients with SSPE presenting with psychosis (11). In reviewing the details of the six previously reported patients in the literature, one striking feature common in all the patients was the absence of a positive family history of psychosis. Three patients were diagnosed with childhood-onset schizophrenia and two were diagnosed with schizophreniform disorder. Delusions, especially of paranoia and persecution, were noted in four patients. Speech

Ictal asystole: A rare cardiac manifestation of temporal lobe epilepsy, treated with epilepsy surgery

Seizures are associated with fascinatingly varied cardiac and autonomic manifestations, of which ictal tachycardia is common, and asystole and bradycardia are rare. Ictal asystole (IA), an often unsought autonomic phenomenon, occurs most commonly with temporal followed by frontal lobe seizures. Prolonged IA may lead to cerebral anoxic ischemia. As the mysteries of sudden unexplained death in epilepsy are unraveled, it is quite possible that the key to it lays within these seizure-induced cardiac rhythm abnormalities. We present a case of a young male with temporal lobe epilepsy due to left mesial temporal sclerosis with prolonged IA, which was successfully managed with epilepsy surgery.

Memory outcomes in mesial temporal lobe epilepsy surgery

INTRODUCTION Decline in verbal memory after dominant mesial temporal lobe surgery is a concern. Outcomes primarily reported by group data analysis do not address issues of practice effects and measurement errors and also do not provide information about individual meaningful change after surgery. Reliable Change Indices (RCIs) are regarded to be robust statistical methods for reporting individual change and have not been hitherto derived in patient populations in India. AIM Report memory outcomes for patients after surgery using group data as well as RCI score analyses using RCI scores derived in a control patient population. METHOD Retrospective data analysis of 106 selected patients who underwent Anterior Temporal Lobectomy (ATL) surgery. RCI scores derived from a control group of 44 non-operated patients. Outcomes based on score shifts on the various measures of two verbal and visual memory tests. RESULTS Group mean score analysis revealed no significant shifts in verbal or visual memory scores after left ATL, but significant improvements in verbal memory after right ATL. RCI score analysis revealed decline and improvements in a small percentage of patients for both left and right ATL groups. Percentage of patients showing decline was much less than reported in western literature although percentage improved was comparable. DISCUSSION Differences in decline percentage may be due to RCI scores and clinical characteristics of our sample (impaired pre-operative functioning, majority seizure free post surgery, moderate hippocampal sclerosis, early onset, long duration of seizures). CONCLUSION Group analyses mask individual change. Therefore, to report memory outcomes and counsel patients about relative risk-benefits of surgery, RCI scores derived from our patient populations should be used.