Awareness of social care needs in people with epilepsy and intellectual disability.

BACKGROUND: Nearly a quarter of people with intellectual disability (ID) have epilepsy with large numbers experiencing drug-resistant epilepsy, and premature mortality. To mitigate epilepsy risks the environment and social care needs, particularly in professional care settings, need to be met. PURPOSE: To compare professional care groups as regards their subjective confidence and perceived responsibility when managing the need of people with ID and epilepsy. METHOD: A multi-agency expert panel developed a questionnaire with embedded case vignettes with quantitative and qualitative elements to understand training and confidence in the health and social determinants of people with ID and epilepsy. The cross-sectional survey was disseminated amongst health and social care professionals working with people with ID in the UK using an exponential non-discriminative snow-balling methodology. Group comparisons were undertaken using suitable statistical tests including Fisher's exact, Kruskal-Wallis, and Mann-Whitney. Bonferroni correction was applied to significant (p < 0.05) results. Content analysis was conducted and relevant categories and themes were identified. RESULTS: Social and health professionals (n = 54) rated their confidence to manage the needs of people with ID and epilepsy equally. Health professionals showed better awareness (p < 0.001) of the findings/recommendations of the latest evidence on premature deaths and identifying and managing epilepsy-related risks, including the relevance of nocturnal monitoring. The content analysis highlighted the need for clearer roles, improved care pathways, better epilepsy-specific knowledge, increased resources, and better multi-disciplinary work. CONCLUSIONS: A gap exists between health and social care professionals in awareness of epilepsy needs for people with ID, requiring essential training and national pathways.

Tackling increased risks in older adults with intellectual disability and epilepsy: Data from a national multicentre cohort study.

PURPOSE: People with intellectual disabilities (ID) suffer multimorbidity, polypharmacy and excess mortality at a younger age than general population. Those with ID and epilepsy are at higher risk of worse clinical outcomes than their peers without epilepsy. In the ID population the health profile of those aged ≥40 years can be compared to those aged over 65 in the general population. To date there is limited data available to identify clinical characteristics and risk factors in older adults (≥40 years) with ID and epilepsy. METHODS: The Epilepsy in ID National Audit (Epi-IDNA) identified 904 patients with ID and epilepsy from 10 sites in England and Wales. This subsequent analysis of the Epi-IDNA cohort compared the 405 adults over 40 years with 499 adults ≥18 years aged under 40 years. Comparison was made between clinical characteristics and established risk factors using the Sudden Unexpected Death in Epilepsy (SUDEP) and Seizure Safety Checklist. RESULTS: The older adults' cohort had significantly higher levels of co-morbid physical health conditions, mental health conditions, anti-seizure medications (median 5), and antipsychotics compared to the younger cohort. The older group were significantly less likely to be diagnosed with a co-morbid neurodevelopmental disorder, and to have an epilepsy care plan. CONCLUSION: This is the largest study to date focused on adults with ID and epilepsy over 40 years. The ≥40 years cohort compared to the younger group has higher levels of clinical risk factors associated with multi-morbidity, potential iatrogenic harm and premature mortality with worse clinical oversight mechanisms.

Posterior circulation stroke following embolization of glomus tympanicum?relevance of anatomy and anastomoses of ascending pharyngeal artery. A case report.

SUMMARY: Embolization in the territory of the ascending pharyngeal artery (APA) can be unsafe even after detailed pretherapeutic angiographic evaluation due to changes in haemodynamics and opening of anastomotic channels. A 60-year-old woman underwent angiogram and embolization for glomus tympanicum tumour. The glomus tympanicum tumour was embolized using contour PVA particles of 150-250 um. At the end of the particulate injection the patient had posterior circulation stroke. The check angiogram showed near total devascularisation of the tumour and in addition filling of the left vertebral artery through an anastomotic channel. MRI confirmed the infarct in the posterior circulation. The ascending pharyngeal artery has potential anastomoses to all neighbouring major arteries, and the anastomoses to the vertebral artery in our case were through the musculospinal artery. This case highlights the importance of potential vascular anastomotic channels as a cause of ischaemic complication during the embolization procedure. It also highlights the fact that dangerous anastomoses may only be visualised in the later phase of embolization probably due to changes in the haemodynamic pressure. The angiographic anatomy of APA is reviewed with potential communications with the internal and external carotid and vertebrobasilar systems.

Albendazole therapy for single small enhancing CT lesions (SSECTL) in the brain in epilepsy.

BACKGROUND: Single small enhancing CT lesions (SSECTL) of the brain with or without perifocal oedema are common in patients with symptomatic epilepsy in India. Solitary cysticercus granuloma, a benign form of parenchymal neurocysticercosis, is considered to be the most common aetiology for SSECTL. Definite information is lacking regarding the effectiveness of antiparasitic treatment on resolution of these lesions and on long term seizure recurrence. OBJECTIVE: To evaluate the response to albendazole treatment in patients who had SSECTL and new onset seizures treated with antiepileptic drugs (AED) in a prospective clinical trial. METHODS: 43 patients who presented with new onset seizures and were documented to have SSECTL were alternatively allocated to receive albendazole 15 mg/kg/day for 2 weeks or no cysticidal therapy. All patients were treated with AED and followed for at least 6 months for seizure recurrence, and serial CT scans were obtained at 4 weeks, 3 months and at study completion. RESULTS: 28 (65%) patients were aged 5-25 years and 31 (72%) presented clinically with partial motor seizures with or without generalisation. Most of the SSECTL were ring lesions (75%) and located in and around the sensory-motor cortex at the gray-white junction (65%). In the albendazole group, 56% of patients compared with 35% in the control group showed resolution of SSECTL (p = 0.154) at 1 month. 22 of 23 patients (95.6%), who received albendazole, compared with 14 of 20 patients (70%) in the control group, demonstrated radiological resolution on study completion (p = 0.03). Punctate residual calcification and seizure recurrence were observed in four patients (9.3%) in the control group and in three (7%) patients in the albendazole group (p = 0.47). CONCLUSIONS: In patients presenting with seizures due to single viable parenchymal neurocysticercosis, albendazole hastens the resolution of SSECTL if treatment is given in the early phase of the illness.

Possible relationship between phenylthiocarbamide taste sensitivity and epilepsy.

The study was based on the data of a sample of 400 epileptic patients (200 idiopathic and 200 symptomatic) and 100 normal healthy individuals serving as controls. The PTC threshold distribution was bimodal. The number of non-tasters among idiopathic epileptics (35.5%) and symptomatic epileptics (32.5%) was significantly higher than controls (20%). The relative incidence of non-tasters in idiopathic and symptomatic epilepsies was 2.20 and 1.93 respectively. There is evidence that non-tasters tend to ingest a greater quantity of bitter tasting goitrogenic substances present naturally in edible plants which in turn exert greater thyroid stress in non-tasters or less sensitive tasters. Such a stress during intrauterine or early childhood growth and development might have affected neurological maturation which in turn made them more susceptible to epilepsy than tasters, who faced lesser stress.

Occipital seizures presenting with bilateral visual loss.

Transient visual loss may occur with occipital seizures as an ictal or post-ictal phenomenon. Its duration varies from less than one minute to days, or can be permanent. We describe a 61-year-old man presenting with headache, vomiting and bilateral visual loss. EEG revealed persistent spike discharge in the occipital lobes suggesting occipital seizures. His vision improved with carbamazepine.

Acute symptomatic seizures due to single CT lesions: how long to treat with antiepileptic drugs?

The duration of anti epileptic drug therapy for single small enhancing CT lesions (SSECTL) presents a major dilemma. We studied the efficacy of short duration (6 months) antiepileptic drug therapy as compared to long duration (2 years) drug therapy. Seventy three patients presenting with seizures and showing SSECTL on cranial CT scans (plain and contrast) were randomized into group A (6 months therapy) and group B (2 years therapy). There were 47 patients in group A and 26 patients in group B. Patients were followed up for one year after withdrawal of anti epileptic drugs. CT Head (plain and contrast) was repeated after 3 months, or earlier in cases of recurrence to rule out reinfection. 53.2% in group A and 53.8% in group B showed complete resolution and were seizure free on one year follow up. Punctate residual calcification was seen in 46.8% in group A and 46.2% in group B. Eight patients (17%) in group A and three (11.5%) in group B had a recurrence. The difference in recurrence of seizure between the two groups was not statistically significant (p<0.77) in the calcified lesion subset. Since none of the patients in total resolution subset showed recurrence, the difference between calcified and total resolution subset was highly significant. The study shows that a short duration (6 months) AED therapy in patients with total resolution of lesion on follow up scan, may be adequate in comparison to those who have calcific speck as a residue. However, a longer duration of therapy in case of calcific group probably does not alter their chances of recurrence.

Detection of antigen B of Cysticercus cellulosae in cerebrospinal fluid for the diagnosis of human neurocysticercosis.

Neurocysticercosis (NCC) is a major cause of morbidity and mortality in developed and developing countries. The diagnosis of this disease remains a problem. We report the detection of specific antigenic fraction (antigen B) of Cysticercus cellulosae by enzyme-linked immunosorbent assay (ELISA) in various fractions of cerebrospinal fluid (CSF) obtained by high performance liquid chromatographic (HPLC) separation, for the diagnosis of human NCC. Forty patients attending or admitted to Nehru Hospital, Chandigarh were included in the study: 10 with suspected NCC, 20 with other neurological diseases and 10 undergoing surgery under spinal anaesthesia for non-neurological conditions, who served as controls. CSF samples collected from all patients and controls were subjected to chromatographic separation on an HPLC system. Antigen B (AgB) was detected in separated fractions by an ELISA test and compared with the detection of antibody response in CSF samples by indirect haemagglutination (IHA) technique. Antigen B was detected in 9 out of 10 patients with suspected NCC based on clinical symptoms and radioimaging reports, but in none of the control subjects. However, antigen B was also detected in 9 out of 20 patients with other neurological disorders, mostly tubercular meningitis. Antibody response by IHA was found positive in only 2 of 10 cases clinically suspected of NCC. In conclusion, antigen B detection in CSF samples may be a useful adjunct to clinical suspicion and radiological reports for the diagnosis of NCC as there is no gold standard criteria to confirm this disease. However, the test needs to be evaluated on more patients in countries where tuberculosis and cysticercosis are endemic due to the high cross reactivity with samples from tubercular meningitis patients.

Albendazole therapy for solitary persistent cysticercus granuloma.

Use of Albendazole therapy for the treatment of patients having persisting intracranial solitary cysticercus granuloma is controversial. Most of the times these patients are treated empirically with variety of drugs for variable period. Some authors advocate biopsy before definitive treatment. 25 patients having radiologicaly persistent solitary cysticercus granuloma (>6 months) were given 15 days course of oral albendazole (15 mg/kg body wt). Cranial CT scan was repeated one month after the completion of albendazole therapy. It was evaluated for complete resolution, partial response (> 50% decrease in size of lesion) or no change as compared to previous scan. 12 patients (48%) showed complete resolution, 4 patients (16%) showed a partial response, while 9 patients (36%) did not show any change on follow up. Albendazole therapy may be useful for patients having persistent cysticercus granuloma.

Cellular immune responses in human neurocysticercosis.

Studies on the role of cell-mediated immune responses in human neurocysticercosis (NCC) are lacking. Various cell-mediated immune responses such as lymphocyte subpopulation, lymphocyte transformation to cysticercus antigens and cytokine profile were carried out in NCC patients. Lymphocyte transformation assays using larval antigens showed significantly higher (3)H-thymidine uptake. Immunophenotyping analysis showed an insignificant increase in B cells and a decrease in total T cells. However, there was a significant decrease (P < 0.05) in CD8+ T cells whereas there was no change in other cells like CD4+, HLA-DR+ and CD16+/CD56+. Cytokine profile revealed significantly higher (P < 0.01) production of Th1 cytokines (gamma-IFN and IL-2) using cysticercal antigens as stimulants for peripheral blood mononuclear cells, while there was no difference in IL-4 levels between NCC patients and healthy controls. The cytokine profile indicated the involvement of Th-1-like responses in NCC patients.

Profile of intractable epilepsy in a tertiary referral center.

This study was undertaken to find out the profile of intractable epilepsy (IE) in a tertiary referral centre. 100 patients (males 67; females 33) with IE attending the epilepsy clinic were evaluated. Detailed history, examination, investigations like EEG and CT scan and details regarding pharmacotherapy were analysed. The age of the patients ranged from 5 to 70 yrs (mean=23.2 yrs). Mean duration of seizures was 11.44 years. Commonest seizure type was partial seizures (74%). Amongst patients with generalised seizures (26%), 14% had multiple seizure types. The seizure frequency was 12.39 +/- 21.57 (mean +/- SD) per month. Fifty seven patients were in the symptomatic group with CNS infections being the leading cause (19%) of epilepsy. Fifty patients had one or more abnormal predictors of IE. There was no difference in the severity of epilepsy in patients with no abnormal feature when compared with patients having abnormal features. EEG was abnormal in 69% cases with background abnormality in 20% and focal abnormality in 36% cases. CT scan was abnormal in 41% cases with commonest abnormality being neurocysticercosis (11%) followed by gliosis (9%) and chronic infarct (9%). Sixty patients were receiving a combination of two drugs, 32 patients 3 drugs and 8 patients were on 4 drugs. There was no difference in seizure control in patients who were on 2 drugs or more than 2 drugs. Partial seizures were the commonest seizure type leading to IE; CNS infection being the leading aetiological factor. The presence or absence of predictors of intractability does not predict severity of epilepsy. Addition of third primary drug to existing combination only increases adverse effects without better control of seizures.

Risk of recurrence of seizures following single unprovoked idiopathic seizure.

A prospective study was conducted to look for various factors that could predict the risk of recurrence of a single unprovoked idiopathic seizure. Seventy six patients with a history of single episode of seizure ultimately completed the study and the data regarding age, sex, duration of seizure, time of occurrence of the ictus, interval between onset and referral, family history of seizure and alcohol consumption were analysed. All patients of symptomatic epilepsy and those with an abnormal scan were excluded. The patients were randomized into two groups, one of which received anti epileptic medication and the other did not. All patients underwent electroencephalography (EEG). Twenty two (M=16, F=6) of the 76 patients (M=56, F=20) had a recurrence of seizure. The duration of seizure at initial presentation was 10.1 +/- 5.2 min. in the recurrence group and 6.5 +/- 4.1 min. in the non-recurrence group. Twelve of the 16 patients with an abnormal EEG had a recurrence whereas only 10 of the 60 patients with a normal EEG had a recurrence (p <0.001). Of the treated cases (n=36), only 4 had a recurrence compared to 18 of the untreated cases (n=40) (p <0.002). Eighteen of the 22 cases having a recurrence did so within three months. Six of the cases with family history of seizure (n=10) had a recurrence, whereas only 16 of the cases without family history of seizure (n=16) had a recurrence (p <0.05). Patients of a single unprovoked idiopathic seizure with a normal CT scan are less likely to have a recurrence if the duration of seizure at presentation is short, EEG is normal, more than 3 months have passed since the first seizure and if treatment has been started. Family history of seizures does have a moderately significant bearing, but alcohol intake does not increase the chances of seizure.

Evaluation of patch test in identification of causative agent in drug rashes due to antiepileptics.

Patch test was evaluated for the identification of causative agent in cutaneous eruptions due to antiepileptics. Patch tests were carried out in twenty patients and ten controls with carbamazepine, phenytoin sodium, phenobarbitone and sodium valproate. Sodium valproate was found tobe irritant in 1 and 5% concentration and further dilution is recommended for patch testing. Patch test was positive in 14 (70%) patients and in 7 with suspected drug alone, and remaining 7 were positive with more than one antiepileptic drug. We recommended patch test for identification of causative drug in rashes due to antiepileptics.

Efficacy of magnesium sulphate and phenytoin in the management of eclampsia.

Fifty pregnant women admitted with diagnosis of eclampsia were randomly allocated to magnesium sulphate (Group A) or phenytoin sodium (Group B) treatment group. Incidence of recurrence of seizures maternal as well as perinatal morbidity and mortality were compared in both the groups. Mean maternal age, parity and gestational age was similar in both the groups. Mean birth weight was significantly lower in Group B compared to Group A. Seizure frequency prior to hospitalization was 5.4 +/- 4.7 in Group A and 4.8-3.6 in Group B. Mean time interval between occurrence of first seizure and hospitalization was 9.6 +/- 3.5 hours in Group A and 11.8 +/- 9.3 hours in Group B, the difference was not statistically significant. Women treated with phenytoin had a higher incidence of recurrent seizures (10/25-40%) than those treated with magnesium sulphate (2/25-8%). Majority of the women treated with phenytoin (6/10-60%) had single convulsion after initiation of anticonvulsant therapy and 1 woman of each group had recurrent convulsions (75). There was no significant difference in perinatal outcome in both the groups. Maternal morbidity was comparable in both the groups and there was no maternal death in either of the groups.

A case control study and one year follow-up of registered epilepsy cases in a resettlement colony of North India, a developing tropical country.

A population-based case control study and monthly follow-up of 121 registered epilepsy cases was conducted during 1995-1997 in a resettlement colony of Chandigarh, India. History of various tentative risk factors, e.g. trauma, febrile seizures, family history of seizures, alcohol intake and other possible causes was elicited. An age- and sex-matched control was selected from the neighbouring families for each case. A discordant pair analysis was done for matched case/controls. History of head injury, febrile seizures and developmental delay was observed exclusively in cases (none present in controls). Odds for epilepsy were higher among people who had positive family history (O.R.= 2.1, chi2 = 5.5, C.I. = 1.1-4.3). All cases were followed up and interviewed for history of seizures and drug intake. Fourteen cases could not be followed up completely. Ninety-four (88%) of the remaining 107 cases did not have any seizures during the follow-up. Of them, 70 (75%) patients were not on medication, 13 patients were on phenytoin and 11 patients received phenobarbitone. Thirteen cases reported seizures during the follow-up. Four patients out of the latter had mental retardation and were not on medication. Cumulative incidence of epilepsy was estimated to be 0.6/1000 person-year exposure.

Primary progressive aphasia : a case report.

Primary progressive aphasia is due to focal left perisylvian degeneration and manifests with progressive decline in language function for two or more years. There is preservation of cognitive functions and activities of daily living continue to be normal. We report a case of progressive aphasia in a 65 year old lady.

Neurological complications of eclampsia.

OBJECTIVE: A prospective study was conducted to evaluate the various neurological (clinical, radiological and EEG) complications in patients of eclampsia. METHODS: Thirty nine patients of eclampsia were studied regarding neurological findings at presentation and electroencephalographic (EEG) tracings were recorded in each patient. Patients with an abnormal neurologic examination and/or focal or lateralizing findings on EEG, underwent a CT scan (n = 18). Foetal and maternal outcome were recorded. RESULTS: The age of the patients ranged from 19-30 (mean +/- SD, 24.2 +/- 3.5) years thirty six patients (92%) had seizures in the antenatal period, 2 (5.4%) patients developed post partum eclampsia and 1 (2.6%) patient had seizures before and after delivery. A diffuse encephalopathy was seen in 9 patients (23.1%), 4 patients (10.2%) had hemiparesis and 1 patient (2.6%) had papilledema. EEG abnormalities were seen in 29 cases (74%) and included generalized slowing (n = 19), generalized sharp waves (n = 9), focal slowing (n = 4), focal sharp waves (n = 2) and spikes (generalized and focal) were seen in 1 patient each. Abnormal CT scan was seen in 10 cases (n = 18). Five patients had generalized infarct was seen in 1 patient each. There were 8 (20.5%) still births and 31 (19.5%) live births and no maternal mortality. CONCLUSIONS: Antenatal seizures occur in > 90% cases of eclampsia and less than 10% cases have seizures after delivery. A diffuse encephalopathy is the commonest clinical abnormality along with generalized slowing on EEG. Although cerebral oedema is common focal infarcts may be seen on CT scan.

Uncommon presentations of neurocysticercosis.

Neurocysticercosis commonly presents with seizures, raised intracranial tension and dementia. The unusual location of the cysts may result in uncommon manifestations mimicking a host of neurological disorders. Ten patients with neurocysticercosis with rare clinical presentations have been described in this series. These include dorsal midbrain syndrome, isolated bilateral ptosis, papillitis, cerebral hemorrhage, painful cervical radiculopathy, progressive swelling of arm, paraplegia due to intramedullary cyst, third ventricular cyst, dystonia and nominal aphasia masquerading as transient ischaemic attacks. The clinical details and possible mechanisms for these rare presentations are discussed.