Cognitive and behavioural concerns in epileptic children.

Cognitive performance in an epileptic child has been a difficult issue to predict in day-to-day clinical practice. Several observations made in early and later part of this century do not provide uniform and convincing answer to this issue. Recent trends in research however, have identified certain variables that are shown to be associated with cognitive decline in epileptic children. Together with associated behavioural problems, the resultant school difficulty is the essence of this concern for the parents. The variables related to cognitive deterioration as identified by several studies include underlying brain pathology (symptomatic epilepsy), early age of onset of seizure, severity and intractability of seizure, repeated head trauma, an episode of status epilepticus, presence of interictal subclinical EEG discharge, adverse psychosocial factor and antiepileptic drug (AED). Association of these variables in a given case cannot only predict adverse cognition outcome but also a preventive management package can be planned aiming at avoiding or minimizing these high risk variables.

Spinal cord tuberculoma and misleading MR imaging : a case report.

A case of multiple spinal cord tuberculomas has been presented because of its rarity and misleading picture on MR imaging. The MRI suggested intradural extramedullary spinal tumours, possibly neurofibromata, because of associated cafe-au-lait spot. Various tubercular processes involving the spinal cord and meninges are discussed in the light of the present case.

Central motor conduction time in malnourished children.

The functional status of the descending motor pathways was assessed in malnourished children using transcranial electromagnetic stimulation of the cortex. Twenty children with different severities of malnutrition and 20 control subjects were studied electrophysiologically. The circular coil of a Dentac MAG 2 magnetic stimulator was applied tangentially over the vertex to stimulate the cortex. The muscle evoked potential in the children's arms was recorded from the abductor pollicis brevis muscle and in their legs from the extensor digitorum brevis muscle on both sides of the body using surface electrodes. The muscle evoked potential of the abductor pollicis brevis and extensor digitorum brevis muscles was further obtained using root stimulation by applying the coil at the cervical and lumbar spine, respectively. The indices of cortical threshold, cortical latency, and central motor conduction time (ms) were evaluated in both arm and leg muscles on both sides. The results showed an increased cortical threshold (mean (SD) 1232.5 (134.06) in the study group v 1147.5 (99.31) in the control group) for the abductor pollicis brevis muscle and for the extensor digitorum brevis muscle (1325.00 (115.70) in the study group v 1190.0 (125.24) in the control group). Similarly, significant prolongation of the central motor conduction time (ms) (study group 6.67 (0.91) v control group 5.71 (0.74)) in the abductor pollicis brevis muscle was seen in malnourished children.

Infantile facioscapulohumeral muscular dystrophy with Coat's syndrome.

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.

The pediatric non-epileptic seizure.

In order to observe the pattern of behaviour, the underlying stressors and possible association with psychosocial factors in Pediatric Non-Epileptic Seizure (NES); 22 children with unusual feature of poor control of their symptoms were studied. Detailed historical account, examination including psychiatric evaluation, EEG and CT Scan in all and video recording with provocative procedures were done wherever feasible. Ten males (45.5%) and 12 females (55.5%) were in the age range from 5 through 18 years. Ten children (45.5%) had uncontrolled symptoms while 12 (55.5%) had unusual symptoms. History and eye witness account was accurate in 15 (68.1%). Separate video recording could be done in 6 (27.3%) while induction with provocative procedures was possible in 7 (31.8%) older children. Pointers to NES were: stress induced events 13 (59.4%), persistence of symptom despite adequate treatment 10 (45%), lack of physical injury 20 (90.0%), symptoms when some one was around 12 (54.5%), lack of postictal phenomenon 12 (54.5%), lack of incontinence 22 (100%), positive gains 6 (27.0%) and gradual onset and offset 12 (54.5%). Nine children (40.0%) had coexisting epilepsy and NES. The present study addresses the need of awareness, provocative procedure, video recording of the event and individualised management of NES in our circumstances where video EEG facility is not available.

Infantile pseudotumor cerebri related to viral illness.

Pseudotumor cerebri (PTC) presents with varied spectrum of manifestations, association with diverse pathological conditions and variable outcome. A preliminary observation on children presenting with PTC is being presented because of certain peculiarities, not observed earlier, like occurring in clusters during particular season, all were infants having close relationship with viral illness and all had extremely benign outcome. Children presenting with raised ICP were hospitalised and evaluated. The relevant investigations carried out included lumbar puncture, cranial ultrasonography, CT Scan and culture studies. Fifteen children, all in infancy with male-female ratio of 2:3 presented in clusters of 11 (73.3%) cases during late summer and early fall presented with bulging fontanelle, vomiting 11, fever 12, respiratory infection 5, diarrhea 5, lateral deviation of eye balls 4 and skin rashes 5 cases. Contact with viral illness in the family was found in 10 cases. Lumbar puncture showed high opening high CSF pressure in all. Results of this study suggest peculiar and perhaps different form of PTC seen recently which affected only the infants. These were closely related to viral illness and had benign outcome.

The offspring of epileptic mother.

The offspring of an epileptic mother is an issue-currently getting attention because of its several implications. A complex interaction between epilepsy during pregnancy and its adverse impact on foetus, labor, neonate, congenital malformation, psychosocial and medico-social concern and treatment challenges of such cases is increasingly being realised. Some of the significant observations has been reviewed extensively in this article. Maternal epilepsy is likely to adversely affect the off-spring at its various stages of development amounting to increased morbidity and mortality. Increased seizure frequency during pregnancy with resultant increased risk is well documented but its mechanism is poorly understood. Low apgar score, increased still birth rates (1.3 to 14%) in offspring of epileptic mother (OEM) is reported. So also, the neonatal and perinatal deaths are twice more common in OEMS than normal control. Small for dates, and prematurity in OEM is reported to be 7 to 10% and 4-11% respectively. Adverse impact on labor and delivery like preclampsia, abruptio placentae, polyhydramnios, assisted delivery, cesarean section and IUGR poses particular challenges to the obstetrician. Pediatrician's alertness is needed to anticipate and deal with the bleeding manifestation due to deficiency of Vit-K dependent clotting factors and various anticonvulsant drug (AED) withdrawal symptoms. Significant risk of developing congenital malformation is the result of epilepsy perse and the AED used during pregnancy. AED exposure leads to other distinct clinical syndromes, the orofacial clefts and cardiac anomalies being the commonest manifestation. Epilepsy in mother but not in father has significant adverse impact. Management strategies in the context of available observation has been discussed.

The dying child and overt parental behavior.

In order to study the immediate grief reaction in parents of children dying in the hospital each parental reaction was scored on a 'grief reaction and intervention' (GRI) scale (minimum 0; maximum 4). The death events of 73 children comprised the study. The GRI score was 0 in 10 (13.7%), 1 in 19 (26.1%), 2 in 21 (28.7%), 3 in 15 (20.5%) and 4 in 8 (11%) cases; the mean (+/- SD) score was 1.89 (+/- 1.20). Crying, weeping spells, hostility, restlessness, denial of death, mutism, impulsivity and destructive behavior were observed. A significantly higher grief reaction was observed among parents of grown up children and those from an urban background. An intense reaction was also seen in cases when the course of illness was acute and death was not anticipated. Parents of male children of the first and second birth order also had higher GRI scores. Our findings suggest that socio-cultural factors may influence the intensity of the parental grief reaction.