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Biomedical subjects

B G Neville

Publications and source records attributed to B G Neville.

At least 37 records · Page 2Linked to original sources
PubMed1998

Language dysfunction in children with Rolandic epilepsy.

Rolandic epilepsy is regarded as the classic example of benign focal epilepsy. However, neuropsychological deficits have been noted in affected children. As Rolandic discharges are mainly distributed over the centrotemporal region, specific interference with language function might be suspected. Therefore, we conducted a standardized neuropsychological assessment in children with Rolandic epilepsy which covered all important aspects of language processing. We measured intelligence Quotient, verbal memory, auditory discrimination, vocabulary, grammar and literacy in 20 children with an active Rolandic focus. Information about performance at school was obtained from teachers by means of a questionnaire. Patients with Rolandic epilepsy failed five of the twelve standardized language tests significantly more often than the normative population and consequently showed impairment of the following functions: reading, spelling, auditory verbal learning, auditory discrimination with background noise and expressive grammar. Thirteen of the 20 children showed language dysfunction with difficulties in two or more of the twelve standardized language tests. In eight of these 13 children the Full Scale Intelligence Quotient was within average range, indicating a specific language deficit. Language dysfunction was closely associated with learning difficulties at school. This study documents a consistent pattern of language dysfunction in children with Rolandic epilepsy which suggests interictal dysfunction of perisylvian language areas.

Adolescent↗
PubMed1998

Developmental perspectives on epilepsy.

This paper reviews current knowledge about epileptogenesis in the developing brain. Animal studies indicate that the maturational balance of excitatory and inhibitory neurotransmitter systems should result in an increased susceptibility to seizures. However, the reason for specific age-locked syndromes and the high rate of impairments secondary to early onset epilepsy remain mysterious. Present research activity is directed to prevention and amelioration of these severe cognitive and psychiatric impairments.

Aging↗
PubMed1998

Epilepsy in very preterm infants: neonatal cranial ultrasound reveals a high-risk subcategory.

The aim of this study was to investigate the association between epilepsy and perinatal brain injury in a cohort of 610 infants born preterm at <33 weeks' gestation. The prevalence of epilepsy in this cohort was 4.3% as determined by a postal questionnaire survey. Most children with epilepsy (16 of 24) had high-risk cranial ultrasound lesions including haemorrhagic parenchymal infarction (HPI), posthaemorrhagic hydrocephalus, and cystic periventricular leukomalacia (PVL). Of all the children in our cohort with high-risk brain lesions, those with epilepsy were more likely to have HPI and significantly less likely to have cystic PVL, although it is possible that PVL was not noticed in some cases. Children with epilepsy and high-risk cranial ultrasound lesions also showed more cognitive impairment than children with high-risk lesions but no epilepsy, which suggested more cortical grey-matter damage. We suggest that brain injury has occurred outside the confines of the periventricular white matter in this group of preterm infants with epilepsy.

Brain↗
PubMed1998

Buccal absorption of midazolam: pharmacokinetics and EEG pharmacodynamics.

PURPOSE: To determine whether buccal/sublingual administration of midazolam (MDL) would lead to detectable venous concentrations and EEG changes in 10 healthy volunteers. METHODS: The study consisted of an open-label and a double-blind phases. Subjects held 10 mg MDL in 2 ml peppermint-flavored fluid or peppermint-flavored placebo in their mouth for 5 min and then spat it out. Cardiorespiratory and EEG monitoring was performed in all subjects. RESULTS: Venous MDL concentrations measured on 10 occasions from 5 to 600 min after administration showed a rapid increase for the first 20-30 min. However, changes in the 8- to 30-Hz frequencies identified by spectral analysis of the EEG showed changes in < or = 5-10 min in test but not in control subjects--more rapid than were expected from the venous absorption data. There were no significant adverse effects. CONCLUSIONS: Our data provide direct evidence of the speed of cerebral effect of a drug. Our results suggest that the buccal/sublingual route of administration should be tested in emergency treatment of seizures as an alternative to the rectal route, over which it has clear practical advantages.

Absorption↗
PubMed1998

Exercise induced steroid dependent dystonia, ataxia, and alternating hemiplegia associated with epilepsy.

This paper describes a 20 year old woman with a new combination of neurological impairments in which the motor phenomena were responsive to corticosteroid treatment. She had lifelong moderate learning impairment. A variable ataxia with cerebellar characteristics was present from early life, with early severe exacerbation when seizures were uncontrolled. Atypical absence and simple and complex partial seizures were present from the first year of life and EEG abnormalities were maximal in the right parietal region, concordant with a mild non-specific abnormality of the white matter in the region of the trigone. Episodes of alternating hemiplegia occurred from 11 years, unassociated with seizures. Exercise induced dystonia occurred from the age of 5. After 10-20 minutes walking, her right foot would turn in and the right leg would stiffen, followed by the left and by falling and inability to get up for several minutes. Prednisolone improved her ataxia and was associated with cessation of both seizures and exercise induced dystonia. This adds a new syndrome to the corticosteroid responsive motor disorders associated with epilepsy.

Adolescent↗
PubMed1997

Epileptic nystagmus in infancy.

Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report an infantile case in which EN was first noted at 10 days of age. Electronystagmography showed a right-beating nystagmus with predominantly linear slow phases that traversed the midline. Neuro-imaging revealed dysplasia of the left middle temporal gyrus extending posteriorly into the parieto-occipital cortex. The right hemisphere and subcortical structures appeared normal. Perfusion studies demonstrated interictal hypoperfusion with ictal hyperperfusion in the left temporal lobe. Electrocorticography demonstrated spiking over the left temporal-parieto-occipital region. Following extensive surgical resection of this area and weaning of anti-convulsants, the child has remained seizure-free without nystagmus. This case demonstrates the cortical origin of EN, and shows that infant cortex has functioning efferent connections to brainstem oculomotor centres from 10 days of age.

Electroencephalography↗
PubMed1997

Surgical treatment of severe autistic regression in childhood epilepsy.

We report 2 children with partial epilepsy who manifested social and language regression and partial recovery after surgical treatment. One child had seizures since the first 2 weeks of life, caused by a right temporal dysembryoplastic neuroepithelial tumor and regression in the latter part of the first year; seizures were relieved and some functions were recovered after temporal lobe resection at 12 months of age. The second child developed epilepsy at 3 years 3 months, and between 5 years 9 months and 6 years 1 month he became aphasic (Landau-Kleffner syndrome) and lost social functioning, manifesting a very severe behavior disorder. He exhibited a significant improvement in communication, social functioning, and behavior after left multiple subpial transections. Both children manifested evidence of subclinical seizure activity in both temporal lobes. Their clinical picture was one of combined language and autistic regression, and the autistic features demonstrated a clear response to surgical treatment. We suggest that in pediatric epilepsy surgical programs, autistic regression should prompt urgent investigation if drug treatment is not effective.

Autistic Disorder↗
PubMed1997

Complex orofacial movements and the disappearance of cerebellar mutism: report of five cases.

A syndrome of mutism and subsequent dysarthria occurs frequently in children after resection of a cerebellar tumour. The role of orofacial and speech motor control in this syndrome has not been studied systematically. We examined simple and complex orofacial movements during the mute phase and shortly after the resumption of speech in five children with mutism and subsequent dysarthria. The recovery of complex orofacial movements coincided with the disappearance of the mutism.

Adolescent↗
PubMed1997

Interictal 99Tc(m) HMPAO SPECT and 1H MRS in children with temporal lobe epilepsy.

PURPOSE: To understand the pathological basis of focal hypoperfusion seen on interictal 99Tc(m) hexamethylpropyleneamine oxime (HMPAO) single-photon-emission computed tomography (SPECT) in intractable temporal lobe epilepsy, and to determine why the technique may be misleading in the localization and lateralization of the seizure focus in some cases. METHODS: Interictal 99Tc(m) HMPAO SPECT and proton magnetic resonance spectroscopy (1H MRS) of the mesial temporal regions were performed in 14 children with intractable temporal lobe epilepsy not caused by a foreign tissue lesion. RESULTS: Hypoperfusion of one temporal lobe ipsilateral to the seizure focus was demonstrated in 10 (71%) of the children; 1H MRS correctly lateralised in eight of these 10. No asymmetry of perfusion of the anterior temporal regions was seen in the remaining four children; on 1H MRS, three of these were bilaterally abnormal but nonlateralising. Repeated SPECT and 1H MRS in three children demonstrated changes over time, the findings from the two techniques being consistent with each other on both the initial and the repeated scans. CONCLUSIONS: Abnormalities demonstrated by 1H MRS correlate well with those seen on interictal SPECT and can help to understand the pathologic basis of these SPECT abnormalities. Furthermore, the presence of bilateral damage can result in an absence of perfusion asymmetry on interictal SPECT.

Adolescent↗
PubMed1997

Ictal cerebral perfusion related to EEG in drug resistant focal epilepsy of childhood.

OBJECTIVES: To evaluate the EEG changes during seizures in children with drug resistant focal epilepsy who demonstrate hypoperfusion at the "seizure focus" interictally, but no perfusion change during the seizure. METHODS: Ictal EEG findings of six children with focal epilepsy who demonstrated hypoperfusion on rCBF SPECT after an interictal injection of (99)Tc(m) HMPAO concordant with the seizure focus, but who did not demonstrate rCBF change after an ictal injection (group 1) were reviewed. These were contrasted with the EEG data of six children matched as closely as possible for age, type of epilepsy, and pathology who did show hyperperfusion at the seizure focus on ictal scan when compared with the interictal study (group 2). RESULTS: The children in group 1 showed slowing of the EEG at the time of the (99)Tc(m) HMPAO injection relative to that seen at the onset of the seizure. Those in group 2 showed rhythmic activity, or sharp waves, or both on EEG at the time of injection. This last change was also seen consistently when the EEG data of a further 13 children who also showed ictal hyperperfusion at the seizure focus were reviewed. CONCLUSION: Ictal rCBF does not invariably increase at the seizure focus in patients with drug resistant focal epilepsy.

Adolescent↗
PubMed1996

Randomised comparative monotherapy trial of phenobarbitone, phenytoin, carbamazepine, or sodium valproate for newly diagnosed childhood epilepsy.

BACKGROUND: The medical treatment of childhood epilepsy is largely influenced by clinical trials in adult patients. We know of only one randomised comparative trial (of two drugs) in newly diagnosed childhood epilepsy. We have undertaken a long-term, prospective, randomised, unmasked, pragmatic trial of the comparative efficacy and toxicity of four standard antiepileptic drugs used as monotherapy in children with newly diagnosed epilepsy. METHODS: Between 1981 and 1987, 167 children aged 3-16 years, who had had at least two previously untreated tonic-clonic or partial seizures, with or without secondary generalisation, were randomly allocated treatment with phenobarbitone, phenytoin, carbamazepine, or sodium valproate. The protocol was designed to conform to standard clinical practice. Efficacy was assessed by time to first seizure after the start of treatment and time to achieving 1-year remission. FINDINGS: The overall outcome with all four drugs was good. 20% of children remained free of seizures and 73% had achieved 1-year remission by 3 years of follow-up. We found no significant differences between the drugs for either measure of efficacy at 1, 2, or 3 years of follow-up. The overall frequency of unacceptable side-effects necessitating withdrawal of the randomised drug was 9%. This total included six of the first ten children assigned phenobarbitone; no further children were allocated this drug. Of the other three drugs, phenytoin (9%) was more likely to be withdrawn than carbamazepine (4%) or sodium valproate (4%). INTERPRETATION Our data will inform choice of drug and outcome with four of the standard drugs available for newly diagnosed tonic-clonic or partial seizures with or without secondary generalisation in children.

Adolescent↗
PubMed1996

Proton magnetic resonance spectroscopy in children with temporal lobe epilepsy.

We performed proton magnetic resonance spectroscopy of the mesial temporal regions in 20 children with intractable temporal lobe epilepsy and compared results with those from 13 normal subjects. Abnormalities of the ratio of N-acetylaspartate to choline plus creatine (NAA/[Cho+Cr]) were seen in 15 patients (75%). The ratio NAA/(Cho+Cr) was correctly lateralizing in 55% and incorrectly lateralizing in none. Bilateral abnormalities were seen in 45%. Overall there was a unilateral decrease in N-acetylaspartate on the side ipsilateral to the seizure focus (mean 19% decrease vs normals, with 5% decrease on the contralateral side), suggesting neuronal loss or dysfunction. There was also a bilateral increase in creatine and choline (mean 18%), consistent with reactive astrocytosis. We conclude that proton magnetic resonance spectroscopy can contribute to lateralization of the seizure focus, and by detection of bilateral abnormalities, can contribute to the understanding of the underlying pathophysiology in temporal lobe epilepsy.

Adolescent↗
PubMed1996

Epileptic akinetic mutism: following temporal lobectomy for Rasmussen's syndrome.

A girl aged 9 years with Rasmussen's encephalitis previously treated by left temporal lobectomy suddenly developed prolonged attacks of extremely rapid respirations associated with poverty of body movements and greatly reduced facial movements with loss of speech. Her comprehension appeared intact and she responded to certain requests. Her eye gaze behaviour suggested a high degree of vigilance. The attacks would last from minutes to hours and were repeated up to a dozen times daily. In the context of the stress generated by the illness and other family problems the attacks had been mistaken for an hysterical elaboration. Their ictal nature was confirmed by EEG/video monitoring. Possible links with other developmental language disorders associated with epilepsy are discussed, including a model of autism.

Akinetic Mutism↗