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Neurology

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G141 THE MANAGEMENT OF CONVULSIVE STATUS EPILEPTICUS IN A DGH

D. Sekaran1, B. Houston1, R. F. M. Chin2. 1Department of Paediatrics, Luton and Dunstable NHS Trust; 2Department of Neurosciences, Institute of Child Health

Aims: The morbidity and mortality associated with convulsive status epilepticus (CSE) is dependent on the duration of CSE. Thus early and effective treatment of CSE is paramount but there has been a lack of consensus on its emergency management. A working party of the British Paediatric Neurology Association (BPNA) has proposed a national guideline for the management of CSE. This study aims to characterise the emergency treatment of CSE in a district general hospital (DGH) that has adopted the BPNA guideline.

Methods: In a 1 year retrospective study, all children aged between 1 month and 16 years presenting to a DGH with CSE were identified through the DGH’s admission database. The database was searched for the ICD-10 diagnostic codes G40.9 (epilepsy, epileptic, epilepsia, fits, convulsions, and seizure), G41.9 (status epilepticus), R56.0 (febrile convulsion), and R56.8 (idiopathic convulsions). Data on patient demographics, diagnosis, choice of emergency antiepileptic drug (AED), AED dosages, intervals between steps, and complications were collated.

Results: Ten patients with CSE accounting for 15 CSE events were identified (mean age 5.4 years, range 0.3–16, M:F 2:3). In most events (75%) diazepam/lorazepam was administered as first line AED. Half of events requiring second (n = 11) and 20% requiring third line (n = 5) AED were treated with the BPNA guideline’s recommended AED for that step. Overall, only a quarter of patients received the recommended AED dose. The mean interval between AEDs was known in 11 events (step1–step 2 13.5 mins, step 2–step 3 12.5 mins).

Conclusions: CSE is frequently inappropriately treated. Recommended second and third line AEDs are frequently overlooked. Few patients receive the appropriate doses of AEDs. This study highlights the importance of audit and modification of local guidelines for the management of CSE.

G142 COMPLICATION RATES OF PAEDIATRIC CEREBRAL ANGIOGRAPHY (CA)

L. W. E. Fung, D. E. Saunders, W. K. Chong, T. Cox, V. Ganesan. Great Ormond Street Hospital for Children NHS Trust and Institute of Child Health

Background: Catheter CA is the gold standard for cerebrovascular imaging. Safety data are derived from adult studies, making it difficult to counsel parents of children undergoing this procedure.

Aims: To evaluate the safety of CA in children with cerebrovascular disease.

Methods: Medical, anaesthetic, and nursing records were reviewed for patients admitted to our institution for CA between January 1998 and July 2003. The following information was retrieved and recorded: age, sex, diagnosis, timing, and purpose of CA, number of vessels catheterised, and local and neurological complications.

Results: 234 CA studies from 171 patients with a median age of 6.8 years (newborn–19 years) were reviewed. Main diagnoses encountered were arterial ischaemic stroke (n 48), intracranial haemorrhage (28) and intracranial vascular malformation (50), vein of Galen malformation (21), and moyamoya syndrome (26). There were 144 four vessel studies, 42 two vessels studies, and 45 single vessel studies. 211 procedures were elective; 172 studies were done for diagnostic reasons, 45 were done for interventional purposes, and 17 procedures combined diagnosis and intervention. Ten local complications were reported. There were eight episodes of bleeding/local haematoma formation. One patient had a decreased pedal pulse on the side of femoral catheterisation but subsequent investigations were normal. Another had a small laceration of the femoral artery but adequate haemostasis was obtained. Only one patient (a child with an arteriovenous malformation undergoing embolisation) had a neurological complication comprising severe headache and visual and speech disturbance after the procedure. Brain imaging did not reveal new changes and his symptoms resolved within 24 hours without permanent deficit.

Conclusion: CA is a safe procedure in children with cerebrovascular disease with a local complication rate of <5% and a neurological complication rate of <1%.

G143 SAFE SEDATION FOR NEUROIMAGING CHILDREN: A PROSPECTIVE CLINICAL AUDIT

W. P. Whitehouse1,2, R. Rushby3, C. Butt1, J. Collier2. 1Department of Paediatric Neurology, 2School of Human Development, 3Paediatric Neurosciences Ward E39, University of Nottingham, Queen’s Medical Centre, Nottingham, UK

Aims: To assess effectiveness, acceptability, and safety of current practice. To inform future practice, as many departments have abandoned all sedated neuroimaging in favour of general anaesthesia.

Methods: A multidisciplinary, prospective clinical audit of consecutive, unselected children attending for sedated neuroimaging using a standard data sheet and sedation nursing observation chart from June 2000 to January 2003 is reported.

Results: Data on 134 (72% male) children aged 4 months to 12 years (mean 3.6 years), 33% with neuro/developmental disabilities are presented. 51% had CT, 49% MRI. Using chloral 50–100 mg/kg for those under 4 years (60%) and quinalbarbitone 7.5–10 mg/kg for older children (40%), 89% of scans were successful and 90% of parents reported the experience was “OK”. 5% of children required airway support (jaw thrust) or other intervention; all in the younger chloral group. There were no serious adverse events (95% CI 0 to 2.9%).

Conclusions: The sedation policy appears satisfactory. Data on a further 50 children are currently being analysed.

G144 LONG TERM EFFECTS OF SEVERE HEAD INJURY (TBI) IN CHILDHOOD: DIFFERENCES IN HEAD SIZE (OFC), BRAIN VOLUME, WHITE MATTER, AND THE HIPPOCAMPUS

R. C. Tasker, A. Gunn-Westland, C. H. Salmond, A. Pena, J. H. Gillard, J. D. Pickard, B. J. Sahakian. University of Cambridge Departments of Paediatrics, Psychiatry, Neuroradiology, Neurosurgery; Wolfson Brain Imaging Centre

Introduction: We are interested in brain growth and neuroplasticity following injury in childhood.

Aim: We have tested the hypothesis that after TBI in childhood subsequent hippocampal volume is related to generally diffuse rather than focal injury.

Methods: We have examined 33 subjects (16 females) who sustained TBI necessitating intensive care when aged mean 9.3 (SD 4.0) years. This cohort was divided, a priori, into control and study groups based on the time course of acute ictus. At follow up, brain MRI was undertaken when aged 15.1 (3.9) years using a Bruker Medspec 30/100 spectrometer attached to an Oxford 3.0T, 910 mm bore, actively shielded magnet. Measurements were blinded and included brain hemisphere, ventricles, hippocampus, and peri-hippocampal volumes; spatial distribution of differences in white matter; and diffusion tensor imaging diffusivity (D) eigen vectors and eigen values.

Results: Data were analysed in three steps: multivariate analysis of variance, ANOVA, and multiple regression analyses. In comparison with controls, study subjects had smaller OFC (SD score 0.48 (0.8) v −0.47 (0.6), p<0.005). A subgroup of the study subjects, identified by voxel-based morphometry, had periventricular white matter loss and maller than expected brain volume for OFC (1413 (106) v 1263 (57) ml, p<0.01), suggesting atrophy; the remainder had expected volume, albeit for smaller OFC, suggesting growth disturbance. Last, right hippocampal volume (corrected for hemisphere volume) was also smaller in the study group compared with controls (3.33 (0.4) v 2.86 (0.5) ml, p<0.05). On multiple regression right hippocampal volume was related to the primary side of injury (that is focal injury), in more severe instances (lower coma score during acute ictus), where a diffuse difference in cerebral volume is produced, and an ipsilateral difference in white matter (D eigen values) ensues (model p<0.001, R2 93%).

Conclusion: Our primary hypothesis is refuted. These data also suggest that injury, even in later childhood, influences subsequent brain development, that is regional volume and white matter milieu.

G145 A REVIEW OF CLINICAL PRACTICE IN A SECONDARY CARE EPILEPSY CLINIC

V. C. A. Kujambal, D. Simkiss. City Hospital, Dudley Road, Birmingham UK

Aims: To identify the proportion of patients without epilepsy among those referred to our epilepsy clinic. To identify patients with recognised epilepsy syndromes and aetiology as per the diagnostic scheme proposed by the International League against Epilepsy (ILAE).

Methods: Retrospective review of casenotes of new patients seen in 2001–2002 by a consultant paediatrician with an interest in epilepsy.

Results: Of the 66 patients referred, 55 casenotes were analysed. Mean age of patients was 6.5 years (range 3 months to 17 years). Forty seven (85%) patients were referred as epilepsy, five (9%) as non-epilepsy, two (3.6%) as febrile convulsions, and one (1.8%) with headache. Thirty patients (54.5%) were diagnosed to have epilepsy, 23 (41.8%) had non-epileptic events, and two had febrile convulsions. Non-epileptic events included fainting (7), cardiac cause (1), breath holding spells (3), reflex anoxic seizures (3), blue episodes (1), delayed parachute reaction (1), dystonic reaction (1), day dreaming (1), headache (1), anxiety headache (1), tremulous episode (1), screaming episode (1), and ritualistic movements (1). Among patients with epilepsy, 22 (73%) had generalised tonic clonic seizures. Five (16%) had epileptic syndromes. Aetiology was recognised in 12 (40%). All, but one, patients were on appropriate medication.

Conclusion: About 40% of patients had non-epileptic events. Epileptic syndromes and aetiology were recognised in fewer patients than expected. In order to produce a robust clinical governance model of care, we intend to monitor our epilepsy practice by using a structured proforma (based on the ILAE criteria) in our clinic. An essential feature will be external revalidation by a paediatric neurologist. This will be reaudited in 3 years.

G146 CORTICOSTEROID USE PROVIDES SHORT TERM IMPROVEMENT IN MOTOR FUNCTIONS IN PATIENTS WITH DUCHENNE MUSCULAR DYSTROPHY (DMD)

A. Ghatak, Z. Bassi, S. Savage, G. Holmes, P. Nicolaides. Royal Liverpool Children’s NHS Trust, Liverpool

Background: DMD is a progressive disorder with no known effective treatment. Corticosteroids have been reported to improve muscle strength and prolong ambulation. We report a 2 year experience of the use of steroids in children with DMD at a tertiary referral centre.

Methods: Consecutive ambulant patients of DMD over the age of 4 years attending the neuromuscular clinic were offered steroids unless clinically contraindicated. Intermittent (10 days on and 10 days off) prednisolone at 0.75 mg/kg was used and all patients underwent detailed physical and physiotherapy assessment (MRC scoring) at baseline, 3 months and then every 6 months. Parental perception of mobility was recorded by a standardised questionnaire.

Results: Eighteen patients (mean (SD) age 7.3 (1.3) years) were treated for a median duration of 8.5 months. At 3 months there was improvement in timed Gower (6.25 sec v 4.8 sec, p 0.03), timed 10 meter walk (12.9 sec v 11.8 sec, p 0.2), motor ability scores (27.2 v 31.6, p<0.001), and forced vital capacity (FVC) (1.24 l v 1.3 l, p 0.01). These improvements were sustained at 6 months. At 3 months, 53% of the carers noted improvement in walking distances. Of the eight patients who continued steroids for over a year, all patients had sustained improvement in FVC but the improvement in motor functioning was maintained in only three (38%) patients. Treatment was withdrawn in 7 of the 18 patients (mean duration of steroid use 7 months) because of side effects (2), loss of effect (2), or because the child became non-ambulant (3).

Conclusions: Intermittent use of prednisolone at 0.75 mg/kg improved subjective and objective motor functioning at 3 and 6 months after commencing treatment in patients with DMD. This improvement was sustained only in a third of the patients at 1 year. However, an improvement in FVC was maintained throughout the duration of use of steroids. Further trials will be required to clarify the role of steroids in patients with DMD.

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