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Biomedical subjects

B G Neville

Publications and source records attributed to B G Neville.

At least 19 recordsLinked to original sources

Neuropathology of biotinidase deficiency.

A patient with biotinidase deficiency and a progressive neurological disorder died just before the biochemical diagnosis was established. Post-mortem examination of the brain and spinal cord revealed necrotising lesions similar to those in Leigh's disease and Wernicke's encephalopathy. Unlike these two conditions, the regions affected included the hippocampus and parahippocampal cortex. In addition there was severe focal oedema in deep cerebral grey matter, the brain stem, and the spinal cord. These lesions appear to result from a number of severe metabolic disturbances, perhaps linked to an underlying disordered pyruvate metabolism. The nature of the pathology explains why a neurological deficit may persist despite treatment.

Amidohydrolases

The spinal cord in neurologically stable spina bifida: a clinical and MRI study.

MRI scans of the brain and spinal cord, together with neurological and urological assessments, were carried out on 20 young people (aged between eight and 22 years) who had had surgical treatment of spina bifida in early life and in whom there had been no deterioration of spinal cord function. 19 were imaged successfully. The cord was tethered in all and it was low-lying in 18. Five had cavities within the cord; other abnormalities included cord thinning, lipomas and diastematomyelia. The relationship of these findings to subsequent deterioration and the use of MRI in these patients are discussed. These abnormalities, found in the absence of clinical deterioration, throw fresh light on the natural history of this condition.

Adolescent

Herpes simplex encephalitis with relapse.

Three children are described in whom herpes simplex encephalitis (HSE) followed a clearly biphasic course. The secondary deterioration may be due to a resurgence of the viral infection and calls into question the adequacy of current treatment regimens for HSE. Alternatively, a postinfectious neuroallergic process may be active in which case immunomodulatory treatment might be more appropriate than further antiviral treatment.

Acyclovir

Transcranial pulsed Doppler ultrasound findings in brain stem death.

Data are presented from transcranial insonation of the middle cerebral artery (MCA) performed at intervals in 23 unconscious children for whom the outcome was subsequently poor. Once an MCA signal had been observed over a 30 minute period with time averaged velocity less than 10 cm s-1 and/or a direction of flow index, DFI, defined as 1 minus the ratio of reverse to forward flow of less than 0.8, recovery to forward flow throughout diastole was never observed and no patient recovered brain stem reflexes. Recovery of forward flow in diastole, and of brain stem function, was seen in cases with time averaged MCA velocity in the range 10 to 25 cm s-1 and with reverse flow but a DFI of greater than 0.8 for short periods of time. All but one of the 13 children fulfilling clinical criteria for brain stem death had MCA signals with time averaged velocity of less than 10 cm/s and DFI of less than 0.8. This type of signal was not observed in five children who were left in a persistent vegetative state.

Adolescent

Anatomical validation of middle cerebral artery position as identified by transcranial pulsed Doppler ultrasound.

The basal cerebral arteries were insonated using transcranial pulsed Doppler ultrasound (TPDU) at 2 MHz. The Doppler sample volume (SV) depths at which signals were obtained which could be attributed to the middle, anterior and posterior cerebral arteries (MCA, ACA and PCA) were compared with measurements in adult cadavers and with B-scan ultrasound studies in infants. The depth of the internal carotid artery (ICA) terminal division into ACA and MCA was closely correlated for both groups. In adults, it was found at 5.6 +/- 1.0 cm using TPDU while in cadavers it was found at 5.3 +/- 0.5 cm from the temporal bone. In infants, it was found at 3.2 +/- 0.3 cm for the right side, and 3.2 +/- 0.2 cm for the left side using TPDU, and at 3.4 +/- 0.4 cm and 3.4 +/- 0.5 cm for right and left sides respectively using B-scan ultrasound. The mean depth of the MCA mid-point in infants as defined by TPDU and B-scan was also closely correlated, with values of 2.8 +/- 0.3 cm and 2.7 +/- 0.3 cm for right and left sides respectively using TPDU and of 2.8 +/- 0.4 cm and 2.7 +/- 0.4 cm for right and left sides respectively using B-scan ultrasound. Values for the most lateral part of the MCA did not correlate. In adults, signals from the ACA and PCA were obtained at greater SV depth than the MCA, thus preventing confusion.

Adult

Hydrocephalus and primary ciliary dyskinesia.

Primary ciliary dyskinesia was shown in a 12 year old boy with bronchiectasis who had developed hydrocephalus in the neonatal period. The possible relevance of his ciliary abnormality is discussed.

Child

Acute autonomic neuropathy following primary herpes simplex infection.

A 13-year-old girl presented with postural hypotension, severe abdominal pain and diarrhoea, parotid pain and a transient encephalopathy. There was evidence of an acute autonomic neuropathy and some electrophysiological evidence of a transient peripheral somatic neuropathy. The likely cause was primary herpes simplex infection.

Acute Disease

Neuropathic vesicourethral dysfunction in children. A trial comparing clean intermittent catheterisation with manual expression combined with drug treatment.

Forty-three children with overt neurological disease and neuropathic vesicourethral dysfunction were entered into a trial comparing clean intermittent catheterisation (CIC) with manual expression combined with drug treatment (non-CIC). The 22 children in the CIC group showed a significantly greater improvement in continence than the 21 children in the non-CIC group (P less than 0.001) without a significant increase in the incidence of urinary tract infections. Neither form of treatment is effective in reducing the risk of deterioration of renal function due to the combination of detrusor-sphincter dyssynergia and vesicoureteric reflux. Neither form of treatment improves continence in the presence of gross sphincter weakness or gross impairment of bladder compliance.

Bethanechol Compounds

Neurological complications of arterial hypertension.

During a 10-year period 45 children were identified as having had neurological complications associated with severe arterial hypertension. Convulsions were the most common complication, occurring in 42 (82%) children. Two (4%) children each presented with a facial palsy and 2 (4%) with alterations in the level of consciousness. Nineteen (42%) presented with epileptic seizures as the first sign of arterial hypertension. The prognosis for children having had a single episode of hypertensive encephalopathy was good. Long-term follow-up showed no permanent neurological deficit on physical examination, and no focal abnormality on brain scan by computerised tomography. Psychometric analysis similarly failed to show any significant difference in cognitive assessment between children having had an episode of hypertensive encephalopathy and a control group with chronic renal disease, although reading skills were generally behind for chronological age and the average IQ was about 90 in both groups.

Adolescent