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Biomedical subjects

R D Hoare

Publications and source records attributed to R D Hoare.

At least 19 recordsLinked to original sources
PubMed1995

Allogeneic bone marrow transplantation for fucosidosis.

Bone marrow transplantation was performed on an 8-month-old boy who was diagnosed as having fucosidosis following the diagnosis of the disease in his older brother. Although he was asymptomatic and his development was normal, abnomalities were found on an MRI scan prior to transplant. In the absence of a suitable related donor, an unrelated volunteer donor was used. Conditioning for the transplant consisted of busulphan and cyclophosphamide. Graft-versus-host disease prophylaxis consisted of in vitro T cell-depletion of the bone marrow and in vivo administration of cyclosporin. The post-transplant period was complicated by moderately severe graft-versus-host disease. Engraftment was documented by the presence of donor levels of alpha-fucosidase, donor blood group and tissue type (difference in the DQ antigen), and chromosomal polymorphism pattern of donor origin. Eighteen months after transplant, there is evidence of mild neurodevelopmental delay. By contrast, his elder sibling showed far greater developmental delay at the same age. The patient's MRI scan shows improvement. We believe this to be the first case of human fucosidosis treated by bone marrow transplantation.

Bone Marrow Transplantation↗
PubMed1992

The evaluation of patients with a suspected pituitary microadenoma: computer tomography compared to magnetic resonance imaging.

OBJECTIVES: To perform a prospective study to compare the ability of magnetic resonance imaging (MRI) and computer assisted tomography (CT) to predict the position of a tumour within the pituitary fossa and to assess the clarity of the image generated by the two modalities in patients with suspected pituitary microadenomas. DESIGN: Nineteen patients were investigated with CT and MRI scans and underwent trans-sphenoidal exploration of the pituitary fossa. Both scans were assessed blind and independently by three neuroradiologists, A, B, and C; A and B examined the CT scans and B and C the MRI scans. The predicted site was then compared to the size and position of the tumour at surgery. PATIENTS: Twenty-five patients were recruited and had both CT and MRI scans, 19 subsequently underwent trans-sphenoidal exploration of the pituitary fossa and formed the study population. RESULTS: The joint opinions of A and B of the CT scans were correct in 10/19, and those of B and C of the MRI scans were correct in 17/19 cases, P = 0.008. Observer agreement was used to assess the clarity of the image. A and B agreed about the site of the tumour on the CT scan in 14 of 19 cases (Kappa statistic 0.556); B and C agreed on the MRI scans in 19/19 cases (Kappa statistic 1), P = 0.025. After the exclusion of five cases found to be macroadenomas at surgery, the joint opinions of A and B of the CT scans were correct in 8/14, and those of B and C of the MRI scans were correct in 12/14 cases, P = 0.133. A and B agreed about the site of the tumour on the CT scan in 12/14 cases (Kappa statistic 0.653), B and C agreed on the MRI scans in 14/14 cases (Kappa statistic 1, no significant difference). CONCLUSIONS: In the assessment of suspected pituitary microadenomas MRI is the imaging technique of choice in view both of its greater diagnostic accuracy and the avoidance of radiation exposure.

Adenoma↗
PubMed1990

The role of diencephalic pathology in human memory disorder. Evidence from a penetrating paranasal brain injury.

A patient (B.J.) is reported who developed severe memory impairment following a penetrating brain injury caused by a snooker cue which entered through his left nostril into the basal regions of the brain. Initially, his memory disorder had the clinical features of a dense amnesic syndrome, with both anterograde and retrograde amnesia, but B.J. subsequently showed significant recovery of memory function. Formal memory testing was carried out 21 months after injury. This demonstrated marked verbal memory impairment, as severe as that seen in patients with the amnesic syndrome. On nonverbal memory tests, his impairment was relatively mild and patchy. His retrograde amnesia had regressed mainly to affect a 6 month period before the injury. On other cognitive tasks, he performed at an average or above average level, and there was no neuropsychological evidence of frontal lobe dysfunction. Neuroradiological investigations at various stages after his injury failed to demonstrate a lesion in any of the thalamic nuclei. Magnetic resonance imaging showed a lesion in the hypothalamus in the region of the mamillary bodies. Our study demonstrates that marked, relatively focal, memory disorder after diencephalic injury can occur without direct pathology to the body of the thalamus. It also indicates that structures in or adjacent to the hypothalamus, such as the mamillary bodies, may play a more important role in human memory functioning than has hitherto been considered.

Adult↗
PubMed1989

Clinical correlation of CT changes in 64 Chinese patients with tuberculous meningitis.

CT changes in 64 Chinese patients with proven tuberculous meningitis were compared with the clinical features at diagnosis. In patients presenting with advanced disease, hydrocephalus and cerebral infarction were common, and the hydrocephalus in these patients was more likely to require surgery at a later date for control of raised intracranial pressure. New infarctions were only seen in follow-up scans of patients who had presented with disturbed consciousness. Meningeal enhancement, the commonest finding, and intracranial tuberculomata were not related to the severity of disease at presentation. A normal scan in a drowsy patient virtually excludes the diagnosis of tuberculous meningitis.

Adolescent↗
PubMed1984

Computed tomography appearances in the linear sebaceous naevus syndrome.

The appearance of C.T. of the head in 11 cases of the linear sebaceous naevus syndrome are presented. From these it is argued that the pathology represents a disorder of neuronal migration and organisation rather than differentiation. Since three of the cases had evidence of CNS tumour in addition to two in the literature, it would appear that a further aspect of this disorder is a failure of growth constraint. This is paralleled by a similar tendency in the skin lesion which should be considered premalignant. Normal intelligence is rare in this condition and co-exists only with a normal C.T. scan. The early onset of fits is not necessarily associated with subsequent mental handicap.

Brain↗
PubMed1984

Clinico-anatomical correlations in uncomplicated stroke.

A method for making clinico-anatomical correlations by computer superimposition of brain maps derived from CT scan images, described in a companion paper, was tested in 19 patients with an uncomplicated left hemiparesis due to acute stroke. Eight patients with an isolated left hemiparesis had small lesions in the most rostral part of the right internal capsule. The composite lesion maps of 11 patients whose left hemiparesis was complicated only by spinothalamic sensory loss showed a larger area of damage more caudally in the posterior part of the posterior limb of the capsule, abutting the thalamus. The close correlation between the results obtained by this method and those obtained in previous clinico-pathological studies of patients with uncomplicated strokes, confirms the validity of this mapping technique.

Basal Ganglia↗
PubMed1984

A microcomputer assisted method for obtaining composite pictures of focal brain damage.

A method used to produce composite drawings of superimposed areas of brain infarction is described. The method uses a microcomputer interfaced to a digitising pad and linear plotter. Areas of brain damage are drawn on standardised maps, digitised, manipulated in computer memory, and summated using computer graphics facilities. The composites show the position of brain damage in groups of patients as a contour map of the extent of lesion overlap. The method can be used to study neurological and neuropsychological deficits using CT-scan, or other imaging techniques. Deep and superficial lesions may be displayed equally well.

Cerebral Infarction↗
PubMed1983

Childhood moyamoya presenting as dementia: report of a case.

A case is presented of moyamoya disease in an Anglo-Saxon child whose principal clinical feature, uniquely, was dementia. CT findings were unexplainable so angiography was performed. Moyamoya is an angiographic entity which has been associated with a wide variety of conditions. No satisfactory hypothesis has yet emerged to link these conditions or to explain the predilection of the Japanese for this disease.

Arterial Occlusive Diseases↗
PubMed1982

Early diagnosis of herpes simplex encephalitis in childhood. Clinical, neurophysiological and neuroradiological studies.

In the initial phase of HSE the clinical symptomatology is more variable and insidious in babies and young children than in older children and adults. Combined clinical, neurophysiological and neuroradiological studies have been carried out in 12 children with proven HSE. Ten patients had the first EEGs taken during the acute phase of the illness and all showed large amplitude irregular slow activity, sharp waves and often spikes with variable distribution; in 7 cases periodic phenomena were recognisable. At a later stage localised low amplitude EEG activities were found in children with focal neurological symptoms. Areas of low attenuation were seen in the CT scans of the 7 children who had this investigation done at an early stage of their illness. Such low density regions persisted at follow-up and eventually cerebral atrophy with irregular features became obvious. Prompt EEG investigations combined with CT scans provide an early diagnostic clue for treatment. Follow-up EEG studies (including VEP) and CT scans may help assess the severity of residual cerebral damage in the survivors.

Adolescent↗
PubMed1982

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↗
PubMed1982

Value of computerised tomography in children with non-specific mental subnormally.

Seventy-six children had computerized tomography scans as part of an investigation of mental subnormality; most of them are severely retarded and all those over age 5 years attend special schools. Seventy-two per cent of the children had normal scans. Twenty per cent showed cerebral atrophy and in only 8% was there a specific abnormality (agenesis of corpus callosum, arachnoid cyst, communicating hydrocephalus). None of these findings had any positive prognostic implication. Sedation or general anaesthesia was required for all except one child. Injection pethidine compound was used for children under age 5 years or less than 30 kg in weight, and trimeprazine orally was used for older children. The radiation exposure was high--5.6 rad per scan, which is 100 times greater than that from a posteroanterior and lateral chest x-ray film. For these reasons computerised tomography scans cannot be recommended as a routine part of the investigation of children with non-specific mental subnormality.

Adolescent↗
PubMed1978

Spinal arachnoid cysts in children.

Five cases of benign spinal arachnoid cysts in children are described with their clinical, neuroradiological, surgical and pathological findings. Intraspinal benign arachnoid cysts may be confidently diagnosed myelographically when there is complete or partial obstruction with multiple oily contrast/cerebrospinal fluid levels as in 3 cases in this series. The multiple fluid levels are probably produced by multiple cysts or a cyst loculated due to partial compression by arachnoid bands either related to the normal septum posticum, congenital or acquired adhesion rather than to trabeculations within the cyst. In this series, the origin of the cysts is considered to be congenital.

Adolescent↗
PubMed1976

Radiographic measurements of the normal spinal cord in childhood.

110 normal air myelograms of children were reviewed and the ratio of the spinal cord width to the subarachnoid space width (cord/SAS ratio) was calculated in the sagittal and transverse planes. This ratio proved to be independent of age and sex. The mean values at different vertebral levels are in close agreement with those previously reported for adults. The cord/sas ratio is helpful in evaluating changed cord dimensions, especially that of cord atrophy.

Adolescent↗
PubMed1976

Sturge-Weber syndrome with bilateral intracranial calcification.

Four children affected by Sturge-Weber syndrome and demonstrating bilateral intracranial calcification are described, bringing up to 21 the number of similar reported cases. The frequency of bilateral hemisphere involvement in this syndrome is not known, but it might be as high as 15%. If present, neurosurgical intervention is, in our opinion, contraindicated.

Adolescent↗
PubMed1975

Incomplete, false, and true diastematomyelia: radiological evaluation by air myelography and tomography.

Six cases of possible diastematomyelia studied by air myelography with tomography were thought to involve fibrous or bony septa, but in each case no septum was found. Surgical findings included prominent midline posterior fissures in the cord associated with fibrous bands, an unusual tentlike extension of the arachnoid over the spinal cord, and hemicords with no intervening septum. Recognition of an osseous septum on the plain film does not necessarily mean that the cord is completely divided by bone, as other structures derived from the mesoderm, such as fat or cartilage, may contribute to the septum. The importance of defining tethering of the cord posteriorly at the septum or conus level is stressed. Air myelography with appropriate projections appears to show posterior fixation of the cord to best advantage.

Child↗