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Biomedical subjects

B Kendall

Publications and source records attributed to B Kendall.

At least 73 records · Page 4Linked to original sources

Embolisation techniques in neuroradiology.

Embolisation is a definitive treatment for selected arteriovenous fistulas, aneurysms and both extra- and intra-axial angiomatous malformations. It is an effective emergency treatment for many cases of intractable epistaxis. It is useful prior to surgery to reduce blood loss from hypervascular tumours, including juvenile angiofibromas, paragangliomas and basal meningiomas, and from angiomas or soft tissue or of bone prior to excision, dental extraction or laminectomy. Embolisation is a satisfactory palliative treatment for angiomas involving superficial tissues, in which the cosmetic effects of surgery are unlikely to be satisfactory, and of mucosal surfaces causing intractable bleeding. In inoperable tumours, embolisation can relieve pain, bleeding, pulsatile tinnitus or discomfort due to mass effect.

Embolization, Therapeutic↗

Intracranial calcification in paediatric computed tomography.

An analysis of the computed tomograms of 18000 children examined consecutively form the basis of an assessment of the diagnostic significance of intracranial calcification. The low incidence of physiological calcification in the pineal and choroid of about 2% up to the age of 8 years, but increasing 5-fold by the age of 15 years, is confirmed. Pathological calcification occurred in 1.6%, the commonest causes being neoplasms (43%), neuroectodermal syndromes (20%) and infections (12%). Diffuse basal ganglia calcification (15%) bore little relation to the diverse clinical symptomatology, and routine biochemical studies showed a disorder of metabolism to be present in only 6 cases. Calcification has not been previously noted in acute haemorrhagic leukoencephalitis, Pertussis or Cocksackie encephalitis, infantile neuraxonal dystrophy, Marinesco-Sjögren syndrome or in the basal ganglia in neurofibromatosis.

Acute Disease↗

Iohexol in paediatric myelography. An open non-comparative trial.

Iohexol was introduced by lumbar puncture in a series of 148 consecutive children aged between 5 days and 16 years referred for myelography; no patient was excluded. Initially, iohexol 180 mgI/ml was used in dosage proportional to body weight varying between 5 ml and 15 ml. During the later part of the trial concentration of iodine was increased to 240 mg/ml for cases in which the dorsal region was of particular interest (69 patients) and to 300 mg/ml for 8 cervical studies. The total dose ranged up to 4.8 g and varied between 0.03 g and 0.51 gI/kg body weight. In all patients, neurological examinations were performed before and at 24 h and observations for adverse reactions continued over a period of 48 h. The contrast medium was run up to the foramen magnum or basal cisterns in 128 patients and to the upper dorsal region in the other 20. In the first 62 patients vital studies were performed over the period of the myelogram and for 24 h following, and an additional limited neurological examination was made at 6 h, and in the first 26 cases of the series EEG's were done before and at 24 h after the myelogram. Minor variations in pulse rate and blood pressure were observed but these were not of sufficient magnitude to be of clinical significance. In 7 patients there was minor, generally slow wave abnormality on the EEG taken after the procedure, but no spike or epileptogenic activity was observed. There were no unexpected or severe adverse reactions.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Spinal angiography with iohexol.

Iohexol 300 was used for 55 selective spinal angiograms in 50 patients; all were of good quality. There was no significant change in vital signs during the angiographic procedure in any case. Two patients with spinal dural arteriovenous fistula draining into a coronal venous plexus developed transient subjective slight increase in their disability during the immediate post-angiographic period. Eight cases embolised at the time of angiography showed improvement in their neurological deficits within 24 h of the study. The other patients were unaffected by the procedure. Iohexol 300 is a very suitable contrast medium for spinal angiography.

Adolescent↗

High density on computed tomography in infantile Krabbe's disease: a case report.

In a child with a compatible clinical presentation and course of illness, Krabbe's disease was proven by white blood-cell galactocerebrosidase estimation in the homozygous range. A CT head scan performed at five months of age revealed symmetrical high density in the thalami, posterior limbs of the internal capsules and corona radiata. It also showed low density in the deep white-matter of the cerebral and cerebellar hemispheres and minor cerebral atrophy. Similar CT findings have been recorded in four other cases of infantile Krabbe's disease, and the authors consider that they are sufficient to alert the clinician to the probability of this diagnosis when the clinical signs are appropriate.

Atrophy↗

High resolution CT scanning of the pituitary gland in growth disorders.

We have performed 217 GEC 8800 CT scans of the hypothalamus and pituitary glands of 202 children with disorders of growth and development. Pituitary morphological abnormalities were common. Intrapituitary low density lesions were found in 17% of the whole series and in 58% of children with tall stature. Seventy-seven children with idiopathic growth hormone deficiency could be divided on the basis of pituitary morphology seen on CT scan into pituitary aplasia (n = 11) and pituitary hypoplasia (n = 53). Patients with pituitary aplasia had an absent adenohypophysis which probably dated from early intrauterine life and therefore could not be related to birth trauma. We have found a high incidence of evolving endocrinopathy in children with pituitary insufficiency: thus, if a short child is investigated the initial endocrine findings need to be repeated as the pattern of pituitary insufficiency changes with time. An evolving endocrinopathy starting in later childhood is suggestive of the presence of a cerebral tumour. Children with subnormal growth velocities and a normal growth hormone response to pharmacological tests have a wide spectrum of pituitary morphological abnormalities which may be associated with growth hormone neurosecretory dysfunction.

Adolescent↗

Dysgenesis of the corpus callosum and hypopituitarism.

A high resolution CT scan demonstrated the unexpected finding of dysgenesis of the corpus callosum in addition to the expected finding of pituitary hypoplasia in three children who presented with growth failure secondary to hypopituitarism. Several of the clinical features reported in association with dysgenesis of the corpus callosum may be ascribed to hypothalamo-pituitary dysfunction. These children emphasize the need to assess the endocrine function of patients with midline structural defects. It should not be assumed that the clinical manifestations are related solely to the anatomical abnormality.

Agenesis of Corpus Callosum↗

Clinical role of pteridine therapy in tetrahydrobiopterin deficiency.

In most patients with deficiency of tetrahydrobiopterin (BH4) continuous administration of BH4 or of a synthetic analogue such as 6-methyltetrahydropterin (6-MPH4) lowers plasma phenylalanine concentrations to the therapeutic range. The effective dose of BH4 varies from 1 to 2 mg kg-1 daily in patients with defective biopterin synthesis, to 5 mg kg-1 or more in patients with dihydropteridine reductase (DHPR) deficiency. The cost of 2 mg kg-1 day-1 of BH4 is comparable to the cost of a low phenylalanine diet. Higher doses of pterins given orally (20 mg kg-1) raise the levels of tetrahydropterin in cerebrospinal fluid (CSF) to normal in patients with defective biopterin synthesis in whom initial concentration of biopterin species are low. In some, but not all, such patients pterin therapy also raises CSF amine metabolite concentrations and ameliorates symptoms. High dose therapy does not appear to be effective in raising CSF pterin levels in patients with DHPR deficiency who already accumulate dihydrobiopterin (BH2) in CSF. Central folate deficiency is an additional cause of neurological deterioration in patients with DHPR deficiency who require supplementation with folate as folinic acid. It is suggested that the accumulation of BH2 in such patients competitively interferes with folate metabolism.

Amino Acid Metabolism, Inborn Errors↗

Multiple sclerosis in childhood: a new look.

Five patients (four female, one male) with multiple sclerosis were managed at the Hospital for Sick Children, London, between January 1978 and June 1983. The age at which first symptoms occurred varied from three to 14 years. Focal seizures occurred in three cases and an encephalopathic process in three cases, and there was evidence of progressive intellectual deterioration in all five children. Computed tomography showed low-density lesions in the white matter of all five. Electroencephalograms were abnormal in all cases, and in two the disturbance was severe. Oligoclonal bands were present in the cerebrospinal fluid of two children. Four of the five children had abnormal responses to pattern-reversal stimulus.

Adolescent↗

Intracerebral haemorrhage complicating dural arteriovenous fistula: a report of two cases.

Two patients presented with unilateral peri-orbital pain, proptosis, chemosis and external ophthalmoplegia. They were shown to have dural arteriovenous fistulae related to the cavernous sinus. Intracerebral haemorrhage occurred in both patients within 18 months of presentation; this gave rise to focal seizures and signs of unilateral hemisphere dysfunction. The haematomas were in the region drained by the superficial middle cerebral vein ipsilateral to the shunt and are presumed to have been the result of locally raised venous pressure.

Aged↗

Cerebral angiography in vasculitis affecting the nervous system.

Of 199 patients with clinico-laboratory evidence of vasculitis, 42 were submitted to cerebral angiography; 35 angiograms were abnormal, but only 21 were characteristic of the particular disease process. A pattern consistent with vasculitis diagnosed in 19 angiograms, was due to neoplasm in 4 and neurofibromatosis, DXT and amphetamines each in 1 case. Moya-Moya type collaterals shown in 20 angiograms were due to the idiopathic disease in 10 but due to non-inflammatory pathologies in 7. Fibromuscular dysplasia shown in 28 angiograms was an incidental finding in 20 of these. Of 56 cerebral angiograms for ischaemic stroke in patients under 15 years old, 17 were normal and only 8 vascular lesions were likely to have been of inflammatory origin. In routine clinical practice cerebral angiography, though essential in the management of mycotic aneurysm and in the diagnosis of Moya-Moya disease isolated vasculitis and fibromuscular dysplasia, plays only a limited role in the diagnosis of other types of vasculitis.

Adolescent↗

Cervical myelography with iohexol.

An open, non comparative study of cervical myelography in 68 adult patients using iohexol (Omnipaque) containing 300 mg I/ml is reported. Satisfactory visualisation was achieved in all cases. Minor adverse effects occurred in 13 patients (19%); they were headache (8), neck or back pain (3), vomiting (3), nausea (1), dizziness (1) and nystagmus (1) and were of minor degree in most and moderate in a few, lasting more than 24 hours in only one patient. EEG performed in 39 patients before and 24 h after the myelogram showed no seizure activity or significant change. Iohexol is a very satisfactory drug for all types of myelography.

Adolescent↗

Dural arteriovenous malformations of the spine. Clinical features and surgical results in 55 cases.

The clinical and angiographic findings of 55 patients with a spinal dural arteriovenous malformation (AVM) are reviewed, and the results of surgery assessed. The symptoms of dural AVM are usually gradual in onset, and hemorrhage from this type of AVM is less common than in true spinal cord angiomas. Other clinical features and the myelographic findings are similar to those of spinal cord angiomas. On angiography, the nidus of dural AVM's usually projected lateral to the spinal cord. Clipping of communicating vessels between the AVM and the coronal plexus was carried out in 50 patients, and decompressive laminectomy only in five cases. Surgery led to improvement of disturbed gait or arrest of a previously progressive course in 85% of those managed by clipping communicating vessels. The pathophysiology and surgical treatment of dural AVM's are discussed.

Adult↗

Diagnosis of masses presenting within the ventricles on computed tomography.

The radiological and clinical features of 90 histologically verified intraventricular masses were reviewed. Computed tomography (CT) and plain X-rays were available in all and angiograms in over half the cases. The localisation, effects on the adjacent brain substance and the presence and degree of hydrocephalus was evident on CT. Two-thirds of colloid cysts presented as pathognomonic anterior third ventricular hyperdense masses and the other third were isodense; an alternative diagnosis should be considered for low density masses in this situation. Plexus papillomas and carcinomas mainly involved the trigone and body of a lateral ventricle of young children and caused asymmetrical hydrocephalus; the third ventricle was occasionally affected also in children and the fourth ventricle more frequently and usually in adults. Two-thirds were hyperdense, one-third of mixed or lower density. The meningiomas were dense trigonal tumours of adults generally arising in the choroid plexus, but two tentorial meningiomas passed through the choroidal fissure and caused a predominantly intraventricular mass. Gliomas frequently thickened the septum and generally involved the frontal segments of the lateral ventricles. They may be supplied by perforating as well as by the choroidal arteries, which supply most other vascularised masses within the ventricles. Only 10% of our cases did not fall into one of the former categories; these included low density non-enhancing dermoid or epidermoid tumours and higher density enhancing metastatic or angiomatous masses.

Adolescent↗