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Biomedical subjects

D Calver

Publications and source records attributed to D Calver.

9 recordsLinked to original sources
PubMed2005

Visual failure without headache in idiopathic intracranial hypertension.

AIM: To determine the differences in outcome in a group of children with idiopathic intracranial hypertension (IIH) who do not present with headaches. METHODS: Differences in epidemiological and aetiological factors, clinical presentation, and visual outcome were investigated in children with a diagnosis of IIH presenting with and without headaches to the Paediatric Neurology and Paediatric Ophthalmology Services at Guy's & St Thomas' Hospitals NHS Trust between 1997 and 2002. RESULTS: Compared to the 29 children with headaches, the 12 children in the non-headache group were younger (7.3 v 9.5 years), presented with more neurological signs (33% v 10%), and were more likely to present with severe visual failure (33% v 4%), with a tenfold increased risk of an enlarged blind spot or field defects (50% v 5%). Permanent visual failure affected a third of all children in the non-headache group, but was rare in children presenting with headaches (33% v 3%), with one patient registered blind and two severely visually impaired. CONCLUSION: The management of IIH is difficult in the absence of headache. Visual surveillance is vital. These children were treated with an aggressive management programme to reduce cerebrospinal fluid pressure by repeated lumbar puncture, medication, and early surgical intervention if required. Non-invasive monitoring techniques might contribute to a better understanding of the natural history of IIH, improved management, and visual outcome.

Child↗
PubMed1996

Extra-articular features of benign joint hypermobility syndrome.

To define the phenotype of patients with benign joint hypermobility syndrome (BJHS), we studied 58 consecutive patients (mean age 37 yr) presenting to a rheumatology clinic and 30 controls. Patients underwent rheumatological and ophthalmic examination, hypermobility scoring, echocardiography, measurement of bone mineral density (BMD), and skin thickness, elasticity and light transmissibility. The median hypermobility score was 5/9 Beighton and 31/56 Contompasis. Eighteen (31%) patients complained of significant arthralgia. Six (10%) patients and two (7%) controls had mitral valve prolapse (MVP) (chi(2) = 0.27, P = NS). Neither MVP nor aortic diameters showed a correlation with hypermobility score. There was no significant reduction in BMD. There was a significant correlation between hypermobility and light transmissibility of the skin (r = 0.71, P < 0.0001 Contompasis; r = 0.47, P < 0.05 Beighton) and skin stretchiness (r = 0.49, P < 0.05 Contompasis; r = 0.39, P < 0.05 Beighton). On ophthalmic examination, 14 (41%) patients had upper eyelid laxity. Thus, patients with BJHS do not have an increased prevalence of significant cardiac, bone, skin or eye abnormalities, helping differentiate BJHS from other more serious hereditary disorders of connective tissue.

Adolescent↗
PubMed1993

Congenital sensory neuropathy in association with ichthyosis and anterior chamber cleavage syndrome.

Two patients with a congenital neuropathy are described. Both had atypical features including: ichthyosis and a mild anterior chamber cleavage syndrome. Both had severely reduced, or absent, sensation for light touch, vibration, position and temperature. Pain sensation was mildly reduced. There was some evidence of motor involvement but this was relatively minor compared with the sensory involvement. Nerve action potentials were small or absent and sural nerve biopsies showed almost complete absence of myelinated nerve fibres with multiple bundles of abnormally arranged axons and Schwann cell processes. These patients appear to have an undescribed syndrome in which the large sensory neurons and the anterior chamber of the eye did not develop properly. This may reflect a failure of migration, differentiation or proliferation of neural crest cells.

Action Potentials↗
PubMed1993

Irreversible blindness in systemic lupus erythematosis.

We describe a patient with systemic lupus erythematosis and renal involvement and who developed visual impairment in the absence of hypertension and anti-cardiolipin antibodies. This was attributed to occlusive vascular disease and choroidal infarction in the context of active lupus. Complete and irreversible blindness developed rapidly despite treatment and the patient died of cerebral haemorrhage 2 months after discharge.

Adult↗
PubMed1989

Cocaine, pregnancy, and postpartum intracerebral hemorrhage.

An increase in cocaine abuse by pregnant women has been associated with a range of maternal/fetal cardiovascular complications. Intracerebral hemorrhage has been reported as a cocaine-related complication, but has not previously been associated with pregnancy. We report a case of cocaine-associated intracerebral hemorrhage in the postpartum period which complicated the differential diagnosis and management of hypertension and seizures.

Adult↗
PubMed1986

Histopathology of mitochondrial cytopathy and the Laurence-Moon-Biedl syndrome.

Clinical and histopathological studies of two patients with distinctly different inherited juvenile retinal dystrophies indicate that the ocular defect in mitochondrial cytopathy involves the underlying pigment epithelium, whereas in the Laurence-Moon-Biedl syndrome the photoreceptor cells are primarily affected.

Child↗
PubMed1986

Oral vitamin E supplements can prevent the retinopathy of abetalipoproteinaemia.

Six patients with abetalipoproteinaemia are described who received large doses of oral vitamin E for between 12 and 18 years in addition to a low fat diet and supplements of the other fat soluble vitamins. The progressive retinopathy observed in untreated abetalipoproteinaemia was substantially modified and most probably prevented by this therapy. Angioid streaks were noted in one patient. Treatment with vitamin A alone did not prevent or arrest the progression of the retinal lesion.

Abetalipoproteinemia↗
PubMed1981

The extra digit. A pointer to the eye?

The syndrome associated with an extra digit which is commonly seen by the ophthalmologist is that of Laurence-Moon-Biedl, with its well-known association with retinal dystrophy. However, there are several other syndromes in which there is polydactyly and an ocular malformation. Many have colobomata and microphthalmos. Other syndromes show various orbital and eyelid abnormalities, such as ptosis, hypertelorism, and lateral displacement of the canthi. In three syndromes other than the Laurence-Moon-Biedl there is a retinal dystrophy. In Trisomy 13 there is a severe retinal dysplasia, and in Bloom's syndrome excrescences on Bruch's membrane have been described. There have been several cases of asphyxiating thoracic dystrophy (Jeune's syndrome) described with a retinal dystrophy, and we present a case of the closely-related Ellis-van Creveld syndrome with a retinal dystrophy. We also present a case with a chromosomal defect (an addition to the short arm of chromosome 2), polydactyly, and a retinal dystrophy.

Abnormalities, Multiple↗