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Biomedical subjects

D P Kingsley

Publications and source records attributed to D P Kingsley.

At least 19 recordsLinked to original sources

Brain structure and neurocognitive and behavioural function in adolescents who were born very preterm.

BACKGROUND: Infants born very preterm (<33 weeks) are at increased risk of neurocognitive deficits. Their neurodevelopmental outcome up to age 8 years can be predicted by neonatal ultrasonography, but little is known of their later function. We investigated the effect of very preterm birth on brain structure and neurocognitive and behavioural functioning in adolescence. METHODS: A cohort of 105 infants born before 33 weeks of gestation in 1979-80 had ultrasonographic scans at University College Hospital, London, and were prospectively examined at 1, 4, and 8 years. At age 14-15 years, 72 of those who remained in UK (cases) and 21 age-matched full-term controls underwent brain magnetic resonance imaging (MRI), as well as neurological, cognitive, and behavioural assessment. MRI images were assessed by two neuroradiologists unaware of ultrasonographic findings or case or control status. FINDINGS: Of the 72 cases, 40 had unequivocally abnormal MRI and 15 had equivocal scans. Of the 21 controls, one had abnormal and five equivocal MRI. Abnormalities of ventricles, corpus callosum, and white matter were especially common in cases. More brain lesions were identified by MRI than by neonatal ultrasonography. The cases had significantly more reading, adjustment, and neurological impairments than controls, but their behaviour was significantly related to MRI abnormality. INTERPRETATION: Individuals born very preterm show an excess of neurocognitive and behavioural problems in adolescence, and more than half have abnormal MRI brain scans.

Adolescent↗

Quantitative MRI in patients with clinically isolated syndromes suggestive of demyelination.

OBJECTIVE: To assess the long-term predictive value of quantitative lesion load measurement on brain MRIs in patients after a 10-year follow-up who presented initially with a clinically isolated syndrome of the optic nerve, brainstem, or spinal cord. BACKGROUND: Quantitative MRI measurement is being used in treatment trials as a surrogate marker in MS, but there is a lack of long-term MRI follow-up data in assessing the natural course of the disease from the earliest stages. METHODS: Using a semiautomated threshold technique, the total lesion volume (TLV), the course of the disease, and disability were assessed in 58 patients at onset and after 5 and 10 years. RESULTS: The TLV at presentation correlated significantly (r = 0.81, p = 0.0001) with the TLV and also with the Expanded Disability Status Scale (EDSS) score (r = 0.45, p = 0.001) at 10-year follow-up. In contrast there was no correlation of the TLV at 5 years with subsequent change in EDSS score over the next 5 years (r = 0.18, p = 0.12). The change in TLV over the first 5 years in patients who developed clinically definite MS (CDMS) differed significantly according to the type of disease course (relapsing-remitting with disability, secondary progressive, or benign) manifesting at 10-year follow-up. CONCLUSION: Quantification of changes detected by T2-weighted brain MRI at the earliest clinical stages is strongly predictive of the subsequent development of CDMS as well as the clinical course and level of disability 10 years later.

Adolescent↗

The prognostic value of brain MRI in clinically isolated syndromes of the CNS. A 10-year follow-up.

A definitive diagnosis of multiple sclerosis cannot be made at presentation on patients with a clinically isolated syndrome of the optic nerve, spinal cord or brainstem suggestive of demyelination, as dissemination in time is not established. To determine the long-term risk of abnormalities on brain MRI for the development of multiple sclerosis and disability we performed a 10-year follow-up on 81 such patients who had T2-weighted brain MRI at presentation. Initial brain MRI was abnormal in 54 (67%). Follow up of those patients with an abnormal MRI revealed progression to clinically definite multiple sclerosis in 45 out of 54 (83%), of whom 11 (20%) had relapsing/remitting disease (EDSS > 3), 13 (24%) secondary progressive and 21 (39%) benign (relapsing/remitting with EDSS < or = 3) disease. For those with a normal MRI progression to clinically definite multiple sclerosis occurred in only three out of 27 (11%), all benign. There was a significant relationship between the number of lesions at presentation and both EDSS (r = 0.45, P < 0.001) and the type of disease at follow-up (P < 0.0001). Brain MRI at presentation with a clinically isolated syndrome is predictive of the long-term risk of subsequent development of multiple sclerosis, the type of disease and extent of disability.

Adolescent↗

Cognitive deficits associated with frontal-lobe infarction in children with sickle cell disease.

This study examined the cognitive manifestations of frontal-lobe infarction in a population of children with sickle cell disease (SCD). Forty-one patients with SCD underwent MRI. Five patients with stroke symptoms had large infarcts encroaching on the tissue of the frontal lobes. Four patients without symptoms had smaller frontal-lobe infarcts. The patients with stroke were significantly impaired on measures of intelligence, memory, and frontal-lobe function (Wisconsin Card Sorting Test, WCST) compared with both the patients with normal MRI scans (N=30) and a group of sibling controls (N=15), who did not differ from each other. Patients with covert infarction obtained scores on the intelligence tests and the WCST that fell in between those of the stroke patients and the other two groups. This trend toward impairment suggests that patients with covert infarction are at similar risk for cognitive deficits to those with stroke.

Adolescent↗

Offspring recurrence rates and clinical characteristics of conjugal multiple sclerosis.

BACKGROUND: There has been no previous systematic study of conjugal multiple sclerosis. This study of conjugal pairs with complex traits investigated disease transmissibility and the genetic contribution to frequency and clinical course. METHODS: We studied 45 conjugal pairs concordant for multiple sclerosis from 58 pairs recorded in a national register of familial disease, 86 offspring of the 45 pairs were individually assessed for clinical evidence of neurological disease; those over age 16 underwent cranial magnetic resonance imaging. Clinical features were compared in 33 pairs in whom neither member had symptoms before they met. FINDINGS: Of the 86 offspring, five (6%) had clinically definite multiple sclerosis. A further five children had either characteristic imaging abnormalities or clinical symptoms consistent with demyelination, but did not meet the criteria for clinically definite disease. There was no evidence of clinical concordance, clustering at year of onset, or distortion of the expected pattern of age of onset in the second affected spouse from 33 pairs. The crude recurrence in children of conjugal pairs (1 in 17) is significantly higher than previously reported population-based risk for offspring of single affected parents (1 in 200). INTERPRETATION: Taken with the low prevalence of multiple sclerosis in the spouses of affected individuals, and the lack of concordance for age at onset in these families, the disparity in crude recurrence between children of conjugal pairs and those of single affected parents shows that the recurrence risk in children is determined by genetic factors inherited from both parents.

Adolescent↗

"Dural tail" adjacent to a giant posterior cerebral artery aneurysm: case report and review of the literature.

The "dural tail" sign on gadolinium (Gd-DTPA)-enhanced MRI has been described in association with meningiomas. Various series with histopathological correlation have shown that in some cases there is tumour invasion into the dura mater, but in the majority of cases it represents a hypervascular, non-neoplastic reaction. While this sign was originally thought to be specific for meningioma, subsequent case reports have described the presence of a dural tail in other intra- and extra-axial lesions. We present a patient with a giant aneurysm arising from the P2 segment of the right posterior cerebral artery, adjacent to the tentorium, with a prominent dural tail on Gd-DTPA-enhanced MRI. In this location, differentiation of an aneurysm from a meningioma was critical.

Adult↗

MRI in children receiving total parenteral nutrition.

Cranial MRI was obtained in 13 of a group of 57 children receiving long-term parenteral nutrition, who were being investigated for hypermanganasaemia. Increased signal intensity on T1-weighted images has been reported in adult patients on long-term parenteral nutrition and with encephalopathy following chronic manganese exposure in are welding. It has been postulated that these changes are due to deposition of the paramagnetic trace element manganese. In excess manganese is hepato- and neurotoxic and we present the correlation of whole blood manganese levels with imaging findings. The age range of our patients was 6 months to 10 years, and the duration of therapy 3 months to 10 years. In 7 children we found characteristic increased signal intensity on T1-weighted images, with no abnormality on T2-weighted images. All patients had elevated whole blood manganese levels, suggesting that the basis for this abnormality is indeed deposition of manganese within the tissues.

Brain↗

Clinical, CSF, and MRI findings in Devic's neuromyelitis optica.

OBJECTIVES: Since Devic's original description of neuromyelitis optica in 1894 there has been much debate regarding its aetiology. A specific cause has been identified in a minority of cases but in most the question has arisen whether or not Devic's neuromyelitis optica is a variant of multiple sclerosis. This study was undertaken to help clarify this issue. METHODS: Neuromyelitis optica was defined as (1) a severe transverse myelitis; (2) an acute unilateral or bilateral optic neuropathy; (3) no clinical involvement beyond the spinal cord or optic nerves, and (4) a monophasic or multiphasic illness. The clinical and autoantibody status was documented. Patients underwent CSF examination and MRI of brain and spinal cord. RESULTS: Twelve patients, with a mean age of presentation of 35.1 years, were seen. Eleven were women; vision was reduced to counting fingers or worse in 10 patients and seven became confined to a wheelchair. Examination of CSF showed local synthesis of oligoclonal bands in only two patients and a neutrophil pleocytosis in two. A possible aetiology was identified in five: a specific connective tissue disorder (two), pulmonary tuberculosis (one), and possible acute disseminated encephalomyelitis (two). Six had non-specific increases in various autoantibodies. Eleven patients underwent MRI of the brain and spinal cord. In 10 there were diffuse abnormalities involving cervical and thoracic cords with extensive swelling in the acute phase. Brain MRI was normal in five; in five there were multiple deep white matter lesions, and one patient had minor age related changes. CONCLUSION: It is proposed that Devic's neuromyelitis optica is a distinctive disorder with some clinical, CSF, and MRI features different from those found in classic multiple sclerosis. In most cases a specific aetiology is not identified, but an immunological mechanism of tissue damage seems likely.

Adolescent↗

Clinical and magnetic resonance imaging predictors of disability in primary and secondary progressive multiple sclerosis.

The role of magnetic resonance imaging (MRI) in predicting disability in multiple sclerosis (MS) remains unclear. In this study 21 patients with primary and secondary progressive MS were reviewed 5 years following a serial MRI study of 6 months duration. In the secondary progressive group (n = 11) there was a significant relationship between the occurrence of enhancing lesions and clinical relapses during the initial 6 months and increase in disability 5 years later. For both groups change in disability over the initial study period was predictive of outcome. These results suggest that the presence and frequency of gadolinium enhancement (a marker of inflammation) and changes in disability over a short period are predictive of future deterioration in progressive patients.

Adult↗

Magnetic resonance spectroscopic study of parkinsonism related to boxing.

Proton magnetic resonance spectroscopy, localised to the lentiform nucleus, was carried out in three ex-professional boxers who developed a parkinsonian syndrome, six patients with idiopathic Parkinson's disease, and six age matched controls. The three ex-boxers all showed a pronounced reduction in the absolute concentration of N-acetylaspartate compared with the patients with idiopathic Parkinson's disease and the control group. This reduction is likely to reflect neuronal loss occurring in the putamen and globus pallidus and supports the hypothesis that the extrapyramidal syndrome that may occur in ex-boxers is a distinct entity from idiopathic Parkinson's disease.

Aged↗

The role of imaging in the follow up of meningiomas.

A retrospective study of 60 patients with meningiomas was conducted to evaluate the role of imaging in postoperative follow up. Using case notes and imaging studies, requests were assessed with reference to the indications for imaging radiological findings and effect on patient management. Patients were divided into three groups: 34 who had undergone a macroscopically complete resection, 18 with known residual tumour, and eight in whom surgery was not performed. These 60 patients underwent a total of 165 CT and 11 MRI studies. In the complete resection group only two patients developed a recurrence, both having highly suggestive symptoms or signs. It is concluded that routine imaging is not indicated in asymptomatic patients after complete tumour clearance. Both CT and MRI contribute to patient management in those with residual disease, MRI probably being the imaging method of choice.

Adult↗

Isolated neurodevelopmental delay in childhood: clinicoradiological correlation in 170 patients.

CT findings on 170 patients presenting with isolated moderate to severe neurodevelopmental delay have been compared with the final diagnosis. MRI was undertaken in 29 patients. Eighty per cent of the patients remained undiagnosed, and although the MRI findings were abnormal in 65.5% compared with only 30% of the CT examinations, imaging uncommonly suggested a specific diagnosis. Biochemical and chromosomal investigations were significantly more diagnostic. The results of these studies should restrict the number of non-contributory neuroradiological examinations. The superior intrinsic contrast of MRI will detect more lesions, particularly in white matter, but these are rarely diagnostic and where access to MRI is limited, CT is usually adequate.

Adolescent↗

EEG features of cortical dysplasia in children.

Two distinctive electroencephalographic abnormalities, very high amplitude rhythmic activity or prominent fast activity, have been described in children with extensive cortical dysplasia. Cases with cortical dysplasia identified on computerised tomography or magnetic resonance imaging, or a characteristic EEG were selected. One hundred and forty electroencephalograms from 94 cases were reviewed and related to the imaging findings. An EEG with very high amplitude rhythmic activity was found to have high specificity for severe cortical dysplasia but low sensitivity (< 50% cases). Abnormal fast activity was not specific and was seen with very diverse pathologies. The EEG features of most cases with localised cortical dysplasia were very variable. The EEG could be normal even when the cortical dysplasia was extensive.

Cerebral Cortex↗

The significance of brain magnetic resonance imaging abnormalities at presentation with clinically isolated syndromes suggestive of multiple sclerosis. A 5-year follow-up study.

A 5-year follow-up study was performed on 89 patients who had undergone brain magnetic resonance imaging (MRI) at presentation with an acute clinically isolated syndrome of the optic nerves, brainstem or spinal cord of a type suggestive of multiple sclerosis. At presentation, MRI was abnormal, revealing one or more asymptomatic cerebral white matter lesions in 57 (64%), and was normal in 32 (36%). At follow-up, progression to clinically definite multiple sclerosis had occurred in 37 out of 57 (65%) with an abnormal MRI and one out of 32 (3%) with normal MRI. Human leucocyte antigen (HLA) typing was performed in 70 patients and cerebrospinal fluid (CSF) was examined at presentation in 36. The presence of HLA-DR2 antigen or cerebrospinal fluid oligoclonal IgG bands were both associated with a significantly increased risk of progression to multiple sclerosis, but MRI was much more powerful in predicting outcome. The presence of four or more MRI lesions at presentation was associated with a higher rate of progression to multiple sclerosis, more frequent development of moderate or severe disabilities and a greater number of new MRI lesions at follow-up. The results indicate that brain MRI at presentation with a clinically isolated syndrome suggestive of multiple sclerosis is a powerful predictor of the clinical course over the next 5 years. This observation, combined with an ability to detect other sometimes treatable disorders which can also cause such syndromes, suggests that MRI is the investigation of choice in evaluating this group of patients.

Adolescent↗

MRI in chronic spinal cord trauma.

Eighty-seven patients aged 16-68 years have been examined by magnetic resonance imaging (MRI) following spinal injury. The MRI findings have been correlated with length of history between trauma and investigation, extent of residual function and site of injury. They include changes at the site of injury consistent with myelomalacia in 37%, a syrinx in 40%, persistent cord compression in 32% and atrophy in 18%. An extensive syrinx can develop within 2 months of injury and it is nearly twice as common in patients with complete paralysis as in those whose paralysis was incomplete. It is suggested that investigation and management of spinal trauma should include early and repeated MRI examinations to detect sequelae at an early stage.

Adolescent↗

Major differences in the dynamics of primary and secondary progressive multiple sclerosis.

In patients with primary and secondary progressive multiple sclerosis (MS), major differences in the pattern and extent of abnormality on cerebral magnetic resonance imaging (MRI) between the two groups have recently been demonstrated. In the present study, 24 patients, matched for age, sex, duration of disease, and disability, had serial gadolinium diethylenetriaminepentaacetic acid-enhanced MRI over a 6-month period. The 12 patients in the secondary progressive group had a total of 109 new lesions over this time (18.2 lesions per patient per year) and 87% of these enhanced. Enhancement also occurred within and at the edge of preexisting lesions. In contrast, only 20 new lesions were seen in the primary progressive group (3.3 lesions per patient per year) and only one of these enhanced. There was no difference in the degree of clinical deterioration between the two groups over the 6-month period. These findings may indicate a difference in the dynamics of disease activity between the two forms of progressive MS, particularly in relation to the inflammatory component of the lesions, and have important implications for the selection of patients and the monitoring of disease activity in therapeutic trials.

Adult↗