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Biomedical subjects

C E Clarke

Publications and source records attributed to C E Clarke.

At least 55 records · Page 3Linked to original sources
PubMed1999

Inherited prion disease with an alanine to valine mutation at codon 117 in the prion protein gene.

A large English family with autosomal dominant segregation of presenile dementia, ataxia and other neuropsychiatric features is described. Diagnoses of demyelinating disease, Alzheimer's disease, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker syndrome have been attributed to particular individuals at different times. An Irish family, likely to be part of the same kindred, is also described, in which diagnoses of multiple sclerosis, dementia, corticobasal degeneration and new variant CJD have been considered in affected individuals. Molecular genetic studies have enabled the classification of this disease at the molecular level as one of the group of inherited prion diseases, with the substitution of valine for alanine at codon 117 of the prion protein gene (PRNP). Only three other kindreds have been described world-wide with this mutation and only limited phenotypic information has been reported. Here we describe the phenotypic spectrum of inherited prion disease (PrPA117V). The diversity of phenotypic expression seen in this kindred emphasizes the logic of molecular classification of the inherited prion diseases rather than classification by specific clinicopathological syndrome. Indeed, inherited prion disease should be excluded by PRNP analysis in any individual presenting with atypical presenile dementia or neuropsychiatric features and ataxia, including suspected cases of new variant CJD.

Adult↗
PubMed1999

Ovarian hyperstimulation syndrome and benign intracranial hypertension in pregnancy after in-vitro fertilization and embryo transfer: case report.

Ovarian hyperstimulation syndrome (OHSS) is a dangerous and sometimes life-threatening complication of ovulation induction with exogenous gonadotrophins. While many complications of severe OHSS are recognized we have only identified one review detailing neurological problems. This report concerns a 32-year-old patient with bilateral tubal blockage who achieved her first pregnancy following in-vitro fertilization (IVF) and embryo transfer. Shortly after embryo transfer she developed clinical signs of moderate OHSS with symptoms which were later diagnosed as benign intracranial hypertension (BIH). The BIH was treated effectively using repeated lumbar puncture and diuretics. Spontaneous labour and delivery occurred at 40 weeks' gestation. There was no neurological sequel and no recurrence of the BIH 2 years after the pregnancy. The possible link between OHSS and BIH is discussed as well as the risks of further pregnancy.

Adult↗
PubMed1998

Referral criteria for speech and language therapy assessment of dysphagia caused by idiopathic Parkinson's disease.

OBJECTIVE: To examine the prevalence of dysphagia in idiopathic Parkinson's disease (IPD) in the outpatient setting and to determine what assessment criteria to use to select patients with dysphagia for referral to speech and language therapists. MATERIAL AND METHODS: Sixty-four patients with IPD and 80 age-matched controls were interviewed in clinic about their swallowing history and an objective swallowing test administered. All patients were assessed over the next few weeks by an experienced speech and language therapist and dysphagia rated according to a modified Rehabilitation Institute of Chicago Dysphagia Rating Scale and a novel global rating scale. The ability of various clinic criteria to predict patients with severe dysphagia were examined. RESULTS: Dysphagia for food was found in 30% of patients, significantly more than in controls. Swallowing speed and bolus volume were significantly lower in patients compared with controls and were correlated with declining Hoehn and Yahr score. Swallowing speed fell significantly on withdrawal of medication. The therapist's global rating score and Chicago score declined with Hoehn and Yahr score and duration of disease. However, only 10% of patients required dietary advice and none needed gastrostomy or tracheostomy. Discriminant analysis showed that various combinations of clinic selection criteria were no better than the presence of dysphagia for food at predicting which patients had significant dysphagia requiring advice from a therapist. CONCLUSIONS: Patients with idiopathic Parkinson's disease should be questioned about dysphagia for food on a regular basis and, if present, should be referred to a speech and language therapist for further assessment and treatment. The outcome of this protocol should be tested prospectively.

Aged↗
PubMed1998

Guidelines for the management of Parkinson's disease. The Parkinson's Disease Consensus Working Group.

Parkinson's disease is a chronic and disabling illness and there is currently wide variation in its management. This article presents the first UK-specific guidelines for the management of Parkinson's disease and it contains a treatment decision free to aid the physician in deciding when and how to treat patients. We hope this document will prove useful to all those involved in the planning and delivery of care to patients with Parkinson's disease.

Aged↗
PubMed1997

Unchanged basal ganglia N-acetylaspartate and glutamate in idiopathic Parkinson's disease measured by proton magnetic resonance spectroscopy.

The lentiform nucleus of five patients with idiopathic Parkinson's disease (IPD) was studied by quantitative magnetic resonance spectroscopy (MRS), both before and after administration of apomorphine, and the spectra were compared with those from a group of age-matched normal subjects. The concentrations of the three major metabolites, choline, creatine, and N-acetylaspartate (NAA), were quantified using tissue water content as an internal concentration reference. Glutamate concentration was assessed as the (glutamate + glutamine; GLX)/creatine peak area ratio. In normal subjects, the mean +/- SD concentrations of the the three metabolites were 2.4 +/- 0.4 mumol/g wet wt for choline, 11.5 +/- 0.8 mumol/g for creatine, and 14.7 +/- 2.8 mumol/g for NAA. The Glx/creatine ratio was 1.26 +/- 0.12. There was no significant difference in these parameters in the lentiform nucleus of patients with IPD either before or after apomorphine. The absence of detectable differences in IPD in this study implies that the changes in glutamate metabolism in the basal ganglia predicted by animal work are more subtle than those currently observable by MRS.

Aged↗
PubMed1997

Movement Disorders Cochrane Collaborative Review Group.

Evidence-based medicine is the process of systematically finding, appraising, and applying contemporaneous research findings as the basis for clinical decisions. The individual clinician is too busy to perform this long and arduous task across the whole of their specialty, so they must rely on the highest quality reviews available in the literature. The Cochrane Collaboration is an international network of individuals preparing, maintaining, and disseminating systematic reviews of trials of all health care interventions. We examine the work of the Collaboration and the major method it uses to disseminate systematic reviews through the Cochrane Library. The rationale for the Cochrane Movement Disorders Group is discussed, and its management structure outlined.

Databases, Factual↗
PubMed1996

Economic and social impact of migraine.

We sent an anonymous self-administered questionnaire to 4200 employees of a Trust hospital, 1903 of whom returned it. We identified 158 migraine sufferers according to International Headache Society (IHS) criteria. These sufferers estimated 2.0 days/year absence from work, and an equivalent of 5.5 days/year lost by reduced effectiveness at work, caused by their migraine at an estimated financial cost of over 50,000 pounds to the Trust. An additional 220 individuals who had received a diagnosis of migraine from a doctor but fulfilled only 3/4 IHS criteria lost the equivalent of 6.7 days off work at a further cost of 63,000 pounds). Few patients had consulted their general practitioner about their migraines in the last 3 months. Most (78%) were using only over-the-counter medication. Migraine patients should be encouraged to seek medical attention.

Cost of Illness↗
PubMed1995

Does levodopa therapy delay death in Parkinson's disease? A review of the evidence.

Studies examining the mortality from Parkinson's disease have been reviewed to assess the impact of levodopa therapy. These include national mortality data taken from death certificate returns, cohort studies comparing the deaths observed in a group of patients with those expected from population statistics, and case-control studies comparing deaths in patients with those in a matched control group. It is concluded that the decrease in crude mortality rate in the early 1970s in several western countries was mirrored by a decrease in observed to expected mortality rates in cohort studies over the same period. The rise in crude mortality in the late 1970s and early 1980s paralleled an increase in observed to expected mortality in cohort studies. These trends are compatible with the hypothesis that levodopa delayed the death of a cohort of frail elderly parkinsonian patients who succumbed approximately 5 years later, leading to an apparent "catch-up" increment in national mortality data. Whilst crude mortality in England and Wales does not appear to be increasing as fast as before the introduction of levodopa, recent cohort and case-control studies report mortality ratios comparable to those seen in the prelevodopa era. Further case-control studies are required to determine the position with current treatment regimens.

Aged↗
PubMed1994

Prognostic value of cortical magnetic stimulation in spinal cord injury.

Cortical magnetic stimulation was performed in a consecutive series of 10 patients presenting within 15 days of traumatic spinal cord injury. In those patients with complete paraplegia or quadriplegia, motor evoked potentials at presentation were absent below the level of the lesion. Six months after the injury, potentials had returned in the biceps brachii and abductor pollicis brevis muscles in some quadriplegic cases, but remained absent from the tibialis anterior in all of this group. None of those with a complete lesion made a significant functional recovery. Of the three patients with incomplete quadriplegia, two showed a significant recovery after 6 months. Motor evoked potentials were recordable below the level of the lesion at presentation in these cases, although the latencies were prolonged. In the remaining patient who failed to improve, potentials were unrecordable throughout the study. This small pilot study suggests that cortical magnetic stimulation may be useful in refining the prognosis in patients with an incomplete spinal cord injury.

Adolescent↗
PubMed1993

Sensitivity of PCR in detecting monoclonal B cell proliferations.

AIMS: To evaluate the rapid detection of various forms of monoclonal B cell proliferations by using the polymerase chain reaction (PCR) to identify clonal immunoglobulin heavy chain genomic rearrangements. METHODS: Thirty four B cell lymphomas defined by morphology, immunophenotyping, and positive immunoglobulin heavy chain gene rearrangements detected by Southern blot analysis were examined. An additional 22 cases representing miscellaneous lymphoproliferative and non-lymphoproliferative disorders were also studied. RESULTS: Monoclonal rearrangements were identified in 19 (56%) cases of B cell lymphoma. The method was less sensitive in the detection of follicular centre cell lymphomas (15 of 28, or 54%) than non-follicular centre cell lesions (four of six, or 67%). Monoclonal rearrangement was not identified in 19 control cases, including T cell lymphomas, Hodgkin's disease, reactive lymphadenopathy and metastatic carcinoma. Three cases showed positive immunoglobulin gene rearrangement by PCR but were negative on Southern blotting. Two of these cases had definite clinical, morphological, and immunophenotypic evidence of monoclonal B cell proliferation suggesting that PCR could, on occasion, pick up cases missed by Southern blotting and that the two methods are complementary in clonal lymphoproliferative disease diagnosis. The third case represented a "false positive" PCR reaction involving a colonic adenocarcinoma. CONCLUSIONS: PCR analysis, using the primer sequences outlined in this study, will detect about 55% of clonal lymphoproliferative proliferations with increased sensitivity for non-follicular centre cell lesions. With these levels of detection in mind, this testing strategy can still be especially useful in cases which prove diagnostically problematic with standard morphological and immunophenotypic analysis, and in instances where the quantity and type of diagnostic material is limiting (needle aspirates and cellular fluids).

B-Lymphocytes↗
PubMed1993

Mortality from Parkinson's disease in England and Wales 1921-89.

Mortality from Parkinson's disease in England and Wales was re-examined using published government statistics. The 300% increase in the crude mortality rate between 1921 and 1989 was largely attributable to the increase in the elderly population who suffer a higher prevalence of the disease. The dramatic fall in 1940 and rise in 1984 were artefacts caused by changes in certification. Age-specific mortality fell after 1940 in all groups below 70 years and in both sexes, but increased in those over 75 years. Analysis by birth cohort showed a progressive decline in mortality at younger ages with successive cohorts but stable mortality in the elderly. Younger patients were probably misclassified cases of "late-onset" Parkinsonism following encephalitis lethargica who can be differentiated from older cases of idiopathic Parkinson's disease. Mortality decreased in all age groups in the 1970s but then increased in the early 1980s. This is comparable to trends in the United States and Scandinavia and suggests that the beneficial effects of levodopa delay death for several years.

Age Factors↗