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R G Will

Publications and source records attributed to R G Will.

18 recordsLinked to original sources
PubMed1992

Creutzfeldt-Jakob disease in Scotland and Northern Ireland 1980-1989.

The epidemiological and clinical features of Creutzfeldt-Jakob disease have never before been studied in Scotland and Northern Ireland. Case records for those dying with this diagnosis were obtained for the period 1980-89. Over the ten year period, 25 definite or probable cases were identified, giving an annual incidence of 0.37 cases/million. There were more cases in the second half of the decade, and this was most likely due to increased ascertainment. One pair of cases occurred in close proximity to each other. Sex distribution showed an excess of males (male:female ratio = 1.8:1). Mean age at onset was 65.2 years, and mean duration of disease was 5.3 months. The presenting symptoms and clinical features were similar to those noted in previous studies of other populations. There was no excess of cases in occupations linked to food, farming, or medical/paramedical work.

Aged↗
PubMed1991

Epidemiological surveillance of Creutzfeldt-Jakob disease in the United Kingdom.

Systematic epidemiological surveillance of Creutzfeldt-Jakob disease was carried out in England and Wales covering the years 1970-1984. The results of this work provide a basis from which to assess any change in the pattern of Creutzfeldt-Jakob disease following the occurrence of Bovine Spongiform Encephalopathy. The methodology, early results and problems of the current systematic national survey of CJD, initiated in May 1990, are discussed.

Creutzfeldt-Jakob Syndrome↗
PubMed1991

The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraproteinaemia. Comparative clinical, immunological and nerve biopsy findings.

Observations have been made on a consecutive series of 62 patients with peripheral neuropathy associated with benign monoclonal paraproteinaemia. The paraprotein class was IgM in 46 cases, IgG in 11 and IgA in 5. Although showing variations between patients, the clinical picture was similar for those with either IgM or IgG paraproteins, usually consisting of a late-onset, slowly progressive, distal sensorimotor demyelinating polyneuropathy, often with tremor and ataxia as prominent features. Tremor was slightly more common in patients with IgM paraproteins, in whom there was a male preponderance. The patients with both paraprotein classes were indistinguishable clinically and electrophysiologically from chronic idiopathic demyelinating polyneuropathy. In the 5 patients with an IgA paraprotein, there was a distal sensorimotor neuropathy in 4 which was demyelinating in 1. In 1 there was proximal demyelinating motor neuropathy. Immunoglobulin deposition on myelin was observed only in the patients with IgM paraproteinaemia, more commonly with a kappa light chain. No deposition of immunoglobulin in the endoneurium was seen. IgM deposits on the perineurium are a feature of normal nerve and were present in all cases. Widely spaced myelin was confined to cases with IgM paraproteins in which immunoglobulin deposition was detected on myelin. The response to treatment could not be assessed systematically but, in general, the patients with IgG and IgA paraproteins responded more satisfactorily (to corticosteroids, cytotoxic drugs, or plasma exchange) than did those with an IgM paraprotein.

Adult↗
PubMed1990

Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-84.

Two hundred and sixty seven patients with a definite or probable diagnosis of Creutzfeldt-Jakob disease (CJD) were identified in an attempt to ascertain all cases of the disease in England and Wales in the period 1970-84. No evidence of space-time clustering of dates and places of onset was found for these cases. Two age- and sex-matched controls were selected for 72 of the 122 cases diagnosed in the period 1980-84 and life histories of places of residence were obtained for these cases and the controls. No evidence was found that cases had lived closer to each other than had controls at any time in their lives, except that there was an excess of cases born in London. The study does not provide supportive evidence for case-to-case transmission of CJD being an important component in the aetiology of the disease.

Adult↗
PubMed1988

Familial multiple sclerosis.

Siblings of patients with multiple sclerosis have an increased risk of developing the disease. In this report we describe 3 families with multiple affected members, representing the largest published aggregation of cases in first degree relatives. In the 2 families in which HLA tissue-typing was performed the affected individuals shared part of the haplotype HLA-DR2 (+ HLA-DQW1), BfS (+ C2C), C4A3, C4B1. The implications of these findings for the aetiology of multiple sclerosis are discussed.

Adolescent↗
PubMed1988

Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors.

An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls.

Adult↗
PubMed1988

A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome.

A patient presenting with the characteristic clinical features of Steele-Richardson-Olszewski syndrome is described, in whom neuropathological examination revealed atypical features, including extensive cortical and subcortical gliosis. The clinical and pathological features are discussed with particular reference to Creutzfeldt-Jacob disease and it is proposed that the case should be classified as progressive subcortical gliosis.

Astrocytes↗
PubMed1986

A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-1979. II: Epidemiology.

A comprehensive search yielded 121 confirmed cases of Creutzfeldt-Jakob disease who died in England and Wales in the decade 1970-1979, 31 probable cases and 10 possible cases. Descriptive epidemiological data are presented. The average annual incidence was 0.3 cases/million. An unexpected female excess was found. There was no evidence of space-time clustering of cases and no associations with occupation or past medical treatment were apparent. There was statistically significant variation in incidence rates in different parts of the country but no relationship was discovered between incidence and population density.

Adult↗
PubMedDate not supplied

A report of Froin's syndrome in five ovine thoracolumbar epidural abscess cases.

An epidural abscess involving the thoracolumbar spinal cord was considered the probable cause of pelvic limb paresis in those sheep where analysis of lumbar cerebrospinal fluid (CSF) samples revealed a significant increase in protein concentration (P less than 0.05) and some increase in total white blood cell concentration and neutrophil percentage. There was no significant increase in protein concentration in the corresponding cisternal samples. This phenomenon caused by blockage to CSF flow is not dissimilar to that of Froin's syndrome in man which has been reported as a result of localized spinal meningitis (Brain, 1985).

Abscess↗