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Biomedical subjects

A Nicolson

Publications and source records attributed to A Nicolson.

15 recordsLinked to original sources
PubMed2006

An association between type 1 diabetes and idiopathic generalized epilepsy.

OBJECTIVE: Idiopathic generalized epilepsies (IGEs) account for approximately 30% of all patients with epilepsy. Both the IGEs and type 1 diabetes mellitus (T1D) represent serious worldwide problems, because of related medical and social management costs. Clinical experience suggested the two conditions were seen in individuals more frequently than might be expected by chance. METHODS: We compared the population prevalence of T1D in 15- to 30-year-olds to a cohort of 518 15- to 30-year-olds with IGE. RESULTS: We found a highly significant excess of T1D in our IGE cohort, with an odds ratio of 4.4 (95% confidence interval, 2.1-9.2). INTERPRETATION: Our results suggest that the prevalence of T1D is increased by a factor of four in young adults with IGE. To our knowledge, this is the first published association between the two conditions and expands the diseases known to be associated with T1D.

Adolescent↗
PubMed2005

Epilepsy in the UK: misdiagnosis, mistreatment, and undertreatment? The Wrexham area epilepsy project.

OBJECTIVE: To assess the diagnostic and therapeutic difficulties in patients with epilepsy who had never come into contact with specialist services. METHODS: Assessment was offered to 676 patients diagnosed as having epilepsy and receiving anti-epileptic drug therapy (AED), who had no previous contact with the local epilepsy services. Two hundred and seventy-five patients gave consent and attended for reassessment. We identified the proportion of patients (a) who had previously seen a neurologist, (b) in whom the diagnosis of epilepsy was not secure, (c) in whom planned AED withdrawal could be considered (d) in whom seizure control could be improved. RESULTS: 53/275 (19.3%) of those attending for review had previously been seen by a neurologist. 87/275 (31.6%) patients ultimately received continued specialist care. Diagnostic doubt was expressed in 3/53 (5.6%) and 42/222 (18.9%) of patients diagnosed by neurologist and non-specialist, respectively. Of 133/219 (60.7%) of patients whose epilepsy was in remission, only 6 elected to withdraw or change medication. Of 18 patients with diagnostic doubt who accepted follow-up, 12 successfully stopped treatment. 17/55 (30.9%) patients with active epilepsy (10 partial, 7 generalised) achieved at least a 1 year remission consequent upon treatment in this clinic. In 15 cases this was a first ever remission. CONCLUSION: Approximately 55% of the population of adults receiving treatment for epilepsy have never received specialist advice. Reassessment of these patients uncovers diagnostic uncertainty, failure to classify (leading to sub-optimal therapy) and lack of information and advice about all aspects of epilepsy care. The development of integrated services for people with epilepsy (PWE) must take account of this hidden need. The new General Medical Services contract for general practitioners will bring this need to our attention, and our experience will help predict the measures required to deal with the under-treatent and mistreatment of this group. The majority of PWE, not currently receiving shared care, merit reassessment and approximately one-third will require continued specialist care. Existing services do not have the capacity to process a marked increase in rate of referral. This project informs prioritisation of referrals and service reorganisation.

Adolescent↗
PubMed2004

A comparison of adult onset and "classical" idiopathic generalised epilepsy.

OBJECTIVES: To report the characteristics of a population of patients with idiopathic generalised epilepsy (IGE) with age of onset over 20 years, and compare them with patients with "classical" IGE. METHODS: Data were collected from a computerised database of all patients with IGE attending a regional adult epilepsy clinic. Demographic data, epilepsy characteristics, and treatment outcomes were recorded. RESULTS: 72 patients with IGE of a total population of 844 had an age of onset over 20 years (8.5%). There was similar incidence of family history of epilepsy, EEG findings, and remission rates between those with a younger and older age of onset of IGE. There was a lower incidence of previous febrile convulsions in patients with adult onset. There were fewer patients with absence seizures in the adult onset group (15.3% v 46.4% in the "classical" group). CONCLUSIONS: IGE with onset later than the third decade was rare in the population studied. Prolonged EEG in selected patients may be helpful in diagnosing adult onset IGE, but the diagnosis of epilepsy remains clinical. Adult onset IGE shares many features with "classical" IGE, including EEG findings and prognosis, and is likely to represent a genetic epilepsy.

Adolescent↗
PubMed2004

The relationship between treatment with valproate, lamotrigine, and topiramate and the prognosis of the idiopathic generalised epilepsies.

OBJECTIVE: To examine a large population with idiopathic generalised epilepsy (IGE), and estimate the overall remission rates for the IGEs and subsyndromes in a clinic based sample. Remission rates on valproate, lamotrigine, topiramate, and combinations of these antiepileptic drugs were estimated and factors predicting outcome examined. METHODS: All patients with IGE were identified from a computerised database and EEG records at large adult and paediatric epilepsy clinics. Data were recorded retrospectively on demographics and clinical information, seizure types and syndrome diagnosis, antiepileptic drug treatment details, and remission rates. RESULTS: 54.3% of 962 patients had achieved a one year period of remission; this was most likely with valproate monotherapy (52.1%), with lower rates for lamotrigine and topiramate (16.7% and 34.6%, respectively). The combination of valproate and lamotrigine achieved a remission rate of 15.3%. The factor most predictive of a response to a particular antiepileptic drug regimen was the rank order in which it was given. Relapse rate was high (79.9%) after antiepileptic drug withdrawal in remission, particularly with juvenile myoclonic epilepsy (93.6%). CONCLUSIONS: Valproate may be the most effective antiepileptic drug in the treatment of the IGEs. Combination therapy should be initiated if an adequate trial of valproate monotherapy is not effective, rather than switching to alternative monotherapy. Antiepileptic drug treatment needs to be lifelong in many adult patients with IGE.

Adolescent↗
PubMed2002

The legacy of vigabatrin in a regional epilepsy clinic.

OBJECTIVES: To review all patients who had received vigabatrin at the Walton Centre to determine the incidence of visual field defect, seizure outcome if vigabatrin had been stopped, and adherence to guidelines on the use of vigabatrin in clinical practice. METHODS: Retrospective review of 583 patients prescribed vigabatrin at any time between 1989 and 2001 from a regional and satellite epilepsy clinic. Data were collected on dose and duration of treatment, results of quantitative perimetry, and reasons for, and outcome of, discontinuation. RESULTS: The visual fields were abnormal with no alternative cause in 42 of the 98 tested (43%). There was no clear relation between the cumulative dose of vigabatrin received and the occurrence of a visual field abnormality. Fifty patients continued taking vigabatrin, and a further 84 were lost to follow up while taking vigabatrin. In 75 patients who had stopped vigabatrin due to a visual field abnormality or concern over this potential adverse effect, the seizure control was no different or had improved in 66 (88%), while it had deteriorated in only 7 (9%). CONCLUSIONS: This study confirms the previously reported high incidence of asymptomatic visual field defects associated with vigabatrin. Many patients taking vigabatrin may not have been counselled about the risks, and there are significant cost implications in tracing and assessing those patients lost to follow up. Switching over to another antiepileptic drug usually does not result in deterioration in seizure control, but in clinical practice an individual risk to benefit ratio needs to be taken into consideration.

Ambulatory Care Facilities↗
PubMed2001

Future prospects for the drug treatment of epilepsy.

Great progress has been made in the last 150 years in the pharmacological management of epilepsy, and, despite the increasing number of technological advances available, antiepileptic drugs (AEDs) remain the mainstay of treatment for the vast majority of patients with epilepsy. This review looks at possible avenues of development in the drug treatment of epilepsy. The strengths and weaknesses of those AEDs which are currently licensed are examined, and ways in which their use may be improved are discussed (e.g. rational combinations, use of new formulations). Potentially new targets that may allow the development of effective treatments are highlighted (neuroimmunological manipulation, decreasing inherent drug resistance mechanisms, and modification of adenosine neurotransmission), and a summary of the most promising AEDs currently in development is provided [e.g. carabersat, ganaxolone, harkoseride, MDL 27192, safinamide (NW 1015), pregabalin, retigabine, talampanel, valrocemide, losigamone and BIA 2093].

Animals↗
PubMed2000

Brown-Vialetto-Van Laere syndrome: case report and literature review.

We describe a case of the Brown-Vialetto-Van Laere syndrome, which is a rare disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. More than 30 cases have been reported since the first case was described in 1894. We review the literature of this condition, comparing our case with those reported in the literature and emphasizing important features to improve our understanding of this syndrome.

Adult↗
PubMed1998

Clinical associations of lymphopenia in elderly persons admitted to acute medical and psychiatric wards.

Lymphopenia is a common finding in old people admitted to medical and psychiatric wards. We describe a pilot study of the clinical associations, and prognostic significance of lymphopenia in elderly persons admitted to acute medical and psychiatric wards. Consecutive patients admitted to acute medical and psychiatric wards were prospectively selected according to initial peripheral lymphocyte count (PLC) into lymphopenic (PLC < 1.0 X 10(9)/litre, n = 41), and non-lymphopenic (PLC > 1.5 x 10(9)/litre, n = 23). Results of routine haematological and biochemical investigations were recorded, as well as drug history and medical diagnoses. Anthropometric measurements, assessment of functional ability (Barthel ADL Index), and cognitive function (Mini-Mental State Examination) were then performed by investigators blind to lymphocyte status. Patients were contacted between 3 and 6 months following recruitment into the study. Lymphopenia was associated with functional ability as measured by a lower Barthel score (p = 0.004), and cognitive impairment as measured by the Mini-Mental State Examination (p = 0.02). No association was found with medical diagnostic groupings, drugs known to cause lymphopenia, nutritional status, or survival. Lymphopenia may be a significant marker of vulnerability, and a larger study is required to elucidate the veracity and mechanisms of lymphopenia-associated debility.

Aged↗
PubMed1996

The prevalence of severe growth hormone deficiency in adults who received growth hormone replacement in childhood [see comment].

OBJECTIVE: The few previous studies in which reassessment of GH status has been carried out in young adults treated with GH therapy for childhood GH deficiency concentrated on determining the incidence of 'transient GH deficiency'. As the benefits of GH replacement therapy in adults become increasingly appreciated, it is likely that GH therapy started in childhood for GH deficiency will be continued into adult life in many of those with severe GH deficiency. The aim of this study is to determine how many patients who received GH replacement therapy in childhood until completion of growth have GH deficiency severe enough to be considered for GH replacement therapy in adult life. DESIGN: Retrospective analysis of the peak GH responses to provocative stimuli performed at the time of diagnosis of GH deficiency in childhood and at the completion of growth after GH replacement therapy had been stopped. PATIENTS: Eighty-eight adults (49 male, 39 female) who had received GH therapy in childhood for a diagnosis of GH deficiency. The aetiology of the GH deficiency included craniopharyngioma, radiation induced associated with either a cerebral tumour or acute lymphoblastic leukaemia, histiocytosis-X and idiopathic. MEASUREMENTS: In childhood the original diagnosis of GH deficiency was based biochemically on the failure of the peak GH response to reach 20 mU/l during two provocative tests in 59 of the 88 patients and to a single test in the remaining 29. A total of 147 tests were performed, the most common being an insulin tolerance test (ITT, n = 72) and an arginine stimulation test (AST, n = 53). At reassessment in young adult life 146 tests were performed (74 ITT, 64 AST). Severe GH deficiency was defined arbitrarily as a peak GH response of less than 9 mU/l to a single (n = 33) or to two (n = 55) pharmacological stimuli. RESULTS: By definition all patients were considered GH deficient at the time of initial diagnosis. A peak GH response of less than 9 mU/l was seen in 64.8% at initial assessment and 60.2% at reassessment. Analysis in aetiological terms, however, showed that between assessments the incidence of severe GH deficiency increased in the group of radiation induced (48.8 vs. 55.8%) but decreased in the idiopathic group (78.1 vs. 53.1%). Out of the 55 patients who underwent two tests at reassessment, 47.3% of those with a GH peak less than 9 mU/l at one test had a GH peak greater than 9 mU/l at the second test. Fifteen of the 55 patients had additional pituitary hormone deficiencies and all 15 had a GH peak below 9 mU/l in both tests. CONCLUSIONS: Our study suggests that all children who have received GH replacement therapy in childhood should undergo reassessment of GH status in young adult life. Between 40 and 60% of such patients merit consideration for GH therapy in adult life depending on the definition of severe GH deficiency in use. Patients with isolated GH deficiency should undergo two provocative tests of GH secretion, but those with additional anterior pituitary hormone deficiencies require only one test at reassessment.

Adolescent↗
PubMed1994

Interpretation of pressure-sore prevalence.

Pressure sores result in patient morbidity and mortality as well as commanding considerable resources within the British National Health Service. To assess the impact of preventive measures, accurate and reliable rates of prevalence and incidence are required. This information is likely to be used as a measure of quality of care as well being included in future purchaser/provider contracting. A number of prevalence studies have been carried out in the United Kingdom using a variety of populations and different methods. The methods employed in these studies need to be considered before any meaningful comparisons can be drawn. In particular, there are variations in the reported rates possibly related to the under-reporting of pressure sore prevalence. This paper describes a recent point prevalence study which suggested that this may be the case. The need for caution when comparing crude rates is highlighted and the importance of standardizing the methods used for determining prevalence rates is discussed.

Cross-Sectional Studies↗