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Biomedical subjects

I F Pye

Publications and source records attributed to I F Pye.

At least 19 recordsLinked to original sources
PubMed2004

Peripheral neuropathy--a rare complication of paediatric systemic lupus erythematosus.

Peripheral neuropathy is an uncommon complication in paediatric systemic lupus erythematosus (SLE). We report the case of a 10-year-old Chinese girl who developed peripheral neuropathy within 3 months of the onset of SLE and presented with bilateral foot drop and sensory symptoms of both hands and feet. There was no involvement of the central nervous system at the time of presentation. The patient was negative for anticardiolipin antibodies, but positive for lupus anticoagulant. She was treated with intravenous methylprednisolone followed by oral steroids, methotrexate, gabapentin and amitryptyline. Although peripheral neuropathy is a rare complication of paediatric systemic lupus erythematosus, one should be vigilant for this entity as part of the neurological spectrum. It may not be associated with involvement of the central nervous system. Antiphospholipid antibodies may have role in the pathogenesis of SLE associated peripheral neuropathy. We speculate that routine nerve conduction studies may have a role in detecting sub-clinical cases.

Child↗
PubMed2000

New variant Creutzfeldt-Jakob disease: three case reports from Leicestershire.

Since a report in 1996 of 10 cases of Creutzfeldt-Jakob disease (CJD) with onset in a younger than usual age, a pattern of the disease has emerged. This includes early neuropsychiatric features and sensory symptoms and neurological signs such as ataxia and involuntary movements later in the course of the disease. Three patients with varied clinical presentations and disease course seen at a single neurology unit are described. The first patient was characterised by cognitive and psychiatric symptoms together with neurological signs. The second patient presented with unusual behavioural disturbance and episodes of collapse. The third patient exhibited striking psychomotor retardation and had abnormal CSF and MRI findings. All patients succumbed in a state of akinetic mutism and myoclonus. All three patients had the methionine/methionine genotype at codon 129 of the PrP gene and in two of the three patients a tonsil biopsy was performed with positive results. These two patients also tested positive for the 14.3.3. protein in the CSF. Whereas late features of the disease seem very similar in all cases, the initial presentation was variable and underlines the uncertainty of the range of the clinical phenotype. Successful diagnosis demands a high index of clinical suspicion.

Adolescent↗
PubMed1998

Randomized study of carotid angioplasty and stenting versus carotid endarterectomy: a stopped trial.

BACKGROUND: Carotid angioplasty (CA) has been suggested to be a safer and more cost-effective alternative to carotid endarterectomy (CEA) in the management of symptomatic severe internal carotid artery (ICA) disease. METHODS: The study was conducted as a prospective consecutive randomized trial of CEA versus CA for symptomatic severe ICA disease in a university teaching hospital. All patients were assessed before and after surgery by a neurologist. The study consisted of 23 patients with focal carotid territory symptoms and severe ICA stenosis (> 70%) who were randomized to either CEA or CA. However, only 17 had received their allocated treatment before trial suspension. CEA with patching or CA with stenting were used as interventions. The main outcome measures were death or disabling or nondisabling stroke within 30 days. RESULTS: All 10 CEA operations proceeded without complication, but 5 of the 7 patients who underwent CA had a stroke (P=.0034), 3 of which were disabling at 30 days. CONCLUSIONS: After referral, the Data Monitoring Committee invoked the stopping rule and the trial was suspended. The investigators and the Ethics Committee subsequently concluded that the trial could not be restarted--even in an amended format-primarily because of problems with informed consent. We review many of the ethical dilemmas encountered in the performance of this study. If future trials do suggest a selected role for CA, it is essential that both the inclusion and the exclusion criteria are fully documented.

Aged↗
PubMed1995

Color-coded ultrasound diagnosis of vascular occlusion in acute ischemic stroke.

A combination of extracranial and transcranial color-coded sonography was used to identify the patterns of vascular occlusion in 47 patients with acute ischemic stroke. Total anterior circulation infarction (n = 20) was associated with internal carotid artery (ICA) or middle cerebral artery (MCA) occlusion (n = 8 and 9, respectively), or with significant reduction in ipsilateral MCA velocities (n = 5). Patients with partial anterior circulation (n = 22) infarction had patency of the ipsilateral ICA and MCA. In this group, significant reduction of ipsilateral MCA velocities (n = 7) was associated with more extensive infarcts on conventional neuroimaging (n = 6), suggesting multiple MCA branch occlusions. Ultrasound imaging was unable to identify underlying vascular pathology in patients with posterior circulation infarction or with lacunar infarction (n = 5). An ultrasound-based approach enables noninvasive identification of major vascular pathology of the anterior cerebral circulation in patients with acute cerebral infarction. It may be useful for the rapid identification of patients most and those least likely to benefit from acute intervention, and for monitoring their response.

Acute Disease↗
PubMed1993

Plumboporphyria (ALAD deficiency) in a lead worker: a scenario for potential diagnostic confusion.

A lead worker developed bilateral wrist drop. At first this seemed to be a lead neuropathy but all his screening tests for blood and urine toxicity had been within the accepted safety limit during employment. Detailed investigation showed that he had plumboporphyria (ALAD deficiency) which had been symptom free until he was exposed to lead. Details of his porphyrin metabolism are presented.

Adult↗
PubMed1993

Posterior paroxysmal discharge: an aid to early diagnosis in Lafora disease.

Lafora body disease is a rare neurometabolic disorder of autosomal recessive inheritance. Symptoms begin in the second decade with progressive myoclonic epilepsy and survival is unusual beyond the age of 30. We report an electroencephalographic study in four cases of histologically proven Lafora body disease. Posterior epileptiform discharges were found even in the early stages of the disease and may assist in early diagnosis.

Adult↗
PubMed1992

Lafora disease: a progressive myoclonus epilepsy.

Lafora disease is a rare inborn error of metabolism resulting in storage of a polyglucosan in tissues including the brain, skin and liver. Four children are described with progressive myoclonus epilepsy and intellectual deterioration in whom this diagnosis was made. In two the diagnosis was confirmed by the presence of periodic acid schiff (PAS) positive, diastase resistant, colloidal iron staining inclusion material in the liver when they were referred to a paediatric gastroenterologist with abnormal liver function tests. In one, the diagnosis was made from cerebellar biopsy, although on retrospective review the liver biopsy performed at this time was abnormal. In a fourth child, whose sibling was affected, histological diagnosis was confirmed by skin biopsy, although clinical and EEG findings had been highly suggestive for several years. The disease has autosomal recessive inheritance, is progressive and the prognosis is poor. Paediatricians should be aware of this diagnosis, which is often delayed, as early histological diagnosis allows prognostic and genetic counselling and optimal treatment. Although the diagnosis was made by liver or brain biopsy in three cases, skin biopsy offers a reliable, less invasive means of diagnosis.

Adolescent↗
PubMed1991

The apomorphine test in parkinsonian syndromes.

The dopamine receptor agonist apomorphine has been used successfully to treat on-off swings in Parkinson's disease. Its value as a predictor of dopa responsiveness in idiopathic Parkinson's disease (IPD) was assessed and its potential role in differentiating IPD from the Parkinsonian plus syndromes (PPS) of multisystem atrophy, progressive supranuclear palsy and olivopontocerebellar atrophy was investigated. The response to an injection of apomorphine was observed in 20 patients with IPD and eight with PPS after being off levodopa for 12 hours. Patients were reassessed after taking levodopa for one month. Nineteen of the 20 patients (95%) with IPD showed a positive response to apomorphine and 18 (90%) to oral levodopa. In the PPS group, two patients (25%) responded to the apomorphine injection but not to oral levodopa. Apomorphine produced severe drowsiness in the PPS patients. It is suggested that the test can predict dopa responsiveness in IPD and may be of help in confirming a doubtful diagnosis. It has potential value in differentiating IPD from PPS.

Aged↗
PubMed1984

Manifesting heterozygosity in sex-linked spastic paraplegia?

An unusual form of hereditary spastic paraplegia is described. Affected females have a late-onset slowly progressive spastic paraparesis. Affected males show oligophrenia with a rapidly progressive spastic quadriplegia. The mode of inheritance is consistent with sex-linkage, with partial manifestation in female carriers.

Adolescent↗
PubMed1982

Interrelations between cerebrospinal fluid and plasma inorganic ions and glucose in patients with chronic renal failure.

The concentrations of inorganic ions and glucose in the plasma and CSF of 11 patients with "steady-state" chronic renal failure have been measured and their CSF: plasma interrelations studied. The results have been compared with the corresponding data from 34 control subjects. In the patients with renal failure, there was a positive correlation between raised CSF and plasma potassium concentrations. In contrast to the impaired potassium homeostasis, normal CSF magnesium and calcium concentrations were observed despite wide variations in the plasma concentrations of these ions.

Adolescent↗
PubMed1982

CSF and plasma GABA levels in Parkinson's disease.

CSF gamma-aminobutyric acid (GABA) levels were reduced in patients with idiopathic Parkinson's disease when compared with age matched controls, but the difference was not significant. However, when the Parkinsonian patients were subdivided, CSF GABA levels were lower in the levodopa treated group than in the untreated group and the controls. There was no difference in plasma GABA levels between Parkinsonian patients and controls.

Adult↗
PubMed1981

Effect of freezing on gamma-aminobutyric acid levels in human cerebrospinal fluid.

Using a radioreceptor assay, the concentration of gamma-aminobutyric acid (GABA) in human cerebrospinal fluid (CSF) was found to be elevated significantly following a single deep-freeze to --70 degrees C and thaw. Mean CSF GABA (+/- SD) in unfrozen CSF was 173 +/0 73 pmol/ml (n = 24). After a single deep-freeze, the mean level was 243 +/- 106 pmol/ml (p less than 0.02). Subsequent freeze-thaw cycles resulted in further irregular and unpredictable elevations in CSF GABA. Mean level after two freezes was 379 +/- 125 pmol/ml and after three freezes 654 +/- 411 pmol/ml. These changes could result in the incorrect interpretation of results in patients suffering from neurological diseases.

Adult↗