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Biomedical subjects

R Appleton

Publications and source records attributed to R Appleton.

At least 19 recordsLinked to original sources
PubMed1999

Best practice guidelines for the management of women with epilepsy. The Women with Epilepsy Guidelines Development Group.

Clinical guidelines for the treatment of epilepsy have been published. A statement on management issues for women with epilepsy has recently been produced by the American Academy of Neurology which has raised awareness of the issues facing women with epilepsy. The communication presented here aims to review current literature on specific issues relating to women with epilepsy, and proposes graded recommendations for its management within a UK health care framework.

Abnormalities, Drug-Induced↗
PubMed1999

Gabapentin as add-on therapy in children with refractory partial seizures: a 12-week, multicentre, double-blind, placebo-controlled study. Gabapentin Paediatric Study Group.

PURPOSE: To evaluate the efficacy and safety of gabapentin (Neurontin; GBP) as add-on therapy for refractory partial seizures in paediatric patients aged 3-12 years. METHODS: After a 6-week baseline period, 247 patients (54 centres) entered a 12-week double-blind phase and were randomized to receive either GBP (t.i.d., titrated to 23-35 mg/kg/ day) or placebo. Seizure activity and type were recorded daily. Efficacy variables included Response Ratio (RRatio), responder rate, and percentage change in frequency (PCH) for all partial seizures; PCH and RRatio for individual types of partial seizures; and investigator and parent/guardian global assessments of seizure frequency and patient well-being. RESULTS: RRatio for all partial seizures was significantly lower (better) for GBP-treated patients (p = 0.0407). Responder rate favored GBP, but the difference between treatment groups was not statistically significant. Median PCH for all partial seizures for the GBP treatment group (-17.0%) was better than that for the placebo group (-6.5%). Median PCH for specific seizure types showed GBP to be most effective in controlling complex partial seizures (-35%) and secondarily generalized seizures (-28%) when compared with placebo (-12%, +13%, respectively). A greater percentage of GBP-treated patients exhibited improvement according to investigator and parent/guardian global assessments, with a statistically significant difference observed in the parent/guardian global assessment of seizure-frequency reduction (p = 0.046). Three GBP patients and one placebo patient were seizure free during the double-blind treatment period. GBP was well tolerated. CONCLUSIONS: GBP was effective and well tolerated as an add-on therapy for partial seizures in paediatric patients with previously drug-resistant seizures.

Acetates↗
PubMed1999

Use of new antiepileptic drugs in the treatment of childhood epilepsy.

The management of epilepsy in children requires careful evaluation, classification, and pharmacologic treatment. With classic antiepileptic drugs (AEDs), at least 25% of children remain refractory to appropriate therapy. The past decade has allowed the introduction of a number of newer AEDs for treatment of both adults and children with epilepsy. These include felbamate, gabapentin, lamotrigine, topiramate, tiagabine, and vigabatrin. Emerging information regarding the efficacy of these AEDs in treating childhood epilepsy syndromes suggests advantages for many patients. Limited data are available that define the optimal use of new AEDs in pediatric patients. Further research must be completed to validate the positive effects described in existing clinical trials of the new AEDs in the treatment of childhood epilepsy.

Acetates↗
PubMed1998

An audit of children referred with suspected epilepsy.

Members of the British Paediatric Neurology Association were invited to participate in a national audit of children presenting with a possible diagnosis of epilepsy. The audit was based on a 'standard' or set of pre-determined questions drawn up by an advisory audit group. The audit form comprised a total of 30 questions divided into four sections addressing history, examination, investigation, treatment and communication. Information for the audit was obtained retrospectively from the child's case notes. Each participating centre or consultant was asked to audit the case notes of 20 children. At the end of the 12-month recruitment period three centres responded, contributing audit forms on 50 children. The required information was provided for the majority of the questions in each of the four sections, thereby meeting the audit 'standard'. Within the history section, only 32 of the 50 (64%) case notes had recorded whether or not there was 'any obvious provoking factor or circumstance to the episodes'. Communication was the least satisfactorily completed section with between none and 48% of the case notes documenting that the child's family had been informed of the existence of a voluntary epilepsy organization. Despite the simplicity of the audit form, the response for this national audit was considerably lower than anticipated.

Adolescent↗
PubMed1996

Clinicians' vs. technicians' history when obtaining an EEG.

The practical value or usefulness of any investigation is dependent upon the clinical information provided and subsequent interpretation; this is particularly important in the investigation and classification of the epilepsies. For two months the histories from clinicians and EEG technicians were prospectively evaluated from 255 consecutive patients. The histories were interpreted by a single paediatric neurologist who was blinded to their origin. The histories were reported as "adequate' or "inadequate', and "diagnostic' or "non-diagnostic' (of epilepsy). Overall 92% and 40% of technicians' and 41% and 13% of clinicians' histories were considered to have been "adequate' and "diagnostic', respectively. These results have implications for the electro-clinical interpretation of the EEG.

Adolescent↗
PubMed1996

The management of febrile seizures in the Mersey Region.

The management of children with febrile seizures admitted to hospital within the Mersey region was evaluated by case note review. The patient groups were demographically similar in all the participating hospitals. Most children were admitted for less than 48 hours and nearly all received paracetamol as an antipyretic. There was a marked variation in the number of investigations performed in each hospital, with venepunctures for blood cultures and white blood cell counts ranging from 6 to 56% and from 8 to 70%, respectively. The majority of children had a urinalysis. 23 to 78% of children were prescribed antibiotics. Further studies are required on the value of hospital admission and the appropriate use of investigations and antibiotics in children with febrile seizures.

Acetaminophen↗
PubMed1996

Downgaze paresis following severe head trauma in a child.

A 4 1/2-year-old girl developed a downgaze paresis following severe head trauma. Magnetic resonance imaging showed evidence of peri-aqueductal lesions in the rostral midbrain in the region of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF). Twenty five weeks following the injury, the downgaze paresis remained unchanged but she developed convergence retraction nystagmus on attempted upgaze. Repeat imaging did not show any change in the lesions in the rostral midbrain. This report provides further evidence for the riMLF in the control of downgaze, and a synkinesis is postulated for the development of the convergence retraction nystagmus.

Child, Preschool↗
PubMed1995

Preserved merosin M-chain (or laminin-alpha 2) expression in skeletal muscle distinguishes Walker-Warburg syndrome from Fukuyama muscular dystrophy and merosin-deficient congenital muscular dystrophy.

The merosin M-chain (or laminin-alpha 2) is one of three subunits of laminin-2 which is highly expressed in striated muscle and peripheral nerve. Complete lack of laminin-alpha 2 expression in skeletal muscle is the hallmark of one form of congenital muscular dystrophy which is characterized by dysmyelination of the central nervous system (CNS), links to chromosome 6q2 and is common among Caucasians. Laminin-alpha 2 expression was also found to be significantly reduced in Fukuyama congenital muscular dystrophy which links to chromosome 9q3. We report consistently preserved laminin-2 expression, including laminin-alpha 2, as detected by immunofluorescence in skeletal muscle from five patients with Walker-Warburg syndrome which is characterized by congenital muscular dystrophy and, in addition, type II lissencephaly or pachygyria, defective CNS myelination, and ocular dysgenesis. These findings show that in spite of partial phenotypic overlap between Fukuyama CMD and Walker-Warburg syndrome the two disorders are nosologically separate disease entities. They also exclude that Walker-Warburg syndrome is allelic to the common form of congenital muscular dystrophy with laminin-alpha 2 deficiency.

Antibodies↗
PubMed1995

Deletion status and intellectual impairment in Duchenne muscular dystrophy.

The authors collected Verbal, Performance and Full-scale IQs for 74 patients in whom complete analysis of the dystrophin gene for deletions and duplications had been performed. There was a significant difference in the mean Full-scale IQ between patients with deletions at the 5' and 3' ends of the gene, with no patients with 5' deletions having mental retardation. No relationship was established between mental retardation and the presence or absence of deletions or length of deletions, and similar deletions were observed in the presence and absence of mental retardation. Although distal deletions were more commonly associated with mental retardation, there was no clear evidence for a particular region of the dystrophin gene being specifically responsible for IQ. The intellectual deficit seen in DMD may be a consequence of cerebral hypoxia, ue to malfunction of smooth muscle dystrophin.

Child↗
PubMed1995

Lorazepam versus diazepam in the acute treatment of epileptic seizures and status epilepticus.

Lorazepam was compared with diazepam for the treatment of acute convulsions and status epilepticus in 102 children in a prospective, open, 'odd and even dates' trial. Convulsions were controlled in 76 per cent of patients treated with a single dose of lorazepam and 51 per cent of patients treated with a single dose of diazepam. Significantly fewer patients treated with lorazepam required additional anticonvulsants to terminate the seizure. Respiratory depression occurred in 3 per cent of lorazepam-treated patients and 15 per cent of diazepam-treated patients. No patient who received lorazepam required admission to the intensive care unit for either respiratory depression or persisting status epilepticus. Rectally administered lorazepam appeared to be particularly valuable (100 per cent efficacy) when venous access was not possible.

Administration, Rectal↗
PubMed1994

Congenital peripheral neuropathy presenting as apnoea and respiratory insufficiency.

A seven-week-old girl presented with recurrent apnoeic episodes and respiratory insufficiency. The child was hypotonic, weak, areflexic and had a paradoxical movement of the right hemidiaphragm. Cranial nerve and sphincter function was normal; there was no fasciculation. Nerve conduction studies showed a severe axonal sensory and motor peripheral neuropathy. Biopsy of the sural nerve revealed marked axonal atrophy. The infant is now over two years of age, is ventilator-dependent and has clinical and electrophysiological evidence of further progression of one of the earliest reported presentations of a congenital peripheral neuropathy.

Apnea↗
PubMed1994

Mitochondrial DNA 8993 (NARP) mutation presenting with a heterogeneous phenotype including 'cerebral palsy'.

The mitochondrial DNA (mtDNA) mutation 8993 is an important cause of Leigh's encephalopathy. A family is reported where other affected members have presented with non-specific delayed development or cerebral palsy. The diagnosis should be considered not only in children with Leigh's encephalopathy, but also in those with mild neurological dysfunction (including cerebral palsy) if there is a pigmentary retinopathy or a family history of neurological or ophthalmological disease. There was some correlation in this family between the disease severity and the proportion of mutant mtDNA in the blood. This mutation appears to segregate to high levels of mutant mtDNA rapidly within pedigrees and the mother of a severely affected child has a high risk of having further children with a high proportion of mutant mtDNA and a severe phenotype.

Adolescent↗
PubMed1993

The loss of a baby at birth: the role of the bereavement officer.

OBJECTIVE: To illustrate and emphasise the role of the Bereavement Officer in the management of perinatal death, as recommended and envisaged by a report of the Royal College of Obstetricians and Gynaecologists in 1985. DESIGN: A description of the training and responsibilities of the officer and an account of her involvement with all perinatal deaths within a given period. SETTING: Perinatal deaths in the 12 months, 1 October 1989 to 30 September 1990 from a regional neonatal intensive care unit within a maternity hospital. SUBJECTS: A total of 59 registerable deaths, three late terminations (severe fetal abnormality) and 12 late mid-trimester miscarriages were dealt with by the Bereavement Officer in this 12 month period. RESULTS: The introduction of this service to our unit has led to an improvement in our management of both perinatal and fetal deaths. CONCLUSION: The service has justified fully the recommendations made by the Royal College of Obstetricians and Gynaecologists. Reducing parents' distress will facilitate their grieving and any subsequent bereavement counselling.

Bereavement↗
PubMed1988

Amaurosis fugax in teenagers. A migraine variant.

Sudden, transient loss of vision in one eye (amaurosis fugax) is associated frequently with atherosclerosis of the internal carotid artery in adults and may herald a stroke. Thus, cerebral angiography is often performed. Amaurosis fugax in children is uncommon and an underlying cause is rarely demonstrated. Recurrent episodes of amaurosis fugax occurred in five adolescents. A characteristic evolution and pattern of visual loss, consistent with choroidal ischemia as the underlying mechanism, was described by four of them. Although none of the episodes were accompanied by headache, four patients had a history of common migraine at other times or a family history of migraine. These episodes of visual loss may represent a migraine variant, and cerebral angiography is not indicated in adolescents with such a history.

Adolescent↗
PubMedDate not supplied

Head injury rehabilitation for children.

The Royal Liverpool Children's Hospital (RLCH), Alder Hey, has established a comprehensive programme and team to rehabilitate children who have suffered head injuries. The team is known as the Head Injury Rehabilitation Team (HIRT). The purpose of this paper is to outline its work.

Child↗