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Biomedical subjects

P Humphreys

Publications and source records attributed to P Humphreys.

At least 37 records · Page 2Linked to original sources
PubMed1990

Isolated angiitis of the central nervous system in childhood.

Isolated angiitis of the central nervous system, a rare inflammatory condition of the nervous system, characterized by vasculitis of the small vessels, has not, to the best of our knowledge, been described in childhood. It usually presents with a diversity of neurological symptoms in the fifth to eighth decades of life. We reviewed the case of an 8 year old male with autopsy proven isolated cerebral angiitis, to encourage the consideration of this disorder in children; and to emphasize the lack of sensitivity of present modalities of neurological investigation in the diagnosis.

Brain Diseases↗
PubMed1990

Clobazam as an add-on drug in the treatment of refractory epilepsy of childhood.

We report the results of a double-blind cross-over study comparing clobazam and placebo in the treatment of refractory childhood epilepsy. Fifty-two percent of patients had greater than 50% reduction in their seizure frequency when taking the clobazam. During the placebo phase no patient recorded a greater than 50% reduction in seizure frequency. Sex, age, seizure type, intellect did not appear to differentiate clobazam responsive from nonresponsive patients. Only 2/21 patients had behavioral changes on the drug sufficiently severe to require the patient to drop out of the study prematurely. Drug interactions between clobazam and the other anticonvulsant medicines did not occur.

Adolescent↗
PubMed1989

Routine screening of blood and urine for severe reactions to anticonvulsant drugs in asymptomatic patients is of doubtful value.

Severe or fatal reactions to anticonvulsant agents are fortunately rare. We examined the value of routine screening of blood and urine to detect early signs of such reactions in asymptomatic patients. The basic assumptions of this type of screening program have been faulty or unproven, and the results of studies, although not definitive, have not supported the value of such programs. Our recommendations, approved by the Canadian Association for Child Neurology, suggest that asymptomatic patients not undergo routine screening of blood and urine but, rather, be informed of the early symptoms of severe toxic reactions and be asked to report them immediately to a physician.

Adult↗
PubMed1989

Flunarizine as a supplementary medication in refractory childhood epilepsy: a double-blind crossover study.

We report a double blinded cross-over study involving Flunarizine versus placebo in the treatment of refractory childhood epilepsy. The patients studied were between the ages of 2 and 18; and were having more than 4 seizures per month not responsive to regular anticonvulsant medications. Of the 34 patients treated, 8 had a 50% decrease in their seizures during the placebo phase, 5 had a 50% decrease during the Flunarizine phase, and 1 patient had a 50% increase in seizures while taking Flunarizine. The remaining 25 patients showed no change in seizure activity in either phase. Patients having partial seizures with secondary generalization tended to do better on Flunarizine than those with other seizure types. Monitoring serum Flunarizine levels showed no significant difference between patients having improved seizure control and those who were unimproved. No significant side effects were noted with this medication, nor were any significant drug interactions noted.

Adolescent↗
PubMed1989

Recurrent headaches in children and adolescents: diagnosis and treatment.

Recurrent headache is a frequent problem in children and adolescents. With a careful history and physical examination few errors in diagnosis will be made. Fortunately, few headaches are of pathological origin. The most common headaches are migraine and muscle contraction headaches. For many sufferers, a few simple suggestions and proper use of analgesics will provide effective management. The best documented treatments for more difficult headaches are stress management therapies. Recently stress management treatments that can be administered by professionals not trained in psychology have made these approaches more accessible.

Adolescent↗
PubMed1988

Relaxation prophylaxis for childhood migraine: a randomized placebo-controlled trial.

A randomized controlled trial was used to evaluate the effectiveness of relaxation training in the treatment of paediatric migraine. Relaxation training was compared with-two control groups (psychological placebo and 'own best efforts') in a total of 99 children and adolescents with frequent migraine. Daily recording of the headaches following treatment, three months after treatment and at the one-year follow-up indicated that all three treatments were equally effective. The importance of the use of adequate control conditions which generate equivalent expectancies in pain treatment research was confirmed.

Adolescent↗
PubMed1988

Cervical spinal cord stimulation for spasticity in cerebral palsy.

A prospective double-blind study of high cervical spinal cord stimulation conducted in eight moderately disabled, spastic, cerebral palsied children failed to demonstrate any significant improvement over base line function during chronic spinal cord stimulation at either optimal stimulation parameters or random placebo parameters. Chronic stimulation included 4 consecutive months of stimulation for 24 hours each day. Stimulators were randomly programmed at optimal parameters for 2 of the 4 months and at placebo parameters for the remaining 2 months. At the end of each month of chronic stimulation, subjects were assessed with a multidisciplinary test battery that included a self-assessment, specific clinical examinations, tests of gross and fine motor control, neuropsychological and neurophysiological tests, a detailed gait analysis, and video recordings. By 6 months after the completion of the study, only 1 of the 8 subjects continued to use his stimulator on a regular basis, with minimal benefit.

Adolescent↗
PubMed1987

Evaluation of information-processing speed and neuropsychological functioning in patients with myotonic dystrophy.

Patients with myotonic dystrophy (MD) were compared to a control group, matched to the patients in important demographic variables including IQ, on the Sternberg Memory Scanning procedure, to investigate the hypothesis of a selective change in speed of information processing in MD patients. The neuropsychological functioning of these MD patients was also compared to normative data to provide a descriptive picture of their abilities; these results were correlated to the factors of age of onset and duration of the disease. Finally, the MD patients were also compared to the defined control group on the neuropsychological measures. There was little evidence of selective slowness of information processing or particular deficit independent of overall IQ. Neuropsychologically, the MD patients as a group performed at the low average level. There was, however, a wide range of abilities, suggesting that MD patients are not a unitary group in terms of neuropsychological functioning. Age of onset of the disease was important, at least for certain results. Further research of the neuropsychological functioning of MD patients must account for the wide range of results, with more precise measures of actual onset of the disease and muscular weakness, in a longitudinal evaluation.

Attention↗
PubMed1987

Representing risks: supporting genetic counseling.

This paper discusses issues involved in decisions to bear a child given the knowledge that the child may be subjected to a genetic risk. It argues that the problem of whether to take the risk or not is predicated on the way the prospective parents have conceptualised this risk and its effects. Five levels of abstraction are described at which this risk can be represented and the implications of conceptualising the problem at each of these levels are outlined. Recommendations are made about how to explore the way--or different ways--parents conceptualise the problems involved and how to go about helping them make the decision which they will be least likely to regret subsequently, whatever the actual outcome.

Attitude↗
PubMed1984

Fulminating haemophilus influenzae b meningitis.

Haemophilus influenzae type b (HIb) is the most common cause of bacterial meningitis in children with a mortality rate ranging from 1.6% to 14%. Most patients have a 2-3 day history of symptoms prior to admission. A few have fulminating disease with rapid neurological deterioration. Review of 191 cases of HIb meningitis revealed a mortality rate of 2.1% but all who died had fulminating meningitis (FM). Four of six patients with FM died. FM patients had symptoms for less than 24 hours before rapid neurological deterioration with increased ICP, seizures, coma and/or respiratory arrest. Review of 10 FM cases revealed that on admission, 5 had hypotension, 3 had thrombocytopenia, and 8 had coma. Typical CSF changes were seen in only 7. All fatal cases died within 24 hours. Brain swelling and tonsillar herniation were found at autopsy. SDS-PAGE outer membrane protein subtyping did not show one "killer strain". Animal and autopsy data suggest that diminished CSF outflow and cerebral edema contribute to increased ICP. To improve survival of FM patients, initial treatment must (1) decrease ICP below levels impairing cerebral perfusion, (2) maintain adequate ventilation and blood pressure, and include (3) LP when stable, (4) antibiotics, and (5) close monitoring. Utilizing these principles, two FM patients survived without major sequelae.

Child, Preschool↗
PubMed1981

Friedreich's ataxia with nephrotic syndrome and convulsive disorder: clinical and neurophysiological studies with renal and nerve biopsies and an autopsy.

In a sibship of four, Friedreich's ataxia and minimal lesion nephrotic syndrome occurred in two siblings, a third sibling had Friedreich's ataxia, but no evidence of nephrotic syndrome; the fourth sibling had neither condition. The chance of Freidreich's ataxia and minimal lesion nephrotic syndrome occurring in two siblings is small, and suggested a common immunological abnormality. High dose prednisone and antimetabolites given for the nephrotic syndrome did not appear to affect the course of Friedreich's ataxia. The two siblings with Friedrich's ataxia and nephrotic syndrome developed epilepsy at age 15 years. All three children with Friedreich's ataxia had abnormal electroencephalograms (EEGs). These epileptiform EEG abnormalities were probably inherited from the mother, who had spike wave epilepsy. The neurologic deficits of Friedreich's ataxia, in turn, may have allowed the EEG trait to be expressed as a seizure disorder. The progressive ataxia and epileptic, sometimes myoclonic, seizures in these patients and the dentate nucleus changes in the autopsied patient were consistent with the diagnosis of dyssynergia cerebellaris myoclonica. This suggested that the latter disorder may represent a coincidence of two genetic entities: Friedreich's ataxia and spike wave epilepsy.

Adolescent↗
PubMed1981

Fisher syndrome in childhood.

Three children, age 2, 7, and 12, developed Fisher syndrome: inability to walk because of ataxia, complete areflexia, and ophthalmoplegia. Ptosis was prominent in all the children, but the pupillary response to light was affected only in one child. Limb weakness was never present, and sensation normal. One patient was obtunded for several days. Two had prodromal upper respiratory tract illnesses, and the third patient was bitten by an insect 2 days before her symptoms began. Cerebrospinal fluid (CSF) protein content was moderately increased in all patients, but only one child's CSF had a pleocytosis. CSF gamma globulin levels were normal. In the acute phase, F waves and H responses were absent in two cases but returned to normal with clinical recovery. All three patients had marked electroencephalographic (EEG) abnormalities that later improved. The EEG pattern suggested a brainstem disorder. These findings plus the obtundation, gaze paralysis, and ataxia indicate that in Fisher syndrome there is parenchymal involvement of the central nervous system with or without nerve root involvement.

Ataxia↗