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Biomedical subjects

P Humphreys

Publications and source records attributed to P Humphreys.

15 recordsLinked to original sources

Freezing lesions of the developing rat brain: a model for cerebrocortical microgyria.

Cerebrocortical microgyri were induced by placing a freezing probe on the skull of P0 and P1 rat pups. Freezing lesions resulted in laminar necrosis of the infragranular layers and the subsequent migration of supragranular neurons through the region of damage. The result was most often a region of four-layered microgyric cortex consisting of a molecular layer, a thickened layer ii, a lamina dissecans (corresponding to the necrotized layers IV, V, and VIa), and a neuronal layer iv which corresponded to layer VIb of the intact cortex. Immunocytochemical investigation of the microgyric cortex with antibodies to neurofilament, glial fibrillary acidic protein and glutamate showed more widespread disruption of neocortical architecture than could be seen from Nissl preparations. In contrast, vasoactive intestinal peptide-containing neuronal bodies appeared to be distributed normally in the microgyric region although their processes were sometimes distorted. These results are considered in the light of previous research on induced microgyria, and possible implications for the behavioral consequences of focal, developmental neuropathologic lesions are discussed.

Animals

Developmental dyslexia in women: neuropathological findings in three patients.

Brains from male cases with dyslexia show symmetry of the planum temporale and predominantly left-sided cerebrocortical microdysgenesis. We now report on three women with dyslexia. In all brains, the planum temporale was again symmetrical. Also, in two of the brains, multiple foci of cerebrocortical glial scarring were present. In both women, many of the scars were myelinated, suggesting origination during late intrauterine or early postnatal life. In one, scars were mainly left perisylvian and involved portions of the vascular border zone of the temporal cortex. In the other, scars were more numerous and occurred in the border zone of the anterior, middle, and posterior cerebral arteries symmetrically. All three cases showed to a variable extent brain warts, molecular layer ectopias, and focal architectonic dysplasia identical to those seen in the male cases. Two women had primary brain neoplasms, an oligodendroglioma and a low-grade astrocytoma, respectively, and two women showed small angiomas. Reexamination of previously reported male cases disclosed one with myelinated glial scars. Two control brains with asymmetrical plana temporale showed myelinated glial scars as well. The significance of the anatomical findings is discussed, and possible etiological factors are considered with known effects of autoimmune diseases on the nervous system.

Adult

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

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

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

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

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

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

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

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

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

Folie à quatre.

A case of folie à quatre is described affecting a proband and his wife, sister and brother. The three family members retained their symtoms of paranoid schizophrenia. The wife was not mentally ill and her delusions disappeared in time (folie imposée). Coincidentally, there were several cases of Huntington's chorea amongst her relatives. Different categories of folie à deux are demonstrated in the various family members and relationships.

Adult