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Biomedical subjects

J I Manson

Publications and source records attributed to J I Manson.

35 records · Page 2Linked to original sources

Testing hearing with the brain-stem evoked response.

The brain-stem electroencephalograph (EEG)-evoked response (BSER) constitutes a reliable and objective method of testing hearing in infants, and in uncooperative, or retarded patients. The procedure is harmless and painless, and involves recording the EEG brain-stem responses to clicks presented by headphones. The patient may be sedated, or even anaesthetized, if necessary. Because of the equipment and time involved, the procedure is not recommended as a routine screening procedure, but constitutes a valuable adjunct to conventional audiological testing, especially in doubtful cases.

Adolescent↗

Electron microscopy of skin and peripheral blood lymphocytes in infantile (Santavuori) neuronal ceroid lipofuscinosis.

Skin punch biopsies and peripheral blood lymphocyte preparations from two siblings with early infantile (Santavuori) neuronal ceroid lipofuscinosis have been examined by electron microscopy. In both cases characteristic osmiophilic inclusion bodies were found in various cells in the skin and in lymphocytes. In one case, lymphocyte inclusions were detected before the onset of any symptoms as a result of a family study. This indicates the possibility of screening lymphocytes of siblings of affected patients as a result of a family study. This indicates the possibility of screening lymphocytes of siblings of affected patients with a view to early detection of the disorder.

Ceroid↗

Two-dimensional echoencephalography in paediatric neurology.

Two-dimensional echoencephalography is a useful procedure in screening young infants for hydrocephalus and cerebral malformation. The procedure is noninvasive and easily repeated. The procedure is less reliable in detecting mass lesions except insofar as they disturb the anatomy of the ventricular system. The procedure can be considered as complementary to computerised axial tomography in the investigation of cerebral anatomy in paediatric neurology.

Brain↗

Bioavailability of phenytoin from various pharmaceutical preparations in children.

In children, the blood level of phenytoin was found to be significantly higher when 100 mg capsules rather than 100 mg tablets were administered. When, on the other hand, 30 mg capsules and tablets were compared; the situation was reversed; tablets produced significantly higher blood levels of phenytoin than did the capsules. The significance and possible explantation of these findings are discussed.

Adolescent↗

Ataxia-without-telangiectasia. Progressive multisystem degeneration with IgE deficiency and chromosomal instability.

Three of five siblings developed a progressive neurological disorder during infancy or early childhood characterized by cerebellar ataxia, choreoathetosis and peripheral neuropathy. Immunological studies revealed a marked selective deficiency of serum IgE in all three affected siblings. There was evidence of chromosomal instability in the three affected siblings and in one of the parents. One of the affected siblings also developed acute lymphoblastic leukaemia. In spite of many resemblances, this syndrome differs from classical or complete ataxia telangiectasia in that oculocutaneous telangiectases were lacking, the serum IgA and alpha-fetoprotein levels in this family were normal, there was no gonadal dysgenesis, and the cytogenetic findings were atypical.

Adolescent↗

Temporal lobe epilepsy in childhood: reappraisal of etiology and outcome.

This article reports the neurologic and psychologic findings, seizure characteristics, family histories, and etiology of clinically and electroencephalographically defined temporal lobe epilepsy in 63 children who were studied retrospectively. Subsequent data were available for 53 patients (84%), 15 of whom had undergone temporal lobectomies; 38 patients had been managed conservatively for at least 2 years. Previous, complicated febrile convulsions were the most common predisposing factor, occurring in 13 patients (21%), while 6 patients had tumors (10%). Of the 10 children whose onset of temporal lobe seizures occurred before 2 years of age, 5 had tumors. The presence of emotional or behavioral problems was related significantly to the presence of borderline or low intelligence, but not to the frequency of seizures. Although there was a tendency for a reduction in seizure frequency over time, only 10% of those managed by medical therapy alone were seizure-free at a mean subsequent examination interval of 6.6 years.

Adolescent↗

Corpus callosotomy for intractable epilepsy: seizure outcome and prognostic factors.

We reviewed the outcome of corpus callosal section in 64 adult and pediatric patients to identify factors associated with a good outcome: 48% of patients had a favorable outcome for overall seizure frequency. Improvement was noted in several seizure types and was most likely for drop attacks, particularly in the setting of a unilateral focal cerebral lesion or a true generalized epilepsy of Lennox-Gastaut type. Poor outcomes for drop attacks were more likely if there was associated severe intellectual handicap or bilateral independent spikes on interictal EEG. Complex partial seizures (CPS), most commonly of frontal lobe origin, also responded favorably. The complications of callosal section were usually mild and transient. New focal seizures occurred in only 2 patients and were not as frequent or disabling as preoperative seizures types. A worthwhile improvement in seizure outcome was achieved by completion of the callosotomy in 6 of 10 patients with unsatisfactory results from anterior callosotomy.

Adolescent↗