Heber de Souza Maia Filho

Juvenile Human T Lymphotropic Virus Type 1-Associated Myelopathy

We report the cases of 5 adolescents with human T lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis, acquired in all but 1 case from the mother. The first symptom in all patients was difficulty in running, which was present for many years before the final diagnosis was made. Follow-up showed an indolent progression, regardless of treatment strategy.

Anti-epileptic drug intake adherence: the value of the blood drug level measurement and the clinical approach

It was evaluated the patient antiepileptic drug (AED) intake adherence in a pilot cross-sectional study carried out at a neurologic out-patient clinic of a university hospital. Ninety-three AED blood concentration (phenobarbital, phenytoin, carbamazepine) were analyzed from 24 patients. The variability of the AED blood level was measured (in the steady state period by means of the variation coefficient) and compared with the self-reported antiepileptic medication non-adherence, AED blood level according to the range (therapeutic or not), and the seizure control. It was not observed any strong correlation between the higher value of variability and the other three parameters of no adherence. The highest correlation was with the blood drug level (therapeutic or not). The evaluation of blood drug measurement alone, except in cases of extreme low adherence and variability of drug intake, is not enough for the recognition of incorrect drug intake, but the clinical markers and the self-reported adherence have to be also considered for this sort of evaluation.

Medication-taking behavior and drug self regulation in people with epilepsy

OBJECTIVE To study the most important characteristics of antiepileptic drug (AED) taking behavior in epileptic people. METHODS In a cross-sectional study, 45 consecutively seen patients answered a standardized questionnaire including questions about drug intake behavior. RESULTS Both genders were equally represented (22M x 23F). The mean age was 30.2 years. No specific characteristic were presented in all patients. The self-reported non-use of the drug at any moment one week before (self-reported non-adherence) was 40.0%. Patients took the drug more than once in most cases (75.0%), and the only precipitating factor of seizures more frequently avoided was alcohol intake (66.7%). Forty-four percent said to be afraid of becoming addicted to the medicine, 61.4% reduced or stopped the medicine just to see what would happen, and 47.7% changed the prescription with the same purpose. There is no relationship among socio-demographic, behavior aspects or treatment characteristics, and self-reported non-adherence. CONCLUSIONS Several patients aspects do not seem to be strongly correlated with self-reported adherence. Nevertheless, drug self-regulation is probably related to the drug-intake behavior, and it is important for the physician to understand this parallel influence on treatment for a more realistic approach.

Management of rheumatic chorea: an observational study

BACKGROUND Rheumatic chorea (RC) has recently been linked to an antibody-mediated immune mechanism. OBJECTIVE/METHOD To verify if this knowledge reflected in management changes we conceived a descriptive study. RESULTS The medical charts of 20 children (13 females) aged 6 to 12 years (mean 8 years), diagnosed as RC from June 1996 to June 1999, were reviewed. All patients received some medical treatment. Haloperidol was the most prescribed medication (15 patients - 75 %). Sulpiride, diazepam and valproate were also used as symptomatic treatment. Immune-modulating therapy with prednisone was prescribed for seven children. The shortest course of chorea (16 days) occurred in a patient treated with prednisone. CONCLUSION Prednisone has been prescribed for rheumatic chorea besides the traditional symptomatic approach. A great variety of antichoreic drugs are being employed.

Epilepsia e saúde mental na infância

OBJECTIVE: To review the current literature about neuropsychiatry co-morbidities of children and adolescents with epilepsy and the impact of these problems in the quality of life of these patients. METHODOLOGY: Literature review in journals indexed through MEDLINE, from January 2000 to December 2005. We selected from the abstracts, original studies (epidemiology, diagnoses and therapeutics), meta-analyses, and reviews in Portuguese and English. RESULTS: The rates of mental disorders in the patients studied are higher than in normal children or even in children with other chronic diseases. Once the epilepsy is a heterogeneous syndrome, multiple factors can determine the development of psychopathology. This paper reviews the relationships among epilepsy and mental retardation, pervasive disorder, language and learning disorders and attention deficit disorder, anxiety and mood disorders, psychoses, as well as diagnoses, therapeutic and prognostic aspects. CONCLUSIONS: The modern treatment of children with epilepsy goes beyond the simple control of the epileptic seizures and should include the appropriate diagnosis and treatment of the psychiatric co-morbidities in order to improve their life quality.

Development and validation of a health related quality of life questionnaire for Brazilian children with epilepsy: preliminary findings

PURPOSE To construct a multidimensional questionnaire that analyses the epileptic child quality of life from the parental point of view. METHOD The pilot questionnaire was composed of 157 questions distributed in several dimensions. Fifty-one epileptic childrens parents answered the questionnaire. The instrument was tested in its diverse properties: frequency of endorsement, homogeneity (Cronbach alpha), criterion and face validity, and later it was reduced. RESULTS Endorsement frequency excluded 65 questions that did not attain a minimum of 5% response per item. Cronbach alpha was as follows: physical (0.93), psychological (0.91), social (0.91), familiar (0.70), cognitive (0.92), medical (0.30) and economical (0.37). Patient groups, in relation to seizure control, significantly differed only in physical domain and total score, although there was a trend to differences in other domains. The final questionnaire (QVCE50) has 50 items, with good homogeneity in the physical, psychological and cognitive domains. CONCLUSION QVCE-50 is a promissing Brazilian HRQL questionnaire for children with epilepsy. It needs to be applied in a larger population to confirm its psychometric properties.

Quality of life among Brazilian children with epilepsy: Validation of a parent proxy instrument (QVCE-50)

PURPOSE To analyze the validity and reliability of an epilepsy specific health related quality of life (HRQL) instrument (QVCE-50), constructed for Brazilians. The QVCE-50 comprises the following domains: physical (9 items), psychological (18 items), social/familial (7 items), and cognitive/educational (16 items). Items were scored on a four point scale. Domains were equally weighted using percent scores. The questionnaire ends with a quality of life scale scored 0-10 and a space for free observations. METHODS A total of 77 children with epilepsy were consecutively seen in a neuropaediatric ambulatory unit. Parents or caregivers responded to the QVCE-50 and a Portuguese version of ICIS (Impact of Childhood Illness Scale). QVCE-50 was analyzed for internal consistency, reliability, content and concurrent validity. Clinical and socio-demographic variables were also analyzed. RESULTS Socio-demographic and clinical variables that differed in at least one domain were age, sex, time since diagnosis, epilepsy family history, and antiepileptic drug used. Internal consistency, analyzed by Cronbachs alpha, showed good results for total and domain scores: physical (0.68), psychological (0.86), socio-familiar (0.70) and cognitive-educational (0.91). Total scores on the QVCE-50 and ICIS are inversely correlated (Pearsons r=-0.74, p<0.0001). The Intraclass Correlation Coefficients for test-retest reliability were acceptable: physical (0.51), psychological (0.62), socio-familiar (0.66), cognitive-educational (0.85) and total (0.77), p<0.0001. CONCLUSIONS QVCE-50 has good psychometric properties. It is a useful tool for analyzing HRQL in children with epilepsy for Portuguese speakers, especially Brazilians. Other properties should be further tested, such as responsiveness to drug and surgical treatment, capacity of distinguishing among seizure control categories and etiology (with a larger sample) and impact of psychiatric and cognitive co-morbidities.