Biomedical subjects

Maria J Marques-Dias

Publications and source records attributed to Maria J Marques-Dias.

3 recordsLinked to original sources

PubMed2006

Randomized double-blind study with prednisone in Sydenham's chorea.

In this randomized, double-blind, parallel study of a group of 22 children and teenagers, prednisone efficacy in acute Sydenham's chorea was assessed. Use of prednisone (2 mg/kg/day during 4 weeks, followed by a gradual discontinuation) in the 22 patients and in a placebo group (n = 15) was evaluated by a chorea intensity score based on presence, distribution, and interference of choreic movement on daily activities. Each patient was evaluated by the same pediatric neurologist weekly during the first month, followed by evaluation on weeks 8 and 12, with further evaluations as necessary if choreic movements persisted. Although initial chorea intensity was similar in both groups, a significant difference was observed after 1 week of medication (P < 0.001) with a larger reduction in the prednisone group, that continued until the end of the study. Percentage decrease in chorea intensity scale score also was persistently and significantly (P < 0.001) greater in the prednisone group. Chorea complete remission time with prednisone (mean 54.3 days) was significantly shorter (P < 0.001) when compared with the placebo group (mean 119.9 days). Seven patients presented recurrences, with no difference between groups (13.6% and 26.7% in the prednisone and placebo groups, respectively). Severe adverse events to prednisone were not observed.

Adolescent↗

PubMed2005

Angelman syndrome: uniparental paternal disomy 15 determines mild epilepsy, but has no influence on EEG patterns.

The authors describe the electroclinical phenotype of four patients with Angelman syndrome (AS) determined by its rarest genetic mechanism-uniparental disomy (UPD). The analysis of ours and published patients showed that in UPD, when epilepsy occurred, it was milder compared to patients with deletion, although a suggestive EEG was observed in most patients. We found that UPD patients do not completely fit the scenario delineated for AS, suggesting that patients determined by different mechanisms should be distinctly addressed, for a better understanding of this syndrome.

Angelman Syndrome↗

PubMed2003

Angelman syndrome: difficulties in EEG pattern recognition and possible misinterpretations.

PURPOSE: This study aimed to evaluate the sensitivity of the EEG in Angelman syndrome (AS), to verify the age at onset of suggestive EEGs and to study EEG patterns, analyzing variations and comparing our findings with nomenclature previously used. METHODS: Seventy EEG and 15 V-EEGs of 26 patients were analyzed. Suggestive EEG patterns of AS were classified in delta pattern (DP), theta pattern (TP), and posterior discharges (PDs). Generic terms were used to simplify the analysis. RESULTS: Suggestive EEGs were observed in 25 (96.2%) patients. DP occurred in 22 patients with four variants-hypsarrhythmic-like: irregular, high-amplitude, generalized delta activity (DA) with multifocal epileptiform discharges (EDs); slow variant: regular, high-amplitude, generalized DA with rare EDs; ill-defined slow spike-and-wave: regular, high-amplitude, generalized DA with superimposed EDs characterizing a slow wave, with notched appearance; triphasic-like: rhythmic, moderate-amplitude DA over anterior regions with superimposed EDs. TP was observed in eight patients, as generalized or over the posterior regions. PDs were seen in 19 patients as runs of sharp waves or runs of high-amplitude slow waves with superimposed EDs. TP was the only age-related pattern (younger than 8 years) and observed only in patients with deletion. In 15 patients who had an EEG before the clinical diagnosis, 60% had a suggestive tracing. CONCLUSIONS: Although some EEG descriptions are not very detailed, and every author describes findings in a slightly different manner, obviously a common denominator must exist. In this context, EEG seems to be a very sensitive method for the diagnosis of AS, offering an opportunity to corroborate this etiologic diagnosis. Conversely, we do not believe that these patterns may be accounted as specific, except for the delta pattern, which seems to be extremely unusual in other syndromes. Other EEG patterns observed in AS, such as theta activity and PDs, occur in a wide variety of disorders. Nonetheless, their importance for the EEG diagnosis of AS is supported by the fact that they are associated with other features and may be helpful in a proper clinical setting.

Adolescent↗