Fibrodysplasia ossificans progressiva: case report.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of the connective tissue and congenital malformation of the big toes. We report on a nine-year-old girl with clinical and radiological features of FOP. She was born with bilateral hallux valgus and at the age of nine presented an indurate mass in the left cervical region that was painful. A significant decreased range of motion in all levels of the spine and shoulder girdle was found. The radiographs showed heterotopic ossification in the thoracic region. The patient had two outbreaks of the disease ("flare-ups") that were treated with prednisone 2 mg/kg/day for four days. After the "flare-ups", she had a continuous therapy with a Cox-2 inhibitor (25 mg/day) and a leukotriene inhibitor, montelukast (10 mg/day).

[Translation and validation of an instrument for evaluation of severity of symptoms and the functional status in carpal tunnel syndrome]. / Tradução e validação do questionário de avaliação de gravidade dos sintomas e do estado funcional na síndrome do tú nel do carpo.

The objective of the present study was to translate, to do cultural equivalence and validation of the Levine et al. (1993) or Boston Carpal Tunnel Questionnaire (BCTQ) to Portuguese. The BCTQ application to patients, selected by the question "What was the reason that brought you to look for a physician and that led him to ask this examination?" showed very good reproducibility. The validity was measured through the comparison of the severity symptoms scores (SSS) and the functional status scores (FSS) with the results of grip forces, Minnesota, two point discrimination, Semmes-Weinstein filaments perception and sensory conduction at the median nerve. The internal consistency was evaluated through Chronbach's alpha coefficient comparing the SSS and the FSS. The measuring properties were evaluated through paired t-test between pre and pos-surgical scores. Reproducibility, internal consistency, validation and measuring properties of the translated BCTQ were similar to those found by Levine et al. with the original version.

[Paresthesia and/or pain affecting the hand and/or wrist as a referral for electrodiagnostic studies]. / Parestesia e/ou dor nas mãos e/ou punhos como motivo de encaminhamento para estudo eletroneuromiográfico.

To study the reasons for referral to electrodiagnostic evaluation, 490 patients referred for electrodiagnostic evaluation to two laboratories (UNIFESP and UNEF) answered the following question: "What was the reason that brought you to look for a physician and that led him to ask this examination?" Paresthesia and/or pain were answered as the main reason by 175 of them (26% UNIFESP and 40% UNEF). The electromyographical examinations were normal in 30.8% and 35.3% and involvement of the median nerve at the wrist was detected in 5 9% and 51.5% of them. Nocturnal symptoms and paresthesia were commonly noted in both groups of patients; they, however, were not able to separate the patients with and without median nerve compression at the wrist. The question asked seems to be a useful selection instrument, to select two similar symptomatic samples of patients from two laboratories serving with very different target populations.