Fibrodysplasia ossificans progressiva: case report

Arquivos de Neuro-Psiquiatria

Andre Leite Gonçalves¹  Marcelo Rodrigues Masruha¹  Carmelinda Correia De Campos¹  Patricia Longo Ribeiro Delai¹  Luiz Celso Pereira Vilanova¹ 

[1] ,Federal University of São Paulo Department of Neurology and Neurosurgery São Paulo SP ,Brazil

关键词: fibrodysplasia ossificans progressiva;    myositis ossificans progressiva;    myositis ossificans;    heterotopic ossification;    fibrodisplasia ossificante progressiva;    miosite ossificante progressiva;    miosite ossificante;    ossificação heterotópica;   

DOI  :  10.1590/S0004-282X2005000600032

来源: SciELO

【 摘 要 】

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).

【 授权许可】

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