Myositis Ossificans Progressiva, Munchmeyer' Disease

Year 1996, Volume: 1 Issue: 2, 42 - 45, 30.12.1996

Güntekin Güner Nurzat Elmalı Kadir Ertem

Abstract

Fibrodysplasia ossificans progressiva
is a rare hereditary connective tissue disorder. Patients with fibrodysplasia
ossificans progressiva develop progressive ossification of muscle and
connective tissue associated with pain and disability. Onset is typically in
childhood, and congenital feet anomalies are the early signs of this condition.
Pain and stiffness of the spine or an inflammatory mass are common presenting
features of fibrodysplasia ossificans progressiva. Studies on twins and
families suggest that fibrodysplasia ossificans progressiva is a genetically
inherited autosomal dominant trait with complete penetrance but variable
expressivity. Unfortunately, effective therapy is unavailable. We present a 23
years old man with fibrodysplasia ossificans progressiva with a review of the
literature on the clinical, radiographic, and genetic manifestations of this
disorder.

Keywords

Myositis ossificans, Adult, Diagnosis

Myositis Ossifikans, Progresiva, Munchmeyer Hastalığı

Abstract

Fibrodisplasia (myositis) ossificans
progressiva nadir görülen herediter bir bağ dokusu hastalığıdır. Hastalık
iskelet kaslarının bağ dokusu içerisinde başlangıçta ağrılı olan, progresif
ektopik ossifikasyonlar ve simetrik iskelet malformasyonları ile karakterizedir.
Hastalıkta ilk belirtiler çocukluk yaş gurubunda başlar. Nadir, otozomal
dominant geçişli konjenital bir hastalıktır. Halen etkin bir tedavisi yoktur.
Bu yazımızda büyük eklemlerinde hareket kısıtlılığı ile kliniğimize başvuran
boyun, sırt, omuzlar, kalçalar ve dizlerde kas içerisinde ektopik
ossifikasyonları ve başparmaklarında kısalığı bulunan ekstensif tutulumlu 23 yaşındaki
bir erkek hastayı literatür bilgileri ışığında sunduk.

Keywords

Myositis ossifikans, Erişkin, Tanı

References

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