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Myositis Ossificans Progressiva
Fact Sheet #18
1. Is Myositis Ossificans Progressiva also known by other names?
It is also known as Fibrodysplasia Ossificans Progressiva, or by its abbreviation 'MOP'.
2. What is Myositis Ossificans Progressiva?
MOP is a disabling condition that is due to the formation of bony bars within the muscles of the body. This bone formation is generally first noticed in early childhood as a series of hard lumps in the neck or along the spine. These lumps, which may be tender, gradually shrink in size as the affected muscle is replaced by bone. The appearance of bony lumps in muscles is usually spontaneous but can also be provoked by any injury to the muscles.
3. What causes Myositis Ossificans Progressiva?
The exact cause of MOP is unknown.
4. What are the symptoms?
The disorder is progressive in that more muscles become involved with increasing age, but the rate of progression is very variable from one person to another. Furthermore the disorder tends to show long periods of inactivity (for up to several years in length). Most individuals with MOP have some irregularities of the fingers or toes. The big toes are most commonly involved and are often shortened and deviated. These signs are usually apparent at birth.
5. Which muscles are affected?
Certain muscles are never involved in the disorder. These include the muscles of the eyes, face, tongue, gullet, intestines and the muscles of continence. The heart muscles are also never involved. Chest expansion may be reduced in MOP but the diaphragm, which is the single most important breathing muscle is never involved.
6. Does anything make MOP worse?
Injury to the muscles can often provoke local bone formation. For this reason intra-muscular injections should be avoided, if possible. Similarly, operations on the muscle to remove pieces of bone almost invariably result in increased bone formation. Also, some forms of dental treatment may result in bone formation in the jaw muscles. It is therefore very important that you warn your dentist that you have MOP.
7. How is Myositis Ossificans Progressiva inherited?
MOP is inherited as an autosomal dominant disorder, with each child of an affected parent having a 50% chance of inheriting the disorder. As this is a genetic condition, genetic counselling is strongly recommended. Genetic counselling provides information on the inheritance pattern, risks to other family members, prognosis, psycho-social support, as well as information about diagnostic testing, carrier testing, preclinical and prenatal testing (where available).
8. How is Myositis Ossificans Progressiva diagnosed?
The diagnosis is based on clinical evaluation of the symptoms.
9. Is there a cure?
Unfortunately, there is no cure for this disorder at present.
10. Is there any treatment?
Many types of treatment have been tried in the past and none of these has been shown to be of benefit.
11. Is there a risk during anaesthesia?
It is always recommended that the presence of a muscular disorder be mentioned to your doctor.
12. Has research been conducted on MOP in South Africa?
To date, no research has been conducted in South Africa on this particular disorder.
13. The role of the Muscular Dystrophy Foundation (MDF) in South Africa
The MDF supports individuals affected by muscular dystrophy and their families by offering emotional support, information - including a series of fact sheets, referrals to genetic counselling and other clinics, formation of support groups, assistance with special equipment, when possible, as well as financial support for research projects in muscular dystrophy in South Africa. Creating public awareness for muscular dystrophy is also an important aspect of our work, since the MDF relies solely on contributions from its members and other donors to provide an on-going support service. Through our newsletter members are kept informed of all the activities and receive national and international research updates. Please contact any office of the MDF if you require information about any of our activities or programmes.
14. Support group or contact person
Please contact the MDF for further information.
15. Where can we find assistance?
Please contact your local MDF office for further information.
General Information: Independent Living Centre (ILC): (011) 482-5476
Disability Info and Care (DIC): (011) 917-3284
Parking Concessions: Application to the Traffic Department of our your Local Authority.
Criteria:
- if you need the extra width as provided by the special parking bays, or
- if you have a problem with walking long distances.
Special Equipment: Phone either ILC or DIC for information on where to get special equipment.
MDF Website: Please visit our MDF website (www.mdsa.org.za) for muscular dystrophy news updates.
16. Please note
The treatments and drugs mentioned in this fact sheet are for information purposes ONLY. Please consult your physician or other health care specialist for information regarding the use of any of the above. The MDF encourages duplication of this fact sheet, under the following condition: that it is duplicated in its entirety - including the MDF logo and full text. Only individuals authorised by the MDF may make changes to this fact sheet (the information "updated by" and "last update" should be completed). Alterations to this fact sheet by any other party are strictly prohibited.
This fact sheet was adapted from the following source(s): Fact sheet(s) of the Muscular Dystrophy Group of Great Britain and Northern Ireland.
Compiled by: MDF-Gauteng Branch
Updated by: MDF-Gauteng Branch
Approved and Released by: National Office of the MDF
Last update: 30 May 2000
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