Ziehen-Oppenheim disease


Ziehen-Oppenheim disease

Description, Causes and Risk Factors:

Ziehen-Oppenheim disease refers to a syndrome of involuntary sustained or spasmodic muscle contractions involving co-contraction of the agonist (denoting a muscle in a state of contraction, with reference to its opposing muscle, or antagonist) and the antagonist. The movements are usually slow and sustained, and they often occur in a repetitive and patterned manner; however, they can be unpredictable and fluctuate. The frequent abnormal posturing and twisting can be painful and functionally disabling.

It is generally agreed that Ziehen-Oppenheim disease may be associated with a number of well delineated pathological entities, such as Wilson's disease, various of the encephalitides, vascular malformations, idiopathic calcification of cerebral vessels, and others. In contradistinction to those "symptomatic" forms of dystonia this paper is concerned with the idiopathic form to which the term Ziehen-Oppenheim disease should be confined according to Herz.

The ratio between affected males and females is about 60:40. No explanation for this sex distribution has been offered, as yet. The disease usually begins during childhood or adolescence.

Ziehen-Oppenheim disease00, although precise figures are currently not available. The estimated prevalence in the Ashkenazi Jewish population is approximately five to ten times higher, due to a founder mutation.

Symptoms:

The chief symptoms are dystonic posture and dystonic movements. The latter aretrue hyperkinesias and are characterized by relatively slow, long sustained, powerful,non-patterned, contorting activities of the axial and appendicular muscles. Themuscles most commonly involved are those of the neck, trunk, and proximal portionsof extremities. Involvement of unilateral muscle groups often results in bizarre torsionmovements, hence the alternative term "torsiondystonia" for the disease. Volitionalacts are often seriously compromised. The dystonic movements disappear duringsleep and under general anesthesia. "Dystonic posture" is the term used if the endposition of a dystonic movement is maintained for any length of time. Eventuallythis may lead to contracture deformities.

The onset is gradual, with drawing sensations in axial and appendicular muscles.Gait is usually impaired first, but writer's cramp or torticollis (an unnatural condition in which the head leans to one side because the neck muscles on that side are contracted) may also constitute theinitial symptom. Muscle hypertrophy may result from continuous activity, eventuallyleading to such deformities as kyphoscoliosis, pes equinovarus and others. Speech isoften impaired. Sensation and mentation (the process of using your mind to consider something carefully) are rarely affected. Laboratory findings arewithin normal limits. The course varies, ranging from complete incapacitation to amild nonprogressive illness. Sometimes remissions may occur.

Diagnosis:

History taking and physical examination are necessary, as in all cases of neuromuscular disorders. Family history is important; as many as 44% of patients have a family history of similar or other movement disorders.

Ziehen-Oppenheim disease may be a clinical manifestation of many treatable neurological conditions; therefore, a thorough screening should be performed to exclude hypoxic brain injury, traumatic brain injury, Huntington disease, Leigh disease, lipid storage disease, and Parkinson disease.

A number of medications can induce Ziehen-Oppenheim disease, and a careful investigation of the patient's medication list must be performed to rule out iatrogenic (induced by a physician's words or therapy used especially of a complication resulting from treatment)causes.

Treatment:

Treatment options include botulinum toxin injections for focal symptoms, pharmacological therapy such as anticholinergics for generalized dystonia and surgical approaches such as deep brain stimulation of the internal globus pallidus or intrathecal baclofen application in severe cases. All patients have normal cognitive function, and despite a high rate of generalization of the dystonia, 75% of patients are able to maintain ambulation and independence, and therefore a comparatively good quality of life, with modern treatment modalities.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

0 Comments

Submit a Comment

Your email address will not be published. Required fields are marked *

Cart Preview

Red Wine May Protect Teeth from Cavities and Gum Disease

Red Wine May Protect Teeth from Cavities and Gum Disease

According to a Spanish research, led by Dr. Victoria Moreno-Arribas from the Spanish National Research Council in Madrid, drinking red wine may protect teeth by destroying bacteria causing cavities and gum disease. The researchers completed an experiment where they...

Running will Help Protect Memory from the Effects of Stress

Running will Help Protect Memory from the Effects of Stress

Numerous studies conducted earlier showed that regular exercises have a positive effect on physical and mental health. The new work of American scientists confirms this. They believe that running five kilometers once a day is useful for the hippocampus (the brain...

Quiz about this article

Please answer on few questions to make our service more useful

Featured Products

8 Reasons to Start Riding a Bike

Spring is not far off and very soon you will see many people riding a bike. It's worth to join them and now we will give you the 8 reasons why. Weight control Scientists of the University of Surrey (England) found that it's enough riding a bike for an hour, and, not...

read more

Simple Ways to Keep Fit Without Going to the Gym

We all want to get in shape and keep fit. But not everyone has time for visiting the gym. Give up the elevator. Give up lifts and climb the stairs. At work, at home, at the mall. This simple advice is a great benefit if you follow it daily. Climbing the stairs...

read more