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.00, 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.
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.
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 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.
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