Catamnesis of neurologic patients, especially dystonic patients, features not only the natural history of certain disorder, but the social status of these cripples. Are they always grata in the country of healthy people? We report the catamnesis of two dystonic patients, that testify the society to turn them often into the pariah. Case one concerns with mixed geno – phenotype of generalized dystonia and Strumpel disease. Strumpel disease and dystonia are inherited disorders with clinical picture of spastic paraparesis and hyperkinesis respectively. We present a case of a patient born from parents with these diseases who developed neurologic phenomena uncharacteristic for the classical clinical picture of his parents’ disorders. Patient V., 12, born from father with generalized dystonia and mother with Strumpel disease, has flaccid lower paraplegia along with dystonic hyperkinesis in neck and arms. The flaccid lower paraplegia could be caused by anterior horn lesion. This phenomenon is unclear because anterior horn lesions were not diagnosed in the proband’s parents. When we got in touch with his family to check the progress of the treatment, we were terrified to reveal the brutal murder of his father with generalized dystonia. And when we proceeded to catamnesis of the rest of studied group of patients, it became clear that such violent outcome seems to be rather the rule than exception. Case two concerns with cervical dystonia patient with angioma of the frontal lobe and her terrible death. Structural lesions of CNS, reported to be associated with torticollis, are mostly restricted to cerebellum, brain stem and basal ganglia. In fact, we know only about two documented frontal lobe mass lesions – meningiomas, associated with torticollis. Our observation of frontal lobe cavernous angioma associated with clinical picture of torticollis confirms the role this area could play in the pathophysiology of involuntary movements. We report a case of patient with torticollis associated with cavernous angioma of the right frontal lobe and presuppose causative role of angioma in the development of our patient’s torticollis. These both cases are the observation of very important neurologic phenomena from one side and the contemporary social attitudes to hyperkinetic patients from another.
Author (s) Details
Dr. Vadim Belenky
ArsVita Clinic, St. Petersburg, Russia.
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