Motor restlessness in the form of incoordinated, unsystematized, grotesque uncontrollable movements occurs in a number of morbid conditions, such as Huntington's chorea, double athetosis, Sydenham's chorea, Wilson's lenticular degeneration, Strümpell-Westphal pseudosclerosis and dystonia musculorum deformans, also known as torsion spasm or lenticular dystonia.
The diagnosis of the aforementioned morbid conditions is not difficult if the patient is seen in the fully developed stage of the disease, but when he is seen in an early stage correct diagnosis is almost impossible. Especially is this true of dystonia musculorum deformans, or, as Flatau and Sterling1 proposed to name it, progressive torsion spasm. Not only are the clinical manifestations of this disease obscure and misleading, but even the histologic changes are by no means definite or uniform and are therefore unconvincing. If one considers that reports on the histologic changes of dystonia musculorum deformans are scarce and for the most part pertain
HASSIN GB, PONCHER HG. DYSTONIA MUSCULORUM DEFORMANS: CLINICOPATHOLOGIC REPORT OF A CASE. Am J Dis Child. 1939;57(1):105–115. doi:10.1001/archpedi.1939.01990010114009
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: