We report two siblings who are suffering from a dystonic syndrome, clinically indistinguishable from idiopathic torsion dystonia (dystonia musculorum defor mans) but with cranial computerized tomographic scan findings of basal ganglia lesions, similar to that reported in Wilson's disease.
The occurrence of the disorder in the same sibship suggests an autosomal recessive mode of inheritance and may represent another variety of the syndrome of idiopathic torsion dystonia.
Get full access to this article
View all access options for this article.
References
1.
Fahn S., Eldridge R.Definition and classification of the dystonic states. Adv Neurol1976;14:1-5.
2.
Barbeau A., DuVoisin RC, Gerstenbrand F., et al. Classification of extrapyramidal disorders by a committee on nomenclature. J Neurol Sci1981;51:311-27.
3.
Golden GSDystonia in the black and the puerto rican population. Adv Neurol1976; 14:121-4.
4.
Menkes JHDystonia musculorum deformans—A status report. Bull LA Neurol Soc1976;4:184-8.
5.
Marsden CD, Harrison Mjg.Idiopathic torsion dystonia (dystonia musculorum deformans): A review of forty two patients. Brain1974;97:793-810.
6.
Dobyns WB, Goldstein NP, Gordon H.Clinical spectrum of Wilson's disease (hepatolenticular degeneration). Mayo Clin Proc1979;54:35-42.
7.
Ropper AH, Hatten HP, Davis KRComputed tomography in Wilson's disease: report of 2 cases. Ann Neurol1979; 15: 102-3.
8.
Nelson RF, Guzman DA, Grahovac Z., et al. Computerized cranial tomography in Wilson's disease. Neurology (Minn) 1979 ;29:866-8.
9.
Haneggeli CA , Hauser H., Paunier L.CT in Wilson disease. Neurology (Ny) 198 1;31:1056.
10.
Harik SI, Post Mjd.Computed tomography in Wilson disease. Neurology ( Ny) 1981;31:107-10.
11.
Selekler K. , Kanzu T., Zileli T.Computerized tomography in Wilson's disease . Arch Neurol1981;38:727-8.
12.
Williams Fjb , Walshe JMWilson's disease. An analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents. Brain1981;104;735-52.
13.
Barr AN, Heinze WJ, Dobben GD et al. Bicaudate index in computerized tomography in Huntington disease and cerebral atrophyNeurology (Minn) 1978;28:1196-200.
Dooling EC, Richardson EP, Davis KRComputed tomography in Hallervorden-Spatz disease. Neurology (Ny) 1980;30:1128-30.
16.
Swaiman KF, Smith SA, Trock GL, et al: Sea-blue histiocytes, lymphocytic cytosomes, movement disorder, and 59Fe-uptake in basal ganglia: Hallervorden-Spatz disease or ceroid storage disease with abnormal isotope scan? Neurology ( Cleveland) 1983;33:301-5.
17.
Zimmerman AW , Karimeddini MK, Ramsby GR, et al. Hallervorden-Spatz syndrome: increased cerebral uptake of 59Fe and the demonstration of striatal iron deposition on CT scan (abstract). Neurology (Ny) 1981;3:129.
18.
Roesmann U. , Schwartz JFFamilial striatal degeneration. Arch Neurol1973;29:314-7.
