Hostname: page-component-78c5997874-4rdpn Total loading time: 0 Render date: 2024-11-18T08:39:28.513Z Has data issue: false hasContentIssue false
  • English
  • Français

A Case of Huntington’s Chorea With Unilateral Ectopic Gray Matter

Published online by Cambridge University Press:  18 September 2015

Haring J.W. Nauta  and
M.E. Platts
Haring J.W. Nauta*
Affiliation:
Division of Neurosurgeryand The Playfair Ncuroscicncc Unit, Toronto Western Hospital and The Department of Pathology, The Princess Margaret Hospital, Toronto
M.E. Platts*
Affiliation:
Division of Neurosurgeryand The Playfair Ncuroscicncc Unit, Toronto Western Hospital and The Department of Pathology, The Princess Margaret Hospital, Toronto
*
Playfair Neuroscience Unit, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada, M5T2S8
Playfair Neuroscience Unit, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada, M5T2S8
Rights & Permissions [Opens in a new window]

Summary:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

The authors report a single case of Huntington’s chorea associated with a unilateral focus of ectopic gray matter. The patient’s symptoms began at age 45 and included typical involuntary jerking movements of all extremities and face. Mental deterioration may have proceeded the choreiform movements. The family history was positive for Huntington’s chorea. Pneumoencephalogram showed atrophy of the caudate nuclei bilaterally early in the disease. The patient improved transiently with haloperidol therapy.

The major pathologic features included mild generalized cerebral atrophy with marked atrophy of the caudate nuclei and putamen. Within the white matter of the left frontal lobe, there were irregular nodules of ectopic gray matter with an overall diameter of 2 cm.

The rarity of either unilateral ectopia or Huntington’s chorea alone, makes it impossible to judge if the two lesions might be linked by a common pathologic mechanism. The significance such a linkage might hold is discussed in light of several currently postulate pathologic mechanisms.

Type
Articles
Information
Canadian Journal of Neurological Sciences , Volume 8 , Issue 2 , May 1981 , pp. 181 - 184
Copyright
Copyright © Canadian Neurological Sciences Federation 1981

References

Alzheimer, A. (1911): Huntingtoniche chorea und die choreatishen Bewegungen uberhaput. Zeitschrift fur die gesamte Neuologie und Psychiatrie 3: 891.Google Scholar
Bruyn, G.W., Bots, G. TH. A.M., and Dom, R. (1979) Huntington’s chorea: current neuropathological status. Advances in Neuology 23: 83–93.Google Scholar
Earle, K.M. (1973) Pathology and experimental models of Huntington’s chorea. Advances in Neurology I: 341–351.Google Scholar
Hallervorden, J. (1957) Huntintonische Chorea (Chorea chronica progressiva hereditaria). In: Handbuch Spez. Pathol. Anat. Histol. vol. 13, edited By Lubarsch, O., Henke, F., Rossle, R., and Uhlinger, E.Springer-Verlag, Berlin, pp 793–822.Google Scholar
Jelgersma, G. (1908) Nue anatomische Befunde bei Paralysis agitans und bei chronischer progressiver Chorea. Neurol. Cbl. 27: 995.Google Scholar
Lyle, O.E., and Gottesman, I.I. (1979) Subtle cognitive deficits as 15- to 20-year precursors of Huntington’s disease. Advances in Neurology 23: 227–238.Google Scholar
Neophytides, A.N., Dichiro, G., Barron, S.A., Chase, T.N. (1979) Computed axial tomography in Huntington’s disease and persons at-risk for Huntington’s disease. Advances in Neurology 23: 185–191.Google Scholar
Noronha, A.B.C., Roos, R.P., Antel, J.P. and Arnason, B.G.W. (1979) Huntington’s disease: abnormality of lymphocyte capping. Ann. Neurol. 6: 447–450.CrossRefGoogle ScholarPubMed
Stone, T.T., and Falsten, E.I. (1938) Pathology of Huntington’s Chorea II. J. Nervous and Mental Disease 89: 602–626.CrossRefGoogle Scholar
Tellez-Nagel, I., Johnson, A.B. and Terry, R.D. (1973) Ultrastructural and histochemical study of cerebral biopsies in Huntington’s chorea. In: Barbeau, A., Chase, T.N. and Paulson, G.W. (eds) Advances in Neurology, vol. 1. Raven Press, New York.Google Scholar
Wilson, R.S. and Garron, D.C. (1979) Cognitive and affective aspects of Huntington’s Disease. Advances in Neurology 23: 193–201.Google Scholar