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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
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Summary:

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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

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