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Neurology India, Vol. 58, No. 4, July-August, 2010, pp. 680 Neuroimage "Split thalamus": Internal medullary involvement in Wilson's disease George Uttam, Varte Nunenga, Rathore Shubhra, Jain Vishesh, Goyal Sandeep Department of Radiodiagnosis, Christian Medical College and Hospital, Ludhiana, Punjab Correspondence Address:Department of Radiodiagnosis, Christian Medical College & Hospital, Brown Road, Ludhiana - 141 008, Punjab, ubgeorge@gmail.com Date of Acceptance: 22-Jun-2010 Code Number: ni10187 PMID: 20739831 DOI: 10.4103/0028-3886.68701 A 32-year-old woman presented to psychiatry outpatient department with decreased sleep, irrelevant speech, aggressive and bizarre behavior over a period of 3 months. Investigations showed decreased serum cerruloplasmin, increased 24 h urinary copper levels, and mild elevation of alkaline phosphatase and bilirubin with decrease in total proteins. Slit lamp examination of cornea showed Kayser-Fleischer ring. Brain magnetic resonance imaging (MRI) revealed symmetrical hyperintensities on T2/T2 fluid attenuation inversion recovery images in the putamen [Figure - 1]a, thalami, midbrain, pons, and subcortical white matter of both frontal lobes. Hyperintensity of Fields of Florel and internal medullary laminae was also seen [Figure - 1]b and c. The patient was started on atypical antipsychotics along with a copper chelating agent. No psychiatric manifestations or worsening was observed during the hospital stay and during the 6 month follow-up. Psychiatric manifestations may be the initial presentation in significant proportion of patients with Wilson′s disease (WD). [1] MRI provides an insight into the pathologic and anatomic correlates of clinical manifestations in WD. [2] Based on neuropsychiatric symptoms and structural brain lesions, Oder et al. identified 3 subgroups. Imaging showed dilatation of the third ventricle, focal thalamic lesions, and putaminal and pallidal lesions in the 3 subgroups, respectively. [3] The findings in our case were similar to an MRI study of 100 patients with WD showing atrophic changes of the cerebrum, brainstem, and cerebellum, as well as signal abnormalities in the basal ganglia, thalami, brainstem, cerebellum, and cerebral white matter. [2] An additional interesting, previously unreported finding in our case was the involvement of the Fields of Florel and the internal medullary lamina (IML) between the medial and lateral thalamic groups of nuclei. The IML involvement seen as a curvilinear hyperintensity appeared to divide the thalamus into 2 halves giving a characteristic "split thalamus" appearance. While thalamic involvement in WD is common, no mention of changes in the IML is previously reported. The only MR differential for such IML involvement is Fucosidosis type 1, a rare autosomal recessive lysosomal storage disease. [4] References
Copyright 2010 - Neurology India The following images related to this document are available:Photo images[ni10187f1.jpg] |
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