Wilson’s Disease: Clinical, Brain MRI and Tc-99m HMPAO SPECT Correlation

[摘要] Background: Brain damage secondary to Wilson's disease leads to several psychiatric and neurological manifestations. However, little research has been published about the role of anatomo-functional diagnostic modalities in the evaluation of the disease course. Objective: To evaluate and compare the role of brain MRI and Tc-99m HMPAO SPECT in assessing brain involvement in Wilson’s disease and correlating these findings with the clinical presentations. Methods:  Eighteen patients with established Wilson’s disease were included. The clinical picture was of neurologic type (nWD) in 11 patients (61.1%), and of the hepatic type (hWD) in 7 patients (38.9%). All patients were subjected to clinical assessment, cognitive assessment, ophthalmic slit lamp examination, biochemical tests including urinary copper and serum ceruloplasmin levels, abdominal ultrasound, liver biopsy (only in 5 patients), MRI of the brain and Tc-99m HMPAO brain SPECT. Results: Brain MRI was abnormal in 11/18 patients (sensitivity 61.1%). It was abnormal in 9/11 nWD patients (sensitivity 81.8%) and 2/7 of hWD patients (sensitivity 28.6%). Brain Tc-99m HMPAO SPECT was abnormal in 15 out of 18 patients (sensitivity 83.3%). The scan was abnormal in 10/11 patients with nWD (sensitivity 90.9%) and in 5/7 of patients with hWD (sensitivity 71.4 %). There was a good correlation between the clinical presentation of the disease and radiological and SPECT findings. Conclusion: In Wilson’s disease brain SPECT has a higher sensitivity than brain MRI in detecting brain damage. There was also a good clinico-anatomical correlation but it was still not clear yet in explaining some clinical aspects in some cases.   (Egypt J Neurol Psychiat Neurosurg. 2010; 47(1): 29-35)

[学科分类] 精神健康和精神病学