Objective: To evaluate the sensitivity of different MRI sequences in Wilson disease (WD) with neurological manifestations and its correlation with clinical features and outcome.
Methods: 34 WD patients with neurological manifestation with a median age of 14 years were included. Their Mini Mental State Examination (MMSE) score, movement disorders and laboratory findings were noted. Cranial MRI in T1, T2, FLAIR and DW sequences were done. Outcome at 6 months was categorized into improved (>1 grade improvement), static or worsening.
Results: MRI was abnormal in all and revealed involvement of putamen in 29 (85.3%), caudate in 23 (67.6%), brainstem and globus pallidus in 21 (61.8%) each, thalamus in 20 (58.8%), cerebral cortex in 9 (26.5%), subcortical white matter in 8 (23.5%), and cerebellum in 2 (5.9%) patients. The overall sensitivity of T2 and FLAIR was 97.1% each, DWI 38.2% and T1 31.4%. None had contrast enhancement and 4 had reduced ADC value. Choreoathetosis correlated with thalamic, pallidal and putaminal lesions; MMSE with subcortical white mater. MRI load correlated with age, tremor, psychiatric disorder, choreoathetosis, and severity of WD. At 6 months 9 (26.5%) patients improved, 18 (52.9%) remained static and 6 (17.6%) deteriorated.
Conclusion: In neurologic WD, putaminal involvement is the commonest; T2 and FLAIR sequences have similar sensitivity and number of MRI lesions correlated with disease severity but not with outcome.
Keywords: ADC; Athetosis; Chorea; Cognitive impairment; DRI; FLAIR; MRI; Movement disorder; Wilson disease; Worsening.
Copyright © 2015. Published by Elsevier B.V.