Corticobasal Degeneration Substantiated by Imaging Studies

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TO THE EDITOR: Corticobasal degeneration (CBD) is a slowly progressive disorder, characterized by initially unilateral akinesia, rigidity, apraxia, cortical sensory impairment, postural instability, and the alien hand syndrome. 1 Mathuranath PS Xuereb JH Bak T et al. Corticobasal ganglionic degeneration and/or frontotemporal dementia?. J Neurol Neurosurg Psychiatry. 2000; 68: 304-312 Google Scholar , 2 Graham NL Bak TH Hodges JR Corticobasal degeneration as a cognitive disorder. Mov Disord. 2003; 18: 1224-1232 Google Scholar , 3 Doran M du Plessis DG Enevoldson TP et al. Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci. 2003; 216: 127-134 Google Scholar The diagnosis can be challenging, given the variable presentations that reflect which brain region is affected first. Neuropathology is said to provide a definitive diagnosis, with neuronal achromasia and cortical atrophy of the frontoparietal region. 1 Mathuranath PS Xuereb JH Bak T et al. Corticobasal ganglionic degeneration and/or frontotemporal dementia?. J Neurol Neurosurg Psychiatry. 2000; 68: 304-312 Google Scholar , 3 Doran M du Plessis DG Enevoldson TP et al. Pathological heterogeneity of clinically diagnosed corticobasal degeneration. J Neurol Sci. 2003; 216: 127-134 Google Scholar SPECT can show asymmetrical parietal hypoperfusion, 2 Graham NL Bak TH Hodges JR Corticobasal degeneration as a cognitive disorder. Mov Disord. 2003; 18: 1224-1232 Google Scholar , 4 Slawek J Lass P Derejko M et al. Cerebral blood flow SPECT may be helpful in establishing the diagnosis of progressive supranuclear palsy and corticobasal degeneration. Nuclear Medicine Review Central Eastern Europe. 2001; 4: 73-76 Google Scholar , 5 Zhang L Murata Y Ishida R et al. Differentiating between progressive supranuclear palsy and corticobasal degeneration by brain perfusion SPECT. Nucl Med Commun. 2001; 22: 767-772 Google Scholar and focal atrophy can be seen on MRI. 6 Yekhlef F Ballan G Macia F et al. Routine MRI for the differential diagnosis of Parkinson's disease, MSA, PSP, and CBD. J Neural Transm. 2003; 110: 151-169 Google Scholar We describe a case of CBD in which the patient presented with few and mild symptoms, and where, over time, imaging studies were helpful diagnostic adjuncts.