We describe a patient with corticobasal syndrome in whom neuropathological examination on autopsy revealed Pick and Alzheimer diseases in comorbidity. Corticobasal degeneration is a tauopathy usually associated with asymmetric parkinsonism, parietal lobe involvement, and cognitive impairment. Corticobasal syndrome is the clinical presentation of corticobasal degeneration without neuropathological confirmation. A 66-year-old right-handed man slowly developed speech difficulties, right-hand clumsiness, and forgetfulness. His speech apraxia progressed to mutism with preserved comprehension, and his clumsiness progressed to severe apraxia involving both hands. He developed behavioral changes and severe amnesia. All of these features were consistent with corticobasal syndrome. His loss of episodic, verbal, and visuospatial memory suggested Alzheimer disease; however, beyond his frontotemporal neuropsychological profile, he had few symptoms characteristic of frontal lobe dementia. Magnetic resonance imaging scans showed worsening temporal, frontal, and parietal atrophy, predominant in the left hemisphere. Neuropathological examination at autopsy revealed abundant neuritic plaques and neurofibrillary tangles consistent with fully developed Alzheimer disease, as well as numerous intraneuronal Pick bodies in the frontotemporal lobes. Our findings confirm the importance of clinical and neuropathological correlations in patients with atypical neurodegenerative dementias.
*Department of Neurology, Thomayer Hospital and Institute for Postgraduate Education in Medicine, Prague, Czech Republic
†Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine, Charles University in Prague, and General University Hospital in Prague, Czech Republic
‡Neurocenter Caregroup Rychnov nad Kněžnou, Czech Republic
§Department of Neurology, Faculty of Medicine in Hradec Králové, Charles University in Prague, Czech Republic
∥Department of Pathology and Molecular Medicine, Thomayer Hospital, Prague, Czech Republic
Supported by research program MŠM 0021620849 from the Czech Ministry of Education and grant IGA NT 12094-5/2011 from the Czech Ministry of Health.
The authors declare no conflicts of interest.
Reprints: Robert Rusina, MD, PhD, Department of Neurology, Thomayer Hospital, Vídeňská 800, 140 59 Prague 4, Czech Republic (e-mail: firstname.lastname@example.org).
Received January 8, 2013
Accepted November 8, 2013