Postmorbid learning of saxophone playing in a patient with frontotemporal dementia.

Some patients with frontotemporal dementia (FTD) show an artistic enhancement of musical abilities. However, no patients with FTD, to date, have been reported to be able to learn how to play a musical instrument after disease onset. Herein we describe a patient (J. K.) who had never played any musical instruments premorbidly, but who learned to play the saxophone after being diagnosed with a behavioral variant of FTD. He mastered a repertoire that consisted of 10 pieces of Korean folk songs over a period of three years. Furthermore, his saxophone skills were high enough to outperform other students in his class.

Shape changes of the basal ganglia and thalamus in Alzheimer's disease: a three-year longitudinal study.

BACKGROUND: A large number of Alzheimer's disease (AD) studies have focused on medial temporal and cortical atrophy, while changes in the basal ganglia or thalamus have received less attention. OBJECTIVE: The aim of this study was to investigate the existence of progressive topographical shape changes in the basal ganglia (caudate nucleus, putamen, and globus pallidus) and thalamus concurrent with AD disease progression over three years. This study also examined whether declines in volumes of the basal ganglia or thalamus might be responsible for cognitive decline in patients with AD. METHODS: Thirty-six patients with early stage AD and 14 normal control subjects were prospectively recruited for this study. All subjects were assessed with neuropsychological tests and MRI at baseline and Years 1 and 3. A longitudinal shape analysis of the basal ganglia and thalamus was performed by employing a boundary surface-based shape analysis method. RESULTS: AD patients exhibited specific regional atrophy in the right caudate nucleus and the bilateral putamen at baseline, and as the disease progressed, regional atrophic changes in the left caudate nucleus were found to conform to a distinct topography after controlling the total brain volume. Volumetric decline of the caudate nucleus and putamen correlated with cognitive decline in frontal function after controlling for age, gender, education, follow-up years, and total brain volume changes. CONCLUSION: Our findings suggest that shape changes of the basal ganglia occurred regardless of whole brain atrophy as AD progressed and were also responsible for cognitive decline that was observed from the frontal function tests.

Changes in subcortical structures in early- versus late-onset Alzheimer's disease.

Patients with early-onset Alzheimer's disease (EOAD) are reported to be different from those with late-onset Alzheimer's disease (LOAD) in terms of neuropsychological and neuroimaging findings. In this study, we aimed to compare the longitudinal volume changes of 6 subcortical structures (the amygdala, hippocampus, thalamus, putamen, globus pallidus, and caudate nucleus) between patients with EOAD and LOAD for 3 years. We prospectively recruited 36 patients with probable Alzheimer's disease (14 EOAD, 22 LOAD) and 14 normal control subjects. We analyzed the volume of subcortical structures using an automatic surface-based method. At baseline, there were no differences in the volumes of subcortical structures between patients with EOAD and LOAD. However, over 3 years of longitudinal follow-up, patients with EOAD showed more rapid volumetric decline in the caudate, putamen, and thalamus than patients with LOAD, which is consistent with neuropsychological results. Our findings suggested that the cognitive reserve theory might be applicable to explain different decline rates of the volumes of the basal ganglia and thalamus according to onset age.

Hanja (Ideogram) alexia and agraphia in patients with semantic dementia.

Posterior fusiform gyrus (BA 37) is responsible for Hanja (ideogram) alexia in stroke patients. Patients with semantic dementia (SD) have lesions in the basal temporal area. The close proximity in these two lesions and the fact that reading ideograms requires holistic processing as is necessary in recognition of objects, suggests a possibility that ideogram alexia/agraphia may occur in patients with SD. We established and carried out Hanja and Hangul (phonogram) reading/writing tasks on six SD patients and nine Alzheimer's disease (AD) patients as control to see if these two patient groups show dissociation in the two sets of tests. SPM analysis was performed on the SD patients' PET images to look for any dysfunctions in the posterior fusiform gyrus. The SD patients manifested Hanja alexia/agraphia whereas Hangul reading/writing ability was relatively preserved. There were group differences between SD and AD in the Hanja tasks but not in the Hangul tasks. The SPM analysis revealed no hypometabolism in the posterior fusiform gyrus, but only in the middle and the anterior part of the temporal gyrus. Dysfunction in the middle temporal gyrus (BA 21) may have disrupted the temporal lobe connections preventing the function of the posterior fusiform gyrus.

Serial positron emission tomography findings in a patient with hydrocephalic dementia and Alzheimer's disease.

Comorbidity of normal pressure hydrocephalus (NPH) and Alzheimer's disease (AD) is not uncommon. However, few studies have reported the clinical courses of these patients in depth. A 73-year-old woman was confirmed to have AD by a biopsy performed during a shunt operation for NPH after a head trauma. She was followed for 4 years using serial neuropsychological tests and positron emission tomography (PET). Her clinical symptoms remained improved for 2.5 years and then declined. The 1-year minus the presurgical PET scan highlighted the bilateral frontal area, basal ganglia, and thalamus, which may reflect brain regions associated with the improvement of hydrocephalic dementia. On the other hand, the 1-year minus the 4-year scan highlighted the bilateral temporoparietal area and the posterior cingulate gyrus, which may reflect brain regions associated with the aggravation of AD. This subtraction method may be useful for monitoring the clinical course in patients with NPH and AD.