A 62-year-old right-handed man gradually experienced increasing difficulty with speech and manual dexterity. He had apraxia of speech, buccofacial apraxia, and complex limb apraxia as well as terminal dementia. At autopsy, focal cortical atrophy, neuronal loss, and neuropil rarefaction in the second and third cortical layers were most prominent in the left opercular, lower precentral, superior parietal, and left temporal pole. Numerous Pick bodies were diffusely present in the temporal and posterior frontal lobes and, to a lesser degree, in the superior parietal lobule. This report demonstrates an association between the distribution of Pick's pathology and several apraxic impairments.

Background: Cerebral microbleeds (MBs) have been well investigated in Alzheimer's disease (AD), but not very extensively in non-AD dementias or in dementia with Lewy bodies (DLB). Aims: To elucidate the clinical significance of MBs in DLB. Methods: We compared the prevalence, locations and risk factors for MBs in 59 DLB and 81 AD patients. We visually counted MBs in each of the cortical and subjacent areas (frontal, temporal, parietal and occipital), the basal ganglia and the thalamus, and the brainstem and the cerebellar hemispheres on 1.5-tesla T2*-weighted gradient-recalled-echo MRI images. White matter lesions were semiquantified in fluid-attenuated inversion recovery images according to the Fazekas rating scale. Results: While the prevalence of MBs was comparable, MBs tended to be more abundant in DLB than in AD in all brain areas with the exception of the occipital lobes. The number of MBs was positively associated with the severity of white matter lesions but not with other vascular risk factors in either AD or DLB. The presence of MBs could be associated with cognitive impairment at onset. MB-positive DLB patients showed less impairment on ¹²³I-metaiodobenzylguanidine myocardial scintigraphy (MIBG scintigraphy) images, supporting the notion of an inverse relationship between vascular lesions and Lewy body pathology. Conclusion: It was suggested that an intricate association between Lewy body pathology, AD-type pathologies and vascular lesions seems to be related to the initial symptoms and results of MIBG scintigraphy in DLB.

Visuospatial ability is one of the important contributors to functional status.

The distribution of the basal ganglia lesions, including the amygdala, striatum, and pallidum, were investigated neuropathologically in eight Japanese autopsy cases of Pick's disease with Pick bodies. The lesions were classified as mild, moderate or severe. The degree and distribution of basal ganglia lesions in all eight cases were uniform: the amygdala showed severe to moderate lesions, the caudate nucleus and putamen showed moderate to mild lesions, and the pallidum showed mild lesions. Furthermore, the lesions in the amygdala were more prominent in the basolateral group than in the corticomedial group. In Pick's disease with Pick bodies, the degree and distribution of the lesions within the basal ganglia differs from those reported in both ‘Pick's disease without Pick bodies’ and corticobasal degeneration (CBD), in which severe lesions were present in the pallidum. These neuropathological findings may contribute to the morphological differential diagnosis among Pick's disease with Pick bodies, ‘Pick's disease without Pick bodies’, and CBD.

We investigated six Japanese autopsy cases of Pick's disease with Pick bodies (PDPB) both clinically and pathologically, and examined the distribution of their cerebral cortical lesions using hemisphere and/or bisphere specimens. The lesions were classified into three categories (slight, moderate, and severe). Two patients with a clinical diagnosis of primary progressive apraxia and of slowly progressive aphasia had speech apraxia as their initial signs, and the other two patients were suspected as having Alzheimer's disease, with the clinical diagnosis of the remainder two patients being presenile dementia and depression, respectively. Extrapyramidal signs, believed to be rare in PDPB, were present in four patients. Severe lesions were multicentrically present in the cerebral cortices of all six cases. In two patients with speech apraxia, severe lesions were seen in the primary motor area, which generally has not been regarded as an "atrophic center" in Pick's disease. Furthermore, in a patient with depression, severe lesions were more widespread in the convexity than in the orbital region of the frontal lobe. The parietal lobes, including the postcentral gyrus usually believed to be spared in Pick's disease, were severely involved in three patients. We postulate that the clinical features of PDPB have a much wider spectrum than previously believed. In addition, we believe that the distribution of the cerebral cortical lesions in PDPB is more widespread than previously assumed, and that clinical manifestations of PDPB depend to some extent on the topographic distribution of the cerebral cortical lesions.