Symptomatology and Neuropathology of patients presenting with focal cortical signs

• Published in: Neuropathology Vol. 43; no. 1; pp. 27 - 43
• Main Authors: Ishihara, Kenji, Fukui, Toshiya, Kawamura, Mitsuru, Shiota, Jun‐ichi, Nakano, Imaharu
• Format: Journal Article
• Language: English
• Published: Melbourne John Wiley & Sons Australia, Ltd 01.02.2023; Wiley Subscription Services, Inc
• Subjects: Aged; agraphia; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - pathology; Apraxia; Apraxias - pathology; Cognitive ability; Degeneration; Dementia - pathology; Dementia disorders; Female; Frontal gyrus; Frontal lobe; Humans; Immunohistochemistry; Limbic system; Male; Middle Aged; Motor neurons; Motor Neurons - pathology; Neuropathology; Niemann-Pick disease; Paralysis; Parietal lobe; Pick disease; Precentral gyrus; Speech; Speech disorders; Temporal lobe; Temporal Lobe - pathology; agraphia; Pick disease; amyotrophic lateral sclerosis; apraxia; neuropathology
• ISSN: 0919-6544; 1440-1789
• DOI: 10.1111/neup.12854

Here, we describe two patients who presented with focal cortical signs and underwent neuropathological examination. Case 1 was a 73‐year‐old woman with progressive speech disorder and abnormal behavior. She showed agraphia of the frontal lobe type, featured by the omission of kana letters when writing, other than pyramidal tract signs, pseudobulbar palsy, and frontal lobe dementia. Neuropathological examination, including TAR DNA‐binding protein 43 (TDP‐43) immunohistochemistry, revealed bilateral frontal and anterior temporal lobe lesions accentuated in the precentral gyrus and posterior part of the middle frontal gyrus. Both upper and lower motor neurons showed pathological changes compatible with amyotrophic lateral sclerosis. Case 2 was a 62‐year‐old man with progressive speech disorder and hand clumsiness. He had a motor speech disorder, compatible with apraxia of speech, and limb apraxia of the limb‐kinetic and ideomotor type. Neuropathological examination revealed degeneration in the left frontal lobe, including the precentral gyrus, anterior temporal, and parietal lobe cortices. Moreover, numerous argyrophilic neuronal intracytoplasmic inclusions (Pick body) and ballooned neurons were observed in these lesions and the limbic system. The pathological diagnosis was Pick disease involving the peri‐Rolandic area and parietal lobe. In these two cases, the distribution of neuropathological changes in the cerebral cortices correlated with the clinical symptoms observed.