Pick’s disease with Pick bodies: An unusual autopsy case showing degeneration of the pontine nucleus, dentate nucleus, Clarke’s column, and lower motor neuron

• Published in: Neuropathology 2007-02, Vol.27 (1), p.81-89
• Main Authors: Oda, Tatsuro, Tsuchiya, Kuniaki, Arai, Tetsuaki, Togo, Takashi, Uchikado, Hirotake, De Silva, Rohan, Lees, Andrew, Akiyama, Haruhiko, Haga, Chie, Ikeda, Kenji, Kato, Motoichiro, Kato, Yuji, Hara, Tsunekatsu, Onaya, Mitsumoto, Hori, Koji, Teramoto, Hiroshi, Tominaga, Itaru
• Format: Article
• Language: English
• Subjects: Age of Onset; Autopsy; Brain - pathology; bulging eyes; Cerebellar Nuclei - pathology; Exophthalmos - etiology; Female; Humans; Immunohistochemistry; Middle Aged; Motor Neurons - pathology; myoclonus; Myoclonus - etiology; Pick body; Pick Disease of the Brain - complications; Pick Disease of the Brain - pathology; Pick Disease of the Brain - physiopathology; Pick’s disease; Pons - pathology; tau; Tomography, X-Ray Computed; topography
• ISSN: 0919-6544; 1440-1789
• DOI: 10.1111/j.1440-1789.2006.00744.x

We report a 51‐year‐old female with Pick’s disease with Pick bodies (PDPB) showing a brainweight of 530 g. This case was considered to be a very rare case of PDPB, in which the lesion developed in the temporal and frontal lobes and later spread to the parietal lobe, occipital lobe, brainstem, cerebellum and spinal cord. This case showed very atypical clinicopathological findings. Clinically, bulging eyes and myoclonus were observed. Neuropathologically, Pick bodies were widely distributed beyond the usual distribution areas to the parietal cortices, occipital cortices, dentate nuclei, motor neuron nuclei in the brain stem, and spinal cord. The atypical clinical symptoms and the widespread neuropathological abnormalities observed in this case seem to represent an extremely extended form of PDPB.