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mds25693-sup-0001-suppvideo.mpg7614K%(A) Photograph of the superior surface of the brain (15% buffered formalin at room temperature for 3 weeks), demonstrating atrophy (arrow) of the precentral gyrus. (B) Photograph of a coronal slice (3 mm in agar) through the area affected. The cortical ribbon is notably thin (curved arrow), and the underlying corpus callosum atrophic (arrowhead). (C,D) Photomicrographs of haematoxylin and eosin stained sections through the substantia nigra (C) showing depigmentation and gliosis in association with a relatively low density of neurons and a typical cerebellar folia (D) showing gliosis in the Purkinje layer, but with no neuron loss. (E,F) Photomicrographs of tau-positive (T5530, Sigma, St Louis, Mo. USA, diluted 1:10,000, peroxidase visualisation) glia in the precentral gyrus, stained using immunohistochemistry to tau-II. (G,H) Photomicrographs of tau-positive globose tangle in the subthalamus (G) and inferior olive (H). (I,J) Photomicrographs of the substantia nigra showing pigmented neurons containing tau-positive (arrow, I) and silver (modified Bielschowsky) stained (arrow, J) globose tangles. Normal appearing nigral neurons are indicated (arrowheads, I,J). The neuromelanin pigment has a dark-brown to black appearance, while the abnormal neurofibrillary tangles appear brown. No astrocytic plaques or other ubiquitin (Z0458, Dako, Glostrup, Denmark, diluted 1:200) or α-synuclein (18-0215, Zymed Laboratories Inc., San Francisco, Ca, USA, diluted 1:200) immunopositive pathologies were found. Scales for E-G are equivalent to that in H. Scale for I is equivalent to that in J.
mds25693-sup-0002-suppfig.docx1793KThe video shows the patient in 2001 and demonstrates the fine irregular tremor in his left hand with bursts of myoclonus superimposed.

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