This case was longitudinally enrolled in an ethically approved brain donor program associated with the Sydney Brain Bank at Neuroscience Research Australia. The brain was removed and processed by the Sydney Brain Bank, which has ethics approval for brain tissue banking and is funded by the National Health and Medical Research Council of Australia, the University of New South Wales, and Neuroscience Research Australia.
Cortical limb myoclonus in pathologically proven progressive supranuclear palsy
Article first published online: 4 OCT 2013
© 2013 Movement Disorder Society
Volume 28, Issue 13, pages 1804–1806, November 2013
How to Cite
Kemp, S., Harding, A. J., Halliday, G. M., Mahant, N. and Fung, V. S.C. (2013), Cortical limb myoclonus in pathologically proven progressive supranuclear palsy. Mov. Disord., 28: 1804–1806. doi: 10.1002/mds.25693
Relevant conflicts of interest/financial disclosures: G.M.H. is funded as a Senior Principal Research Fellow of the National Health and Medical Research Council of Australia to perform research.
Full financial disclosures and author roles may be found in the online version of this article.
- Issue published online: 13 NOV 2013
- Article first published online: 4 OCT 2013
- Manuscript Accepted: 28 JUL 2013
- Manuscript Revised: 14 JUL 2013
- Manuscript Received: 23 FEB 2013
Additional Supporting Information may be found in the online version of this article.
|mds25693-sup-0001-suppvideo.mpg||7614K||%(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.docx||1793K||The 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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