Perry, Elaine K

Publication Detail

The publication detail shows the title, authors (with indicators showing other profiled authors), information on the publishing organization, abstract and a link to the article in PubMed. This abstract is what is used to create the fingerprint of the publication. If any grants are referenced by the publication, they will be listed here as well.

Clinical and neuropathologic features of progressive supranuclear palsy with severe pallido-nigro-luysial degeneration and axonal dystrophy.

Zeshan Ahmed; Keith A Josephs; John Gonzalez; Anthony DelleDonne; Dennis W Dickson (Profiled Author: Dickson, Dennis)

Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL, USA.
Brain : a journal of neurology 2008;131(Pt 2):460-72.

Pallido-nigro-luysial atrophy (PNLA) is a rare disorder that in many cases has histopathological features similar to progressive supranuclear palsy (PSP). In a pathological series of over 400 cases of PSP, eight cases were noted to have features similar to those described in PNLA, including severe atrophy and neuronal loss in the globus pallidus, substantia nigra and subthalamic nucleus, in addition to many axonal spheroids in the globus pallidus and substantia nigra. These eight cases of PSP-PNLA were compared to 11 typical PSP cases with quantitative neuropathologic indices and assessment of demographics, clinical features and the timing of clinical features. PSP-PNLA cases were younger, had longer disease duration and more often were not initially diagnosed with PSP; in the end, they did not differ from PSP with respect to any major clinical feature. The clinical course of PSP-PNLA, however, was different, with earlier gait abnormalities and difficulty with handwriting, but later falls, rigidity and dysphagia than PSP. Pathologically, the same types of lesions were detected in both PSP and PSP-PNLA, but there were differences in the distribution and density of tau-pathology, with less tau-pathology in motor cortex, striatum, pontine nuclei and cerebellum in PSP-PNLA. These clinical and pathological findings suggest that PSP-PNLA should be considered a variant of PSP.

5 Originating Grant

• 1.

  Hardy, John A

  GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM

  30 September 1999 - 31 July 2004

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 3,886,270

• 2.

  Dickson, Dennis William

  Genetics and Molecular Biology of Parkinsonism

  30 September 1999 - 31 August 2009

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 8,904,777

• 3.

  Dickson, Dennis W

  GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM

  30 September 1999 - 29 September 2004

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 2,454,521

• 4.

  Hutton, Michael L

  Tau and Neurodegeneration II: A therapeutic target

  1 September 1999 - 31 May 2010

  NATIONAL INSTITUTE ON AGING

  Total Funding: $ 8,997,034

• 5.

  Dickson, Dennis William

  Tau and Neurodegeneration II: A therapeutic target

  1 September 1999 - 31 May 2010

  NATIONAL INSTITUTE ON AGING

  Total Funding: $ 4,182,608

Scientific Context

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