PSPA Talk Alistair Church Dept Neurology Royal Gwent Hospital - - PowerPoint PPT Presentation

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PSPA Talk Alistair Church Dept Neurology Royal Gwent Hospital - - PowerPoint PPT Presentation

Dec 19, 2022 335 likes •673 views

PSPA Talk Alistair Church Dept Neurology Royal Gwent Hospital What are you going to do about it ? How long have I got? What is going on in my Brain? Who is going to help me? Are you sure? PSP What is that? What is going to happen to me?

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PSPA Talk

Alistair Church Dept Neurology Royal Gwent Hospital

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PSP

What are you going to do about it ? Are you sure? Who is going to help me? What is going to happen to me? What can you do for me? What is going on in my Brain? How long have I got? What else could it be? What is that?

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“A chilled, slow, earthy, fixed old man. A cadaverous man of measured speech. An old man who seemed as unable to win, as if his eyelids had been nailed to his forehead. An old man whose eyes – two spots of fire – had no more motion than [sic] if they had been connected with the back of his skull by screws driven through it, and riveted and bolted

  • utside, among his grey hair.”

“He had come in and shut the door, and now he sad down. He did not bend himself to sit, as other people do, but seemed to sink bolt upright, as if in water, until the chair stopped him.”

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Hx of PSP

“It looks like a funny kind of Parkinsonism”

  • Professor Gilbert H. Glaser, 1963

April 1964 Progressive Supranuclear PalsyA Heterogeneous Degeneration Involving the Brain Stem, Basal Ganglia and Cerebellum With Vertical Gaze and Pseudobulbar Palsy, Nuchal Dystonia and Dementia JOHN C. STEELE, MD; J. CLIFFORD RICHARDSON, MD; JERZY OLSZEWSKI, MD

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PSP-RS PSP-P PSP-PGF PSP-F PSP-CBS PSP-OM PSP-SL PSP-C PSP-PLS

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What is that?

  • Progressive Spranuclear Palsy
  • Neurodegenerative disorder
  • Disorder of: gait, balance, eye

movement, bulbar function, cognition.

  • Parkinsonism poorly responsive to L-

dopa

  • Tauopathy
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Why me?

  • Unlucky 5/100,000. male>female. Over 40yrs
  • Sporadic. Less than 1% monogenic related to

Chromosome 17.

  • Genome wide association studies (GWAS)
  • 3 genes. (STX6, EIF2AK3, MOBP)
  • H1/ H1c Haplotype MAPT
  • Environment
  • Epigenetics
  • Guadeloupe
  • Northern France
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How do you know?

  • Clinical diagnosis
  • Diagnostic criteria
  • No good biomarkers
  • Imaging
  • Pathology
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What else could it be?

  • Review of 60 patients with a clinical diagnosis of

PSP

  • 47 PSP (78%)
  • 3 PD with additional Lewy bodies
  • 1 Alzheimer’s
  • 4 Multisystem atrophy
  • 1 each of CBS, Picks, MND, CVD, FTD with PD
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  • Tau deposited in
  • Basal ganglion
  • Brain stem
  • Cortex
  • Cerebellum
  • Spinal cord
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What’s going to happen to me?

  • Progressive
  • Variable
  • Balance
  • Bulbar dysfunction
  • Vision
  • Cognition
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What can you do for me?

  • Diagnosis
  • Education
  • Support
  • Management
  • Research
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Diagnosis

  • History
  • Falls
  • Unresponsive to L-dopa
  • Progressive
  • Examination
  • Parkinsonism- symmetrical
  • Up-right posture
  • Eye-lid apraxia
  • Eye movement disorder
  • Postural instability
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Support services

  • PSP society
  • Physiotherapy
  • Occupational Therapy
  • General Practice
  • Social services
  • Dietician
  • Speech and language therapy
  • Gastroenterology/ Respiratory

medicine

  • Palliative care
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Management - Motor

  • Physiotherapy/ Occupational Therapy
  • L-dopa
  • Amitriptyline
  • Amantadine
  • Botox
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Management - Vision

  • Lubrication
  • Prisms
  • Dark glasses
  • Botox
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Management - Mood

  • Depression
  • SSRI/ SNRI
  • Impulsivity
  • Epilim
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Management – Nutrition/Communication

  • SLT
  • Swallow
  • Hyper-salivation
  • Communication
  • Dietician
  • PEG feeding
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Management – Advance Care

  • Palliative care
  • Advanced Directives
  • Power of attorney
  • Will
  • Caregiver support
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How long have I got?

  • 8.7yrs
  • 12.8yrs (PSP –P, PSP-PGF
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What are you going to do about it?

  • Correct diagnosis
  • Support
  • Education
  • Coordinate care
  • Research
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What are the challenges?

  • Ascertaining patients with rare

disease at an early stage

  • Firm diagnosis at an early stage for

PSP and CBD

  • Monitoring the disease progress
  • Understanding disease biology
  • Research Infrastructure
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Research Network / PROSPECT Study

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Research Network / PROSPECT Study Sites

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PROSPECT PSP

Aims ims Natu atural al h histo istory: : up to 7 years Clinical Assessment/MRI/CSF/Blood biomarkers/Psychology. Cr Cross ss-se sectio ctional al: (“one-stop/light touch”) Clinical Assessment / DNA / Consent to re-contact Lo Longitu itudin inal al: 1yr follow up, biomarkers, CSF 862 p patie atients ts an and co countin ting

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Drug Research

  • Co enzyme Q10
  • Riluzole
  • GSK inhibitors
  • Lithium
  • Tideglusib
  • Microtubule stabilizers
  • Davunetide
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Passport

  • Monoclonal antibody
  • BMS-986168
  • Completed 2 phase 1 studies
  • Plan RCT 459 PSP patients worldwide
  • Closed for recruitment Sept 2018
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ARISE Trial

  • Monoclonal antibody
  • 378 patients
  • Open Jan 2018
  • Stopped July 2019
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Thanks very much

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For more information

Website site www.pspassociation.org.uk He Helplin ine 0300 0110 122 Email mail helpline@pspassociation.org.uk Write ite to to us s at at Freepost PSPA