Chromatic Pupillometry to Assess the Melanopsin-Light Pathway in Progressive Supranuclear Palsy

NCT03330353 | Unknown

• 1 other identifier: 2017p001603
• Study Type: observational
• Target: 56
• Locations: 1 country
• Sites: 1

Brief Summary

The specific aim of this study is to investigate rod, cone and melanopsin driven pupillary light response in individuals with progressive supranuclear palsy (PSP), age-matched healthy controls and individuals with other neurodegenerative diseases using chromatic pupillometry, with special interest in assessing melanopsin-driven post-illumination pupil response (PIPR) as an identifier for PSP. The study addresses the following hypotheses:

1. 1.Chromatic pupil responses, including rod/cone-driven rapid phase constriction and melanopsin-driven PIPR, are reduced in subjects with PSP compared to age-matched normal healthy control subjects,
2. 2.Pupil parameters of the melanopsin-driven PIPR are abnormal in PSP subjects without supranuclear palsy, which is indicative of a subclinical physiological deficit of the OPN in the early stages of PSP.

Conditions

PSP - Progressive Supranuclear Palsy; PD - Parkinson's Disease; AD - Alzheimer's Disease; ALS (Amyotrophic Lateral Sclerosis)

Outcome Measures

Primary Outcomes (2)

• Maximal Pupil Constriction

  The smallest pupil size following light stimulation. This parameter primarily represents rapid phase extrinsic ipRGC activity driven by rods and cones through synaptic input.

  2 years

• Post-illumination pupil response (PIPR)

  Measured pupil diameter over a period of 20 seconds, from 10 to 30 seconds after the offset of light stimulation.

  2 years

Study Arms (1)

Neurodegenerative Diseases

Individuals with neurodegenerative diseases

Diagnostic Test: Pupillometry

Interventions

Pupillometry DIAGNOSTIC_TEST

Use of pupillometry to assess melanopsin-light pathway in patients with neurodegenerative diseases

Neurodegenerative Diseases

Eligibility Criteria

• Age: 55 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Subjects diagnosed with PSP and age-matched control subjects

You may qualify if:

• Individuals that meet the clinical criteria for PSP. Core features include:
• Recurrent falls and unsteady gait
• Axial and nuchal rigidity
• Pseudobulbar palsy
• Bilateral lid retraction
• Supranuclear vertical gaze palsy
• Atrophy of the midbrain tegmentum (the hummingbird sign on brain MRI,
• Individuals that fit the criteria for the second PSP phenotype (which resembles PD) that has asymmetric findings, tremors and poor responses to treatment with Levodopa,
• Individuals that meet the clinical criteria for PD with:
• Progressive bradykinesia
• Postural instability and frequent falls
• Festinating gait with loss of associated movements
• Cogwheel rigidity and mask-like face
• Rest tremor,
• Individuals who carry a diagnosis of Alzheimer' disease who present with progressive impairment of memory and cognitive domains such as language and visuospatial perception.

You may not qualify if:

• Individuals who are frail or in questionable health,
• Individuals with cataracts or with posterior pole ocular pathology such as age-related macular degeneration and optic neuropathies, including open angle high intraocular pressure glaucoma,
• Individuals with photophobia (i.e., painful light sensitivity) when exposed to bright light, including those with ophthalmological conditions such as keratitis (herpes simplex), uveitis or Achromatopsia,
• Individuals with advanced dementia with inability to sit erect, hold the eyes open, incontinence,
• Individuals with epilepsy,
• Individuals diagnosed with major depression or other severe psychiatric disorders

Sponsors & Collaborators

• Massachusetts General Hospital: lead
• NeurOptics Inc.: collaborator
• University of Toronto: collaborator

Study Sites (1)

Massachusetts General Hospital

Boston, Massachusetts, 02114, United States

RECRUITING

MeSH Terms

Conditions

Supranuclear Palsy, Progressive; Parkinson Disease; Alzheimer Disease; Amyotrophic Lateral Sclerosis

Condition Hierarchy (Ancestors)

Basal Ganglia Diseases; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Movement Disorders; Ophthalmoplegia; Ocular Motility Disorders; Cranial Nerve Diseases; Tauopathies; Neurodegenerative Diseases; Paralysis; Neurologic Manifestations; Eye Diseases; Signs and Symptoms; Pathological Conditions, Signs and Symptoms; Parkinsonian Disorders; Synucleinopathies; Dementia; Neurocognitive Disorders; Mental Disorders; Spinal Cord Diseases; Motor Neuron Disease; TDP-43 Proteinopathies; Neuromuscular Diseases; Proteostasis Deficiencies; Metabolic Diseases; Nutritional and Metabolic Diseases

Study Officials

• Shirley H Wray, MD, PhD

  Massachusetts General Hospital

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Shirley H Wray, MD, PhD

CONTACT

617-726-5539; wray@helix.mgh.harvard.edu

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor of Neurology, Harvard Medical School

Study Record Dates

• First Submitted: September 19, 2017
• First Posted: November 6, 2017
• Study Start: November 1, 2017
• Primary Completion: October 1, 2019
• Study Completion: October 1, 2019
• Last Updated: November 7, 2017

Record last verified: 2017-11

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)