NCT06881368

Brief Summary

SUSAC's Syndrome (SS) is characterized by the clinical triad of encephalopathy, hearing loss, and retinal artery branch occlusions. Since the first description of SS in 1979, hundreds of patients with SS, mostly young women, have been reported. However, comprehensive epidemiological, clinical and etiological features of SS have never been specifically addressed so far.

Trial Health

77
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
180

participants targeted

Target at P50-P75 for all trials

Timeline
229mo left

Started Jul 2025

Longer than P75 for all trials

Geographic Reach
1 country

1 active site

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress4%
Jul 2025Feb 2045

First Submitted

Initial submission to the registry

January 29, 2025

Completed
2 months until next milestone

First Posted

Study publicly available on registry

March 18, 2025

Completed
4 months until next milestone

Study Start

First participant enrolled

July 1, 2025

Completed
19.4 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

November 30, 2044

Expected
3 months until next milestone

Study Completion

Last participant's last visit for all outcomes

February 15, 2045

Last Updated

May 7, 2026

Status Verified

April 1, 2026

Enrollment Period

19.4 years

First QC Date

January 29, 2025

Last Update Submit

May 6, 2026

Conditions

Susac Syndrome

Outcome Measures

Primary Outcomes (52)

  • To characterize the epidemiological Susac's Syndrome

    Variables collected at inclusion: Date of birth

    10 years

  • 1. To characterize the epidemiological of Susac's Syndrome

    Variables collected at inclusion: • Place of birth

    10 years

  • 1. To characterize the epidemiological of Susac's Syndrome

    • Variables collected at inclusion: Sex

    10 years

  • 1. To characterize the epidemiological of Susac's Syndrome

    • Variables collected at inclusion : Date of first symptom

    10 years

  • 1. To characterize the epidemiological of Susac's Syndrome

    • Variables collected at inclusion: Date of diagnosis

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at inclusion : • Personal medical history

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at inclusion : • Gynaecological history

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at inclusion : • Surgical history

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at inclusion : • Family history

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    • Variables collected at inclusion : Previous travel and vaccination status

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at inclusion : • Any symptoms (flu-like illness, fever)

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at each visit : • - Weight in kilograms

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at each visit : • - Tobacco consumption

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at each visit : • - Use of oestroprogestogenic contraception

    10 years

  • 2. To characterize the etiological of Susac's Syndrome

    Variables collected at each visit : • - Alcohol consumption

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : Headache

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Neurology: Neurological symptoms: present / absent / date of onset : • Sensory disturbance

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Neurology: Neurological symptoms: present / absent / date of onset : • Motor deficit

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Neurology: Neurological symptoms: present / absent / date of onset : • Osteotendinous pyramidal reflexes

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Neurology: Neurological symptoms: present / absent / date of onset : • Babinski sign

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Neurology: Neurological symptoms: present / absent / date of onset : • Hoffman's sign

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Tact sensitivity disorder

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Thermoal sensitivity deficit

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : Proprioceptive disorder : • If yes, specify : * Ataxia

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : Proprioceptive disorder : • If yes, specify : * Anomaly of the sense of position of the big toe

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Paresthesias

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Confusion

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Obnubilation

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Coma

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Agitation

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Temporo-spatial disorientation

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    1\) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Fluctuating memory disorders

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    • Any sequelae : Results of neurocognitive assessment : BREF

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Any sequelae : • Results of neurocognitive assessment: MMS

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Any sequelae : For mood disorders and quality of life: DSMIV criteria for depression

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Any sequelae : For mood disorders and quality of life: SF36

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Any sequelae : • For disability : Barthel Index

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    • Any sequelae : For disability : Rankin score

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology • Ophthalmological examination results : Fundus

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology • Ophthalmological examination results : Visual field

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology • Ophthalmological examination results : o Angiography

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    • Ophthalmology Ophthalmological symptoms: scotoma

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology : Ophthalmological symptoms: decrease in visual acuity

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophtalmology : Evolution of retinal arterial occlusions: improvement

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology : Evolution of retinal arterial occlusions: stability

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    Ophthalmology : Evolution of retinal arterial occlusions: worsening

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    • Ophthalmology : Sequelae

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    ENT : * Cochlear symptoms: vertigo, tinnitus, ataxia, hearing loss

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    ENT: • Audiogram results

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    ENT • Results of vestibular examinations

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    ENT • Course of deafness: improvement, stability, worsening

    10 years

  • 3. To characterize the clinical of Susac's Syndrome

    ENT : • Possible sequelae (functional discomfort, psycho-social repercussions, follow-up audiograms)

    10 years

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

The population to be studied will be patients with defined Susac syndrome, presenting with at least two of the signs of the clinical triad, having compatible complementary examinations and after elimination of differential diagnoses (see inclusion and non-inclusion criteria below). Patients will be included prospectively. The existence of an associated pathology (autoimmune, tumour, metabolic, etc.) is not a criterion for exclusion.

You may qualify if:

  • Patient over 18 years of age
  • Patient presenting with at least two of the signs of the clinical triad: encephalopathy, cochlear damage authenticated by an audiogram (uni- or bilateral, predominantly in the middle or low frequencies), retinal artery occlusion assessed by fundoscopy or fluorescein retinal angiography.

You may not qualify if:

  • Patient having been individually informed and objecting to the use of his/her data
  • Patient under legal protection (guardianship or curatorship)
  • Differential diagnosis established: multiple sclerosis, mitochondriopathy, CADASIL, primary tumour of the central nervous system, Lyme disease.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Hôpital Bichat

Paris, 75018, France

RECRUITING

MeSH Terms

Conditions

Susac Syndrome

Condition Hierarchy (Ancestors)

Ear DiseasesOtorhinolaryngologic DiseasesCerebrovascular DisordersBrain DiseasesCentral Nervous System DiseasesNervous System DiseasesNeurologic ManifestationsRetinal Artery OcclusionRetinal DiseasesEye DiseasesVision DisordersArterial Occlusive DiseasesVascular DiseasesCardiovascular DiseasesEye ManifestationsSigns and SymptomsPathological Conditions, Signs and Symptoms

Study Officials

  • Karim Sacre, MD, PHD

    Assistance Publique - Hôpitaux de Paris

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Karim Sacre, MD, PHD

CONTACT

+33140258705karim.sacre@aphp.fr

Thomas Papo, MD, PHD

CONTACT

thomas.papo@aphp.fr

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

January 29, 2025

First Posted

March 18, 2025

Study Start

July 1, 2025

Primary Completion (Estimated)

November 30, 2044

Study Completion (Estimated)

February 15, 2045

Last Updated

May 7, 2026

Record last verified: 2026-04

Locations

FR(1)