Susac SyndromeCARESS CohorteNCT06881368
Phenotypic and Etiological Characterization of Susac Syndrome
An observational study of Susac Syndrome, run by Assistance Publique - Hôpitaux de Paris at 1 site in France.
Trial 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. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Susac Syndrome
- How many people
- 180 participants
- Where
- 1 siteFrance
- Study dates
- 1 Jul 2025to 15 Feb 2045
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
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.
Who may be able to join
- 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.
Who cannot join
- 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.
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
Thomas Papo, MD, PHD contact
France1 site, 1 recruiting
- Recruiting
Hôpital Bichat
Paris, 75018
Karim Sacre, MD, PHD, +33140258705, karim.sacre@aphp.fr
About this study
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.
Read the detailed description
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.
The diagnosis of SS is difficult because its characteristic signs often do not occur simultaneously or may be too subtle for the patient to notice. Neurological features of SS may occur several months prior to other symptoms. The retinal artery branch occlusion, by occurring in the peripheral portion of the retina, may remain asymptomatic. Sensorineural hearing loss may also be asymptomatic and disclosed only by audiogram. Besides mild pleocytosis in cerebro-spinal fluid, all performed biological tests are virtually negative. No infectious agent, consistent autoimmune marker, or coagulopathy has been disclosed. Changes seen on brain MRI are well characterized although not specific. The only site from which biopsy material is available for pathological analysis is the brain. The most common finding in brain biopsies is the presence of microinfarcts but brain biopsy is not currently performed.
Although the treatment of SS has not been studied in controlled trials, most patients have a good response to treatment with glucocorticoids, with the addition of antithrobomtic therapy and, for cases in which the disease is refractory to steroids, intravenous immune globulin or cyclophosphamide. The clinical course is characterized by recurrent attacks involving 1 or more components of the triad that characterize the active phase of the disease. Remission usually occurs after the active phase but some patients show residual mild to moderate dementia or gait disturbance, and impaired hearing and vision.
Susac's Syndrome is a vasculopathy causing small infarcts in the cochlea, retina and brain. Proposed explanations include a hypercoagulable state, vasospasm, and vasculitis, none of which are supported by laboratory results or findings on brain biopsies. The unique distribution of arteriolar disease affecting the brain, the retina, and the cochlea suggests selective vulnerability of these three structures. The brain, retina, and cochlea all have a blood-tissue barrier, and the endothelium in these sites shares a common embryologic origin and unique structural and antigenic characteristics. It has therefore been proposed that SS is an autoimmune disease in which the endothelium is the primary target, and damage to the endothelium triggers arteriolar occlusion and microinfarcts. However, the pathogenesis remains unknown.
The objective of this study is to characterize the epidemiological, clinical, and etiological features of Susac's Syndrome. In this aim, we will constitute a national clinical-based cohort including all SS new cases prospectively observed. French Society of Neurology, Ophtalmology and Internal Medicine will be asked to collaborate.The exhaustive and systematic analysis of each case will help to better define different aspects of the disease such as the incidence and prevalence, the clinical presentation, the diagnostic modalities and the impact of treatments.
Because Susac's syndrome is a rare disease, we expect to include 180 patients in this cohort. The constitution of the cohort will last for 19 years (9 years of inclusion and 10 years of follow-up.
The conclusion of the study, based on statistical analysis done once all patients will be included in the cohort, should allow new recommendations in the diagnosis strategy and give new understandings of the therapeutic management of the disease. The result of this study may also give rise to hypothesis for an interventional study.
- Conditions
- Susac Syndrome
- Medical subject headings
- Susac Syndrome
What is being measured
The primary outcome is the main question the study is designed to answer.
To characterize the epidemiological Susac's Syndrome
Variables collected at inclusion: Date of birth
Measured: 10 years
1. To characterize the epidemiological of Susac's Syndrome
Variables collected at inclusion:
- Place of birth
Measured: 10 years
1. To characterize the epidemiological of Susac's Syndrome
- Variables collected at inclusion: Sex
Measured: 10 years
1. To characterize the epidemiological of Susac's Syndrome
- Variables collected at inclusion : Date of first symptom
Measured: 10 years
1. To characterize the epidemiological of Susac's Syndrome
- Variables collected at inclusion: Date of diagnosis
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at inclusion :
- Personal medical history
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at inclusion :
- Gynaecological history
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at inclusion :
- Surgical history
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at inclusion :
- Family history
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
- Variables collected at inclusion : Previous travel and vaccination status
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at inclusion :
- Any symptoms (flu-like illness, fever)
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at each visit :
- - Weight in kilograms
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at each visit :
- - Tobacco consumption
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at each visit :
- - Use of oestroprogestogenic contraception
Measured: 10 years
2. To characterize the etiological of Susac's Syndrome
Variables collected at each visit :
- - Alcohol consumption
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset : Headache
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Neurology: Neurological symptoms: present / absent / date of onset :
- Sensory disturbance
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Neurology: Neurological symptoms: present / absent / date of onset :
- Motor deficit
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Neurology: Neurological symptoms: present / absent / date of onset :
- Osteotendinous pyramidal reflexes
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Neurology: Neurological symptoms: present / absent / date of onset :
- Babinski sign
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Neurology: Neurological symptoms: present / absent / date of onset :
- Hoffman's sign
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Tact sensitivity disorder
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Thermoal sensitivity deficit
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
Proprioceptive disorder :
- If yes, specify :
- Ataxia
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
Proprioceptive disorder :
- If yes, specify :
- Anomaly of the sense of position of the big toe
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Paresthesias
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Confusion
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Obnubilation
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Coma
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Agitation
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Temporo-spatial disorientation
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Neurology: Neurological symptoms: present / absent / date of onset :
- Encephalopathy, If yes, specify the signs present:
- Fluctuating memory disorders
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Any sequelae : Results of neurocognitive assessment : BREF
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Any sequelae : • Results of neurocognitive assessment: MMS
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Any sequelae : For mood disorders and quality of life: DSMIV criteria for depression
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Any sequelae : For mood disorders and quality of life: SF36
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Any sequelae : • For disability : Barthel Index
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Any sequelae : For disability : Rankin score
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology
- Ophthalmological examination results : Fundus
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology
- Ophthalmological examination results : Visual field
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology
- Ophthalmological examination results : o Angiography
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Ophthalmology Ophthalmological symptoms: scotoma
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology : Ophthalmological symptoms: decrease in visual acuity
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophtalmology : Evolution of retinal arterial occlusions: improvement
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology : Evolution of retinal arterial occlusions: stability
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
Ophthalmology : Evolution of retinal arterial occlusions: worsening
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
- Ophthalmology : Sequelae
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
ENT :
- Cochlear symptoms: vertigo, tinnitus, ataxia, hearing loss
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
ENT:
- Audiogram results
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
ENT
- Results of vestibular examinations
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
ENT
- Course of deafness: improvement, stability, worsening
Measured: 10 years
3. To characterize the clinical of Susac's Syndrome
ENT :
- Possible sequelae (functional discomfort, psycho-social repercussions, follow-up audiograms)
Measured: 10 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted18 Mar 2025
- Study start1 Jul 2025
- Primary completion30 Nov 2044 (estimated)
- Study completion15 Feb 2045 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Susac SyndromeStudies of the same condition, open and under way.
- More trials run by Assistance Publique - Hôpitaux de ParisThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 180 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Assistance Publique - Hôpitaux de Paris
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Karim Sacre, MD, PHD, Principal investigator, Assistance Publique - Hôpitaux de Paris
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06881368
- Sponsor's protocol ID
- APHP241173
- Other IDs
- 2025-A00214-45
- Acronym
- CARESS Cohorte
- Status last verified
- Apr 2026
- First submitted
- 29 Jan 2025
- Last updated
- 7 May 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 7 May 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Phenotypic and Etiological Characterization of Susac Syndrome
- ClinicalTrials.gov ID
- NCT06881368
- Acronym / sponsor ID
- CARESS Cohorte / APHP241173
- Conditions
- Susac Syndrome
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 180 participants (estimated)
- Lead sponsor
- Assistance Publique - Hôpitaux de ParisUniversity, hospital or other
- Study start
- 1 Jul 2025 (actual)
- Primary completion
- 30 Nov 2044 (estimated)
- Study completion
- 15 Feb 2045 (estimated)
- First posted
- 18 Mar 2025 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
The diagnosis of SS is difficult because its characteristic signs often do not occur simultaneously or may be too subtle for the patient to notice. Neurological features of SS may occur several months prior to other symptoms. The retinal artery branch occlusion, by occurring in the peripheral portion of the retina, may remain asymptomatic. Sensorineural hearing loss may also be asymptomatic and disclosed only by audiogram. Besides mild pleocytosis in cerebro-spinal fluid, all performed biological tests are virtually negative. No infectious agent, consistent autoimmune marker, or coagulopathy has been disclosed. Changes seen on brain MRI are well characterized although not specific. The only site from which biopsy material is available for pathological analysis is the brain. The most common finding in brain biopsies is the presence of microinfarcts but brain biopsy is not currently performed.
Although the treatment of SS has not been studied in controlled trials, most patients have a good response to treatment with glucocorticoids, with the addition of antithrobomtic therapy and, for cases in which the disease is refractory to steroids, intravenous immune globulin or cyclophosphamide. The clinical course is characterized by recurrent attacks involving 1 or more components of the triad that characterize the active phase of the disease. Remission usually occurs after the active phase but some patients show residual mild to moderate dementia or gait disturbance, and impaired hearing and vision.
Susac's Syndrome is a vasculopathy causing small infarcts in the cochlea, retina and brain. Proposed explanations include a hypercoagulable state, vasospasm, and vasculitis, none of which are supported by laboratory results or findings on brain biopsies. The unique distribution of arteriolar disease affecting the brain, the retina, and the cochlea suggests selective vulnerability of these three structures. The brain, retina, and cochlea all have a blood-tissue barrier, and the endothelium in these sites shares a common embryologic origin and unique structural and antigenic characteristics. It has therefore been proposed that SS is an autoimmune disease in which the endothelium is the primary target, and damage to the endothelium triggers arteriolar occlusion and microinfarcts. However, the pathogenesis remains unknown.
The objective of this study is to characterize the epidemiological, clinical, and etiological features of Susac's Syndrome. In this aim, we will constitute a national clinical-based cohort including all SS new cases prospectively observed. French Society of Neurology, Ophtalmology and Internal Medicine will be asked to collaborate.The exhaustive and systematic analysis of each case will help to better define different aspects of the disease such as the incidence and prevalence, the clinical presentation, the diagnostic modalities and the impact of treatments.
Because Susac's syndrome is a rare disease, we expect to include 180 patients in this cohort. The constitution of the cohort will last for 19 years (9 years of inclusion and 10 years of follow-up.
The conclusion of the study, based on statistical analysis done once all patients will be included in the cohort, should allow new recommendations in the diagnosis strategy and give new understandings of the therapeutic management of the disease. The result of this study may also give rise to hypothesis for an interventional study.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- 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.
Key inclusion criteria (2 listed)
- 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.
Key exclusion criteria (3 listed)
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
To characterize the epidemiological Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion: Date of birth
1. To characterize the epidemiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion: • Place of birth
1. To characterize the epidemiological of Susac's Syndrome
Time frame: 10 years
• Variables collected at inclusion: Sex
1. To characterize the epidemiological of Susac's Syndrome
Time frame: 10 years
• Variables collected at inclusion : Date of first symptom
1. To characterize the epidemiological of Susac's Syndrome
Time frame: 10 years
• Variables collected at inclusion: Date of diagnosis
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion : • Personal medical history
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion : • Gynaecological history
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion : • Surgical history
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion : • Family history
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
• Variables collected at inclusion : Previous travel and vaccination status
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at inclusion : • Any symptoms (flu-like illness, fever)
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at each visit : • - Weight in kilograms
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at each visit : • - Tobacco consumption
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at each visit : • - Use of oestroprogestogenic contraception
2. To characterize the etiological of Susac's Syndrome
Time frame: 10 years
Variables collected at each visit : • - Alcohol consumption
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : Headache
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Neurology: Neurological symptoms: present / absent / date of onset : • Sensory disturbance
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Neurology: Neurological symptoms: present / absent / date of onset : • Motor deficit
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Neurology: Neurological symptoms: present / absent / date of onset : • Osteotendinous pyramidal reflexes
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Neurology: Neurological symptoms: present / absent / date of onset : • Babinski sign
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Neurology: Neurological symptoms: present / absent / date of onset : • Hoffman's sign
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Tact sensitivity disorder
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Thermoal sensitivity deficit
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : Proprioceptive disorder : • If yes, specify : * Ataxia
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : Proprioceptive disorder : • If yes, specify : * Anomaly of the sense of position of the big toe
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Paresthesias
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Confusion
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Obnubilation
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Coma
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Agitation
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Temporo-spatial disorientation
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
1) Neurology: Neurological symptoms: present / absent / date of onset : • Encephalopathy, If yes, specify the signs present: * Fluctuating memory disorders
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
• Any sequelae : Results of neurocognitive assessment : BREF
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Any sequelae : • Results of neurocognitive assessment: MMS
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Any sequelae : For mood disorders and quality of life: DSMIV criteria for depression
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Any sequelae : For mood disorders and quality of life: SF36
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Any sequelae : • For disability : Barthel Index
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
• Any sequelae : For disability : Rankin score
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology • Ophthalmological examination results : Fundus
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology • Ophthalmological examination results : Visual field
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology • Ophthalmological examination results : o Angiography
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
• Ophthalmology Ophthalmological symptoms: scotoma
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology : Ophthalmological symptoms: decrease in visual acuity
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophtalmology : Evolution of retinal arterial occlusions: improvement
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology : Evolution of retinal arterial occlusions: stability
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
Ophthalmology : Evolution of retinal arterial occlusions: worsening
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
• Ophthalmology : Sequelae
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
ENT : * Cochlear symptoms: vertigo, tinnitus, ataxia, hearing loss
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
ENT: • Audiogram results
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
ENT • Results of vestibular examinations
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
ENT • Course of deafness: improvement, stability, worsening
3. To characterize the clinical of Susac's Syndrome
Time frame: 10 years
ENT : • Possible sequelae (functional discomfort, psycho-social repercussions, follow-up audiograms)
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 7 May 2026.
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Thomas Papo, MD, PHD contact
Karim Sacre, MD, PHD principal investigator
Assistance Publique - Hôpitaux de Paris
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Susac Syndrome, run by Assistance Publique - Hôpitaux de Paris at 1 site in France.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 2 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 7 May 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in France. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Karim Sacre, MD, PHD (+33140258705, karim.sacre@aphp.fr), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
To characterize the epidemiological Susac's Syndrome (time frame: 10 years). The study lists 52 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 7 May 2026.