Functional Disorders Moya Moya Young Patients

NCT ID: NCT05772572

Last Updated: 2025-09-12

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 21 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-05-02
• Study Completion Date: 2024-02-09

Brief Summary

Moya Moya disease is a rare condition of the vessels that supply blood to the brain. It normally occurs without apparent cause. In both children and adults, the disease is mainly manifested by strokes. Diagnosis is made on MRI and cerebral angiography. There is no treatment that can prevent the arteries in the brain from narrowing.

Surgical treatment may be a priority, especially in the early forms of the disease. Functional, painful disorders, in particular headaches, may persist after neurosurgical intervention, without any clear predictive factor being found. Otherwise there is a disjunction between the objectification of sequelae on MRI and painful complaints, sometimes a dissociation between the improvement of the objective parameters of perfusion and imaging, and functional somatic complaints.

The study focuses on improving knowledge of post-operative functional disorders in Moya Moya disease in children and adolescents, in order to propose interventions based on this knowledge and making it possible to reduce both functional complaints and depression, the anxiety which accompanies them, to decrease the impact on the quality of life and parental wandering in the installation of adapted accompaniments. For this, a half-day consultation will be intended in order to carry out questionnaires and standardized tests, the results of which will be reported and compared to known rates in the general population and the population of children with chronic diseases.

Detailed Description

Moya Moya disease is a rare condition of the vessels that supply blood to the brain. It normally occurs without apparent cause. In both children and adults, the disease is mainly manifested by strokes. Diagnosis is made on MRI and cerebral angiography. There is no treatment that can prevent the arteries in the brain from narrowing.

Surgical treatment can be considered, especially in the early forms of the disease. Several surgical techniques are used to divert the vessels, including multiple craniotomies, which consists of making several small holes in the cranium so as to pass vessels from the scalp so that they develop towards the poorly irrigated areas of the brain.

Functional, painful disorders, in particular headaches, can persist after neurosurgical intervention, without any clear predictive factor being found. 57% of patients under 17 with Moya Moya disease have persistent functional disorders in without correlation to any of the characteristics of the disease, nor to its surgical treatment. There is a disjunction between the objectification of sequelae on MRI and painful complaints, sometimes a dissociation between the improvement of the objective parameters of perfusion and imaging, and functional somatic complaints. Interviews with families experienced that psychological phenomena may play an important role in postoperative pain and functional complaints: child and parent anxiety, depression, and family functioning and attachment, child cognitive functioning.

Functional complaints and postoperative pain are therefore frequent in children and adolescents with Moya Moya, but poorly documented. The difficulty is to be able to disentangle the pain linked to organic factors such as a recurrence of a vascular accident, and those linked to psychological factors, as they have been described in the disease. The important thing is to be able to avoid or reduce paraclinical explorations in the face of the resurgence of painful complaints; the challenge is to defuse the vicious interactive circle between painful complaints, anxiety-provoking explorations, reinforcing stress and possible increase complaints. Identifying the psychological factors would make it possible to better support functional complaints.

The study focuses on improving knowledge of post-operative functional disorders in Moya Moya disease in children and adolescents, in order to propose interventions based on this knowledge and making it possible to reduce both functional complaints and depression, the anxiety which accompanies them, to decrease the impact on the quality of life and parental wandering in the installation of adapted accompaniments. For this, a half-day consultation will be intended in order to carry out questionnaires and standardized tests, the results of which will be reported and compared to known rates in the general population and the population of children with chronic diseases.

Conditions

Moyamoya Disease

Study Design

• Observational Model Type: CASE_ONLY
• Study Time Perspective: CROSS_SECTIONAL

Study Groups

Patients

Children aged 7 to 17 inclusive, and young adults operated (or re-operated) for a Moya Moya in the previous 7 years and followed at the Necker-Enfants Malades hospital, and their parents.

Questionnaires

Intervention Type: OTHER

Questionnaires are completed only once and assess:

• recorded questions about the disease and the operation for the parents and the patient, human figure drawing test and drawing of the operation for the patient
• psychiatric questionnaires for parents and patients on anxiety and depression
• behavior questionnaires for the parents and the patient: assessment of attachment
• questionnaires for the parents and the patient concerning the family context, the quality of life

Interventions

Questionnaires

Questionnaires are completed only once and assess:

• recorded questions about the disease and the operation for the parents and the patient, human figure drawing test and drawing of the operation for the patient
• psychiatric questionnaires for parents and patients on anxiety and depression
• behavior questionnaires for the parents and the patient: assessment of attachment
• questionnaires for the parents and the patient concerning the family context, the quality of life

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

• Adult patients up to 25 years old and minor patients aged 7 to 17 inclusive and holders of parental authority for minor patients
• Patients operated (or re-operated) for a Moya Moya in the previous 7 years
• Subjects without intellectual disabilities
• Subjects speaking French well enough to answer the questions of the study
• subjects (parent(s) and minor or adult patient) and holders of parental authority informed and who do not object to their participation in the study

Exclusion Criteria

Cognitive level of the patient incompatible with the administration of the questionnaires and evaluations of the study

• Minimum Eligible Age: 7 Years
• Maximum Eligible Age: 25 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

URC-CIC Paris Descartes Necker Cochin

OTHER

Sponsor Role: collaborator

Assistance Publique - Hôpitaux de Paris

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Lisa Ouss, MD

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris

Manoelle Kossorotoff, MD

Role: STUDY_DIRECTOR

Assistance Publique - Hôpitaux de Paris

Locations

Hôpital Necker-Enfants Malades

Paris, , France

Countries

France

Other Identifiers

2021-A02555-36

Identifier Type: OTHER

Identifier Source: secondary_id

APHP220991

Identifier Type: -

Identifier Source: org_study_id