TFMoya : ADAR: Functional Disorders Moya Moya Young Patients
Study Details
Study Description
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.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
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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 multi craniotomia, 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 increasly 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.
Study Design
Arms and Interventions
| Arm | Intervention/Treatment |
|---|---|
| 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. |
Other: 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
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Outcome Measures
Primary Outcome Measures
- The " State trait anxiety inventory ", STAI forme Y [Day 0]
Study the links between postoperative functional disorders by multicraniostomy in children and adolescents with Moya Moya disease, and psychological dimensions such as anxiety. For teenager, adults patients and parents : The " State trait anxiety inventory ", STAI form Y (revised), a self-questionnaire assessing trait anxiety (usual tendency to be anxious) and state anxiety (anxiety felt at a particular time), has 20 items for assessing trait anxiety and 20 for state anxiety on a 4-point Likert scale, scores range from 20 to 80, with higher scores correlating with greater anxiety.
- Revised Children's Manifest Anxiety Scale (RCMAS) [Day 0]
Revised Children's Manifest Anxiety Scale (RCMAS) : self-administered 37-item questionnaire assessing the level and nature of anxiety in children and adolescents aged 9 to 19, not allowing diagnosis of the various anxiety disorders in children. The total anxiety score can range from 0 to 28. The cut-off point for pathological anxiety is more than two standard deviations around the mean (T more than 70).
- Children Depression Inventory (CDI) [Day 0]
Study the links between postoperative functional disorders by multicraniostomy in children and adolescents with Moya Moya disease, and psychological dimensions such as depression. CDI Children Depression Inventory : self questionnaire of 27 items which measures the intensity of depression in children and adolescents from 7 to 17 years old. Each item is rated from 0 (normal behavior for age or absent) to 2 (severe). The addition of the items gives a total between 0 and 54. The higher the score, the more severe the depression symptomatology is.
- Beck = Dépression [Day 0]
Parents and young adult patients : Beck = Dépression multiple-choice questionnaire of 21 items of symptoms and attitudes, used to measure the severity of clinical depression, graded from 0 to 3 by a series of 4 statements (from 0 to 63, higher score correlating with higher severity) reflecting the degree of severity of the symptom.
- FMSS= Five minute speech sample [Day 0]
The Five Minute Speech Sample (FMSS) is a method that can be used to measure both expressed emotion and narrative coherence. The FMSS requires parents to speak about their child and their relationship with their child for five minutes.
- Cards for the evaluation of Attachment and Mentalization in Children (CAME) [Day 0]
Study the links between postoperative functional disorders by multicraniostomy in children and adolescents with Moya Moya disease, and psychological dimensions such as of attachment of child with parents. CAME: Cards for the evaluation of Attachment and Mentalization in Children, from Child Attachment Interview.
- CA-MIR [Day 0]
Parent's attachment. Cards for assessment of parent's attachment.
- Child Behavior Checklist (CBCL) [Day 0]
The Child Behavior Checklist (CBCL) is a checklist parents complete to detect emotional and behavioural problems in children and adolescents. The main scoring for the CBCL is based on statistical groupings of sets of behaviors that typically occur together.
- Representations of disease and surgery [Day 0]
human figure drawing test and drawing of the operation for the patient. Recorded questions about the disease and the operation for the parents and the patient.
- Pediatric Quality of Life Inventory (PedsQL) [Day 0]
The Pediatric Quality of Life Inventory (PedsQL) makes it possible to assess the quality of life in relation to the state of health of healthy children or children suffering from various diseases. The 23 questions relate to 4 different areas, the functioning physical (8 items), emotional (5 items), social (5 items) and school (5 tems).
- WHOQOL - BREF [Day 0]
For young adult patients. The WHO QOL is a 26 items questionnaire. It assesses the quality of life in relation to the state of health, according to 4 different areas: physical, emotional, social and environment.
Secondary Outcome Measures
- Medical and neuropsychological assessment of patients [Day 0]
Neurological examination, functional symptoms, radiological data including cerebral perfusion, surgical history, neuropsychological measures.
Eligibility Criteria
Criteria
Inclusion Criteria:
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Adult patients up to 25 years old and minor patients aged 7 to 17 inclusive and holders of parental authority for minor patients
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Patients operated (or re-operated) for a Moya Moya in the previous 7 years
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Subjects without intellectual disabilities
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Subjects speaking French well enough to answer the questions of the study
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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
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | Hôpital Necker-Enfants Malades | Paris | France | 75015 |
Sponsors and Collaborators
- Assistance Publique - Hôpitaux de Paris
- Tanguy Moy Moya Association
Investigators
- Principal Investigator: Lisa Ouss, MD, Assistance Publique - Hôpitaux de Paris
- Study Director: Manoelle Kossorotoff, MD, Assistance Publique - Hôpitaux de Paris
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- APHP220991
- 2021-A02555-36