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This study investigates cerebrovascular reactivity (CVR) and functional brain connectivity in Dravet Syndrome (DS) patients with convulsive seizures. Using functional MRI (fMRI), we will define differences in brain responses to CO₂ changes before administration of the drug Fintepla (Baseline), with a library of healthy controls and with those obtained after administration of Fintepla (Day ~60). Changes in CVR and their relation to ventilatory responses will also be assessed during fMRI.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Fintepla plus Hypercapnia Challenge using the Respiract Device | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Fenfluramine treatment (Fintepla) | Drug | Participants will receive Fintepla starting at 0.2 mg/kg/day, up to a maximum of 0.6 mg/kg/day (or 5.9 mL/day, whichever is lower), per FDA guidelines. Dosing will be adjusted as tolerated. After Day ~60, the dose will be gradually tapered. |
| Measure | Description | Time Frame |
|---|---|---|
| Change in blood oxygenation level in response to CO₂ | Measures how much blood oxygenation level dependent (BOLD) signal changes in response to CO₂, using fMRI. | Baseline, Day ~60 |
| Change in speed of cerebrovascular response to CO₂ | Measures how quickly the brain responds to CO₂ exposure during fMRI. | Baseline, Day ~60 |
| Measure | Description | Time Frame |
|---|---|---|
| Change in breathing response to CO₂ | Assesses how the participant's breathing rate changes when CO₂ levels are increased. | Baseline, Day ~60 |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Samden Lhatoo, MD, FRCP | Contact | (713) 500-7106 | Samden.D.Lhatoo@uth.tmc.edu | |
| Sandhya Rani | Contact | (713) 500-7499 | M.R.Sandhya.Rani@uth.tmc.edu |
| Name | Affiliation | Role |
|---|---|---|
| Samden Lhatoo, MD, FRCP | The University of Texas Health Science Center, Houston | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| The University of Texas Health Science Center at Houston | Houston | Texas | 77030 | United States |
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| ID | Term |
|---|---|
| D004831 | Epilepsies, Myoclonic |
| D004827 | Epilepsy |
| D000080485 | Sudden Unexpected Death in Epilepsy |
| ID | Term |
|---|---|
| D004829 | Epilepsy, Generalized |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D005277 | Fenfluramine |
| ID | Term |
|---|---|
| D010627 | Phenethylamines |
| D005021 | Ethylamines |
| D000588 | Amines |
| D009930 | Organic Chemicals |
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| Hypercapnia Challenge using the device (RespirAct) | Device | Participants will undergo a hypercapnia challenge using the RespirAct device during fMRI. The protocol includes alternating one minute blocks of controlled CO₂ increases and normocapnia under normoxic conditions. |
|
|
| D000073376 | Epileptic Syndromes |
| D003645 | Death, Sudden |
| D003643 | Death |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |