BEUDAMED
    FDA Adverse Event Injury Summary report: N

    SYNCHROMED II

    MDR report key: 4698380 · Received April 16, 2015

    Report

    Report Number
    3004209178-2015-07339
    Event Type
    Injury
    Date Received
    April 16, 2015
    Date of Event
    December 12, 2014
    Report Date
    April 2, 2015
    Manufacturer
    MDT PUERTO RICO OPERATIONS CO
    Product Code
    LKK
    PMA / PMN Number
    P860004
    Adverse Event
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    IT
    Reporter Occupation
    PHYSICIAN

    Narratives

    Additional Manufacturer Narrative · 1

    THE REPORTED EVENT DATE REFLECTS THE DATE THAT THE ARTICLE WAS PUBLISHED ONLINE. IT WAS NOT POSSIBLE TO MATCH THIS EVENT WITH ANY PREVIOUSLY REPORTED EVENT. (B)(4).

    Description of Event or Problem · 1

    POZZI M, PICCININI L, GALLO M, MOTTA F, RADICE S, CLEMENTI E. TREATMENT OF MOTOR AND BEHAVIOURAL SYMPTOMS IN THREE LESCH-NYHAN PATIENTS WITH INTRATHECAL BACLOFEN. ORPHANET JOURNAL OF RARE DISEASES. 2014; 9:208. DOI: 10.1186/S13023-014-0208-3. SUMMARY: CURRENT THERAPIES FOR THE LESCH-NYHAN SYNDROME ARE OFF-LABEL AND EXPERIMENTAL, OFTEN LEADING TO INCONSISTENT OUTCOMES. THE AUTHORS REPORT THE EFFECTS OF INTRATHECAL BACLOFEN (ITB) THERAPY, CARRIED OUT AT THE SCIENTIFIC INSTITUTE EUGENIO MEDEA (LECCO, ITALY), ON THREE PATIENTS WHO NO LONGER RECEIVED BENEFIT FROM PREVIOUS THERAPIES. THIS TREATMENT, AS EXPECTED, AMELIORATED THE MOTOR SYMPTOMS AND, UNEXPECTEDLY, IT ALSO IMPROVED BEHAVIORAL COMPONENTS. ADDITIONALLY, ITB IMPROVED THE QUALITY OF SLEEP FOR ALL PATIENTS; THIS HAPPENED IN THE ABSENCE OF SERIOUS ADVERSE REACTIONS, PATIENT 3 ONLY EXPERIENCED MILD DROWSINESS AND PERSISTENT MODERATE NAUSEA. THIS RESULT MAY INVOLVE A FUNCTIONAL INTERACTION BETWEEN BACLOFEN AND DOPAMINE, COMPLEMENTED BY AN ANXIOLYTIC EFFECT. THE AUTHOR'S OBSERVATIONS PROVIDE THE RATIONALE FOR THE USE OF INTRATHECAL BACLOFEN ADMINISTRATION IN THE THERAPY OF THE LESCH-NYHAN SYNDROME. REPORTED EVENT: A (B)(6) MALE, AT THE TIME OF REFERRAL, WAS REPORTED TO HAVE NEVER ACHIEVED HEAD CONTROL, CRAWLED SCANTLY, AND NEVER WALKED. THE PATIENT HAD DEVELOPED STRONG RETROPULSIVE REACTIONS, WITH DYSTONIA INVOLVING NECK AND LIMBS AND BALLISM OF THE ARMS. BY PUNCHING, THE PATIENT INJURED HIMSELF AND ATTACKED OTHERS. THE PATIENT WAS WEANED OFF THEIR PREVIOUS THERAPY, AND SUBSEQUENTLY IMPLANTED WITH THE INTRATHECAL DRUG DELIVERY DEVICE SYNCHROMED II - 20 ML. THE ITB DOSAGE WAS UP-TITRATED TO ACHIEVE A SATISFACTORY EFFECT ON THE DYSTONIA. THE DYSTONIA WAS CONTROLLED WITH AN ITB DOSAGE OF 550 MCG PER DAY. ITB IMPROVED THE PATIENT'S QUALITY OF SLEEP, AS EXPECTED. THIS HAPPENED IN THE ABSENCE OF SERIOUS ADVERSE REACTIONS; THE PATIENT ONLY EXPERIENCED MILD DAYTIME DROWSINESS AND PERSISTENT MODERATE NAUSEA. IN ADDITION, WITHIN THREE DAYS, AFTER REACHING THE STABLE ITB DOSAGE, BALLISM WAS ABOLISHED AND AGGRESSION AND SELF-INJURIOUS BEHAVIORS CEASED. THE BENEFICIAL EFFECTS OF ITB THERAPY AT UNCHANGED DOSAGES PERSISTED THROUGHOUT THE FOLLOW UP PERIOD OF FIVE MONTHS. ADDITIONAL INFORMATION WAS LATER RECEIVED. IT WAS INDICATED THAT THE PATIENT¿S SYMPTOMS HAD BEEN CAUSED BY AN EXCESSIVE DOSE AND RESOLVED ONE WEEK AFTER DOSE REDUCTION.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    253762 SYNCHROMED II PUMP, INFUSION, IMPLANTED, PROGRAMMABLE LKK MDT PUERTO RICO OPERATIONS CO 8637-20

    Patients

    Seq Age Sex Outcome Treatment
    1 00020 YR Required Intervention