BEUDAMED
    FDA Adverse Event Injury Summary report: N

    IMPLANTED SUBCORTICAL ELECTRICAL STIMULATOR (MOTOR DISORDERS)

    MDR report key: 6133603 · Received November 29, 2016

    Report

    Report Number
    3007566237-2016-04195
    Event Type
    Injury
    Date Received
    November 29, 2016
    Date of Event
    October 4, 2016
    Report Date
    November 29, 2016
    Manufacturer
    MEDTRONIC NEUROMODULATION
    Product Code
    MRU
    PMA / PMN Number
    H020007
    Adverse Event
    Yes
    Product Problem
    Yes
    Report Source
    Manufacturer report
    Reporter Location
    CA
    Reporter Occupation
    PHYSICIAN

    Narratives

    Additional Manufacturer Narrative · 1

    DATE OF EVENT: PLEASE NOTE THAT THIS DATE IS BASED OFF OF THE DATE OF PUBLICATION OF THE ARTICLE AS THE EVENT DATES WERE NOT PROVIDED IN THE PUBLISHED LITERATURE. THE DEVICE WAS USED FOR AN OFF LABEL INDICATION. CONCOMITANT MEDICAL PRODYCRS: PRODUCT ID NEU_UNKNOWN_LEAD, PRODUCT TYPE: LEAD. A GOOD FAITH EFFORT WILL BE MADE TO OBTAIN THE APPLICABLE INFORMATION RELEVANT TO THE REPORT. IF INFORMATION IS PROVIDED IN THE FUTURE, A SUPPLEMENTAL REPORT WILL BE ISSUED.

    Additional Manufacturer Narrative · 1

    DEVICE PRO CODE CORRECTED TO MRU. NOTE PMA / 510(K) # WAS INCORRECTLY NOTED AS P960009 ON THE INITIAL REPORT. SUPPLEMENTAL BEING SENT TO CORRECT THIS TO H020007. A GOOD FAITH EFFORT WILL BE MADE TO OBTAIN THE APPLICABLE INFORMATION RELEVANT TO THE REPORT. IF INFORMATION IS PROVIDED IN THE FUTURE, A SUPPLEMENTAL REPORT WILL BE ISSUED.

    Description of Event or Problem · 1

    BEAULIEU-BOIRE, I., AQUINO, CC., FASANO, A., POON, YY., FALLIS, M., LANG, AE., HODAIE, M., KALIA, SK., LOZANO, A., MORO, E. DEEP BRAIN STIMULATION IN RARE INHERITED DYSTONIAS. BRAIN STIMULATION. 2016 OCT 4. DOI: 10.1016/J.BRS.2016.07.009. SUMMARY: RARE CAUSES OF INHERITED MOVEMENT DISORDERS OFTEN PRESENT WITH A DEBILITATING PHENOTYPE OF DYSTONIA, SOMETIMES COMBINED WITH PARKINSONISM AND OTHER NEUROLOGICAL SIGNS. SINCE THESE DISORDERS ARE OFTEN RESISTANT TO MEDICATIONS, DBS MAY BE CONSIDERED AS A POSSIBLE TREATMENT. METHODS: PATIENTS WITH IDENTIFIED GENETIC DISEASES (ATAXIA-TELANGIECTASIA, CHOREA-ACHANTOCYTOSIS, DOPARESPONSIVE DYSTONIA, CONGENITAL NEMALINEMYOPATHY, METHYLMALONIC ACIDURIA, NEURONAL CEROID LIPOFUSCINOSIS, SPINOCEREBELLAR ATAXIA TYPES 2 AND 3, WILSON¿S DISEASE ,WOODHOUSE¿SAKATI SYNDROME, METHYLMALONIC ACIDURIA, AND X TRISOMY) AND DISABLING DYSTONIA UNDERWENT BILATERAL GPI DBS (BILATERAL THALAMIC VIM NUCLEUS IN 1 CASE). THE PRIMARY OUTCOME WAS THE DIFFERENCE IN THE BURKE¿FAHN¿MARSDEN DYSTONIA RATING SCALE (BFMDRS) BETWEEN BASELINE, 1 YEAR AND LAST AVAILABLE FOLLOW-UP. PREOPERATIVE FACTORS SUCH AS AGE AT SURGERY, DISEASE DURATION AT SURGERY, PROPORTION OF LIFE LIVED WITH DYSTONIA AND SEVERITY OF DYSTONIA WERE CORRELATED TO THE PRIMARY OUTCOME. RESULTS: ELEVEN PATIENTS WERE OPERATED BETWEEN FEBRUARY 2003 AND DECEMBER 2013. AGE AND DURATION OF DISEASE AT TIME OF SURGERY WERE 30 ± 19 AND 12.5 ± 15.7 YEARS, RESPECTIVELY. DBS EFFECTS ON DYSTONIA SEVERITY WERE VARIABLE BUT OVERALL MARGINALLY EFFECTIVE, WITH A MEAN IMPROVEMENT OF 7.9% (P = 0.39) AT 1-YEAR FOLLOW-UP AND 16.7% (P = 0.46) AT LAST FOLLOW-UP (MEAN 47.3 ± 19.9 MONTHS AFTER SURGERY). NO PREOPERATIVE FACTORS WERE IDENTIFIED TO PREDICT THE SURGICAL OUTCOME. CONCLUSION: OUR FINDINGS SUPPORT THE CURRENT KNOWLEDGE THAT DBS IS MODESTLY EFFECTIVE IN TREATING RARE INHERITED DYSTONIAS WITH A COMBINED PHENOTYPE. HOWEVER, THE BFMDRS MIGHT NOT BE THE BEST TOOL TO MEASURE OUTCOME IN THESE SEVERELY AFFECTED PATIENTS. REPORTED EVENT: PATIENT 8: A (B)(6) PATIENT WITH GLOBUS PALLIDUS INTERNUS (GPI) DEEP BRAIN STIMULATION (DBS) FOR DYSTONIA SECONDARY TO SPINOCEREBELLAR ATAXIA 3 (SCA3) REQUIRED A SECOND SURGERY TO REPOSITION THE ELECTRODES WITHIN THE GPI, ¿WITH MILD BENEFIT.¿ THE AUTHORS ADDED THAT AT LAST FOLLOW-UP THERE WAS SOME DETERIORATION IN THE LEVEL OF DISABILITY OF ALL PATIENTS, WITH A MEAN WORSENING OF 39%, AND THAT ANY TRANSIENT BENEFIT OBSERVED MAY HAVE BEEN DUE TO A PLACEBO AFFECT OR INSERTIONAL EFFECT. IT WAS NOT POSSIBLE TO ASCERTAIN SPECIFIC DEVICE INFORMATION FROM THE ARTICLE OR TO MATCH THE REPORTED EVENT WITH ANY PREVIOUSLY REPORTED EVENT.

    Devices

    Seq Brand Generic Product Code Manufacturer Model Lot UDI-DI
    783482 IMPLANTED SUBCORTICAL ELECTRICAL STIMULATOR (MOTOR DISORDERS) MRU MEDTRONIC NEUROMODULATION NEU_INS_STIMULATOR UNKNOWN

    Patients

    Seq Age Sex Outcome Treatment
    1 47 YR Required Intervention