MAUDE data represents reports of adverse events involving medical devices. This maude entry was filed from a foreign,health professional,l report with the FDA on 2019-05-28 for UNKNOWN IMPLANTABLE NEUROSTIMULATOR NEU_INS_STIMULATOR manufactured by Medtronic Neuromodulation.
[146236813]
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. Li h, zhang c, pan y, sun b, li d, wu y. Status dystonicus in pantothenate kinase-associated neurodegeneration due to internal pulse generator depletion: case study and literature review. J neurol sci. 2019;400:44-46. Doi: 10. 1016/j. Jns. 2019. 02. 038. If information is provided in the future, a supplemental report will be issued.
Patient Sequence No: 1, Text Type: N, H10
[146236814]
Summary: status dystonicus (sd) is defined as a severe, life-threatening episode of a generalized or focal hyperkinetic movement disorder [1]. Many movement disorders may progress to sd either with or without a specific trigger. Unexpected interruption to deep brain stimulation (dbs) treatment due to battery depletion or other factors is increasingly recognized as a precipitant of sd [2,3] (table 1). Here, we describe an adolescent female patient with kinase-associated neurodegeneration (pkan) who was successfully treated with dbs of the subthalamic nuclei (stn) for dystonia until she suddenly deteriorated to sd. It turned out that battery depletion of the dbs internal pulse generator (ipg) was the probable cause of the sd, which resolved after implanting a new battery. Reported events: a female patient with pantothenate kinase-associated neurodegeneration (pkan) who had subthalamic nucleus (stn) deep brain stimulation (dbs) for dystonia experienced an excellent clinical response to the dbs-stn treatment after implant. The burke-fahn-marsden dystonia rating scale (bfmdrs) score was 65/17 (motor/disability) preoperatively and dropped to 22/11 in the next week. At age (b)(6), however, after more than five years of continuous dbs-stn treatment, the patient's clinical status suddenly deteriorated. Within two months, she developed progressively severe episodes of generalized dystonia affecting the orofacial region, neck, trunk, and limbs, which made her unable to walk without using a wheelchair. Upon admission to a local hospital, it was found that the battery of the ipg at the left stn was nearly depleted. Three days later, the patient and her parents visited our hospital for battery replacement. At that time, however, she had developed a dystonic storm. She presented with a fever, sweating, and increasingly frequent episodes of dystonia involving neck, trunk, and limbs. Moreover, she suffered from painful muscle spasms and continuous orofacial dystonia, which compromised swallowing function. Laboratory tests confirmed the presence of leukocytosis and elevated serum creatine kinase. She scored 78/120 on the motor part and 26/30 on the disability part of the bfmdrs (see video, segment 2). Following battery replacement surgery, her dbs-stn treatment was reestablished with the same parameters (left: 6? 7- 90us/ 145 hz/3. 45 v and right: 2? 3- 90us/145 hz/3. 50 v) that were used before the replacement surgery. The patient's condition did not improve until one week after the replacement operation. At one-month followup, however, the bfmdrs score had dropped to 46/17 (motor/disability). Furthermore, the patient regained the ability to sit independently, even though her sit-to-stand ability did not recover to a past level. No life-threatening complications occurred during her dystonic storm.
Patient Sequence No: 1, Text Type: D, B5
| Report Number | 3007566237-2019-01169 |
| MDR Report Key | 8646017 |
| Report Source | FOREIGN,HEALTH PROFESSIONAL,L |
| Date Received | 2019-05-28 |
| Date of Report | 2019-05-28 |
| Date of Event | 2019-02-28 |
| Date Mfgr Received | 2019-05-08 |
| Date Added to Maude | 2019-05-28 |
| Event Key | 0 |
| Report Source Code | Manufacturer report |
| Manufacturer Link | Y |
| Number of Patients in Event | 0 |
| Adverse Event Flag | 3 |
| Product Problem Flag | 3 |
| Reprocessed and Reused Flag | 3 |
| Health Professional | 3 |
| Initial Report to FDA | 3 |
| Report to FDA | 3 |
| Event Location | 3 |
| Manufacturer Contact | LISA WOODWARD CLARK |
| Manufacturer Street | 7000 CENTRAL AVENUE NE RCW215 |
| Manufacturer City | MINNEAPOLIS MN 55432 |
| Manufacturer Country | US |
| Manufacturer Postal | 55432 |
| Manufacturer Phone | 7635263920 |
| Manufacturer G1 | MEDTRONIC NEUROMODULATION |
| Manufacturer Street | 800 53RD AVE NE |
| Manufacturer City | MINNEAPOLIS MN 554211200 |
| Manufacturer Country | US |
| Manufacturer Postal Code | 554211200 |
| Single Use | 3 |
| Previous Use Code | 3 |
| Event Type | 3 |
| Type of Report | 3 |
| Brand Name | UNKNOWN IMPLANTABLE NEUROSTIMULATOR |
| Generic Name | IMPLANTED SUBCORTICAL ELECTRICAL STIMULATOR (MOTOR DISORDERS) |
| Product Code | MRU |
| Date Received | 2019-05-28 |
| Model Number | NEU_INS_STIMULATOR |
| Catalog Number | NEU_INS_STIMULATOR |
| Lot Number | UNKNOWN |
| Operator | HEALTH PROFESSIONAL |
| Device Availability | N |
| Device Eval'ed by Mfgr | * |
| Device Sequence No | 1 |
| Device Event Key | 0 |
| Manufacturer | MEDTRONIC NEUROMODULATION |
| Manufacturer Address | 800 53RD AVE NE MINNEAPOLIS MN 554211200 US 554211200 |
| Patient Number | Treatment | Outcome | Date |
|---|---|---|---|
| 1 | 0 | 1. Hospitalization; 2. Required No Informationntervention | 2019-05-28 |