MAUDE Adverse Event Report: MEDTRONIC NEUROMODULATION IMPLANTABLE NEUROSTIMULATOR; IMPLANTED SUBCORTICAL ELECTRICAL STIMULATOR (MOTOR DISORDERS)

Model Number NEU_INS_STIMULATOR

Device Problem Adverse Event Without Identified Device or Use Problem (2993)

Patient Problems Emotional Changes (1831); Memory Loss/Impairment (1958); Anxiety (2328); Irritability (2421); Cognitive Changes (2551)

Event Date 12/11/2015

Event Type  Injury  

Manufacturer Narrative

Event date: please note this date is based off of the article¿s publication date as the specific event date was not provided in the published literature.

Event Description

Multani, n., moro, e., lang, a., zurowski, m., duff canning, s., tartaglia, m.C.Progression of neuropsychiatric and cognitive features due to exons 2 to 5 deletion in the epsilon-sarcoglycan gene: a case report.Neurocase.2016.22(2):215-219.Doi:10.1080/135547 94.2015.1120312 summary: physical symptoms of myoclonus dystonia due to epsilon-sarcoglycan mutations are well documented; however, the progression of neuropsychiatric and cognitive symptoms remains unclear.We present a case of a (b)(6) woman with early childhood onset of myoclonic jerks, dystonic posture and developmental delay due to exons 2 to 5 deletion in the epsilon-sarcoglycan gene.Over time, she developed neuropsychiatric symptoms.She underwent bilateral deep brain stimulation of the ventral intermediate nucleus of the thalamus for her motor symptoms, which greatly improved but she exhibited slow deterioration of her neuropsychiatric and cognitive symptoms, particularly apathy, aggression and severe executive dysfunction.Reported events: it was reported that a (b)(6) woman with early childhood onset of myoclonic jerks, dystonic posture, and developmental delay due to a deletion of exons 2 through 5 from her epsilon-sarcoglycan gene (sgce) underwent bilateral deep brain stimulation (dbs) of the ventral intermediate thalamic nucleus (vim) for her motor symptoms at the (b)(6).The procedure reportedly greatly improved the patient¿s motor symptoms, though the patient ¿exhibited a slow deterioration of her neuropsychiatric and cognitive symptoms, particularly apathy, aggression, and severe executive dysfunction¿ along with an overall ¿fluctuation of psychiatric issues.¿ it was stated that her ¿cognitive function and psychiatric symptoms declined over time.¿ the patient underwent cognitive and neuropsychiatric evaluation at (b)(6) (three years after dbs implant).It was noted that ¿overall, her cognitive profile was characterized by borderline to low-average intellectual functioning.The symptom checklist-90-revised (scl-90-r) was also conducted and revealed obsessive¿compulsive symptoms, anxiety, paranoid ideation, psychoticism, and some degree of thought disorder.In addition, the patient reported olfactory and tactile hallucinations.After the initial assessment, the patient¿s mother reported progressive decline in her daughter¿s memory.At (b)(6), there was notable worsening of her neuropsychiatric symptoms resulting in two hospitalizat ions for psychotic episodes and aggressive behavior.Moreover, she began to exhibit loss of judgment, engaging in risky behaviors such as shoplifting and drinking excessively.Another assessment was conducted at (b)(6), suggesting overall stability of her cognitive function.The patient¿s behavior worsened around (b)(6), as she began to display aggressive and agitated behavior and was hospitalized for another psychotic episode.The patient was treated with quetiapine (50 mg q.H.S.) and divaloproex (500 mg q.H.S.).In addition, the patient was diagnosed with hypothyroidism and diabetes, which were well controlled¿ at the time the article was written.The patient underwent an additional assessment ¿at (b)(6), approximately 18 months after discontinuation of vim dbs, and it showed further decline in learning and memory but improvement on a measure of novel problem solving.During this evaluation, she remained rigid and concrete in her response style and appeared apathetic throughout the evaluation.¿ an mri was performed and ¿revealed mild atrophy for her age, but no other abnormalities, including white matter changes.¿ it was noted that the patient¿s dbs was turned off at the (b)(6), ¿as her mother felt it was contributing to cognitive and psychiatric issues.¿ the patient experienced ¿further deterioration of her psychiatric and cognitive domains after the dbs was turned off.¿ as mentioned previously, the patient underwent cognitive and neuropsychiatric evaluation at (b)(6) (both with dbs on), and (b)(6) (dbs off) years of age.The patient¿s (b)(6) test-ii ¿ learning subscore changed from ¿borderline,¿ to ¿decline, impaired,¿ and finally to ¿decline, profoundly impaired¿ when evaluated at (b)(6) respectively.Additionally, the patient¿s (b)(6) test-ii ¿ immediate recall subscore changed from ¿stable, borderline¿ at (b)(6) to ¿decline, profoundly impaired¿ at (b)(6) and their (b)(6) test-ii ¿ delayed recall subscore changed from ¿stable, borderline-impaired¿ at (b)(6) to ¿decline, profoundly impaired¿ at (b)(6).The patient¿s (b)(6) test total errors results changed from ¿low average,¿ to ¿significant decline, profoundly impaired,¿ and finally to ¿significant improvement, average¿ at (b)(6) respectively.Both the patient¿s (b)(6) test perseverative responses results and perseverative errors results changed from ¿average,¿ to ¿significant decline, profoundly impaired,¿ and finally to ¿significant improvement, high average¿ at (b)(6) respectively.The patient¿s (b)(6) test total categories results went from ¿borderline-low average,¿ to ¿slight decline, borderline,¿ and finally to ¿significant improvement, average¿ at (b)(6) respectively.Patient testing using the (b)(6) test was not evaluated at (b)(6); however, the patient saw a change in visuospatial constructional ability from ¿low average¿ to ¿significant decline, profoundly impaired¿ at (b)(6) respectively.Assessment of the patient¿s psychomotor speed, executive function, and semantic fluency saw no changes from (b)(6); however, these scores changed from ¿stable, profoundly impaired¿ to ¿decline, profoundly impaired,¿ ¿stable severely impaired¿ to ¿decline, profoundly impaired,¿ and ¿stable, borderline-low average¿ to ¿decline, profoundly impaired¿ respectively with testing at (b)(6).Finally, frontal systems behavior scale scores for apathy, disinhibition, and executive dysfunction decreased over-time both when measured through patient self-assessment and through her mother¿s assessment of her condition.The patient¿s self-assessment found apathy scores of 63, 62, and 55; disinhibition scores of 56, 69, and 46; and executive dysfunction scores of 64, 62, and 54 when assessed at the (b)(6) respectively.The mother¿s assessment of the patient reported apathy scores of 109, 76, and 76; disinhibition scores of 77, 67, and 44; and executive dysfunction scores of 88, 69, and 57 when assessed at the (b)(6) respectively.It was further reported that ¿the patient exhibited several psychiatric symptoms over time, including temporary psychosis and aggressive behavior during two different periods.There was significant improvement in her disinhibition and depression scores; however, [at the time of report], apathy was still a major concern.Although the patient did not endorse any depressive thoughts¿ when evaluated most recently prior to report, the authors noted that ¿her score may be due to lack of insight or presenting overly positive view of herself.¿ the authors went on to state that ¿her cognitive evaluation suggests improved performance on a novel problem solving task; however, deterioration was evident in other domains with age: psychomotor speed, visuospatial ability, verbal fluency, executive function, learning and memory, indicating profoundly impaired function, even after turning the dbs stimulation off.¿ it was reported that ¿this was suggestive of progressive frontal subcortical dysfunction.¿ the authors noted that ¿it was unclear whether some cognitive changes could be attributed to the dbs.Upon turning the dbs stimulation off, the patient¿s cognitive profile was still characterized by further decline in certain domains.¿ it was further reported that ¿there was no improvement in the patient¿s learning and memorability with the dbs on and her performance declined further in these domains at (b)(6).Furthermore, the patient exhibited low verbal fluency during the stimulation-on period and increased impairment in verbal fluency after dbs was turned off.¿ the authors stated that ¿the occurrence of a psychotic episode in the absence of alcohol abuse and dbs suggests another cause.¿ the authors ¿suspected that the decline in certain cognitive domains and presence of certain psychiatric features could not be due to the dbs stimulation and might have resulted from progression of the disease.¿.

Manufacturer Narrative

If information is provided in the future, a supplemental report will be issued.

• Brand Name: IMPLANTABLE NEUROSTIMULATOR
• Type of Device: IMPLANTED SUBCORTICAL ELECTRICAL STIMULATOR (MOTOR DISORDERS)
• Manufacturer (Section D): MEDTRONIC NEUROMODULATION; 800 53rd ave ne; minneapolis MN 55421 1200
• Manufacturer (Section G): MEDTRONIC NEUROMODULATION; 800 53rd ave ne; minneapolis MN 55421 1200
• Manufacturer Contact: diane wolf ; 7000 central avenue ne rcw215; minneapolis, MN 55432 ; 7635263987
• MDR Report Key: 5563323
• MDR Text Key: 42188464
• Report Number: 3007566237-2016-01624
• Device Sequence Number: 1
• Product Code: MRU
• Combination Product (y/n): N
• Reporter Country Code: CA
• PMA/PMN Number: H020007
• Number of Events Reported: 1
• Summary Report (Y/N): N
• Report Source: Manufacturer
• Source Type: foreign,health professional,l
• Reporter Occupation: Other
• Type of Report: Initial,Followup
• Report Date: 04/08/2016

1 Device was Involved in the Event

1 Patient was Involved in the Event

• Date FDA Received: 04/08/2016
• Is this an Adverse Event Report?: Yes
• Is this a Product Problem Report?: No
• Device Operator: Health Professional
• Device Model Number: NEU_INS_STIMULATOR
• Device Catalogue Number: NEU_INS_STIMULATOR
• Device Lot Number: UNKNOWN
• Was Device Available for Evaluation?: No
• Is the Reporter a Health Professional?: No
• Date Manufacturer Received: 03/10/2016
• Is the Device Single Use?: Yes
• Is This a Reprocessed and Reused Single-Use Device?: No
• Type of Device Usage: Initial
• Patient Sequence Number: 1
• Patient Outcome(s): Hospitalization
• Patient Age: 00034 YR