Thavanesan, n., gillies, m., farrell, m., green, a.L., aziz, t.Deep brain stimulation in multiple system atrophy mimicking idiopathic parkinson's disease.Case reports in neurology.2014;6(3):232-237.Doi: 10.1159/000368571.Summary: deep brain stimulation (dbs) is approved for idiopathic parkinson¿s disease (ipd) but has a poor evidence base in parkinson-plus syndromes such as multiple system atrophy (msa).We describe the clinical and neuropathological findings in a man who was initially diagnosed with ipd, in whom dbs was unsuccessful, and in whom msa was unexpectedly diagnosed at a subsequent autopsy.This case report highlights that dbs is often unsuccessful in msa and also demonstrates that msa can masquerade as ipd, which may explain treatment failure in a small group of patients apparently suffering from parkinson¿s disease.Additionally, it also presents a case with an unusually long duration of disease prior to death, comparable only to a handful of other cases in the literature.Reported event: one (b)(6) male patient underwent 2-stage bilateral deep brain stimulation (dbs) implant for parkinson¿s disease in (b)(6) 2008.Stage 1 initially gave positive results barring an episode of confusion, hypotension and reduced glasgow coma scale (gcs) score attributed to overstimulation.Titration was effective, and the patient was switched from regular madopar to prn.Stage 2 was complication free aside from transient calibration paresthesia.Medications were stopped two days postoperatively with good gait, no tremor, no freezing and improved left arm mobility.A health care provider (hcp) and the patient specifically reported improvement in rigidity and gait.The patient was discharged three days later.The patient subsequently deteriorated after two weeks, returning to pre-operative medication doses, with his symptoms worse than preoperatively.The patient experienced confusion, short-term memory impairment and hypertonia in the left arm.Subsequent titration helped minimally.In the subsequent four years until time of death, the patient developed episodes of psychosis, dopamine dysregulation syndrome, iga nephropathy, chronic renal failure and ultimately terminal clostridium difficile colitis.Neuropathological examination, performed six years after surgery during the patient¿s autopsy, confirmed the placement of dbs electrodes in the subthalamic nucleus (stn).The neuropathological findings were those of multiple system atrophy (msa).The pathological diagnosis was based on extensive striatal and subcortical white matter accumulation of ¿-synuclein coupled with profound depletion of the neurons from the striatum and substantia nigra with atrophy and gliosis.Oligodendroglial ¿-synuclein inclusions were also very extensive in cerebral and cerebellar white matter, and the middle cerebellar peduncle also showed prominent ¿-synuclein-positive glial inclusions indicating elements of both a striatonigral and olivopontocerebellar pattern of distribution.Noteworthy was the finding of prominent ¿-synuclein-positive glial inclusions adjacent to the dbs insertion site in the stn.It was noted that incorrect preoperative idiopathic parkinson¿s disease diagnose showed positive initial response to surgery followed by rapid decline secondary to progression of msa and subsequent death.The source literature did not include any specific device information.Further information has been requested; a supplemental report will be submitted if additional information is received.
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Date of death: please note the patient's date of death was unavailable at the time of report.The date reported reflects the date the attached literature article was published online.Other: psychosis.It was not possible to ascertain specific device information from the article or to match the events reported with previously reported events.Correspondence has been sent to the author of the article inquiring about individual patient information and additional information regarding the reported events.Concomitant medical products: product id neu_unknown_lead, lot# unknown, product type: lead.(b)(4).
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