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Subthalamic nucleus deep brain stimulation in a patient with levodopa-responsive multiple system atrophy. Case report
Kelvin L Chou1, Mark S Forman, John Q Trojanowski
1Parkinson's Disease and Movement Disorders Center and Department of Neurosurgery, Penn Neurological Institute at Pennsylvania Hospital, Philadelphia, Pennsylvania 19107, USA.
Journal of Neurosurgery
|March 24, 2004
Summary
Deep brain stimulation (DBS) was ineffective for a patient with multiple system atrophy (MSA), despite levodopa responsiveness. This case highlights challenges in differentiating Parkinson disease (PD) from MSA.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Parkinsonism can present with levodopa responsiveness, mimicking Parkinson disease (PD).
- Differentiating PD from other parkinsonian syndromes like multiple system atrophy (MSA) is clinically challenging.
- Deep brain stimulation (DBS) is a treatment option for advanced PD.
Observation:
- A patient with parkinsonism, initially responsive to levodopa, underwent subthalamic nucleus DBS.
- Postoperatively, the patient showed no response to DBS or medication and developed aspiration pneumonia.
- Autopsy revealed neuropathological features of MSA, including alpha-synuclein inclusions and inflammation around DBS electrodes.
Findings:
- Neuropathological examination confirmed multiple system atrophy (MSA), not Parkinson disease (PD).
- The patient's lack of response to DBS and levodopa postoperatively was associated with alpha-synuclein pathology and inflammation.
- Inflammatory response around DBS electrodes may have contributed to treatment failure in MSA.
Implications:
- Deep brain stimulation (DBS) may be ineffective in multiple system atrophy (MSA), even with initial levodopa sensitivity.
- Accurate diagnostic tools are crucial for distinguishing PD from MSA to guide treatment decisions.
- This case underscores the importance of neuropathological confirmation in atypical parkinsonian syndromes.