Abstract Details

Title Deep Brain Stimulation for Refractory Myoclonus-dystonia: A Case-by-case Systematic Review and Meta-analysis

Topic Epilepsy/Clinical Neurophysiology (EEG)

Presentation(s) P6 - Poster Session 6 (5:00 PM-6:00 PM)

Poster/Presentation
Number 11-011

Objective To perform a case-by-case analysis of deep brain stimulation (DBS) for refractory myoclonus–dystonia (M-D), and identify EEG/EMG phenotypes, DBS targets, programming, and outcomes, including post-hypoxic epileptic myoclonus as seen in Lance–Adams syndrome (LAS).

Background The evidence for DBS as a treatment for myoclonus dystonia is scattered across small reports with variable targets and parameters. To better understand its utility in management, a concise, practice-oriented summary is needed.

Design/Methods We performed a PRISMA-guided search including PubMed, EMBASE, Cochrane and other sources for screening. Our inclusion criteria was based on studies with patients diagnosed with M-D (± LAS) treated with DBS and reporting pre/post outcomes. For analysis, we extracted the electrophysiology, DBS target/laterality; initial/chronic settings (frequency, pulse width, amplitude, configuration); outcomes (UMRS, dystonia/functional), follow-up, adverse events. This was a descriptive case-by-case analysis

Results

In our analysis, we found that most M-D cases used bilateral GPi with ~130 Hz, 60–90 µs, ~2–4 V/mA, showing clinically meaningful, durable improvement. In one detailed case, the patient's UMRS improved from 93→39 with parallel BFMDRS gains at ~3 years. Another 2-patient series reported resolution of myoclonus within 6–12 months on high-frequency GPi. A focal myoclonic-seizure phenotype improved long-term with unilateral STN.

In LAS, bilateral GPi showed frequency dependence: 130–160 Hz could worsen symptoms, whereas low frequency ~35–40 Hz (≈2.5 V, ~130 µs) improved UMRS subscales with sustained benefit. An adult progressive myoclonic epilepsy series favored SNr/STN at ~100–110 Hz with ~30–100% myoclonus reduction; VIM was generally ineffective or limited by side effects.

Adverse events were infrequent and typically parameter-responsive.

Conclusions Bilateral GPi DBS at conventional high frequency—yields meaningful, durable benefit in refractory M-D; STN may help select focal myoclonus. In LAS, low-frequency GPi can outperform high frequency, underscoring target- and frequency-specific programming. Adverse events appear uncommon and manageable. Standardized case reporting and prospective studies are needed to refine selection and programming.

Authors/Disclosures
Hira Burhan, MD PRESENTER	Dr. Burhan has nothing to disclose.
Ajaz Sheikh, MD (ProMedica Neurosciences Center)	Dr. Sheikh has nothing to disclose.
Mehmood Rashid, MD (Neuroscience Center)	Dr. Rashid has nothing to disclose.
Fahima Akter Shammi, Research Associate	Ms. Shammi has nothing to disclose.
Naeem Mahfooz, MD	Dr. Mahfooz has nothing to disclose.
Imran I. Ali, MD, FAAN (University of Toledo COM)	Dr. Ali has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for ABPN.
Judy Y. Zheng, MD (Neuroscience Center)	Dr. Zheng has nothing to disclose.
Jennifer Amsdell, MD (Promedica Neuroscience Center)	Dr. Amsdell has nothing to disclose.
Syed Owais, MD	Dr. Owais has nothing to disclose.

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