2026 American Academy of Neurology Abstract Website

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.