Objective: To describe the effect of Deep Brain Stimulation (DBS) on tremor associated with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) in three patients.

Background: Tremor is a symptom of CIDP and other neuropathies. DBS is a therapy for tremor of various etiologies. Little is known on the efficacy of DBS in tremor due to CIDP.

Method: We report all CIDP patients treated with DBS in our center. DBS was targeted at the dentatorubrothalamic tract (DRT) in the posterior subthalamic area (PSA). To determine the location for DBS electrode implantation, diffusion tension imaging tractography and intraoperative clinical response to macrostimulation were used. Tremor was assessed with the Fahn-Tolosa-Marin tremor rating scale (FTM-TRS, range 0 to 156), which includes assessment of disability due to tremor. Neuropathy associated disability was measured with the Inflammatory Rash-built Overall Disability Scale (I-RODS, range 0 to 48). Assessments were performed pre-operatively and up to 40 months post-operatively. Additional clinical information was available up to 40 months.

Results: Three CIPD patients (all males, ages were 65, 68, 69 years) with neuropathic tremor were treated with bilateral DBS of the DRT/PSA. All patients suffered from a postural and intention tremor and two of them also had tremor in rest. Tremor frequency ranged from 3 to 7 Hz.
Initially, treatment with DBS led to improvement of tremor severity in all patients according to the FTM-TRS scores: pre-operative and follow-up (FU) scores were 52 and 31 for case 1 (10 months FU), 58 and 18 for case 2 (12 months FU), and 76 and 65 for case 3 (6 months FU). Disability due to tremor improved in case 1 and 2, but did not change in case 3. Neuropathy associated disability had improved in case 1 (I-RODS 20 to 23) and case 2 (I-RODS 35 to 45), but had worsened in case 3 (clinical assessment). After longer follow-up, case 1 and case 2 had a deterioration of tremor and associated disability: FTM-TRS scores 65 (36 months FU) and 56 (40 months FU) respectively. I-RODS scores then were 28 for case 1 and 24 for case 2.

Conclusion: DBS of the DRT/PSA effectively improved neuropathic tremor and disability in two out of three CIDP patients, but the long-term response was suboptimal. Further research is needed to substantiate findings.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-for-tremor-in-chronic-inflammatory-demyelinating-polyneuropathy-a-series-of-three-cases/