Objective: To communicate the effectiveness of internal globus pallidus deep brain stimulation (GPi-DBS) on a case of acquired dystonia due to natural gas intoxication.

Background: Dystonia has been described as a possible complication of carbon monoxide poisoning during natural gas intoxication. Symptoms have been attributed to basal ganglia lesions with a poorer prognosis and more challenging treatment than idiopathic dystonia. GPi-DBS has proven to be effective in idiopathic and genetic dystonia. However, acquired dystonia shows an irregular response and to our knowledge no cases treated with GPi-DBS in dystonia due to natural gas intoxication has been reported.

Method: We present a 32-year-old right-handed man with normal developmental milestones and a medical history of a nearly fatal natural gas intoxication at the age of 5. At that moment, he developed myoclonus and cognitive dysfunction, progressing to a multifocal dystonia during his second decade of life. During the following years, dystonic movements interfered with his daily activities and different treatments were prescribed with little response. Brain MRI showed prominent hyperintensities in superior cerebellar peduncles with subtle GPi lesions [figure 1]. Due to the daily functioning interference of the symptoms bilateral GPi-DBS was proposed (Burke‐Fahn‐Marsden Dystonia Rating Scale (BFMDRS): 18/30 on the disability and 63/120 on the movement subscales).

Results: After implantation of directional leads in both GPi, blepharospasm abated and cervical and hand dystonia notably improved. At discharge, a directional program was initiated [table 1]. After this initial response, the patient’s symptoms progressively relapsed. Image guided programming was performed five months after DBS to select the best volume of tissue activated [figure 2], allowing to optimise its configuration [table 1]. Eight months after DBS, the patient improved again, being able to walk safely and use his right-hand for simple movements. The current total scores on the BFMDRS are 12/30 on the disability and 20/120 on the movement subscales.

Conclusion: Acquired cases of multifocal dystonia can be improved by bilateral GPi-DBS, even in those symptomatic to natural gas intoxication. In the event of disabling symptoms, it might be worthwhile to consider it. Image guided programming could be helpful to optimise the effectiveness of DBS.

Figure 1. Pre-surgical MRI.

Table 1. DBS configurations.

Figure 2. Image Guided Programming DBS.

References: 1. Albanese A, Bhatia K, Bressman SB, Delong MR, Fahn S, Fung VS, et al. Phenomenology and classification of dystonia: a consensus update. Mov Disord. 2013;28(7):863-73. doi: 10.1002/mds.25475.

2. Choi IS, Cheon HY. Delayed movement disorders after carbon monoxide poisoning. Eur Neurol. 1999;42(3):141-4. doi: 10.1159/000008088.

3. Wang X, Li Z, Berglass J, He W, Zhao J, Zhang M, et al. MRI and clinical manifestations of delayed encephalopathy after carbon monoxide poisoning. Pak J Pharm Sci. 2016;29(6 Suppl):2317-2320.

4. Quinn DK, McGahee SM, Politte LC, Duncan GN, Cusin C, Hopwood CJ, et al. Complications of carbon monoxide poisoning: a case discussion and review of the literature. Prim Care Companion J Clin Psychiatry. 2009;11(2):74-9. doi: 10.4088/pcc.08r00651.

5. Chen HL, Chen PC, Lu CH, Hsu NW, Chou KH, Lin CP, et al. Structural and cognitive deficits in chronic carbon monoxide intoxication: a voxel-based morphometry study. BMC Neurol. 2013;13:129. doi: 10.1186/1471-2377-13-129.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/effect-of-bilateral-internal-segment-of-the-globus-pallidus-deep-brain-stimulation-on-acquired-multifocal-dystonia-due-to-natural-gas-intoxication/