Case report: Unilateral GPi DBS in secondary myoclonus-dystonia syndrome after acute disseminated encephalomyelitis
Introduction: Deep brain stimulation (DBS) is an established and effective therapy for movement disorders. Here, we present a case of secondary myoclonusdystonia syndrome following acute disseminated encephalomyelitis (ADEM) in childhood, which was alleviated by DBS. Using a patient-specific conn...
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| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Philipps-Universität Marburg
2023
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| Subjects: | |
| Online Access: | PDF Full Text |
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| Summary: | Introduction: Deep brain stimulation (DBS) is an established and effective therapy
for movement disorders. Here, we present a case of secondary myoclonusdystonia
syndrome following acute disseminated encephalomyelitis (ADEM) in
childhood, which was alleviated by DBS. Using a patient-specific connectome
analysis, we sought to characterise the fibres and circuits affected by stimulation.
Case report: We report a case of a 20-year-old man with progressive dystonia,
myoclonic jerks, and impaired concentration following childhood ADEM. Motor
assessments utilising the Unified Myoclonus Rating Scale (UMRS) and the Burke-
Fahn-Marsden Dystonia Rating Scale (BFMDRS) revealed a greater improvement
in dystonia compared to myoclonus following adjustments of DBS parameters.
These adjustments were based on visualisation of electrode position and volume
of tissue activated (VTA) 3 years after surgery. A patient-specific connectome
analysis using the VTA as a region of interest revealed fibre tracts connecting to
the cerebello-thalamo-cortical network and the superior frontal gyrus in addition
to basal ganglia circuits as particularly effective.
Conclusion: Globus pallidus internus (GPi) DBS shows promise as a treatment
for secondary myoclonus-dystonia syndromes. Personalised structural
considerations, tailored to individual symptoms and clinical characteristics, can
provide significant benefits. Patient-specific connectome analysis, specifically,
offers insights into the structures involved and may enable a favourable treatment
response. |
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| Item Description: | Gefördert durch den Open-Access-Publikationsfonds der UB Marburg. |
| Physical Description: | 6 Pages |
| DOI: | 10.3389/fneur.2023.1238743 |