Objective: To report a case of isolated generalized dystonia causing severe cervical trauma

Background: Rotational forces on the cervical spine leading to injury is a theoretical concern; no systematic studies have assessed this. One series of 4 cervical dystonia cases suggested accelerated spinal degeneration and myeloradiculopathy at high cervical levels [1]. No cases describe generalized dystonia causing acute cervical cord injury in our review.

Method: Case report

Results: A 52-year-old man with generalized childhood-onset isolated dystonia (TOR1 negative), who had remote bilateral pallidotomy and GPi DBS without lasting benefits, had responded well for years to botulinum injections to the neck. At his last office visit, he complained of severe right-sided neck pain. Exam revealed his usual tightness of neck muscles but much more on the right. We shifted the injection pattern from left to right to address this. The substantial increase in his symptoms was consistent with status dystonicus requiring hospitalization with additional systemic medications. Over the next three weeks, the left side of the neck (his typical pattern) returned to severe spasms with excessively severe cervical and truncal dystonia. Exam revealed normal strength and sensation throughout with substantial periorbital abrasion from pushing his head against pillows. We gave another round of injections addressing the left side of the neck. The next morning, he had weakness in all limbs with subsequent quadriplegia and respiratory failure prompting intubation but with intact facial and neck strength. MRI showed an old dens fracture and moderate-severe cord compression at C1-3 with central cord signal changes. Rotational displacement with possible C1-2 epidural and C2-6 prevertebral hematomas were noted. Surgical decompression was discussed and he and the family declined given that it would require prolonged neck stabilization in the setting of severe cervical and truncal dystonia with little chance of meaningful recovery. Comfort measures only were provided and he died in the ICU.

Conclusion: We believe his new neck pain may have been related to the older dens fracture; subsequently severe truncal and cervical dystonia caused additional torsional stress on his neck with subsequent cervical cord impingement. Traumatic cervical cord injury with high cervical cord injury led to respiratory failure and quadriplegia. This is an extremely rare complication of generalized dystonia.

References: 1. Neeraja K, Prasad S, Surisetti BK, Holla VV, Sharma D, Kamble N, Kulanthaivelu K, Dwarakanth S, Pruthi N, Pal PK, Yadav R. Cervical Myeloradiculopathy and Atlantoaxial Instability in Cervical Dystonia. World Neurosurg. 2021 Feb;146:e1287-e1292. doi: 10.1016/j.wneu.2020.11.153. Epub 2020 Dec 4. PMID: 33285336.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/severe-acute-spinal-cord-injury-due-to-isolated-generalized-dystonia/