Isolated spinal cord compression syndrome revealing delayed extensive superficial siderosis of the central nervous system secondary to cervical root avulsion

Abstract

Context: Cervical root avulsion secondary to traumatic plexus injury is a rare etiology of superficial siderosis (SS) of the central nervous system (CNS). We describe the case of an isolated progressive compressive myelopathy revealing this complication and discuss the pathogenesis of such a presentation, its clinical and imaging peculiarities with a literature review.

Findings: We report on the case of a 48-year-old man with history of left brachial plexus injury at the age of 2 years. Since the age of 38 years, he had presented with a progressive paraplegia, bladder and erectile dysfunction, neuropathic pain and sensory level. The diagnosis was made by spinal cord and brain magnetic resonance follow-up imaging revealing hypointensity T2-weighted gradient echo linear dark rim around the entire neuraxis and cervical dural pseudomeningoceles. These MRI findings were suggestive of extensive hemosiderin deposition consolidating the diagnosis of SS of CNS.

Conclusion/clinical relevance: Our case report illustrates diagnosis difficulties in unusual or paucisymptomatic presentations of SS. A history of brachial plexus trauma with nerve root avulsion should prompt gradient-echo T2-weighted imaging to bring out such a complication. Superficial siderosis of the CNS should be included in the panel of differential diagnosis of the parethospastic syndromes and compressive myelopathy.

Keywords: Myelopathy; Pseudomeningocoele; Spastic paraplegia; Spinal cord compression; Superficial Siderosis.

Figure 1

Magnetic resonance imaging of the neuraxis. T2-weighted gradient echo showing linear hypointensity due to extensive hemosiderin coating around the entire neuraxis including cerebral falx and sylvian fissures (D), the folia of the posterior fossa and brainstem, together with cerebellar atrophy prevailing on the superior vermis (C) and along the cord surface (F and G). The presence of lesions was ambiguous on T2-weighted MR imaging (A, B and E). Cervical spine MR imaging revealed cervical dural pseudomeningocele (H).