Updating superficial siderosis of the central nervous system: bleeding of a dorsal osteophyte into the subarachnoid space from a perforating artery

Abstract

<jats:p>Superficial siderosis of the central nervous system (SSCNS) is an uncommon and often unrecognized disorder that results from recurrent and persistent bleeding into the subarachnoid space. Currently, there is no effective treatment for SSCNS. The identification and surgical resolution of the cause of bleeding remains the most reliable method of treatment, but the cause of bleeding is often not apparent. The identified sources of recurrent bleeding have typically included neoplasms, vascular malformations, brachial plexus or nerve root injury or avulsion, and previous head and spinal surgery. An association between recurrent bleeding in the CNS and dural abnormalities in the spine has recently been suggested. Dural tears have been identified in relation to a protruding disc or osteophyte. Also in these patients, the exact mechanism of bleeding remains unknown because of a lack of objective surgical data, even in patients who undergo neurosurgical procedures.</jats:p><jats:p>The present case concerns a 48-year-old man who presented with longstanding symptoms of mild hearing loss and mild gait ataxia. A diagnosis of SSCNS was made in light of the patient’s history and the findings on physical examination, imaging, and laboratory testing. MRI and CT detected a small calcific osteophyte in the anterior epidural space of T8–9. The patient underwent surgical removal of the bone spur and dural tear repair. During the surgery, the authors detected a perforating artery, which was on the osteophyte, that was bleeding into the subarachnoid space. This case shows a possible mechanism of chronic bleeding from an osteophyte into the subarachnoid space. In the literature currently available, a perforating artery on an osteophyte bleeding into the subarachnoid space has never been described in SSCNS.</jats:p>

Spinal muscular atrophy type III complicated by spinal superficial siderosis: a case report with molecular and neuropathological findings

“…In some, a connection with the arachnoid space is demonstrable. Also, some cases have been reported with protruding discs, osteophytes, dural calcifications at times in disc [2,13], dural ectasia or diverticula and following anterior cervical spine surgery [4]. In our patient, there was significant dural and spinal distortion generated by the severe scoliosis and the post-surgical procedure with the insertion of a surgical rod, from T4 to S1, level of significant hemosiderosis.…”

“…1 Division of Neurology, Department of Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, Canada. 2 Department of Diagnostic Imaging, The Ottawa Hospital, University of Ottawa, Ottawa, Canada. 3 Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON K1H 8L6, Canada.…”

Progressive superficial siderosis from Chronic CSF leak as a long-term complication of cervical anterior corpectomy: A case report and review of the literature

“…Hemosiderin deposition from chronic subarachnoid hemorrhage in patients with SSCNS deposits in the subpial layers of the brain and spine, most typically superficially along the cerebellum, brainstem, and spinal cord; this results in the symptomology seen in this patient including gait ataxia, progressive loss of hearing and smell, and bilateral extremity weakness. [3] ree theories have been proposed for the cause of bleeding in patients with SSCNS associated with a dural defects. First, brain sagging due to intracranial hypotension may lead to chronic bleeding from bridging veins on the surface of the cerebellum.…”

“…[13,18,22] Brembilla et al posit a third theory, that osteophyte protrusion into the subarachnoid space can introduce bleeding through a ruptured perforating artery. [3] Takai and Taniguchi provide evidence that the second theory is the most likely cause of CSF leak induced bleeding leading to SSCNS. [20] Although there is no one definitive treatment for SSCNS, therapies have focused on iron chelation, surgery for specific etiologies such as dural defects, or symptom management.…”

Superficial siderosis of the central nervous system with epilepsy originating from traumatic cervical injury: illustrative case

“…Other manifestations have been reported, such as diplopia, hyposmia, amnesia, headache, and seizures. 12 , 13 Because most of the damage to the CNS is irreversible, it is vital to determine the etiology and intervene as early as possible. Although extensive diagnostic examinations are used to determine the causative pathologies of bleeding conditions, the etiology of more than 30% of subarachnoid hemorrhage cases remains unknown.…”