Non-traumatic intramedullary hemorrhage (hematomyelia)

Non-traumatic intramedullary hemorrhages (a form of spinal vascular accident), or hematomyelias, are the rarest form of intraspinal hemorrhages. They can occur in isolation or be associated with a spinal subarachnoid hemorrhage (SAH).

They are considered a surgical emergency until proven otherwise.

Etiologies

• Idiopathic (related to hypertension? microangiopathies?)
• Vascular malformations of spinal and/or epidural arteries: the primary non-traumatic cause of hematomyelias
  • Arteriovenous malformations (AVMs), arteriovenous fistulas, vertebro-vertebral fistulas, etc.
• Intramedullary tumors
• Coagulation disorders and anticoagulant or fibrinolytic treatments
• Rarely: secondary to spinal venous thrombosis, or secondary to local inflammatory diseases, etc.

Clinical presentation

The onset is typically sudden, characterized by intense localized spinal pain, which can quickly radiate throughout the spine or be associated with radicular pain.

This is followed by the rapid development of a neurological deficit of varying severity, indicating spinal cord or conus medullaris involvement: para- or tetraparesis, massive flaccid plegia, sensory disturbances, and sphincter dysfunction. Hyporeflexia or areflexia below the level of the lesion is common in the first hours, followed by the return of reflexes and progression to spastic hyperreflexia within 24 hours to a few weeks.

Meningeal signs may be present if an associated SAH is present.

Cases of subacute to chronic progression over several months have also been reported.

Complementary exams

For both diagnostic and etiological purposes:

• Spinal MRI:
  • Demonstrates the hemorrhage:
    • Acute phase: iso/hypointense on T1, hypointense on gradient echo T2 or T2*
    • Often surrounded by an edema halo (hypointense on T1 and hyperintense on T2)
    • Late phase: hyperintense on T1 and T2, surrounded by an edema halo (hypointense on T1 and hyperintense on T2)
  • T2*, angiographic, and venous sequences for etiological investigation
• Blood work: coagulation profile, and other tests based on clinical history

Therapeutic management – Treatments

• Etiological treatment if possible: interventional neuroradiology, correction of coagulation disorders, surgery, etc.
• Systematic neurosurgical consultation: surgical evacuation may be considered depending on the severity, clinical evolution, and time elapsed since the onset of symptoms.
• General management: stress ulcer prophylaxis (PPI "half-dose"), thromboembolic prophylaxis (prophylactic doses of clexane), to be initiated 48 hours after a control CT scan confirming no worsening or rebleeding.
• While not based on evidence-based medicine (EBM), blood pressure control in the acute phase may follow similar protocols to those used in hemorrhagic strokes.

Bibliography

EMC, Traité de Neurologie, 2018

Osborn AG et al, Brain, Elsevier, 2018