Spinal cord hemorrhage in a patient with neurosarcoidosis on long-term corticosteroid therapy: case report

In the case described here, the diagnosis of neurosarcoidosis was definite according to Judson’s criteria 16] and probable according to Zajicek’s 17]. This first report highlights the possibility of spinal cord hemorrhage in patients
with neurosarcoidosis, as suggested by Waubant and colleagues, who described hemosiderin
deposits in spinal-cord sarcoidosis 18].

Common causes of atraumatic hematomyelia 19], 20] include vascular malformations (arteriovenous fistula, cavernoma, capillary telangiectasia,
venous angioma) 21], neoplasms, Gowers’intrasyringal hemorrhage, spinal radiation, anticoagulation and
bleeding disorders. None of these applied to our patient, suggesting that her hematomyelia
was caused by bleeding of a spinal cord granuloma, given the proximity of the first
inflammatory lesion (T8-L1) to the hemorrhage (T10-T11).

Despite the lack of histological confirmation of the source of bleeding, there is
sufficient evidence from cerebral cases 9] to suggest that hemorrhage in neurosarcoidosis may be caused by vascular pathologies
(venous, arteriolar, or micro-arterio-venous malformations) induced by the disease
itself. Sarcoid granulomas in the central nervous system (CNS) follow perivascular
spaces and penetrate brain parenchyma 22]. Post mortem examinations of intracranial hemorrhage in neurosarcoidosis have revealed
perivascular inflammatory infiltrate and vessel wall destruction by granulomas 7], 9]. The enlarged blood vessels noticed by the neurosurgeon in our patient seem to support
the hypothesis of neurosarcoid vasculitis. The frontoparietal hemorrhage that occurred
during the second episode, 3 years before the hematomyelia (Fig. 2a-b), is also suggestive of the fragility of blood vessels in the CNS damaged by the
neurosarcoid vasculitis.

Long-term corticosteroids also increase vascular fragility, owing to reduced collagen
formation in vessel walls 23], but there was no evidence to suggest that steroids directly caused the bleeding
here. Indeed, we decided to increase the corticosteroid dose, as adding an immuno-modulating
agent to control the neurosarcoid vasculitis (and spare corticosteroids) was not possible
because of an infectious complication. There have not been any randomized controlled
trials of pharmacological treatments for neurosarcoidosis, but the algorithm developed
by Nozaki and Judson, has proved useful for severe neurosarcoidosis 24].

Kreppel and colleagues’ meta-analysis 25] is the largest review of reports of spinal hematoma published before 1996. These
authors reviewed 613 patients belonging to four etiological groups of spinal cord
hemorrhage: intramedullary (hematomyelia), subarachnoidal, subdural and epidural.
Hematomyelia was found in only 0.82 % of patients, possibly owing to the dearth of
MRI-based diagnosis prior to 1996. More recently, Leep and colleagues 19] and Heldner and colleagues 20] reviewed the diagnosis, cause and treatment of hematomyelia. Hematomyelia can present
as an acute, subacute, step-wise or chronic myelopathy 19]. As it did in our case, neurological deterioration can occur after the initial hemorrhage,
owing to a secondary tissue response?19]. It is essential to monitor vital signs and neurological status in the intensive
care unit, to prevent complications 20]. However, there have not been any clinical trials comparing different ways of managing
hematomyelia?19], 20], notably the timing of hematoma evacuation. In our case, the decision to operate
was made because of a progressive, rapid and inexorable neurological deterioration.
There was a definite, but not total, clinical improvement following treatment for
this rare and severe complication of neurosarcoidosis.