Rare Case of Spinal Sarcoidosis Provides Guidelines for Diagnosis, Management

A new report describes a woman with a very rare case of sarcoidosis involving only the spine who entered full remission after surgical removal of the lesion followed by treatment with corticosteroids.

Based on this case report, researchers advised that cases of spinal sarcoidosis should be managed by surgery, followed by a spinal cord magnetic resonance imaging (MRI) and total body computed tomography (CT) scan to see whether other body parts are affected, followed by treatment with steroids.

The report, “A rare case of spinal epidural sarcoidosis: case report and review of the literature,” was published in the journal Acta Neurologica Belgica.

Sarcoidosis is a disease marked by an overreactive immune response, leading to the formation of small clumps of inflammatory cells called granulomas. Over time, these clumps can become calcified or bone-like, and cause permanent damage. Sarcoidosis can affect any organ in the body, but the most affected are the lungs, lymph nodes, eyes, and skin.

Now, researchers from Sapienza University of Rome described a rare case of neurosarcoidosis affecting the spine. Neurosarcoidosis occurs in about 5–15% of the cases, where granulomas develop in the nervous system.

Spinal sarcoidosis is rare and can involve either cells within the spinal cord (intramedullary), the thick membrane that covers the spinal cord (intradural extramedullary), or the bones of the spine (epidural), the last being the rarest form.

Typical manifestations of spinal sarcoidosis are back pain and myelopathy (injury to the spinal cord due to severe compression). But the diagnosis can be difficult, especially if no other organs are involved, and because clinical and radiological findings can be confounded with other neurological diseases affecting the spine, such as infectious diseases and tumors.

The present report describes the case of a 45-year-old woman who was referred to the emergency room with back pain and neurological signs, including a sensation of “pins and needles” (paraesthesia) in the lower limbs, and lack of knee and ankle jerk reflexes, both indicative of myelopathy.

Spinal cord MRI revealed a lesion near the lower end of her spinal cord (medullary conus), suggestive of an inflammatory lesion, while blood tests and a chest X-ray looked normal.

She underwent surgery to remove the lesion, and perform decompressive laminectomy to relieve pressure over the affected spinal nerves. A total body CT scan performed two days after surgery did not show other signs of the disease.

After surgery, the patient’s symptoms gradually eased. Microscopic examination of the removed lesion revealed fibrous-collagenous tissue infiltrated by granulomas, confirming the diagnosis of spinal epidural sarcoidosis.

To achieve rapid control of the disease, the patient was treated with corticosteroids — first with high-dose methylprednisolone for three days, followed by prednisone.

The patient responded well to the treatment, with no side effects and no need for steroid-sparing immunosuppressants. At six months after surgery, a CT scan showed full remission of the disease.

Aside from this patient, only five other cases of spinal epidural sarcoidosis, without involvement of other organs, have been described. Like the patient described in the report, all previous patients  experienced myelopathy and back pain, and achieved complete remission of the disease after surgery and adjuvant steroid therapy.

Thus, according to the team, this form of spinal sarcoidosis appears to respond better to steroid medications than other spinal variants of the disease.

Overall, the study “underlines the difference between [spinal epidural] sarcoidosis compared to intradural extramedullary and intramedullary spinal sarcoidosis,” researchers said, emphasizing that “spinal epidural sarcoidosis represents the rarest form of spinal sarcoidosis.”