Woman’s ultra rare spinal condition caused by Cushing’s disease: Report
Spinal decompression surgery successfully restored patient's leg function
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Cushing’s disease was identified as the underlying cause of spinal epidural lipomatosis (SEL) — a rare condition marked by the buildup of fat in the space around the spinal cord — in a 62-year-old woman with progressive leg weakness who had to undergo immediate spinal decompression surgery.
Both that procedure and subsequent pituitary surgery proved successful, the researchers reported, with the woman regaining leg function and having normal hormone levels after one year. Still, the team noted that it is rare to see this spinal condition occurring due to Cushing’s.
“To date, only a few cases of SEL secondary to pituitary Cushing disease have been reported, and surgically treated cases are exceptionally rare,” the researchers wrote, noting that just two such cases had been previously reported in the literature.
“Early recognition and prompt decompression are essential for recovery,” the team added.
The woman’s case was described in a report titled “A rare case of spinal epidural lipomatosis secondary to pituitary Cushing disease presenting with progressive paraparesis,” published in the journal JOS Case Reports by researchers in Japan.
In Cushing’s disease, a tumor in the brain’s pituitary gland produces too much adrenocorticotropic hormone (ACTH), which triggers the overproduction of cortisol by the adrenal glands sitting atop the kidneys. Over time, chronically high cortisol levels can lead to a variety of symptoms, including weight gain and unusual buildup of fat in different parts of the body.
SEL occurs when fat builds up in the space around the spinal cord. This can narrow the spinal canal and compress nerves, leading to weakness, numbness, and difficulty walking.
Woman’s Cushing’s not diagnosed until after spinal surgery
SEL most often develops as a secondary problem, usually linked to long-term steroid use. However, it can also be associated with other conditions, including Cushing’s disease. Still, cases of SEL tied to Cushing’s are “exceedingly rare,” the researchers noted.
Here, a team from the department of orthopedic surgery at the Teikyo University School of Medicines described the case of an older woman in whom Cushing’s disease was identified as the underlying cause of SEL.
The woman sought hospital treatment after developing progressive muscle weakness in both legs over the course of six months. Two weeks before her hospital referral, she was unable to stand or walk. She had a history of high blood pressure, type 2 diabetes, osteoporosis, and high cholesterol, per the report.
A physical examination confirmed muscle weakness and increased muscle tone in both legs, along with sensory loss below the sixth thoracic vertebra located in the mid portion of the spine.
The woman also exhibited typical signs of Cushing’s, including a rounded moon face, a buffalo hump at the upper back, and fat buildup around the abdomen.
Spine X-rays showed markedly low bone density and severe kyphosis — an exaggerated forward bending of the upper back — caused by multiple compression fractures in the mid portion of the spine.
MRI scans showed narrowing of the spinal canal in different regions of the mid and lower spine. In these areas, excess fat had accumulated in the epidural space — the space around the sleeve that protects the spinal cord — putting pressure on the spinal cord and nerves.
Urgent surgery done to relieve pressure on spine
Doctors diagnosed the woman with SEL and performed urgent decompression surgery to relieve pressure on the spinal cord. Surgeons removed part of the vertebral bone from the mid and lower spine and a large amount of epidural fat.
Tissue analysis showed mature fat cells, with no signs of cancer. The surgery was completed without complications, according to the team.
The scientists summed up the process: “Emergency decompression was performed, followed by endocrine evaluation that revealed pituitary [Cushing’s disease].”
Specifically, lab tests performed after surgery showed markedly elevated cortisol levels in the blood and urine, consistent with a diagnosis of Cushing’s. Brain MRI revealed the presence of a pituitary tumor, confirming the diagnosis of Cushing’s disease.
One month after spinal surgery, the woman underwent transsphenoidal surgery to remove the pituitary tumor, which was confirmed to be an ACTH-positive pituitary adenoma.
At one year of follow-up, she had no residual pituitary tumor, hormone levels had normalized, and she had regained normal function in her lower legs, the scientists reported.
According to the researchers, this case highlights that SEL can occur as a rare complication of Cushing’s disease and may cause severe neurological symptoms. The team noted that identifying and treating both the spinal compression and the underlying pituitary tumor early can lead to a good recovery.
Here, Cushing’s was “suspected based on clinical findings,” the researchers wrote, noting “the rewarding outcome” of the case “even after several months of symptoms.”
