Acromegaly presenting with myelopathy due to ossification of posterior longitudinal ligament: a case report

Abstract

Background Acromegaly is a rare disease caused by high serum levels of growth hormone (GH) and insulin-like growth factor 1 (IGF-1), often originating from a pituitary adenoma. Spinal and peripheral joint abnormalities are caused by these hormonal hypersecretions. In particular, the response to GH is involved in the onset of ossification of the spinal ligament in vitro, especially ossification of the posterior longitudinal ligament (OPLL). However, because acromegaly and OPLL are rare diseases, we seldom encounter them in combination. To the best of our knowledge in the English-language literature, this is the first reported case of acromegaly presenting with thoracic myelopathy due to OPLL. Case presentation A 47-year-old woman presented with lower extremity weakness and paresthesia, gait disorder, and bladder disorder without any trauma. The patient’s most remarkable symptom was paraplegia, and we diagnosed myelopathy due to cervical and thoracic OPLL. Furthermore, we suspected acromegaly because of the characteristic facial features, and we found a pituitary adenoma by contrast-enhanced MRI. Cervical and thoracic decompression, posterior fixation, and pituitary adenoma resection were performed. Conclusion We report a case of acromegaly that was detected after the diagnosis of OPLL. The main challenge in acromegaly is delayed in diagnosis. Even in this case, the facial features characteristic of acromegaly had appeared at least 9 years ago. Early diagnosis and treatment of acromegaly improve prognosis and reduce exposure to GH and IGF-1 through early intervention and seem to suppress the progression of ligament ossification. Orthopedic surgeons and neurosurgeons need to keep in mind that acromegaly is associated with bone/joint lesions and ossification of the spinal ligament and should aim to diagnose acromegaly early.

Incidence of ossification of the spinal ligaments in acromegaly patients

“…Although some previous case reports implied an association between acromegaly and spinal ligament ossification ( Kamakura et al, 2021 ; Schmidt et al, 2013 ), they were not conclusive because there have been no reports of a consecutive case series or prospective studies. In the current study, among the 10 consecutive acromegaly patients, 5 (50 %) had spinal ligament ossification and 2 (20 %) had OPLL, so this prevalence was noticeably higher than that of the general population (1.9–4.3 %) ( Sasaki et al, 2014 ; Shingyouchi et al, 1996 ; Stapleton et al, 2011 ; Yoshimura et al, 2014 ), which suggested that acromegaly might have a possible correlation with the development of spinal ligament ossification similar to other previously identified risk factors, including diabetes mellitus or obesity.…”

“…Regarding multifactorial inheritance, in 2014, a genome-wide association study (GWAS) revealed several susceptibility genes for OPLL ( Nakajima et al, 2014 ). There have been some case reports that implied an association between acromegaly and spinal ligament ossification ( Kamakura et al, 2021 ; Schmidt et al, 2013 ). However, there was no method to ascertain whether the reported co-occurrence of these two conditions was a coincidence or causal association, as there has been no report of consecutive cases.…”

Lumbar Spinal Canal Stenosis in Acromegaly: A Case Report and Literature Review

“…We surveyed the literature dealing with spinal canal stenosis and acromegaly. As shown in Table, 15 cases (10 men, 5 women) have been reported since 1982 (12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25). Spinal canal stenosis spanning multiple vertebrae from the cervical to lumbar spine was observed in 7 patients, cervical stenosis was detected in 6 patients, thoracic stenosis was identified in 9 patients, and lumbar stenosis was demonstrated in 10 patients.…”

Role of the NF-kB signalling pathway in heterotopic ossification: biological and therapeutic significance

“…Additionally, the developmental effects of GH are mediated by p65, according to a report in which 2 different mutations impairing NF-κB induction in 2 children resulted in growth deficiency and GH tolerance [ 71 ]. In contrast, patients with acromegaly suffer from ossification of the posterior longitudinal ligament as a result of excessive secretion of GH and IGF-1 [ 75 ]. These findings confirmed that GH and IGF-1 could promote additional chondrogenesis for final ectopic bone ossification in soft tissue, and NF-κB signalling might be involved in this process.…”