Primary Hypoparathyroidism Mimicking Ankylosing Spondylitis in a Young Man with Fahr's Syndrome: A Case Report

Abstract

Patients with chronic idiopathic hypoparathyroidism may develop neurological complications, including calcification of the basal ganglia and other areas of the brain. In Fahr's syndrome, intracranial calcification is associated with an underlying disorder such as hypo or hyperparathyroidism. We report the case of a 37-year-old gentleman, with a history of bilateral cataract surgery and seizures, who presented with a new episode of seizure and was found to have severe hypocalcemia and bilateral symmetric intracranial calcification due to previously diagnosed primary hypoparathyroidism. He had symptoms and signs mimicking ankylosing spondylitis (AS), but with negative radiological and serological findings, not fitting into the diagnosis of axial spondyloarthropathies (SpA), as per standard criteria. Patients with longstanding idiopathic hypoparathyroidism can have severe calcification of soft tissues and bones, including vertebrae and paravertebral soft tissues, causing inflammatory back pain and stiffness. It is vital to report such cases as their occurrence is rare, and physicians should be aware of the possibility while evaluating patients with inflammatory back pain. Treatment in these cases is directed towards hypocalcemia and underlying primary pathology rather than spondyloarthropathy.

The axial spondyloarthritis clinical phenotype in idiopathic hypoparathyroidism: critical review of concept that muscular hypercontractility can induce enthesopathy lesions

“…After 4 years, no definite syndesmophytes or ossification of spinal ligaments were found on x-ray and SIJs were unremarkable. This 37-year-old iHPoPT patient with calcium and vitamin D treatment over 4 years may be considered an example of low dose hypocalcaemia exposure or a forme fruste outcome of iHPoPT/SpA (67). Another unusual case report of documented iHPoPT presenting with a clinical picture of AS, but no x-ray evidence for this, is an 81-year-old male reported in 1979 by Schen (68).…”

“…Longitudinal analysis of separate iHPoPT patients can be critically analysed to determine if the degree and duration of hypocalcaemia was sufficient for the development of associated SpA imaging lesions. Such an example is a 37-year-old male with a 4-year history of intermittent seizures, confirmed hypocalcaemia, and primary hypoparathyroidism reported to clinically mimic AS (67). Over a 4-year interval, he was treated with calcium and vitamin D supplement, but had a significant history of inflammatory lower back pain.…”