Steroid therapy may help to treat autoimmune hypoparathyroidism

Woman, 55, seizure free after one year of treatment: Case study

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by Andrea Lobo |

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People with autoimmune hypoparathyroidism who have anti-CaSR antibodies — which play a role in regulating the parathyroid hormone, known as PTH — may benefit from adding steroid therapy to standard vitamin D and calcium supplementation, a case report suggests.

That report described a 55-year-old woman who’d had seizures for 35 years due to hypocalcemia, or low blood calcium levels, despite treatment with antiepileptic medicines. For one year after therapy with a combination of a steroid and calcium and vitamin D supplements, she was seizure free.

“The addition of prednisone to vitamin supplements showed a better response and corrected the hypocalcemia,” the researchers wrote.

“In the future,” they added, “research should also compare the efficacy of combination therapy and immunosuppressive therapy alone, so that physicians can provide the best treatment for autoimmune hypoparathyroidism patients.”

The study, “Seizures in activating calcium-sensing receptor antibodies positive autoimmune hypoparathyroidism and preponderance of steroids therapy: A case report,” was published in the journal Clinical Case Reports.

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Steroid therapy raised patient’s calcium levels 37%

Hypoparathyroidism is characterized by lower-than-normal levels of the parathyroid hormone (PTH) — a key regulator of calcium levels in the blood. PTH also regulates levels of phosphorus and vitamin D, which helps absorb calcium in the gut.

Low PTH leads to hypocalcemia, which is associated with several symptoms, including numbness in fingers and toes, severe muscle pain, and seizures — periods of abnormal brain electric activity that lead to movement, consciousness, and behavior changes.

Other symptoms of the disease can include cataracts, a condition in which the eye’s natural lens becomes cloudy, and behavioral problems.

Here, a team led by researchers from Pakistan described the case of a middle-aged Afghan woman who was admitted to a hospital with recurrent seizures that had started at age 20, with no obvious cause. The patient was prescribed antiepileptic drugs by a local doctor, but despite strict adherence, her seizures were not resolved for more than three decades.

During that time, the patient did not attend neurology consultations nor receive treatment at a specialized hospital due to limited resources and financial issues.

She also had a history of kidney stones and cataracts. Her family had no cases of seizures or other neurologic or autoimmune diseases.

Now, she was admitted to the hospital with specific signs of hypocalcemia. These were Trousseau’s sign, an involuntary contraction of the hand and wrist after the inflation of a blood pressure cuff, and Chvostek’s sign, which is a twitch in the facial muscles.

Results of blood tests revealed hypocalcemia and low levels of PTH. Brain imaging showed the presence of calcium deposits. Anti-CaSR antibodies targeting calcium-sensing receptors at the surface of parathyroid cells also were detected. CaSR receptors in cells of the parathyroid glands regulate the secretion of PTH .

The data led to the diagnosis of autoimmune hypoparathyroidism, with seizures induced by hypocalcemia. Autoimmune hypoparathyroidism occurs when the immune system wrongly attacks the parathyroid glands, affecting the production of PTH.

“This unique case describes a patient in whom the secondary causes of hypoparathyroidism were absent. Although she had no family history of autoimmune disease, she experienced her first seizure in her 20s which makes autoimmune likely to be the etiology [cause],” the researchers wrote.

“Autoantibodies examinations may be performed when suspecting autoimmune hypoparathyroidism,” they added.

The woman was treated with oral calcium supplements and an antiepileptic (sodium valproate) twice daily, and also given an injection of vitamin D2 weekly for 12 weeks. She also was advised to check her blood calcium levels weekly.

However, her calcium levels did not improve after eight weeks. At that point, prednisone, a corticosteroid that suppresses immune system activity, was added to her treatment regimen.

Adding the steroid therapy improved (raised) her calcium levels by 37% over three weeks, reaching the reference range.

In the future, … research should also compare the efficacy of combination therapy and immunosuppressive therapy alone, so that physicians can provide the best treatment for autoimmune hypoparathyroidism patients.

When sodium valproate and prednisone were gradually withdrawn, the woman’s calcium levels remained stable. The patient maintained calcium and vitamin D supplements daily and was seizure-free for one year.

“The management of this case was unique because the patient did not respond to standard therapy of vitamin D and calcium until the addition of prednisone,” the researchers wrote.

The successful use of steroids to treat autoimmune hypoparathyroidism has been reported previously. In that case, the team did not evaluate the impact of steroid therapy on anti-CaSR antibody levels.

“The addition of immunosuppressive therapy to the supplementations gives a better response compared to replacement therapy alone,” the researchers concluded in this case study.