Objective: To describe the management of patient with calcium disequilibrium presenting with a complex, hyperkinetic, movement disorder emergency akin to chorea paralytica, which was refractory to maximal medical therapy.

Background: Primary hypoparathyroidism is a rare metabolic disorder characterized by hypocalcemia which may result in ectopic intracranial calcifications. These patients may suffer neurological symptoms ranging from mild to several movement disorder phenotypes such as dystonia, chorea, parkinsonism or paroxysmal movement disorders.

Method: We report a 58-year-old lady, a known case of hypoparathyroidism, presented to our center with subacute onset, progressive abnormal flinging movements of both upper limb and lower limbs for 3 months, which increased in severity three days prior to admission. She was conscious and did not have any cranial nerve, sensory or motor deficit at the time of admission to our hospital. Investigations revealed serum hypocalcemia, hypoparathyroidism and elevated serum creatinine, urea and creatine kinase. Extensive bilateral subcortical, basal ganglia and cerebellar calcifications were noticed on a non-contrast CT. No other abnormality was noticed on CT and MRI.

Results: She was started on 6 drugs in dosages appropriate for her weight. However, there was no improvement noticed in her condition. She had to be intubated, mechanically ventilated and initiated on fentanyl and midazolam infusions as her abnormal movements were not controlled and she was going into renal failure secondary to rhadomyolysis. Improvement in renal function was noticed over time following improvement in abnormal movements but the movements returned as soon as it was tried to reduce fentanyl and midazolam infusion rates. As the medical measures were not helping, she underwent simultaneous bilateral palldotomy using radiofrequency ablation. The choreo-ballistic movements subsided remarkably post pallidotomy, and the patient was weaned off infusions and extubated within next few days. She can do her activities of daily living independently with movements under control at the time of last follow up. She has some dysphagia.

Conclusion: Calcium metabolism derangements may result in complex hyperkinetic movement disorders and present as life-threatening movement disorder emergencies. Medical measures may not be able to control symptoms and one may need emergent surgical procedure.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/emergency-pallidotomy-in-a-patient-with-chorea-paralytica-in-primary-hypoparathyroidism/