A new case report published in Revista Española de Anestesiología y Reanimación has revealed that early and prompt diagnosis and treatment of thyroid storm are important in improving the survival in patients with Friederich’s ataxia (FA).

The study found that symptomatic treatment, definitive treatment, treatment of cardiovascular, neurological, and hepatic manifestations and thyrotoxicosis, and measures to suppress the stimuli triggering can be key therapeutic strategies for early diagnosis of thyroid storm in FA.

Read more about FA therapies

In the present study, the researchers demonstrated the case of a 26-year-old male with a history of FA associated with structural cardiac involvement. Despite treatment with amiodarone and several electrical cardioversion therapies, he was admitted to the hospital multiple times due to decompensation subservient to atrial fibrillation/atrial flutter with rapid ventricular response and impaired ventricular function.

In addition, the patient exhibited significant hyperthyroidism upon using the amiodarone drug, which was later discontinued. Further analysis revealed the presence of symptoms similar to thyroid storm, following which antithyroid treatment and plasmapheresis were started, which improved the symptoms and heart rate to a certain extent; however, regulating the thyroid hormone levels was a challenging task.

Following that, the patient underwent total intravenous anesthesia and was administered various medications before the induction. All his vital signs remained stable during the first 100 minutes of the intervention, except for fluctuations in the bispectral index. However, after that, the patient’s body temperature and heart rate gradually increased, and his blood pressure dropped.

Results of the Burch-Wartofsky Point Scale analysis showed that the patient was experiencing a new episode of thyroid storm; therefore, supportive measures were initiated to control the patient’s temperature and heart rate. Furthermore, the patient was transferred to the postoperative critical care unit for monitoring, where he was extubated 4 hours after surgery and showed good postoperative recovery. Antithyroid treatment was continued, and he did not show new episodes of instability during his stay.

“We believe that early identification of the clinical picture and rapid implementation of the treatment strategies are the key to increasing the patient’s likelihood of surviving a thyroid storm,” the authors wrote.

FA is an autosomal recessive disorder affecting the pyramidal and corticospinal tracts. The presence of ataxia, dysarthria, oculomotor dysfunction, spasticity, and muscle weakness mainly characterize the disease. Half of all FA patients are believed to present supraventricular and ventricular arrhythmias.


Closa MS, Requena AP, García SS, et al. Anaesthetic management of thyroid storm in a patient with Friederich’s ataxia. a case report. Rev Esp Anestesiol Reanim (Engl Ed). Published online June 3, 2023. doi:10.1016/j.redare.2023.06.002