Endocrinology Research and Practice
Case Report

Two Siblings with Triple-A Syndrome: Endocrinologic and Neurologic Features

1.

Ege University Faculty of Medicine, Department of Endocrinology and Metabolism, İzmir, Turkey

2.

Ege University Faculty of Medicine, Department of Internal Medicine, İzmir, Turkey

3.

Ege University Faculty of Medicine, Department of Neurology, İzmir, Turkey

4.

Ege University Faculty of Medicine, Department of Gastroenterology, İzmir, Turkey

Endocrinol Res Pract 2018; 22: 45-49
DOI: 10.25179/tjem.2017-56501
Read: 2714 Downloads: 727 Published: 01 March 2018

ABSTRACT

Triple-A syndrome is a rare, multi-systemic disease and is characterized by adrenal insufficiency, achalasia, or alacrima. The symptoms may also involve neurologic manifestations, including pyramidal findings and peripheral motor neuropathy. Other findings include autonomic dysfunction and cerebellar ataxia. In the present study, we present the case of two siblings with triple-A syndrome with neurologic manifestations. The neurologic abnormalities can lead to misdiagnosis owing to resemblance to the manifestations of other neurologic disorders. Therefore, the cases with triple-A syndrome should be carefully evaluated and examined for clinical and neurophysiologic signs.

 

 

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