Ehlers Danlos, POTS, and Occult Cerebrospinal Fluid Leak: A Case Report

Abstract

Anuj Aggarwal and Ian Carroll

Ehlers Danlos syndromes (EDS) are associated with spontaneous intracranial hypotension (SIH) and postural orthostatic tachycardia syndrome (POTS). We hypothesized that some POTS patients might in fact have occult SIH due to unappreciated EDS. We describe a 26-year-old female with a history of POTS and headache who presented to us with negative imaging for cerebrospinal fluid leak (CSF). Upon examination with clinical evidence of EDS and supportive history, we performed an epidural blood patch despite repeat imaging evidence negative for CSF leak resulting in resolution of the patient’s POTS, headache, and cognitive symptoms. Patients with POTS and connective tissue disorders associated with dural weakness may be suffering from occult chronic CSF leaks. MRI imaging may be falsely reassuring and dissuade clinicians from performing epidural blood patching among patients who can be cured.

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