Idiopathic intracranial hypertension and Ehlers-Danlos syndrome: a case series.

Abstract

Background: Ehlers Danlos syndrome (EDS) is a group of 13 heritable connective tissue disorders with various ophthalmic manifestations. Idiopathic intracranial hypertension (IIH) is the presence of raised intracranial pressure (ICP) without specific neurological disease and deficits along with normal neuroimaging. The relationship between EDS and IIH has not been evaluated. In this report, we present a series of three confirmed cases of EDS with features of IIH.

Case series: Case 1 was a 23 year old female patient with EDS who presented with intractable headaches. Her lumbar puncture (LP) had an elevated opening pressure of 53 cm H₂0 and an MRI showed signs of raised ICP. Similarly, Case 2 was a 38 year old female who presented with worsening headaches in the setting of clinically proven EDS. LP had an elevated opening pressure of 37 cm H₂0 and an MRI showed signs of raised ICP. Optical coherence tomography (OCT) showed ganglion cell loss. Case 3 was a 55 year old female with EDS who presented with diplopia and pituitary microadenoma in the setting of chronic IIH. OCT showed nerve fiber layer and ganglion cell loss. We did not observe papilledema in any of the cases.

Conclusion: This is the largest series of cases linking IIH to EDS. Hyperextensibility may make meninges more pliable and tolerant to raised ICP. There may be a potential association between IIH and EDS, and IIH may be considered as one of the rare manifestations of EDS.