A Case of Hemiplegia Vegetativa Alterna, Paroxysmal Sympathetic Hyperactivity and Ogilvie’s Syndrome: The Role of Central Sympathetic Pathways in Their Pathophysiology

Abstract

We describe a patient with aneurysmal subarachnoid hemorrhage who developed hemiplegia vegetativa alterna (HVA), a rare brainstem syndrome characterized by contralateral hemiplegia, hemisensory loss, hemihyperhidrosis and ipsilateral Horner's syndrome. The patient also experienced paroxysmal sympathetic hyperactivity (PSH) and colonic pseudo-obstruction, known as Ogilvie's syndrome. A magnetic resonance imaging (MRI) scan revealed a focal lesion at the anterolateral mesencephalon. It is believed that lesions of this region can interrupt central sympathetic pathways and account for this unique combination of syndromes. This first documented case provides an opportunity to review the relevant neuroanatomy and contributes to cumulative clinical evidence verifying the excitatory-inhibitory ratio (EIR) disconnection theory for PSH.