Reverse Locked-In Syndrome

Pooja Raibagkar, Ram V. Chavali, Tamara B. Kaplan, Jennifer A. Kim, Meaghan V. Nitka, Sherry H.-Y. Chou, Brian L. Edlow

Basilar artery occlusion can cause locked-in syndrome, which is characterized by quadriplegia, anarthria, and limited communication via eye movements. Here, we describe an uncommon stroke syndrome associated with endovascular recanalization of the top of the basilar artery: "reverse locked-in syndrome." We report the case of a patient with atypical neurological deficits caused by acute ischemic stroke of the midbrain tegmentum. We perform neuroanatomic localization of the patient's infarcts by mapping the magnetic resonance imaging (MRI) data onto a brainstem atlas. A 61-year-old man presented with acute coma and quadriplegia due to top of the basilar artery occlusion. He underwent emergent endovascular thrombectomy, with successful recanalization of the basilar artery at 4 h and 43 min post-ictus. The patient regained consciousness and purposeful movement in all four extremities, but the post-procedure neurological examination demonstrated bilateral ptosis with complete pupillary and oculomotor paralysis. MRI revealed infarction of the bilateral oculomotor nuclei in the midbrain tegmentum. At 9-month follow-up, he had anisocoria and dysconjugate gaze, but was living at home and required minimal assistance in performing all activities of daily living. Since the patient's deficits were the exact opposite of those described in locked-in syndrome, we propose the term "reverse locked-in syndrome" to describe this neurological entity characterized by bilateral ptosis, non-reactive pupils, and ophthalmoplegia with preservation of consciousness and extremity motor function.