Locked-in Syndrome Secondary to Vertebro-basilar Dolichoectasia - An Unfortunate Complication of a Rare Pathology in a Young Patient
Ahsan Ali1, Hira Pervez2, Aizaz Ali3, Paige Prologo-Richardson4, Syed Muhammad Owais2
1SLUCare Academic Pavilion, 2University of Toledo, 3Somo Apartments, 4University of Toledo College of Medicine and Life Sciences
Objective:
Dolichoectasia; a rare progressive arteriopathy observed in the posterior circulation, with a prevalence 3% in the general population. Basilar-ectasia is defined as >4.5mm at mid-pontine level. Patients presentation is from recurrent transient ischemic attacks, vestibular symptoms and highly morbid locked-in syndrome, commonly due to vascular events or compressive effects on the brain stem/cranial nerves due to the tortuous vessel.
Background:
We present a case of 39-year-old male, with a history of joint laxity who presented to the emergency department with tonic posturing and seizure like activity without resolution with Lorazepam and Levetiracetam. He was admitted to the neuro ICU following intubation for declining neurological status. The physical exam demonstrated right upper extremity flexor posturing and an asymmetric withdrawal to pain. Initial CT and CTA head and neck revealed dolichoectasia of the left vertebral artery with extension to the basilar artery and occlusion of right vertebral artery. An EEG remained negative for electrographic or clinical seizures. A stat MRI brain w/wo contrast showed acute ischemia involving the cranial para-midline medulla, Ponto-medullary junction, cranial and caudal pons without hemorrhage. A diagnostic cerebral angiogram demonstrated diffusely large and mildly dysplastic vessels, right vertebral artery occlusion at the origin and missing right PCA, about 70mm x 10 mm dolichoectasia extending from V4 up to AICA origin without reflux into the right V4 segment. Serial physical exam remained unchanged with intact level of consciousness, vertical eye movements, asymmetric extensor posturing left greater than right and triple flexion with spasticity in bilateral lower extremities suggestive of locked in syndrome.
Conclusions:
Vertebro-basilar dolichoectasia represent a challenging pathology with no treatment guidelines regarding medical management versus stenting, with variable outcome in different studies. Considering the higher risk of ischemic complications, further studies need to be done to guide treatment plan.