1519 - Cortical Ribbon Sign: A Sign of New Onset Seizures

Thursday, February 22, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

ER

Erick Rosenvall, MD

Resident
ECU Health Medical Center
Greenville, North Carolina, United States

Co-Author(s)

EZ

Evan Zeldin, MD

Clinical Assistant Professor
University of Cincinnati
Cincinnati, Ohio, United States

Presenter(s)

CC

Cole Carter, DO

Resident
ECU Health Medical Center
Greenville, North Carolina, United States

Case Diagnosis: 50-year-old female with cortical ribbon sign secondary to new onset seizure

Case Description: Patient presented after being found comatose at home with bouts of seizure-like activity. MRI brain revealed cortical laminar necrosis with radiology suggesting possible Creutzfeldt-Jakob Disease (CJD). Prior to admission, the patient denied typical CJD symptoms such as progressive memory problems, ataxia, or myoclonus. She had no history of seizures. Despite her clinical course suggesting against a diagnosis of CJD, the patient was misdiagnosed with sporadic CJD.Patient was admitted to IPR with significant neurological deficits including left homonymous hemianopia and left hemiparesis. Neurology was consulted and believed the MRI findings were more consistent with sequela from prolonged seizure or status epilepticus as well as the rapid progression of symptoms being inconsistent with sporadic CJD. The patient improved with intensive inpatient therapies.

Discussions: Cortical ribbon sign is due to cortical laminar necrosis (CLN) with associated neurological deficits is a rare finding due to status epilepticus. CLN is radiologically defined as high intensity cortical lesions on T1 weighted MRI images following a gyral distribution. Other radiographic clues for the diagnosis of CLN are diffusion restriction not respecting vascular territories, hyperperfusion, and frequent involvement of the mesial temporal structures as well as the pulvinar thalami. When comparing radiographic findings of CLN with sporadic CJD, penetrance of restricted diffusion is higher and FLAIR changes are much brighter in CLN. Additionally, pathology seen on MRI in sporadic CJD will always involve pulvinar thalami as well as the rest of the basal ganglia. In this patient, the basal ganglia was spared.

Conclusions: Cortical ribbon sign from cortical laminar necrosis with associated neurological deficits due to status epilepticus is a rare finding, however important to be aware of for proper diagnosis. It is important not to disregard the clinical course regardless of symptomatology and objective findings such as imaging.