Abstracts

Rationale: Severe excitotoxic brain injury with crossed cerebellar diaschisis have been reported in cases of prolonged status epilepticus. MRI findings are usually very focal or bilateral increased T2/FLAIR signal with some edema. These regions may show corresponding DWI/ADC changes. These changes are usually transient and reversible, and rarely provoke hemispheric syndromes. Hemiconvulsion-hemiplegia syndrome (HHS), however, is an uncommon outcome of prolonged febrile focal status epilepticus in children under the age of 4, causing hemispheric syndromes and impressive radiologic evidence of acute cytotoxic edema in the affected side. We share a case of hemiconvulsion-hemiplegia like syndrome in an 18-year-old male.

Methods: Case report

Results: An 18-year-old male with a history of epilepsy presented to an outside hospital (OSH) due to presumed status epilepticus. The patient’s last known normal was the night prior, at around 11pm. He was then found on the floor, unresponsive, at 3pm of the following day. Upon arrival to OSH ER, he was in multi-organ failure and was loaded with levetiracetam. He was intubated for airway protection and transferred to our hospital for CVEEG. On arrival to our hospital, labs were significant for Cr 7.2, CPK 70,000, lactic acidosis, and leukocytosis. Echocardiogram revealed cardiac dysfunction with EF of 25%. He was connected to a CVEEG which showed a slow nonreactive background with intermittent right frontotemporal slowing and occasional right temporal sharps. On day 2, he was noted to have left hemiplegia. MRI Brain showed restricted diffusion in the temporal lobes, right thalamus and limbic structures, and right cerebral cortex. CSF findings: RBC 64, WBC 8, Protein 91, Glucose 72. CSF meningoencephalitis, autoimmune and paraneoplastic panels were negative. On day 6, when extubated, patient was alert, disoriented, complaining of right-sided headache, and had dense left hemiplegia with hemineglect and hemianopia. Repeated MRI brain showed subtle increased restricted diffusion with corresponding T2/FLAIR hyperintense signal involving the right cortex, right hippocampus, caudate nucleus, and thalamus with a 7 mm midline shift to the left. Also noted crossed cerebellar diaschisis. MRA head and neck were unremarkable. Due to worsening of MRI findings and lack of clinical improvement, a 5-day course of solumedrol was given. On day 12, patient was clinically more alert and oriented, however dense left hemiplegia and neglect persisted. MRI Brain showed improvement in midline shift, restricted diffusion, and FLAIR changes in the cortex, with more involvement of the subcortical areas. Mitochondrial DNA was positive for variant of unknown significance (VUS) in the WFS1 gene and genetic epilepsy panel for VUS in ANKRD11 gene. On day 20, patient was discharged to inpatient rehab with persistent dense left hemiplegia and neglect.

Conclusions: Prolonged ictal activity provoking inflammation and increase in blood-brain-barrier permeability play a role in the pathogenesis of severe cases of excitotoxic injury resulting in neuronal death in the seizure circuit. Early recognition and treatment of status epilepticus is imperative to prevent permanent cerebral injury.

Funding: Please list any funding that was received in support of this abstract.: None.