456 - Hemispherotomy in Adult Rasmussen Encephalitis: Multidisciplinary Inpatient Rehabilitation Approach

Friday, February 23, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

BH

Bachar S. Hazim, MD

Resident
Corewell Health
Taylor, Michigan, United States

Co-Author(s)

SK

Shaima Khandaker, MD

Resident
Corewell Health
Taylor, Michigan, United States
RS

Riley Smith, MD

Fellowship Director of Brain Injury Medicine
Corewell Health
Taylor, Michigan, United States
PR

P. Tyler Roskos, PhD

Director of Research
Corewell Health
Taylor, Michigan, United States

Case Diagnosis: Adult Rasmussen Encephalitis resulting in right-upper extremity dystonia and intractable epilepsy treated with left hemispherotomy warranting postoperative multidisciplinary inpatient rehabilitation (IPR).

Case Description: A 23-year-old left-handed female presented with intractable epilepsy associated with right-upper extremity dystonia. Her dystonia started at age 12 while seizures started at age 20. Despite several antiepileptic medications, she continued to have persistent worsening seizures. Left stereoelectroencephalography implantation showed irritative zones in the frontal and temporal lobes. The patient underwent left temporal lobectomy and amygdalohippocampectomy. Pathohistological results suggested Rasmussen's Encephalitis. Neuropsychological evaluation showed progressive declines in verbal memory and language functioning associated with left hemisphere dysfunction. She continued having frequent and severe seizures ultimately necessitating left hemispherotomy to achieve seizure freedom. Postoperatively, she was nonverbal with increased right-sided motor deficits. She participated in acute care physical therapy and was transferred to IPR. During her rehabilitation course, she worked with a multidisciplinary team including speech therapy and neuropsychology. She was also started on donepezil for language and cognitive deficits. Assessments showed improvements in verbal expression, transfers, and dressing assistance.

Discussions: Rasmussen’s encephalitis is a rare and severe progressive inflammatory disease that typically affects one hemisphere of the brain in children and adolescents. Limited cases have been reported regarding IPR in adults after hemispherotomy to manage intractable epilepsy in Rasmussen Encephalitis. In this case, the patient benefitted from a multidisciplinary rehabilitation program leading to improvements in left cerebral hemisphere language and motor functions, as well as activities of daily living.

Conclusions: This case of Rasmussen Encephalitis in an adult female who underwent left hemispherotomy demonstrates the importance of post-operative multidisciplinary rehabilitation to treat functional motor and language deficits in this rare neurological disorder.