Clinical Sciences/Health Conditions
Rebecca P. Liu, MD
Resident Physician
UT Southwestern
Dallas, Texas, United States
Rajashree Srinivasan, MBBS
Associate professor
UTSW
Plano, Texas, United States
Patient A is an 11 year old female with history of seizures complicated by status epilepticus and epilepsy partialis continua starting at age 3 years. MRI brain showed atrophy of the right hemisphere. Rasmussen encephalitis was diagnosed 5 months after seizure onset. Patient B is a 6 year old female with seizure onset at age 3 years. Serial brain MRIs showed gradual atrophy of the left hemisphere. Rasmussen encephalitis was diagnosed 10 months after seizure onset.
Case Description:
Patient A underwent right hemispherotomy 1 month after diagnosis for treatment of seizures. Deficits included left hemiparesis, cognitive impairment and left eye neglect. After 31 days of inpatient rehab with all 3 therapy disciplines, patient demonstrated active left arm movement and improvement in left-sided attention, expressive language and social engagement. WeeFIM scores for transfers and locomotion also improved.
Patient B underwent left hemispherotomy 1.5 years after diagnosis for treatment of seizures. Deficits included right hemiparesis, dysarthria and cognitive impairment. After 36 days of inpatient rehab with all 3 therapy disciplines, WeeFIM scores for self care domains, transfers and locomotion improved. Cognitive and Linguistic Scale (CALS) scores advanced from 66 at admission to 80 at discharge.
Discussions:
Rasmussen encephalitis is a rare CNS disorder characterized by chronic progressive inflammation of one cerebral hemisphere resulting in epileptic seizures and cerebral destruction. Diagnosis requires clinical and imaging findings. Limited literature exists on functional recovery in patients with Rasmussen encephalitis. Literature on this topic depicts possibility of functional recovery post-hemispherotomy in those with Rasmussen encephalitis.
Conclusions:
These cases show that patients with Rasmussen encephalitis who undergo hemispherotomy can gain functional recovery in the setting of inpatient rehab where patients’ deficits are continuously re-evaluated and therapies modified to accommodate for evolving functional levels. Standardized scoring systems such as WeeFIM and CALS allow for objective evaluation of functional gains throughout admission.
