Bilateral Rasmussen encephalitis: postmortem documentation in a five-year-old.
A case of Rasmussen encephalitis with bilateral involvement and onset at age 2 years is reviewed. Rasmussen encephalitis is a rare progressive disease that causes intractable seizures, cognitive decline, and inflammatory changes in the brain. The neurologic involvement is characteristically unilateral. Bilateral involvement in this case was suspected within 5 months of presentation and was confirmed by bilateral frontal lobe biopsies. The severity and progression of the disease in this case was remarkable and resulted in the patient's death by age 5 years, despite numerous attempted therapeutic interventions. Autopsy findings confirmed the diagnosis of Rasmussen encephalitis with bilateral involvement.
Tobias
Sharon M
SM
Pathology Department, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire 03756-0001, USA.
Robitaille
Yves
Y
Hickey
William F
WF
Rhodes
C Harker
CH
Nordgren
Richard
R
Andermann
Frederick
F
D001284:Atrophy; D001921:Brain / Q000473:pathology; D016923:Cell Death / Q000502:physiology; D002675:Child, Preschool; D018450:Disease Progression; D004292:Dominance, Cerebral / Q000502:physiology*; D004660:Encephalitis / Q000473:pathology*; D017036:Epilepsia Partialis Continua / Q000473:pathology*; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D009474:Neurons / Q000473:pathology