Fulminant Encephalopathy with Marked Brain Edema and Bilateral Thalamic Lesions

Abstract

We encountered two children with acute encephalopathy associated with unique clinical manifestations. Both the patients had status epilepticus at onset and neuroimaging studies revealed marked brain edema and bilateral thalamic lesions. Although they were treated with steroids and immunoglobulin, their outcomes were very poor. A thermolabile variant of carnitine palmitoyltransferase II and an elevated interleukin-6 level in cerebrospinal fluid were observed in one patient each. The constellation of clinical and neuroimaging findings in our patients is apparently not consistent with any established subtype of acute encephalopathy/encephalitis.

A Fatal Case of Encephalitis

“…Ando et al [3] recently reported two cases of acute encephalopathy with distinctive clinical manifestations. Both of their patients had SE and marked brain edema with bilateral thalamic lesions on the MRI.…”

“…His cranial MRI revealed findings concordant with left temporal lobe encephalopathy. His blood tests were, CRP: 101 mg/L, white blood count (WBC): 11000 mm 3 , neutrophils: %82, lymphocyte: %10, platelet: 269000, Hgb: 13.5 g/dl, Urea: 34 mg/dl, creatine: 0.9 mg/dl, alanine transaminases (ALT): 34IU/L, aspartate transaminases (AST): 32IU/L, ESR:15 mm/h, blood glocose:83 mg/dl, anti-HIV: negative. His electrolyte levels were normal.…”

“…Acute encephalitis has wide variety of clinical manifestations. Patients may have mild symptoms like diminished consciousness or they may have mortal clinical course complicating with coma, seizures and even multi-organ failure [3]. Infections are common causes of encephalitis.…”