Response: must acute necrotizing encephalopathy of childhood and acute bilateral striatal necrosis be differentiated?

Abstract

I would like to thank Dr. Wang for his interest in our article and comments. This is indeed a confusing area and it is not clear whether acute necrotizing encephalopathy (ANE) and acute bilateral striatal necrosis are entirely different diseases or only different presentations of the same disease. As we addressed in our article (see Table 1) [ [1] Sazgar M. Robinson J.L. Chan A.K.J. Sinclair D.B. Influenza B acute necrotizing encephalopathy: A case report and literature review. Pediatr Neurol. 2003; 28: 396-399 Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar ], the basal ganglia seems to be selectively vulnerable to a number of metabolic, infectious, and neurodegenerative diseases. This would suggest that it is the properties of the region rather than the etiology which is the main determining factor in these diseases. The case presented by Dr. Wang [ [2] Wang H.S. Huang S.C. Acute necrotizing encephalopathy of childhood. Chang Gung Med J. 2001; 24: 1-10 PubMed Google Scholar ] is very different from ours in several ways, including clinical presentations (dystonia), timing (postinfectious), and site and extent of injury (white matter involvement). The postinfectious time course and white matter changes in Dr. Wang's case (Fig 1) would suggest postinfectious autoimmune disease like acute disseminating encephalomyelitis (ADEM) rather than an acute inflammatory disease such as ANE. Until the etiology and mechanism of injury in ANE can be established, the “lumping or splitting” of these diseases of the basal ganglia is just a question of philosophy.