A Little Girl’s Legacy to an ANE Pioneer

Dr. Masashi Mizuguchi, Professor, Department of Developmental Medical Sciences at the The University of Tokyo, Japan answer some questions for us. Dr Mizuguchi is a pioneer of ANE research and first proposed it as its own entity in 1995. Here he gives the reasons he chose to study ANE.

Q1: What do you find most fascinating about ANE?

In the year 1981, soon after I became a pediatrician, I encountered for the first time a patient with ANE, a 5-year-old Japanese girl. In the evening, she visited the emergency room (ER) of a municipal hospital where I was on duty. According to the prior phone call from the ambulance crew, the diagnosis was supposed to be febrile seizures. Soon after her arrival to ER, however, I realized that she had some critical condition which I had never seen or even heard of. I was shocked by all her findings: physical findings of very high body temperature (41℃), coma and decorticate rigidity, laboratory findings of exceedingly high serum transaminases (>50,000 units) and cerebrospinal fluid protein (yellow, viscous and clotting spontaneously), and CT findings of symmetrical lesions in the bilateral thalamus. Subsequently, she never recovered consciousness and died two weeks later. Since that night, I have been studying this devastating syndrome, ANE, for more than three decades. I feel as if the patient has told me to do so from the paradise.

Q2: If you could advice to doctors working in Emergency Room what would it be?

When infants and young children have a viral infectious disease (such as influenza and exanthema subitum) with fever and convulsion, the diagnosis is febrile seizures in the vast majority of them. In the case of prolonged coma following the convulsion, however, please consider the possibility of encephalitis and encephalopathy syndromes, including ANE.

Q3: What advice would you give parents when they first present to the ER with neurological change in their child to get the deserved attention?

If your child recovers consciousness within 30 minutes or 1 hour after convulsion, please relax. It’s only febrile seizures. If not, ask the doctor again about the next step of diagnosis and treatment.

Department of Developmental Medical Sciences
The University of Tokyo

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