Neurological Manifestations of Enterovirus 71 Infection in Children during an Outbreak of Hand, Foot, and Mouth Disease in Western Australia

Neurological Manifestations of Enterovirus 71 Infection in Children during an Outbreak of Hand, Foot, and Mouth Disease in Western Australia
EV71 is an endemic enterovirus with a worldwide distribution [3, 13] and a propensity to cause epidemics with a high frequency of HFMD and/or neurological disease [2–4, 6]. There is significant variation in the spectrum of neurological disease associated with EV71 epidemics. Certain outbreaks have been associated with a predominance of aseptic meningitis (97%) [8], a high frequency of poliomyelitis-like paralysis (21%) [4, 5, 13], and, more recently, fatal brain-stem encephalitis [12, 14, 15]. Although the clinical presentation of acute neurological disease in the Perth outbreak was similar to that observed in previous epidemics [2–4, 6, 8], immunopathological mechanisms, rather than primary gray matter disease, appeared to be responsible for many cases of neurological disease in our cohort.
The tropism of EV71 for spinal cord and brain-stem gray matter is well established [5, 12, 16]. In our study, patient 9 developed acute cerebellar ataxia and mutism and had changes shown on MRI that were consistent with primary gray matter disease, including the presence of cytotoxic edema in the right cerebellar hemisphere on diffusion-weighted imaging. Cerebellar mutism is a rare, transient syndrome of unknown pathogenesis, most frequently associated with the trauma caused by surgical removal of midline cerebellar tumors [17]. To our knowledge, this is the first case of cerebellar mutism associated with enterovirus infection.

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