P212: Clinical spectrum of sensory ganglionopathy in eight patients

Zuberbuhler, Paz; Young, Park Woo; Bandeo, L.; Cejas, L.V. León; Finn, Bridgid; Bruetman, Julio E; Calandra, Cristian; Fulgenzi, E.; Akly, Manuel Pérez; Pardal, Ana; Reisin, Ricardo

doi:10.1016/s1388-2457(14)50346-x

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“…13 Another study treated SNN with IVIG and/or high-dose steroids, but reported poor responses in 4 of 10 patients, neurological improvements in 5 of 10 patients, and no change in 1 patient. 14 Antoine et al suggested that the optimal therapeutic window for treating acute/subacute inflammatory SNN is within 8 months of disease onset for stabilization and within 2 months of disease onset for symptom improvement. 15 In our case, no treatment was administered for financial reasons and no spontaneous improvements in neurological deficits were observed over 1 year of careful follow-up clinical monitoring.…”

Section: Discussionmentioning

confidence: 99%

Acute Sensory Neuronopathy following Enterovirus Infection in a 3-Year-Old Girl

Chiu

Yang

et al. 2017

Neuropediatrics

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Acute sensory neuronopathy (SNN) is a rapidly developing peripheral nervous system disease that primarily affects sensory neurons in the dorsal root ganglion or trigeminal ganglion, leading to the impairment of sensory axons. SNN is notably uncommon in childhood; only three cases of childhood or adolescent SNN have been reported to date. Moreover, SSN has never been reported in association with enterovirus infection. Here, we report the case of a 3-year-old girl who was initially diagnosed with enterovirus infection based on the presentation of fevers, rashes on all extremities, and ulceration over the posterior pharynx. Nine days later, she presented with ataxic and wide-based gait and dysmetria affecting the extremities, with an absence of sensory nerve action potentials in the upper and lower limbs. The patient was diagnosed with acute SNN based on the criteria developed by Camdessanché et al in 2009. To our knowledge, this is the youngest case of SNN reported to date. In addition, this case reveals that enterovirus infection can be associated with acute SNN in children in rare cases. Accurate diagnosis relies on clinical suspicion, comprehensive knowledge of the patient's history, and careful characterization of abnormal findings in electrodiagnostic studies.

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Section: Discussionmentioning

confidence: 99%