Auditory brainstem function in microcephaly related to Zika virus infection.

OBJECTIVE: To study the effect of prenatal Zika virus (ZV) infection on brainstem function reflected in brainstem auditory evoked potentials (BAEPs). METHODS: In a cross-sectional study in 19 children (12 girls) with microcephaly related to ZV infection, aged between 12 and 62 weeks, the brainstem function was examined through BAEPs. The latencies of wave peaks I, III, and V of the left and right ears (n = 37) were standardized according to normative data, and compared between them by 2-tailed t test. The confounding variables (cephalic perimeter at the born and chronological age) were correlated with the normalized latencies using Pearson test. RESULTS: All patients showed, in general, clear waveforms, with latencies within 3 SDs of the normative values. However, statistically increased latencies of waves I and III (I > III, p = 0.031) were observed, relative to wave V (p < 0.001), the latter being closer to respective normative value. The latency of wave I was observed to increase with age (r = 0.45, p = 0.005). The waves, in turn, did not depend on cephalic perimeter. CONCLUSIONS: These results are consistent with the functional normality of the brainstem structure and its lack of correlation with microcephaly, suggesting that the disruption produced by the ZV infection does not act in the cell proliferation phase, but mostly in the processes of neuronal migration and differentiation in the telencephalon.

Spinal cord compression in young children with type VI mucopolysaccharidosis.

Spinal cord compression (SCC) is a known complication of mucopolysaccharidosis type VI (MPS VI) secondary to atlantoaxial subluxation, craniovertebral stenosis, posterior longitudinal ligament hypertrophy, or dural thickening. SCC is expected to occur in the natural history of the disease, regardless of enzyme replacement therapy (ERT), as intravenous enzyme does not cross the blood-brain barrier. We describe six MPS VI children with SCC, all diagnosed before 7years of age. Within this group, four of the children were diagnosed with SCC after the introduction of ERT. We hypothesize that these patients may illustrate the previously undetected risk of increased joint mobility caused by ERT which may have contributed to increased cervical instability by loosening the neck joint, thus leading to or unmasking SCC. We reinforce the need for close follow-up of SCC, periodic neurological assessment, spine imaging, and neurophysiology in all MPS VI patients before and during ERT. Neurophysiological abnormalities may precede changes in MRI images (as shown in patients 4 and 5 from this sample) and should, therefore, be accessed in MPS VI patient evaluations, allowing for timely intervention and better prognosis. We recognize the limitations of these data due to the small sample size and recommend further investigation into this patient population.