Table 1. Clinical PHH phenotypes recapitulated by this neonatal LPA-induced mouse model.
Hydrocephalus characteristics |
Clinical studies | Neonatal model |
Bleeding/hemorrhage | Yes (1–3) | Mimicked |
Ventricular dilation | Yes (1–3) | Yes |
Elevated CSF pressure | Yes (1–3) | Yes |
Early lethality | 75% untreated (41) and 20% treated (3) |
80% by 12 weeks |
Loss of ependymal layer |
Yes (15, 16) | Yes |
Ciliary defects | Yes (42) | Yes |
Aqueductal occlusion | Sometimes (43) | No |
Monocyte infiltration | Implicated in shunt failure (44) and systemic inflammation (45) |
Yes |