Summary
The neuropathology of congenital hydrocephalus in SUMS/NP mice was investigated in this study. Hydrocephalus occured with an incidence of 16.5% and was evident in the form of an enlarged head soon after birth. The condition was progressive; affected mice gradually became noticeably smaller than normals, acquired functional disturbances and died before ever breeding. Hydrocephalic mice exhibited enormous dilatation of the lateral ventricles, thinning of the cortex and compression and distortion of the diencephalon and striatum. Examination of the midbrain in these animals revealed that the proximal cerebral aqueduct was diminished in size, occluded or absent. Evidence of its anomalous development was seen as early as day 15 of foetal development. This is in substantial agreement with previous reports on this strain. Although the mechanism by which this occurs is still unknown, abnormal development of the adjoining diencephalon and/or rostral midbrain is likely to be involved.
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References
Beemer FA, Ertbruggen IV (1984) New syndrome: Peculiar facial appearance, hydrocephalus, double-outlet right ventricle, genital anomalies, and dense bones with lethal outcome. Am J Med Genet 19: 391–398
Berry RJ (1961) The inheritance and pathogenesis of hydrocephalus—3 in the mouse. J Pathol Bacteriol 81: 157–167
Bickers DS, Adams RD (1949) Hereditary stenosis of the aqueduct of Sylvius as a cause of congenital hydrocephalus. Brain 72: 246–262
Borit A, Sidman RL (1972) New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuropathol 21: 316–331
Bruni JE, Del Bigio MR, Clattenburg RE (1985) Ependyma: normal and pathological. A review of the literature. Brain Res Rev 9: 1–19
Bryan JHD, Hughes RL, Bates TJ (1977) Brain development in hydrocephalic-polydactyl, a recessive pleiotropic mutant in the mouse. Virchows Arch A 374: 205–214
Clark FH (1932) Hydrocephalus, a hereditary character in the house mouse. Proc Nat Acad Sci 18: 654–656
Dignan PSJ, Warkany J (1974) Congenital malformations: hydrocephaly. Mental Retard 6: 44–83
Green CG (1970) The developmental effects of congenital hydrocephalus (ch) in the mouse. Dev Biol 23: 585–608
Gruneberg H (1943 a) Congenital hydrocephalus in the mouse, a case of spurious pleiotropism. J Genet 45: 1–21
Gruneberg H (1943 b) Two new mutant genes in the house mouse. J Genet 45: 22–28
Hollander WF (1966) Hydrocephalic-polydactyl, a recessive pleiotropic mutant in the mouse. Am Zool 6: 588
Jones HC (1984) The development of congenital hydrocephalus in the mouse. Z Kinderchir [Suppl] II, 39: 87–88
Jones HC (1984) The resistance to drainage of the cerebrospinal fluid in normal mice and in mice with congenital hydrocephalus. J Physiol 357: 41
Jones HC (1985) Cerebrospinal fluid pressure and resistance to absorption during development in normal and hydrocephalic mutant mice. Exp Neurol 90: 162–172
Jones HC, Dack S, Ellis C (1987) Morphological aspects of hydrocephalus in a mouse mutant (SUMS/NP). Acta Neuropathol 72: 268–276
Kuzniecky RI, Watters GV, Watters L, Meagher-Villemure K (1986) X-linked hydrocephalus. Can J Neurol Sci 13: 344–346
Laurence KM (1984) Genetic aspects of uncomplicated hydrocephalus and its relationship to neural tube defect. Z Kinderchir [Suppl] 2, 39: 96–99
Lorber J (1984) The family history of uncomplicated congenital hydrocephalus: an epidemiological study based on 270 probands. Br Med J 289: 281–283
Lycka BAS, Chichak VR (1985) Congenital hydrocephalus and the basal cell nevus syndrome. Can Med Assoc J 132: 1037–1038
McLone DG (1979) Cerebrospinal fluid pathways in a murine mutant. Child's Brain 5: 293–303
McLone DG, Bondareff W, Raimondi AJ (1971) Brain edema in the hydrocephalic hy-3 mouse: submicroscopic morphology. J Neuropathol Exp Neurol 30: 627–637
Punt J, Pickard J, Weller R, Mitchell JM, Mather C, Perry S, Hunt R (1982) The hydrocephalic mutant mouse. J Neurol Neurosurg Psychiat 45: 280
Raimondi AJ, Bailey OT, McLone DG, Lawson RF, Echeverry A (1973) The pathophysiology and morphology of murine hydrocephalus in hy-3 and Ch mutants. Surg Neurol 1: 50–55
Raimondi AJ, Clark SJ, McLone DG (1976) Pathogenesis of aqueductal occlusion in congenital murine hydrocephalus. J Neurosurg 45: 66–77
Richardson RR (1985) Congenital genetic murine (ch) hydrocephalus. A structural model of cellular dysplasia and disorganization with the molecular locus of deficient proteoglycan synthesis. Child's Nerv Syst 1: 87–99
Richardson RR, Rees MG (1984) Renal dysplasia and chondrodysplasia in the ch hydrocephalic mouse: a cellular model of defective differentiation and organization. Mount Sinai J Med 51: 188–196
Waller PE, Aarskog D (1980) Syndrome of hydrocephalus, costovertebral dysplasia and and Sprengel anomaly with autosomal dominant inheritance. Neuropediatrics 11: 291–297
Weller RO, Mitchell J, Griffin RL (1981) Cerebral ventriculitis in the hydrocephalic mouse: a histological and scanning electron microscope study. Acta Neuropathol [Suppl] VII: 160–161
Winter RM, Campbell S, Wigglesworth JS, Nevrkla EJ (1987) A previously undescribed syndrome of thoracic dysplasia and communicating hydrocephalus in 2 sibs, one diagnosed prenatally by ultrasound. J Med Genet 24: 204–206
Wozniak M, McLone DG, Raimondi AJ (1975) Micro- and macrovascular changes as the direct cause of parenchymal destruction in congenital murine hydrocephalus. J Neurosurg 43: 535–545
Zimmerman K (1933) Eine neue Mutation der Hausmaus: “Hydrocephalus” Z Indukt Abstamm Vererb 64: 176–180
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Bruni, J.E., del Bigio, M.R., Cardoso, E.R. et al. Neuropathology of congenital hydrocephalus in the SUMS/NP mouse. Acta neurochir 92, 118–122 (1988). https://doi.org/10.1007/BF01401981
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DOI: https://doi.org/10.1007/BF01401981