Summary
Two cases are reported of the rare anatomical abnormality of fusion of the cerebellar hemispheres with agenesis of the vermis. The cases are the more unusual since they show prolonged survival and the absence of other major fusion abnormalities in the nervous system.
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References
Bartelmez GW, Dekaban AS (1962) The early development of the human brain. Contrib Embryol Carnegie Inst 37: 13–32
Friede RL, Bolthauser E (1978) Uncommon syndromes of cerebellar vermis aplasia. I. Joubert syndrome. Dev Med Child Neurol 20:758–763
Gross H (1959) Die Rhombenkephalosynapsis, eine systemisierte Kleinhirnfehlbildung. Arch Psychiatr Nervenkr 199:537–552
Gross H, Jellinger K, Kaltenback E (1978) Rhombencephalosynapsis eine seltene Dysraphieform. Zentralbl Allg Pathol Pathol Anat 122:577
Kepes JJ, Clough C, Villanueva A (1969) Congenital fusion of the thalami (atresia of the third ventricle) and associated abnormalities in a 6-month-old infant. Acta Neuropathol (Berl) 13:97–104
Lemire RJ, Loeser JD, Leech RW, Alvord RC Jr (1975) Normal and abnormal development of the human nervous system. Harper and Row, Hagerstown, pp 319–336
Marks TA, Worthy WC, Staples RE (1980) Teratogenic potential of phencyclidine in the mouse. Teratology 21: 241–246
Michand J, Mizrahi EM, Urich H (1982) Agenesis of the vermis with fusion of the cerebellar hemispheres, septo-optic dysplasia and associated abnormalities. Acta Neuropathol (Berl) 56:161–166
Obersteiner H (1916) Ein Kleinhirn ohne Wurm. Arb Neurol Inst Wien Univ 21:124–136
Schachenmayr W, Friede R (1982) Rhombencephalosynapsis: a Viennese malformation? Dev Med Child Neurol 24:178–182
Williams B (1982) Aqueduct stenosis. In: Recent advances in neuropathology, vol 2. Churchill-Livingstone, Edinburgh, pp 273–296
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Supported in part by an MRC Training Fellowship in Neuropathology (MI)
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Isaac, M., Best, P. Two cases of agenesis of the vermis of cerebellum, with fusion of the dentate nuclei and cerebellar hemispheres. Acta Neuropathol 74, 278–280 (1987). https://doi.org/10.1007/BF00688192
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DOI: https://doi.org/10.1007/BF00688192