Rhombencephalosynapsis
As Rhombencephalosynapsis is called a rare malformation of the posterior fossa . There is a malformation of the cerebellum , which is characterized by a fusion of the two cerebellar hemispheres, associated with agenesis or hypogenesis of the cerebellar worm, as well as a fusion of the dentate nucleus and the superior cerebellar peduncle. Associated with this, there may be various supratentorial pathologies such as aqueduct stenosis . This syndrome was first described by Obersteiner in a 28-year-old patient. The name comes from Gross and Hoff.
clinic
Described Stammataxie , dysarthria , strabismus , choreatic-athetoid dyskinesia , epilepsy . The severity of these symptoms varies widely and depends on the type and severity of the supratentorial lesions. The majority of them have cognitive retardation .
Web links
- S. Caffier, T. Kittner, M. Laniado: The Rhombencephalosynapsis as a rare anomaly in the posterior cranial fossa. In: RöFo - Advances in the field of X-rays and imaging processes. 173, p. 850, doi: 10.1055 / s-2001-16973 .
- JL Mendonca, MR Natal, SL Viana, PP Coimbra, MA Viana, M. Matsumine: Rhombencephalosynapsis: CT and MRI findings. In: Neurol India. 52, 2004, pp. 118-120.
Individual evidence
- ↑ H. Obersteiner: A cerebellum without a worm. In: Arb Neurol Inst. (Vienna) 21, 1914, pp. 124-125.
- ^ H. Gross, H. Hoff: Sur les dysraphies crâniocéphaliques. In: G. Heuyer, M. Feld, J. Gruner (Eds.): Malformations Congénitales du Cerveau. Masson, Paris 1959, pp. 287-297.
- ^ SP Toelle et al.: Rhombencephalosynapsis: Clinical Findings and Neuroimaging in 9 Children. In: Neuropediatrics. 33 (4), Aug 2002, pp. 209-214.