This paper is only available as a PDF. To read, Please Download here.
Abstract
A case of human rhombencephalosynapsis diagnosed in vivo by magnetic resonance imaging
(MRI) is presented that demonstrates fusion of the dentate nuclei and agenesis of
the cerebellar vermis. To our knowledge, this condition in its isolated form has been
previously diagnosed only at necropsy. The developmental features of the cerebellum
are discussed and correlation with cerebellar function is also presented. Recognition
of this anomaly in the living patient may be useful in further understanding the clinical
significance of segmentation of the rhombencephalon.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Clinical ImagingAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Two cases of agenesis of the vermis of cerebellum, with fusion of the dentate nuclei and cerebellar hemispheres.Acta Neuropathol (Berl). 1987; 74: 278-280
- Ein Kleinhirn ohne Wurm.Arb Neurol Inst Wien Univ. 1916; 21: 124-136
- Etudes sur les dyraphies cranio-encephaliques. II. Angenesie du vermis cerebelleux. Dysraphie rhombencephalique mediane (rhomboschizis).Monatsschrift fur Psychiatrie and Neurologie. 1955; 129: 321-344
- Die Rhombenkephalosynapsis, eine systemisierte Kleinhirnfehlbidung.Arch Psychiatr Nervenkr. 1959; 199: 537-552
- Rhombencephalosnaps is eine seltene Dysraphieform.Zentralbl Allg Pathol Pathol Anat. 1978; 122: 577
- Rhombenkephalosynapsis: a Viennese malformation: Dev Med Child Neurol. 1982; 24: 178-182
- Agenesis of the vermis with fusion of the cerebellar hemispheres, septo-optic dysplasia and associated anomalies. Report of a case.Acta Neuropathol (Berl). 1982; 56: 161-166
- Familial agenesis of the cerebellar vermis. A syndrome of episodic hyperpnea, abnormal eye movements, ataxis, and retardation.Neurology. 1969; 19: 813-825
- Uncommon syndromes of cerebellar vermis aplasia. I: Joubert Syndrome.Dev Med Child Neurol. 1978; 20: 758-763
- Uncommon syndromes of cerebellar vermis aplasia. II: tecto-cerebellar dysraphia with occipital encephalocele.Dev Med Child Neurol. 1978; 20: 764-772
- Congenital fusion of the thalami (atresia of the third ventricle) and associated abnormalities in a 6-month-old infant.ACTH Neuropathol (Berl). 1969; 13: 97-104
- The early development of the human brain.Carnegie Inst. Wash. Publ. 621, Contribs Embryol. 1962; 253: 15-32
- Angel dust: possible effects on the fetus.Pediatrics. 1980; 65: 18-20
- The cerebellum in sagittal plane-anatomic-MR correlation: 1. The vermis.AJR. 1989; 153: 829-835
- The cerebellum in sagittal plane-Anatomic-MR Correlation: 2. The cerebellar Hemispheres.AJR. 1989; 153: 837-846
- The cerebellum: 3. Anatomic-MR Correlation in the Coronal Plane.AJR. 1990; 154: 593-602
Article info
Identification
Copyright
© 1991 Published by Elsevier Inc.