Summary
A system is presented for the classification of chronic herniations of the cerebellar tonsils in the absence of space-occupying intracranial lesions, based on a survey of the literature and 13 own cases. The Arnold-Chiari malformation in adults typically involves herniation of the cerebellar tonsils instead of herniation of the vermis as is typical when it occurs in infancy. Identification of chronic tonsillar herniation with the Arnold-Chiari malformation in adults was thought to require at least one other sign of the Arnold-Chiari complex, e. g. a medullary deformity. Cases for which chronic herniation and sclerosis of the cerebellar tonsils present as the only nervous lesion are classified as “chronic tonsillar herniation”. Such cases may manifest with neurological symptoms during adult life, or they may be found incidentally at autopsy. Attention is drawn to the occurence of chronic tonsillar herniation in 7 infants and children where it apparently represent a cause of sudden unexpected death. The overlap between chronic tonsillar herniation and the Arnold-Chiari malformation of adults is discussed in regard to the frequence of associated osseous anomalies, hydrocephalus and syringomyelia.
Similar content being viewed by others
References
Adams, R. D., Schatzki, R., Scoville, W. B.: The Arnold-Chiari malformation. New Engl. J. Med.225, 125–135 (1941)
Alvord, E. C., Jr.: The pathology of hydrocephalus. In: Disorders of developing nervous system, pp. 343–412. Springfield, Ill: Ch. C. Thomas 1961
Appleby, A., et al.: The diagnosis and management of the Chiari anomalies in adult life. Brain91, 131–139 (1968)
Aring, C. D.: Cerebellar syndrome in adult with malformation of cerebellum and brain stem (Arnold-Chiari malformation) with note occurrence of “torpedos” in cerebellum. J. Neurol. Psychiat.1, 100–109 (1938)
Arnold, J.: Myelocyste, Transposition von Gewebskeimen und Sympodie. Zieglers Beitr. path. Anat.16, 1–28 (1894)
Banerji, N. K. Millar, J. H. D.: Chiari malformation presenting in adult life. Its relationship to syringomyelia. Brain97, 157–168 (1974)
Bedi, S. S.: Arnold-Chiari malformation presenting as syringomyelia. Ann. phys. Med.10, 16–17 (1969)
Bucy, P. C., Lichtenstein, B. W.: Arnold-Chiari deformity in an adult without obvious cause. J. Neurosurg.2, 245–250 (1945)
Carmel, P. W., Markesbery, W. R.: Arnold-Chiari malformation in an elderly woman. Arch. Neurol. (Chic.)21, 258–262 (1969)
Chaouat, Y., Kanovitch, B., Faures, B., Aubin, M., Aboulker, J.: La Malformation de Chiari. A propos de 7 observations decouvertes dans un service de Rhumatologie. Rev. Rhum.39, 115–122 (1972)
Chiari, H.: Über Veränderungen des Kleinhirns, der Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Großhirns. Dtsch. med. Wschr.17, 1172–1175 (1891)
Chiari, H.: Über Veränderungen des Kleinhirns, Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Großhirns. Denkschr. Kaiserl. Akad. Wiss. Math. Naturw. Classe63, 71–115 (1896)
Cleland: Contributions to the study of spina bifida, encephalocele and anencephalus. J. Anat. Physiol.17, 257–292 (1883)
Cogan, D. G., Lawrence, J. B.: Platybasia and the Arnold-Chiari malformation. Arch. Ophthal.52, 13–29 (1954)
Colclough, J. A.: Stimulation of herniated cervical disc by Arnold-Chiari deformity, presentation of 2 cases. Surgery28, 874–880 (1950)
Collier, J.: The false localising signs of intracranial tumour. Brain27, 490–508 (1904)
Cossa, P., Duplay, M., Martin, J., Aubert, D., Hemmi, M.: Malformation d'Arnold-Chiari. Impression basilaire, invagination basilaire; platybasie; convexobasie; aplasie de lame basilaire; à propos de deux observations. Rev. neurol.85, 392–398 (1951)
Cushing, H.: Some experimental and clinical observations concerning states of increased intracranial tension. Amer. J. med. Sci.124, 375–400 (1902)
Custis, D. L., Verbrugghen, A.: Basilar impression resembling cerebellar tumor. Arch. Neurol. Psychiat. (Chic.)52, 412–415 (1944)
Earle, K. M., Baldwin, M., Penfield, W.: Incisural sclerosis and temporal lobe seizures produced by hippocampal herniation at birth. Arch. Neurol. Psychiat. (Chic.)69, 27–42 (1953)
Epstein, B. S.: Pantopaque myelography in the diagnosis of the Arnold-Chiari malformation without concomitant skeletal or central nervous system defects. Amer. J. Roentgenol.59, 359–364 (1948)
Godlewski, St.: Quelques apports récents sur les anomalies congenitales de la charnière cervico-occipitale. Sem. Hôp. Paris48, 1635–1679 (1972)
Golding, D. J.: Cerebellar ectopia presenting in adult life. Brit. med. J.1969 II, 119
Gordon, D. S.: Neurological syndromes associated with craniovertebral anomalies. Proc. roy. Soc. Med.62, 725–727 (1969)
Gustafson, W. A., Oldenberg, E.: Neurological significance of platybasia. Arch. Neurol. Psychiat. (Chic.)44, 1184–1198 (1940)
Hankinson, J.: Syringomyelia and the surgeon. In: Modern trends in neurology ed. D. Williams Vol. 5, pp. 127–148. New York: Appleton-Century-Crofts (1970)
Hurteau, E. F.: Arnold-Chiari malformation. J. Neurosurg.7, 282–284 (1950)
Labsan, H. F., Proctor, L. D.: Syringomyelia associated with Arnold-Chiari malformation. Henry Ford Hosp. Bull.8, 157–162 (1960)
Larson, S. J., Sances, A., Baker, J. B., Reigel, D. H.: Herniated cerebellar tonsils and cough syncope. J. Neurosurg.40, 524–528 (1974)
List, C. F.: Neurological syndromes accompanying developmental anomalies of occipital bone, atlas, and axis. Arch. Neurol. (Chic.)45, 577–616 (1941)
Malis, L. I., Cohen, I., Gross, S. W.: Arnold-Chiari malformation. Arch. Surg.63, 783–798 (1951)
McConnell, A. A., Parker, H. L.: Deformity of hind-brain associated with internal hydrocephalus; its relation to Arnold-Chiari malformation. Brain61, 415–429 (1938)
McHenry, L. C., Jr., Ewald, R. A., Talbert, W. M., Jr., Elwell, R. H.: Bilateral cortical cerebellar sclerosis. Residuum of cerebellar swelling or displacement early in life. Arch Path.78, 665–672 (1964)
Meyer, A.: Herniation of the brain. Arch. Neurol. Psychiat. (Chic.)4, 387–400 (1920)
Newton, E. J.: Syringomyelia as a manifestation of defective fourth ventricular drainage. Ann. roy. Coll. Surg.44, 194–213 (1969)
Norton, T., Pawl, R. P.: The Arnold-Chiari deformity in elderly patients: Diagnosis and treatment. Neurochirurgia15, 153–159 (1972)
Ogryzlo, M. H.: Arnold-Chiari malformation. Arch. Neurol. Psychiat. (Chic.)48, 30–46 (1942)
Pryse-Davies, J., Beard, R. W.: A necrospy study of brain swelling in the newborn with special reference to cerebellar herniation. J. Path.109, 51–74 (1973)
Ray, B.: Platybasia with involvement of the central nervous system. Ann. Surg.116, 231–250 (1942)
Schmidt, H.: Die Einklemmung der Kleinhirntonsillen bei Hirnatrophie und ihre Bedeutung. Wien. Z. Nervenheilk.30, 289–290 (1972)
Schuster, P.: The Arnold-Chiari malformation. Dis. nerv. Syst.15, 264–267 (1954)
Sirois, J., Belanger, C.: Malformation d'Arnold-Chiari. Laval méd.18, 1033–1041 (1953)
Smith, J., Ridley, A.: Cerebellar ectopia presenting in adult life. Brit. med. J.1969I, 353–355
Spillane, J. D., Pallis, C., Jones, A. M.: Developmental abnormalities in the region of the foramen magnum. Brain80, 11–48 (1957)
Sunder-Plassmann, M., Salah, S., Jellinger, K.: Late death in cases of internal hydrocephalus treated during early childhood. Neuropädiatrie6, 162–175 (1975)
Swanson, H. S., Fincher, E. F.: Arnold-Chiari deformity without bony anomalies. J. Neurosurg.6, 314–319 (1949)
Teng, P., Papatheodorou, C.: Arnold-Chiari malformation with normal spine and cranium. Arch. Neurol. (Chic.)12, 622–624 (1965)
Tobin, W., Sencer, W.: Neurological management of congenital malformations of the brain stem and upper cervical spinal cord. J. Mt Sinai Hosp.30, 503–506 (1963)
Trotot, M. R.: Anomalies cranio-vertebrales avec signes bulbaires, étude clinique et radiologique. Rev. neurol.85, 523–527 (1951)
Walsh, M. N., Camp, J. D., McCraig, W. K.: Basilar invagination of the skull (so-called platybasia): report of a case with operation. Proc. Mayo Clin.16, 449–452 (1941)
Wertheimer, P., Allegre, G.: Trois observations de malformation d'Arnold-Chiari. Rev. neurol.82, 186–190 (1950)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Friede, R.L., Roessmann, U. Chronic tonsillar herniation. Acta Neuropathol 34, 219–235 (1976). https://doi.org/10.1007/BF00688677
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00688677