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Keyhole Aqueduct Syndrome

Keyhole Aqueduct Syndrome Abstract • Communicating syringes confined to the brain stem are extraordinarily rare. Two patients, presenting with signs and symptoms of cerebellar dysfunction, later developed evidence of brain-stem disease with dysarthria, nystagmus, deafness, and internuclear ophthalmoplegia. The condition of both patients had been diagnosed clinically as multiple sclerosis, but at autopsy they had a striking keyhole-shaped syrinx in the midbrain and upper pons, which communicated with the aqueduct and fourth ventricle without associated syringomyelia. In addition, both patients had marked atrophy and gliosis of the cerebellum, one with extension of the syrinx into cerebellar folia. The unique character of these lesions coupled with the similarity of the clinical features of the cases prompted us to name this disorder—"keyhole aqueduct syndrome." References 1. Banerji NK, Millar JHD: Chiari malformation presenting in adult life: Its relationship to syringomyelia . Brain 1974;97:157-168.Crossref 2. Barnett HJM, Jousse AT: Case records of author's series of post-traumatic syringomyelia , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 165-173. 3. Barnett HJM, Rewcastle NB: Syringomyelia and tumours of the nervous system , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 261-301. 4. Savoiardo M: Syringomyelia associated with postmeningitic spinal arachnoiditis . Neurology 1976;26:551-554.Crossref 5. Escobar A, Vega J: Syringomyelia and syringobulbia secondary to arachnoiditis and fourth ventricle blockage due to cysticercosis: A case report . Acta Neuropathol 1981; (suppl 7) , pp 389-391. 6. Gimenez-Roldan A, Esteban A, Benito C: Communicating syringomyelia following cured tuberculous meningitis . J Neurol Sci 1974;23:185-197.Crossref 7. Barnett HJM: Syringomyelia associated with spinal arachnoiditis , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 220-244. 8. Foster JB, Hudgson P: The pathology of communicating syringomyelia , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 79-103. 9. Rivera LL, Arroyo LS: Siringomielia y siringobulbia: Comunicación de un caso con cavitación talamica . Rev Med Hospital General 1964;24:363-370. 10. Berry RG, Chambers RA, Lublin FD: Syringoencephalomyelia (syringocephalus) . J Neuropathol Exp Neurol 1981;40:633-644.Crossref 11. Hardy RC, Stevenson LD: Syringomesencephalia: Report of a case with signs of Parkinson's disease having a syrinx of the substantia nigra . J Neuropathol Exp Neurol 1957;16:365-369.Crossref 12. Samples JR, Howard FM Jr, Okazaki H: Syringomesencephalia: Report of a case . Arch Neurol 1983;40:757-759.Crossref 13. Williams B: On the pathogenesis of syringomyelia: A review . J R Soc Med 1980;73:798-806. 14. Gardner WB: Hydrodynamic mechanism of syringomyelia: Its relationship to myelocele . J Neurol Neurosurg Psychiatry 1965;28:247-259.Crossref 15. Newman PK, Terenty TR, Foster JB: Some observations on the pathogenesis of syringomyelia . J Neurol Neurosurg Psychiatry 1981;44:964-969.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1986 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1986.00520090056017
Publisher site
See Article on Publisher Site

Abstract

Abstract • Communicating syringes confined to the brain stem are extraordinarily rare. Two patients, presenting with signs and symptoms of cerebellar dysfunction, later developed evidence of brain-stem disease with dysarthria, nystagmus, deafness, and internuclear ophthalmoplegia. The condition of both patients had been diagnosed clinically as multiple sclerosis, but at autopsy they had a striking keyhole-shaped syrinx in the midbrain and upper pons, which communicated with the aqueduct and fourth ventricle without associated syringomyelia. In addition, both patients had marked atrophy and gliosis of the cerebellum, one with extension of the syrinx into cerebellar folia. The unique character of these lesions coupled with the similarity of the clinical features of the cases prompted us to name this disorder—"keyhole aqueduct syndrome." References 1. Banerji NK, Millar JHD: Chiari malformation presenting in adult life: Its relationship to syringomyelia . Brain 1974;97:157-168.Crossref 2. Barnett HJM, Jousse AT: Case records of author's series of post-traumatic syringomyelia , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 165-173. 3. Barnett HJM, Rewcastle NB: Syringomyelia and tumours of the nervous system , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 261-301. 4. Savoiardo M: Syringomyelia associated with postmeningitic spinal arachnoiditis . Neurology 1976;26:551-554.Crossref 5. Escobar A, Vega J: Syringomyelia and syringobulbia secondary to arachnoiditis and fourth ventricle blockage due to cysticercosis: A case report . Acta Neuropathol 1981; (suppl 7) , pp 389-391. 6. Gimenez-Roldan A, Esteban A, Benito C: Communicating syringomyelia following cured tuberculous meningitis . J Neurol Sci 1974;23:185-197.Crossref 7. Barnett HJM: Syringomyelia associated with spinal arachnoiditis , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 220-244. 8. Foster JB, Hudgson P: The pathology of communicating syringomyelia , in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia . Philadelphia, WB Saunders Co, 1973, pp 79-103. 9. Rivera LL, Arroyo LS: Siringomielia y siringobulbia: Comunicación de un caso con cavitación talamica . Rev Med Hospital General 1964;24:363-370. 10. Berry RG, Chambers RA, Lublin FD: Syringoencephalomyelia (syringocephalus) . J Neuropathol Exp Neurol 1981;40:633-644.Crossref 11. Hardy RC, Stevenson LD: Syringomesencephalia: Report of a case with signs of Parkinson's disease having a syrinx of the substantia nigra . J Neuropathol Exp Neurol 1957;16:365-369.Crossref 12. Samples JR, Howard FM Jr, Okazaki H: Syringomesencephalia: Report of a case . Arch Neurol 1983;40:757-759.Crossref 13. Williams B: On the pathogenesis of syringomyelia: A review . J R Soc Med 1980;73:798-806. 14. Gardner WB: Hydrodynamic mechanism of syringomyelia: Its relationship to myelocele . J Neurol Neurosurg Psychiatry 1965;28:247-259.Crossref 15. Newman PK, Terenty TR, Foster JB: Some observations on the pathogenesis of syringomyelia . J Neurol Neurosurg Psychiatry 1981;44:964-969.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1986

References