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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1988 Sep;51(9):1126–1133. doi: 10.1136/jnnp.51.9.1126

Granulomatous angiitis of the central nervous system: protean manifestations and response to treatment.

E H Koo 1, E W Massey 1
PMCID: PMC1033014  PMID: 2906362

Abstract

Granulomatous angiitis is an uncommon necrotising vasculitis of unknown cause restricted to vessels of the central nervous system. Five tissue-proven cases emphasise the protean manifestations of this disease and the difficulties encountered in reaching a diagnosis. One patient presented with a temporoparietal mass, the second, a progressive dementia, the third suggested herpes simplex encephalitis, the fourth mimicked multi-infarct state; and the fifth presented with a cerebellar mass lesion. In four cases with CSF examination, protein was elevated (81-193 gm/l) and three patients had mononuclear pleocytosis (12-800 WBC/mm3). Cerebral arteriogram suggested vasculitis in only one of four cases. Diagnosis was made by brain biopsy in three cases and all three were treated successfully. The diagnosis in the two other cases was made at postmortem examination.

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Selected References

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  1. Arthur G., Margolis G. Mycoplasma-like structures in granulomatous angiitis of the central nervous system. Case reports with light and electron microscopic studies. Arch Pathol Lab Med. 1977 Jul;101(7):382–387. [PubMed] [Google Scholar]
  2. BUDZILOVICH G. N., FEIGIN I., SIEGEL H. GRANULOMATOUS ANGIITIS OF THE NERVOUS SYSTEM. Arch Pathol. 1963 Sep;76:250–256. [PubMed] [Google Scholar]
  3. Beresford H. R., Hyman R. A., Sharer L. Self-limited granulomatous angiitis of the cerebellum. Ann Neurol. 1979 May;5(5):490–492. doi: 10.1002/ana.410050516. [DOI] [PubMed] [Google Scholar]
  4. Burger P. C., Burch J. G., Vogel F. S. Granulomatous angiitis. An unusual etiology of stroke. Stroke. 1977 Jan-Feb;8(1):29–35. doi: 10.1161/01.str.8.1.29. [DOI] [PubMed] [Google Scholar]
  5. CRAVIOTO H., FEIGIN I. Noninfectious granulomatous angiitis with a predilection for the nervous system. Neurology. 1959 Sep;9:599–609. doi: 10.1212/wnl.9.9.599. [DOI] [PubMed] [Google Scholar]
  6. Craven R. S., French J. K. Isolated angiitis of the central nervous system. Ann Neurol. 1985 Aug;18(2):263–265. doi: 10.1002/ana.410180216. [DOI] [PubMed] [Google Scholar]
  7. Cupps T. R., Moore P. M., Fauci A. S. Isolated angiitis of the central nervous system. Prospective diagnostic and therapeutic experience. Am J Med. 1983 Jan;74(1):97–105. doi: 10.1016/0002-9343(83)91125-7. [DOI] [PubMed] [Google Scholar]
  8. De Reuck J., Crevits L., Sieben G., De Coster W., vander Eecken H. Granulomatous angiitis of the nervous system: a clinicopathological study of one case. J Neurol. 1982;227(1):49–53. doi: 10.1007/BF00313547. [DOI] [PubMed] [Google Scholar]
  9. Faer M. J., Mead J. H., Lynch R. D. Cerebral granulomatous angiitis: case report and literature review. AJR Am J Roentgenol. 1977 Sep;129(3):463–467. doi: 10.2214/ajr.129.3.463. [DOI] [PubMed] [Google Scholar]
  10. Greco F. A., Kolins J., Rajjoub R. K., Brereton H. D. Hodgkin's disease and granulomatous angiitis of the central nervous system. Cancer. 1976 Nov;38(5):2027–2032. doi: 10.1002/1097-0142(197611)38:5<2027::aid-cncr2820380527>3.0.co;2-h. [DOI] [PubMed] [Google Scholar]
  11. Griffin J., Price D. L., Davis L., McKhann G. M. Granulomatous angiitis of the central nervous system with aneurysms on multiple cerebral arteries. Trans Am Neurol Assoc. 1973;98:145–148. [PubMed] [Google Scholar]
  12. Harrison P. E., Jr Granulomatous angitis of the central nervous system. Case report and review. J Neurol Sci. 1976 Oct;29(2-4):335–341. doi: 10.1016/0022-510x(76)90182-9. [DOI] [PubMed] [Google Scholar]
  13. Hilt D. C., Buchholz D., Krumholz A., Weiss H., Wolinsky J. S. Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: diagnosis and management approaches. Ann Neurol. 1983 Nov;14(5):543–553. doi: 10.1002/ana.410140509. [DOI] [PubMed] [Google Scholar]
  14. Hughes J. T., Brownell B. Granulomatous giant-celled angiitis of the central nervous system. Neurology. 1966 Mar;16(3):293–298. doi: 10.1212/wnl.16.3.293. [DOI] [PubMed] [Google Scholar]
  15. Jellinger K. Giant cell granulomatous angiitis of the central nervous system. J Neurol. 1977 Jun 13;215(3):175–190. doi: 10.1007/BF00312476. [DOI] [PubMed] [Google Scholar]
  16. Kjeldsen M. H., Reske-Nielsen E. Pathological changes of the central nervous system in giant-cell arteritis. Acta Ophthalmol (Copenh) 1968;46(1):49–56. doi: 10.1111/j.1755-3768.1968.tb02494.x. [DOI] [PubMed] [Google Scholar]
  17. Kolodny E. H., Rebeiz J. J., Caviness V. S., Jr, Richardson E. P., Jr Granulomatous angiitis of the central nervous system. Arch Neurol. 1968 Nov;19(5):510–524. doi: 10.1001/archneur.1968.00480050080008. [DOI] [PubMed] [Google Scholar]
  18. Kristoferitsch W., Jellinger K., Böck F. Cerebral granulomatous angiitis with atypical features. J Neurol. 1984;231(1):38–42. doi: 10.1007/BF00313650. [DOI] [PubMed] [Google Scholar]
  19. Linnemann C. C., Jr, Alvira M. M. Pathogenesis of varicella-zoster angiitis in the CNS. Arch Neurol. 1980 Apr;37(4):239–240. doi: 10.1001/archneur.1980.00500530077013. [DOI] [PubMed] [Google Scholar]
  20. McCORMICK H. M., NEUBUERGER K. T. Giant-cell arteritis involving small meningeal and intracerebral vessels. J Neuropathol Exp Neurol. 1958 Jul;17(3):471–478. doi: 10.1097/00005072-195807000-00007. [DOI] [PubMed] [Google Scholar]
  21. Moore P. M., Cupps T. R. Neurological complications of vasculitis. Ann Neurol. 1983 Aug;14(2):155–167. doi: 10.1002/ana.410140202. [DOI] [PubMed] [Google Scholar]
  22. Nurick S., Blackwood W., Mair W. G. Giant cell granulomatous angiitis of the central nervous system. Brain. 1972;95(1):133–142. doi: 10.1093/brain/95.1.133. [DOI] [PubMed] [Google Scholar]
  23. REWCASTLE N. B., TOM M. I. Non-infectious granulomatous angitis of the nervous system associated with Hodgkin's disease. J Neurol Neurosurg Psychiatry. 1962 Feb;25:51–58. doi: 10.1136/jnnp.25.1.51. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Rajjoub R. K., Wood J. H., Ommaya A. K. Granulomatous angiitis of the brain: a successfully treated case. Neurology. 1977 Jun;27(6):588–591. doi: 10.1212/wnl.27.6.588. [DOI] [PubMed] [Google Scholar]
  25. Rawlinson D. G., Braun C. W. Granulomatous angiitis of the nervous system first seen as relapsing myelopathy. Arch Neurol. 1981 Feb;38(2):129–131. doi: 10.1001/archneur.1981.00510020087015. [DOI] [PubMed] [Google Scholar]
  26. Reik L., Jr, Grunnet M. L., Spencer R. P., Donaldson J. O. Granulomatous angiitis presenting as chronic meningitis and ventriculitis. Neurology. 1983 Dec;33(12):1609–1612. doi: 10.1212/wnl.33.12.1609. [DOI] [PubMed] [Google Scholar]
  27. Reyes M. G., Fresco R., Chokroverty S., Salud E. Q. Viruslike particles in granulomatous angiitis of the central nervous system. Neurology. 1976 Aug;26(8):797–799. doi: 10.1212/wnl.26.8.797. [DOI] [PubMed] [Google Scholar]
  28. Russi E., Aebi M., Kraus-Ruppert R., Mumenthaler M. Intracranial giant cell arteritis. J Neurol. 1979 Oct;221(4):219–224. doi: 10.1007/BF00314638. [DOI] [PubMed] [Google Scholar]
  29. Sandhu R., Alexander W. S., Hornabrook R. W., Stehbens W. E. Granulomatous angiitis of the CNS. Arch Neurol. 1979 Jul;36(7):433–435. doi: 10.1001/archneur.1979.00500430063009. [DOI] [PubMed] [Google Scholar]
  30. Serdaru M., Chiras J., Cujas M., Lhermitte F. Isolated benign cerebral vasculitis or migrainous vasospasm? J Neurol Neurosurg Psychiatry. 1984 Jan;47(1):73–76. doi: 10.1136/jnnp.47.1.73. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Valavanis A., Friede R., Schubiger O., Hayek J. Cerebral granulomatous angiitis simulating brain tumor. J Comput Assist Tomogr. 1979 Aug;3(4):536–538. doi: 10.1097/00004728-197908000-00019. [DOI] [PubMed] [Google Scholar]
  32. Vincent F. M. Granulomatous angiitis. N Engl J Med. 1977 Feb 24;296(8):452–452. doi: 10.1056/NEJM197702242960815. [DOI] [PubMed] [Google Scholar]
  33. Wilkinson I. M., Russell R. W. Arteries of the head and neck in giant cell arteritis. A pathological study to show the pattern of arterial involvement. Arch Neurol. 1972 Nov;27(5):378–391. doi: 10.1001/archneur.1972.00490170010003. [DOI] [PubMed] [Google Scholar]

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