Hostname: page-component-848d4c4894-x5gtn Total loading time: 0 Render date: 2024-05-22T16:00:49.004Z Has data issue: false hasContentIssue false
  • English
  • Français

A Syndrome of Brain, Inner Ear and Retinal Microangiopathy

Published online by Cambridge University Press:  18 September 2015

D.J. MacFadyen ,
R.J. Schneider  and
I.A. Chisholm
D.J. MacFadyen*
Affiliation:
Departments of Clinical Neurological Sciences, University Hospital, Saskatoon
R.J. Schneider
Affiliation:
Departments of Ophthalmology, University Hospital, Saskatoon
I.A. Chisholm
Affiliation:
Departments of Ophthalmology, University Hospital, Saskatoon
*
Department of Clinical Neurological Science, University Hospital, Saskatoon, Saskatchewan, Canada S7N 0X0
Rights & Permissions [Opens in a new window]

Abstract:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

We report the seventh case of a self-limiting syndrome of brain, inner ear and retinal microangiopathy which affects adult women producing mild to moderate mental status and personality changes, mild pyramidal and cerebellar dysfunction, patchy and asymmetrical visual loss secondary to non-inflammatory retinal infarctions, and asymmetrical neurosensory hearing loss. There is no clinical or laboratory evidence of involvement beyond the brain, the eyes and the ears. Magnetic resonance imaging or brain biopsy in previously reported cases best outline the brain pathology: multiple microinfarcts. All cases have been treated with steroids or cyclophosphamide. The etiology is unknown but may be related to systemic lupus erythematosus.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 14 , Issue 3 , August 1987 , pp. 315 - 318
Copyright
Copyright © Canadian Neurological Sciences Federation 1987

References

REFERENCES

1.Susac, JO, Hardman, JM, Selhorst, JB. Microangiopathy of the brain and retina. Neurology 1979;29: 313–316.CrossRefGoogle ScholarPubMed
2.Coppeto, JR, Currie, JN, Monteiro, MLR, et al. A syndrome of arterial-occlusive retinopathy and encephalopathy.Am J Ophthalmol 1984; 98: 189–202.CrossRefGoogle ScholarPubMed
3.Monteiro, MLR, Swanson, RA, Coppeto, JR, et al. A microangiopathic syndrome of encephalopathy, hearing loss and retinal arteriolar occlusions. Neurology 1985; 35: 1113–1121.CrossRefGoogle ScholarPubMed
4.Verriest, G, VanLaethem, J. Uvijls A. A new assessment of the normal ranges of the Farnsworth Munsell 100-Hue test scores. Am J Ophthalmol 1982; 93: 635–642.CrossRefGoogle ScholarPubMed
5.Pfaffenbach, DD, Hollenhorst, RW. Microangiopathy of the retinal arterioles. JAMA 1973; 225: 480–483.CrossRefGoogle ScholarPubMed
6.Weidauer, H, Tenner, A. Bilateral acute hearing loss with a transient occlusion of the retinal artery on both sides. Z Larngol Rhinol Otol 1973; 52: 121–128.Google ScholarPubMed
7.Tan, EM, Cohen, AS, Fries, JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis … Rheumatism 1982; 25: 1271–1277.CrossRefGoogle ScholarPubMed
8.Feinstein, DI. Lupus anticoagulant, thrombosis and fetal loss. NEJM 1985, 313: 1348–1350.CrossRefGoogle ScholarPubMed