ABSTRACT: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. RESUME: Micro-angiopathies localisees au cerveau, a Poreille interne et a la retine. Nous rapportons le septieme cas d'un syndrome comportant des micro-angiopathies a localisation bien circonscrite, soit au niveau du cerveau, de I'oreille interne et de la retine. Ce syndrome atteignant les femmes d'age adulte, provoque une alteration de legere a mod6r6e de l'6tat mental, des modifications de la personnalite, une legere dysfonction pyramidale et cer£belleuse, une perte de vision en plaques disseminees et asymetrique qui est secondaire a des infarcissements rdtiniens non-inflammatoires et une surdite neurosensorielle asymetrique. Aucune atteinte autre qu'au cerveau, aux yeux et aux oreilles ne peut etre mise en evidence par la clinique ou les examens de laboratoire. L'imagerie par resonance magnetique ou la biopsie cerebrale sont les deux examens qui demontrent le mieux la pathologie cerebrale sousjacente a cette entite, soit les nombreux micro-infarctus. Tous les cas ont et6 traites au moyen de steYoi'des ou de cyclophosphamide. L'etiologie de ce syndrome est inconnue, mais elle pourrait etre reliee au lupus 6rythemateux aigu diss6mine\Can. J. Neurol. Sci. 1987; 14:315-318 To date six patients, all young women, have been described with a remarkably similar and distinctive syndrome of retinal arteriolar infarction, neurosensory hearing loss especially in the lower frequencies (presumably due to infarction of the cochlear apex), and small multifocal central nervous system white and grey matter infarcts. The course of the disease seems to be biphasic or multiphasic with a duration of several months followed by partial recovery and a lengthy remission. The first two cases were described in 1979 by Susac et al. A 31 year old pregnant woman (para III, gravida IV) developed intermittent numbness of all four extremities in March 1981 followed by slurring of speech. In late April she noted a persistent "blind spot" in the left eye. Her corrected visual acuity was 20/20. She had an exophoria on near vision and an area of left retinal infarction just superior to the macula within the area of supply of a cilioretinal artery. Goldman perimetry revealed two dense scotomas both at about 20° of nasal eccentricity -one above and one below the horizontal meridian. One week later she developed ho...

No abstract

No abstract