Recurrence of Susac syndrome (retinocochleocerebral vasculopathy) after remission of 18 years

Susac syndrome (retinocochleocerebral vasculopathy) is a syndrome of unknown pathogenesis. The triad of multifocal encephalopathy, visual loss, and hearing loss is caused by microangiopathy of the brain, retina, and cochlea. The illness tends to be monophasic, and to our knowledge, recurrence after years of remission has not been reported. We describe a 51-year-old woman with symptoms, signs, and brain magnetic resonance imaging findings consistent with recurrence of Susac syndrome 18 years after remission. Clinicians should be aware of the possibility of late recurrence of Susac syndrome when evaluating patients with a distant history of the syndrome who present with complaints referable to the brain, retina, and cochlea.     

Autoimmune inner ear disease: diagnostic and therapeutic approaches in a multidisciplinary setting

Autoimmune Inner Ear Disease (AIED) is a clinical syndrome of uncertain pathogenesis. It is associated with bilateral rapidly progressive hearing loss. The hearing loss may be associated with vestibular symptoms. Autoimmunity has been proposed as the pathogenesis of this sort of hearing loss, although the mechanism of the disease is poorly understood. It is well accepted that the endolymphatic sac is an immunocompetent organ and circulating autoantibodies against inner ear antigens have been reported, as have viral antigens in the endolymph, although the sensitivity, specificity, and roles of those antibodies in a disease process are poorly defined. We will describe the clinical aspects of the disease, the histopathology, the immunologic indicators, the types of presentation, both from the audiologic and vestibular point of view, clinical trials for treatment and the follow-up. One of our conclusions is that many of these patients respond favorably to the treatment Methotrexate.     

Involvement of apoptosis and interferon-gamma in murine toxoplasmosis

PURPOSE: A murine toxoplasmosis model has been developed that results in central nervous system (CNS) and ocular inflammation characterized by encephalitis with numerous brain tissue cysts and milder inflammation with rare tissue cysts in the eye after 4 weeks of Toxoplasma gondii infection. In this model IFN gamma and inducible nitric oxide (iNO) are protective against T. gondii infection. In this study, the role of apoptosis in the pathogenesis of toxoplasmosis was investigated. METHODS: C57BL/6 (wild-type mice), B6MRL/lpr, and B6MRL/gld (defective Fas or FasL expression, respectively) mice were infected intraperitoneally with 20 to 30 tissue cysts of the ME-49 strain of T. gondii. Mice were killed at days 0, 14, or 28 after infection. The eyes and brains were harvested for histologic, immunohistochemical, and molecular studies. Analysis included immunostaining for Fas, FasL, Bcl-2, and Bax; in situ apoptosis detection (TUNEL assay); RT-PCR amplification for IFN gamma; and measurement of ocular nitrite levels. The control mice were na?ve mice of each strain that received no inoculation or injection. RESULTS: Wild-type mice appeared to constitutively express apoptotic molecules at higher levels in the eye than in the brain. Consequently, during T. gondii infection, apoptosis was greater in the eyes than in the brain. Untreated na?ve lpr and gld mice showed no expression of Fas and FasL, respectively. After infection, a slightly higher number of tissue cysts (lpr, 11.8 +/- 2.4; gld, 10.3 +/- 3.4) were found in the brains of the mutants than in the control animals (8.8 +/- 2.9). However, no significant differences between the number of apoptotic cells, inflammatory scores, or number of tissue cysts were noted in the eyes. IFN gamma mRNA in control mice was detected at day 28 after infection, whereas in both mutants, mRNA production occurred earlier, at day 14. Ocular nitrite levels were higher in lpr and gld mice than in wild-type mice. CONCLUSIONS: No significant difference in the degree of ocular inflammation and apoptosis was detected between the wild-type and Fas or FasL mutant mice. However, there was an earlier and subjectively greater expression of IFN gamma in the brain and eye and a higher level of nitrite in the ocular tissue of mutant strains than in the wild type. Multiple factors are likely to be involved in the pathogenesis of ocular toxoplasmosis.     

Interobserver variation in the interpretation of abdominal radiographs

A total of 140 sets of abdominal radiographs were reviewed independently by four qualified diagnostic radiologists. The degree of interobserver agreement was determined by calculating kappa values for 19 commonly used radiographic signs and diagnoses. There was fair to excellent interobserver agreement for 11 signs and diagnoses and poor agreement for the remaining eight. The signs and diagnoses for which agreement is poor cannot be considered reliable and include particularly large bowel obstruction and nonspecific gas pattern.