Confusion, dissociation and bizarre behaviour as the onset of an early Susac syndrome.

A 47-year-old woman presented an episode of confusion and disorientation. According to remarkable psychiatric records, she had been treated for major depression and obsessive-compulsive disorder; however, no other relevant background was known. After preliminary examinations, blood analysis and neurological tests were unspecific and inconclusive. Therefore, the case was treated as a possible psychiatric episode related to her previous psychiatric disorders. However, due to the atypical presentation of the case, a cerebral MRI was performed, which demonstrated multiple central lesions of the corpus callosum ('snowball lesions'), as well as several supratentorial white matter lesions. As a result of the follow-up of the case, sensorineural hearing loss and branch retinal artery were detected, which concluded in the classic triad and the confirmation of the diagnosis of a Susac syndrome.

Delirium as presentation of late-onset and relapsing Susac syndrome.

A 67-year-old patient, only known for bilateral presbycusis, presented with subacute onset of delirium. Clinical examination showed multifocal neurological deficits, all together suggesting subcortical frontal dysfunction together with cerebellar and corpus callosum involvement.Cerebral MRI demonstrated supratentorial and infratentorial subcortical and periventricular T2-hyperintense lesions with cerebellar gadolinium enhancement and multiple central lesions of the corpus callosum (snowball lesions). The diagnosis of Susac syndrome was made and the patient treated with intravenous methylprednisolone, followed by a prednisone maintenance dose over 8 weeks. After a clinical improvement, a relapse was noticed during corticosteroid tapering. The patient was again treated with intravenous methylprednisolone followed by a prednisone maintenance therapy with simultaneous introduction of mycophenolate mofetil acid and one cycle of intravenous rituximab. The patient recovered rapidly. At 11-month follow-up, only mild executive dysfunction and persistent right postural tremor was noted, MRI showed partial regression of subcortical and juxtacortical lesions.

Susac's syndrome: a case of simultaneous development of all three components of the triad.

Susac's syndrome is characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. We here report a case of 46 year old woman with a sudden diminution in visual acuity at the left eye, headache and decreased hearing at the right ear with mild confusion considered as Susac's syndrome with complete triad. Although it's a relatively rare entity, it should be kept in mind in differential diagnosis of the neurological disorders, especially in women in 20- to 40-year of age presenting with acute hearing loss.

Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.

Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and anecdotal evidence has suggested a therapeutic role for monoclonal antibodies (Rennebohm et al. 2008, Lee and Amezcua 2009) [3,4]. We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab.

Letter: It is Dermatosis neglecta.

The case presented in the article "Severe retention hyperkeratosis occurring with Susac syndrome" in October 2010 of DOJ (16:10) is very dramatic. Although it was implied in the article, the authors did not state that this is actually Dermatosis neglecta, the same entity reported by Poskitt et al 15 years ago. The description of the case in the article, histopathology, mechanism of pathogenesis, and treatment are consistent with Dermatosis neglecta, only with a different name.