Abstract
A 50-year-old right-handed woman with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS) presented with progressive impairments of the dorsal visual system and other focal cognitive functions that resulted in ultimate dementia.
A definitive diagnosis of SLE and APS was made in 1993 based on a medical history of difficult pregnancies and recurrent seizures, presence of hematological abnormalities and various autoantibodies, and results of renal and skin biopsies. By 1996, the patient developed Balint's and Gerstmann's syndromes, dressing and constructional difficulties, ideomotor and ideational apraxia, mild amnesia, word finding difficulty, left-side sensorimotor neglect and executive dysfunction, and added afterwardsle signe de la main etrangere” and dementia. Longitudinal magnetic resonance (MR) imaging of the brain revealed diffuse progressive atrophy and parietal white matter changes that extended to the frontal lobes. Single photon emission computed tomography showed diffuse progressive uptake defects that were most prominent in the borderzones. MR angiography was negative. We presume that the underlining immunological abnormalities resulted in progressive microvasculopathy and microinfarcts especially in the bilateral parieto-occipital regions including the dorsal visual system. We also suggest that the dorsal visual system may be associated with the neural fibers passing through the posterior boderzone among three major cerebral arteries and, more specifically, the hemodynamic terminal zone between cortical and deep penetrating arteries
