Progressive Multifocal Leukoencephalopathy

Noncompaction in a HIV-positive with cortical blindness as initial manifestation of progressive multifocal leucencephalopathy

“…PML is associated with HIV-infection in up to 86% of the cases [2], but occasionally occurs together with transplanted patients [3,4], or in patients under an immune-modulating therapy with natalizumab or rituximab [4,5]. Though PML has been reported to manifest with visual impairment [1,3,[6][7][8][9][10][11] or as bilateral visual loss as the presenting manifestation in single patients [3,8], it has not been described in association with left ventricular-hypertrabeculation (LVHT)/noncompaction [12].…”

“…PML is a demyelinating disease of the CNS caused by an opportunistic infection with the JC papovavirus in patients with impaired cellular immunity [13]. The multiplying virus destroys oligodendrocytes, resulting in extensive white matter demyelination [6]. Though initially described in patients with hematological malignancies, it has been found meanwhile predominantly in association with HIV-infection, and patients under immunosuppressive or immune-modulating therapy [13].…”

“…Visual impairment as a manifestation of PML was first described in a patient with chronic lymphatic leukemia, who had developed bilateral blurring from occipital PML [6]. In another patient, who lastly ended up with cortical blindness, blurred vision and hemianopsia were the initial manifestations [7].…”

Isolated cortical blindness without simultaneous neurological involvement in progressive multifocal leukoencephalopathy in a patient with human immune deficiency virus infection

“…Most common initial manifestations are hemiparesis, mental deterioration, dysarthria, and dysphasia [2,5]. Although 20% to 30% of patients with HIV/AIDS can present with associated ocular manifestations at the time of diagnosis of PML [9], ocular involvement as the initial manifestation is rarely seen [3,10]. Patients presenting as hemianopic field defects and retrochiasmal field defects have been reported [5-9].…”

“…It is caused by John Cunningham virus, a human polyomavirus (DNA virus) belonging to Papovaviridae group [1]. PML was originally described in patients with chronic diseases associated with compromised immune response or with immunosuppressive drugs [2,3]. However, the disease has been most frequently documented in patients with acquired immunodeficiency syndrome (AIDS) [4,5].…”