Visual symptoms after lung transplantation: a case of progressive multifocal leukoencephalopathy

Abstract

After solid organ transplantation, signs and symptoms of the central nervous system may present a diagnostic challenge. A 43-year-old patient developed a decrease in vision 15 months after bilateral lung transplantation. The initial diagnosis was a left posterior cataract, but left eye cataract extraction did not improve his vision. Seizures led to investigation of a broader differential diagnosis (cyclosporine intoxication, post-transplant lymphoproliferative disorder, infectious disease, chronic lymphatic leukemia). The clinical diagnosis of progressive multifocal leukoencephalopathy (PML) was confirmed by demonstration of JC virus in the cerebrospinal fluid and by autopsy findings. Modulation of the immunosuppressive regimen was unsuccessful. This case illustrates that decreased vision in immunocompromised patients may be the first manifestation of PML.

Viral infection and reactivation in autoimmune disease

“…JCV is neurotropic, and reactivation can lead to progressive multifocal leukoencephalopathy (PML), a fatal demyelinating condition. The occurrence of PML is restricted to patients with primary or secondary immunosuppression; it has been reported, for example, in recipients of heart, kidney, or liver transplants (61)(62)(63)(64).…”

Molecular and clinical perspectives of polyomaviruses: Emerging evidence of importance in non-kidney transplant populations

“…Progressive multifocal leukoencephalopathy (PML) is a disease of the central nervous system characterized by multiple foci of demyelination caused by lytic JCV infection of oligodendrocytes. PML has been reported among heart, kidney, and liver transplant recipients, [15][16][17][18] but its incidence in these patient groups is not known. In addition, JCV has also been associated with polyomavirus-associated nephropathy (PVAN) 8,14 and shown as a coagent with BKV in some cases of PVAN in renal transplant patients.…”

“…Cases have occurred among heart, lung, liver, and kidney transplant groups. 2,[15][16][17][18] In the majority of the cases among organ transplant recipients, PML occurred within 24 months of transplantation and tended to present later in the kidney transplant group as compared to heart, liver, and lung transplant patients. 17 Hemiparesis, apathy, and confusion have been the most frequently clinical features among these patients.…”

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

“…Blurred vision together with hemiparesis and ataxia were also the initial manifestations of PML in a HIV-infected female with an enhancing lesion in the left middle cerebellar peduncle on MRI [13]. Visual impairment was the initial manifestation of JC-positive PML in a patient who had undergone bilateral lung transplantation 15 months earlier [3]. Left hemianopsia and left-sided hemiparesis were also the initial manifestations in an HIVpositive in whom the cerebral lesions and clinical manifestations from JC-positive PML significantly improved after HAART [1].…”

“…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].…”