ABSTRACT
INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) is a rare, lethal, demyelinating disease classically seen in profoundly immunosuppressed individuals. It is caused by intracerebral infection by John Cunningham polyomavirus (JCV). We report a rare case of PML in a man with presumed immunocompetence at presentation experiencing bilateral painless visual impairment. CASE DESCRIPTION: A 60-year-old man with a 3-week history of bilateral painless visual impairment attended our ophthalmology department. Unusually, he navigated around the room well and was able to read 4 of 13 Ishihara test plates in spite of a best-corrected visual acuity of counting fingers at 1 m bilaterally. Slit lamp examination, routine blood tests and optical coherence tomography (OCT) of the maculae and discs were unremarkable. Diffuse hyperintense white matter lesions on T2-weighted magnetic resonance imaging of the brain and detection of JCV within the parietal lobe tissue obtained by biopsy confirmed PML. Additional investigations identified an underlying hypogammaglobulinaemia, which may have initiated PML. He received intravenous immunoglobulin but passed away 2 months after diagnosis. CONCLUSIONS: To our knowledge this case is one of only a handful worldwide to describe PML developing in a patient with presumed immunocompetence at presentation - there was no previous history of recurrent, chronic, or atypical infections. There has only been one other report of visual symptoms presenting as the primary complaint. The case illustrates the importance of ruling out organic, central nervous system pathology in patients presenting with visual loss and normal objective visual function tests such as slit lamp examination and OCT.
Subject(s)
Agammaglobulinemia , JC Virus , Leukoencephalopathy, Progressive Multifocal , Agammaglobulinemia/complications , Agammaglobulinemia/diagnosis , Agammaglobulinemia/pathology , Brain/pathology , Humans , Leukoencephalopathy, Progressive Multifocal/complications , Leukoencephalopathy, Progressive Multifocal/diagnosis , Leukoencephalopathy, Progressive Multifocal/pathology , Magnetic Resonance Imaging , Male , Middle Aged , Vision Disorders/complicationsABSTRACT
Ligneous conjunctivitis is a rare form of chronic recurrent membranous conjunctivitis with reduced plasminogen activity. It is characterized by the formation of characteristic firm ("woody") membranes on the tarsal conjunctiva. Similar lesions may occur on other mucous membranes. When treated with local excision, ligneous conjunctivitis is invariably associated with recurrences. Various therapeutic modalities, including topical heparin, cyclosporine, fresh frozen plasma (FFP), plasminogen, and amniotic membrane transplantation have been reported to reduce postoperative recurrences. We present 2 cases of recurrent ligneous conjunctivitis in children successfully managed with surgical excision under cover of FFP transfusion, amniotic membrane grafting, and combined with concomitant postoperative administration of topical heparin, steroids.
Subject(s)
Anticoagulants/administration & dosage , Conjunctivitis/therapy , Heparin/administration & dosage , Plasma , Administration, Topical , Child , Conjunctivitis/complications , Conjunctivitis/etiology , Female , Humans , Infant , Male , Plasminogen/deficiency , Skin Diseases, Genetic/complications , Treatment OutcomeSubject(s)
Cerebral Infarction/complications , Diplopia/etiology , Occipital Lobe/pathology , Parietal Lobe/pathology , Vision, Binocular , Visual Fields/physiology , Cerebral Infarction/diagnosis , Diplopia/diagnosis , Diplopia/physiopathology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Visual AcuitySubject(s)
Brain Neoplasms/diagnosis , General Practice/methods , Referral and Consultation , Retinal Neoplasms/diagnosis , Retinoblastoma/diagnosis , Strabismus/diagnosis , Brain Neoplasms/complications , Child , Child, Preschool , Disease Management , Esotropia/diagnosis , Esotropia/etiology , Esotropia/therapy , Exotropia/diagnosis , Exotropia/etiology , Exotropia/therapy , Humans , Infant , Practice Guidelines as Topic , Retinal Neoplasms/complications , Retinoblastoma/complications , Strabismus/etiology , Strabismus/therapyABSTRACT
Wagner syndrome is a rare inherited vitreoretinopathy. We describe 3 related patients with Wagner syndrome who presented with congenital glaucoma at age 3 months and required multiple surgical interventions to control their intraocular pressure. All experienced visual loss and glaucomatous optic neuropathy.