Neuro-Ophthalmic Manifestations of HIV Infection.

Purpose: To review the broad spectrum of clinical neuro-ophthalmic presentations associated with human immunodeficiency virus (HIV) infection. Methods: Critical review of the literature regarding neuro-ophthalmic consequences of HIV infection and its sequelae. Results: Neuro-ophthalmological diseases are common in both asymptomatic HIV-positive patients and those who profound immunosuppression with acquired immune deficiency syndrome (AIDS). Neuro-ophthalmic manifestations of HIV infection can involve the afferent or efferent visual pathway. Common clinical presentations include headache, papilledema, chorioretinitis, optic nerve involvement, meningitis, and cranial nerve palsies. Other neuro-ophthalmic manifestations include involvement of the visual pathway in the brain producing visual field defects such as occur in progressive multifocal encephalopathy. Pupil abnormalities have also been reported. Discussion: Neuro-ophthalmic consequences of HIV are important to recognize as it is critical to identify underlying neoplastic or infectious diseases which could be amenable to treatment.

Primary varicella infection presenting with headache and elevated intracranial pressure.

Primary varicella infection may be associated with neurologic complications, such as cerebritis and meningoencephalitis. Several cases of varicella infection with elevated intracranial pressure have been reported. We describe a 13-year-old immunocompetent girl who presented with a clinical picture of headaches and elevated intracranial pressure as the only manifestation of primary varicella zoster infection. The working diagnosis at first was pseudotumor cerebri based on complaints of headache of 2 weeks' duration, in addition to vomiting and papilledema, without fever or skin eruption. On lumbar puncture, opening pressure was 420 mmH2O, but mild pleocytosis and mildly elevated protein level ruled out the diagnosis of pseudotumor cerebri. Our patient had no history of previous varicella infection, and she did not receive the varicella zoster vaccine. Serology tests, done on admission and repeated 2 months later, suggested primary varicella infection. The literature on varicella infection associated with pseudotumor cerebri or elevated intracranial pressure is reviewed.

[Papillitis due to Epstein-Barr virus infection in an adult patient]. / Papilitis por infección del virus de Epstein-Barr en paciente adulto.

CASE REPORT: A 70-year- old woman who consulted due to poor vision in her right eye for 2 weeks. The examination showed a visual acuity (VA) of 0.6 and 1.0, with normal anterior pole and intraocular pressure and a relative afferent pupillary defect. Some papillitis was observed in the fundus of her right eye. The high levels of acute phase reactants led to an initial diagnosis of arteritic anterior ischemic optic neuropathy (AAION) and treatment with corticosteroids was started. DISCUSSION: According to the age of the patient, the fundus and the high levels of acute phase reactants, the initial diagnosis was AAION. However, the atypical papillitis features, with good AV and non-specific perimetry, together with a suitable medical history, and a profile of viral characteristics, with laboratory confirmation, led to the diagnosis of a primary papillitis infection due to Epstein-Barr virus, a very rare case due to the advanced age of the patient.

Viral-induced intracranial hypertension mimicking pseudotumor cerebri.

BACKGROUND: Pseudotumor cerebri or idiopathic intracranial hypertension is characterized by normal spinal fluid composition and increased intracranial pressure in the absence of a space-occupying lesion. METHODS: This study describes a subgroup of 10 patients with the same typical presenting symptoms (headache, vomiting, and papilledema) but without nuchal rigidity, meningeal signs, or change in mental status. Patients had normal neuroimaging studies and intracranial hypertension but also pleocytosis in the cerebrospinal fluid, suggesting central nervous system infection. From the results it can be hypothesized that those children represent a unique subgroup of viral-induced intracranial hypertension when comparing their risk factors, clinical course, treatment, and outcome with 58 patients who had idiopathic intracranial hypertension. RESULTS: All patients with viral-induced intracranial hypertension presented with papilledema but none had reduced visual acuity or abnormal visual fields, compared with 20.7% of patients who had idiopathic intracranial hypertension. They also responded better to treatment with acetazolamide, needed a shorter duration of treatment (7.7 ± 2.6 months vs 12.2 ± 6.3 months, P = 0.03), and had no recurrences. CONCLUSIONS: The results suggest that children who fulfill the typical presenting signs and symptoms and all diagnostic criteria for pseudotumor cerebri other than the normal cerebrospinal fluid component may represent a unique subgroup of viral-induced intracranial hypertension and should be managed accordingly. The overall prognosis is excellent.

[Benign but not harmless intracranial hypertension: a case report]. / Hypertension intracrânienne bénigne, mais pas anodine. À propos d'un cas.

Benign intracranial hypertension (BIH) is characterized as an intracranial pressure increase occurring in the absence of brain tumour, sinus thrombosis or hydrocephaly. But contrary to what its designation might suggest, it threatens the visual prognosis. We report the case of a 15-year-old girl with lymphocytic meningitis, developing secondary a BIH. Cerebrospinal fluid pressure was 70cm water, without enlargement of the cerebral ventricles. Along with the progression, bilateral 6th nerve palsy, impairment of visual acuity and bilateral papilledema appeared. No cause was found after a complete assessment. Treatment consisted in oral acetazolamide and 9 depletive spinal taps. Clinical examination, fundus examination and Goldmann visual field normalized after 8 weeks. No relapse occurred after a 1-year follow-up. This case shows that BIH, which is not a well-known disorder, is incorrectly referred to as benign: both prompt diagnosis and proper management are of major importance.

Immune-recovery uveitis in patients with cytomegalovirus retinitis taking highly active antiretroviral therapy.

PURPOSE: To investigate the clinical features associated with immune recovery in human immunodeficiency virus (HIV)-infected patients with cytomegalovirus retinitis who are taking highly active antiretroviral therapy. METHODS: Sixteen patients were evaluated prospectively at the National Eye Institute, Bethesda, Maryland. Evaluation included a medical history and a complete ophthalmologic examination. The examination included best-corrected visual acuity score measured by means of logarithmic charts, slit-lamp biomicroscopy, dilated retinal examination, retinal photography, and fluorescein angiography. Immune-recovery uveitis was defined as the ocular inflammation associated with clinical immune recovery in patients taking potent antiretroviral regimens. The ophthalmic characteristics of immune-recovery uveitis were identified, and their effect on visual acuity was statistically analyzed. RESULTS: The mean CD4+ T-lymphocyte count for the 16 patients taking highly active antiretroviral therapy at the time of evaluation was 393 cells/microl (range, 97-1,338 cells/microl). Immune-recovery uveitis was characterized by vitreitis and optic disk and macular edema. Clinically important complications of immune-recovery uveitis included cataract and epiretinal membrane formation. The visual acuity scores were significantly worse in the 23 eyes with cytomegalovirus retinitis (mean, 67.2 letters, 20/50) than in the nine eyes without cytomegalovirus retinitis (mean, 89.8 letters, 20/16) (P <.001). Regression analysis showed that a lower visual acuity score was associated with the presence of moderate to severe macular edema on fluorescein angiography and vitreous haze (P < or =. 001). CONCLUSIONS: Immune-recovery uveitis is an important cause of visual morbidity in HIV-infected patients with cytomegalovirus retinitis in the era of highly active antiretroviral therapy. Although immune recovery associated with highly active antiretroviral therapy has allowed some patients to discontinue specific anticytomegalovirus therapy, the rejuvenated immune response can be associated with sight-threatening inflammation.

Adenoviral gene therapy with catalase suppresses experimental optic neuritis.

OBJECTIVE: To determine if adenoviral-mediated transfer of the gene for catalase (CAT), the reactive oxygen species scavenger, suppresses experimental optic neuritis. CLINICAL RELEVANCE: Gene therapy with CAT delivered by an adeno-associated viral vector was previously shown to suppress experimental optic neuritis. Because the transduction of protein expression with recombinant adeno-associated viral vector is relatively slow, taking weeks to reach full levels, we studied the effects of replication-deficient adenovirus containing CAT in suppressing experimental optic neuritis. Transduction with adenovirus occurs within days of inoculation, thus, it may be more applicable for the treatment of patients with acute optic neuritis. MATERIALS AND METHODS: Replication-deficient adenovirus containing CAT was injected above the right optic nerve heads of SJL/J mice that were simultaneously sensitized for experimental allergic encephalomyelitis. For controls, the left eyes were injected with the replication-deficient adenovirus without CAT or no virus. The histological effects of CAT on the lesions of experimental allergic encephalomyelitis were measured by computerized analysis of the myelin sheath area (for demyelination), optic disc area (for optic nerve head swelling), the extent of the cellular infiltrate, extravasated serum albumin labeled with immunogold (for disruption of the blood-brain barrier), and the in vivo hydrogen peroxide reaction product. RESULTS: After 1 month, cell-specific catalase activity, evaluated by the quantitation of catalase immunogold, was increased about 2-fold each in endothelia, oligodendroglia, astrocytes, and axons of the CAT-inoculated right optic nerves compared with the control left optic nerves. The increased cellular levels of catalase reduced demyelination by 30%, optic nerve head swelling by 25%, cellular infiltration by 26%, disruption of the blood-brain barrier by 61%, and in vivo levels of hydrogen peroxide by 81%. CONCLUSIONS: Adenoviral-mediated gene transfer increased catalase levels in all optic nerve cell types, and it persisted for 1 month after inoculation. The increased cellular levels of catalase suppressed demyelination and blood-brain barrier disruption at the foci in the optic nerve where prior magnetic resonance imaging and histopathologic studies have demonstrated the demyelinating inflammation of experimental and human optic neuritis. Together, they suggest that gene therapy with CAT may be helpful in the treatment of patients with optic neuritis.