Intact survival of refractory CMV limbic encephalitis in a patient with severe aplastic anemia after unrelated bone marrow transplantation.

We described a patient of refractory cytomegalovirus (CMV) limbic encephalitis who received matched unrelated bone marrow transplantation. Pyrosequencing study on serial cerebrospinal fluid samples revealed the emergence of resistant strains associated with exposure of antiviral agents. Combinations of antiviral agents had a role in partial suppression of CMV viral load but the clearance of virus mainly relied on the recovery of host's immunity and resulted in intact survival of host. Donor's CMV-seronegative status may contribute to the delay in controlling this serious infection. Prompt identification of drug-resistant mutant helps in selection of antiviral agents.

[Rift Valley fever]. / Fièvre de la vallée du Rift.

Rift Valley Fever (RVF) is a zoonotic arbovirosis. Among animals, it mainly affects ruminants, causing abortions in gravid females and mortality among young animals. In humans, RVF virus infection is usually asymptomatic or characterized by a moderate fever. However, in 1 to 3% of cases, more severe forms of the disease (hepatitis, encephalitis, retinitis, hemorrhagic fever) can lead to the death of infected individuals or to major sequels. The RVF virus (Bunyaviridae, genus Phlebovirus) was identified for the first time in the 1930s in Kenya. It then spread over almost all African countries, sometimes causing major epizootics/epidemics. In 2000, the virus was carried out of Africa, in the Middle East Arabian Peninsula. In 2007-2008, Eastern-African countries, including Madagascar, reported significant episodes of RVF virus, this was also the case for the Comoros archipelago and the French island of Mayotte. This ability to spread associated with many vectors, including in Europe, and high viral loads in infected animals led the health authorities worldwide to warn about the potential emergence of RVF virus in areas with a temperate climate. The awareness has increased in recent years with climate changes, which may possibly modify the vector distribution and competence, and prompted many RVF virus-free countries to better prepare for a potential implantation of RVF.

[Severe presentations of Rift Valley Fever in Madagascar]. / Les formes graves de la fièvre de la vallée de Rift pendant l'épidémie à Madagascar.

OBJECTIVE: The authors describe clinical and epidemiologic characteristics of severe presentations of Rift valley fever (RVF) during the 2008 epidemic in Madagascar. METHODOLOGY: The diagnosis was confirmed by RVF virus polymerase chain reaction (PCR), or detection of specifics antibodies by Elisa. RESULTS: Sixteen cases of severe RVF were recorded. The sex-ratio was 7/1 and median age was 32 years (20/59 years). The risk factors of infection were: contact with infected animals or their meat (n=8), and travelling to a risk area (n=2). Hemorrhagic, neurological, and ocular manifestations were observed respectively in 87.5%, 43.8% and 6.3% of cases. All patients who died (n=4) presented with a hemorrhagic form of the disease. CONCLUSION: The hemorrhagic form was the most frequent presentation of RVF and was responsible for a high level of mortality. Epidemiologic surveillance must be implemented.

2.5 years survival with sequential ganciclovir/foscarnet treatment in a patient with acquired immune deficiency syndrome and cytomegalovirus retinitis.

The case with the longest survival time (30 months) after the diagnosis of cytomegalovirus retinitis in a group of 53 patients with the acquired immune deficiency syndrome and cytomegalovirus retinitis (median survival time 8.4 months) is described. The patient developed cytomegalovirus retinitis in his left eye and received intravenous virustatic treatment for 29.5 months. Treatment was started with ganciclovir. After withdrawal from maintenance treatment, a relapse of cytomegalovirus retinitis occurred, which was again successfully treated with ganciclovir. A secondary cataract developed, and cataract extraction was performed. After 12.5 months on treatment with ganciclovir, a change to foscarnet was necessary because of neutropenia. Maintenance treatment with foscarnet was well tolerated for 17 months. The cytomegalovirus retinitis showed no signs of reactivation during this period. Vision in the right eye was preserved until death, and no sign of cytomegalovirus retinitis developed in the right eye. This case report demonstrates that an unusual long survival time is possible in a patient sequentially treated with ganciclovir and foscarnet.

Repair of retinal detachment caused by cytomegalovirus retinitis in patients with the acquired immunodeficiency syndrome.

Twenty-two eyes of 19 patients with the acquired immunodeficiency syndrome who had pars plana vitrectomy and silicone-oil injection after retinal detachment caused by cytomegalovirus retinitis were studied. All patients but one were monitored until time of death. The postoperative survival time and the factors that predicted anatomic success (retinal attachment) and functional success (visual acuity) were analyzed. No intraoperative complications were encountered. The mean survival time after surgery was four months. Of all of the preoperative and intraoperative factors studied, only the duration of cytomegalovirus retinitis was predictive of survival (P less than .03). The anatomic success rate was 89.5% (17 of 19 patients). None of the factors showed a trend or statistical significance in relation to anatomic success. Fifteen of 19 patients (79%) had lost at least two lines of Snellen visual acuity at time of death. Vision declined in a bimodal pattern (within the first postoperative month and after four months postoperatively). The optic nerve was pink and well perfused preoperatively in 16 of 19 patients (81.8%), but optic-nerve atrophy was observed postoperatively in 18 of 19 patients (95.5%). There was a trend for functional success to be influenced by increased intraocular pressure and optic-nerve atrophy, although our sample size was too small for statistical significance.

Survival of patients with AIDS and cytomegalovirus disease treated with ganciclovir or foscarnet.

We reviewed the hospital charts of 168 patients with AIDS and cytomegalovirus (CMV) disease diagnosed at San Francisco General Hospital between July 1985 and October 1989. One hundred and thirty-three patients had CMV retinitis, 33 had CMV gastrointestinal disease, and two had CMV lung disease. We found a trend towards longer survival from time of CMV disease diagnosis in patients with more recent dates of diagnosis. The median survival of patients diagnosed with CMV disease prior to 30 September 1987 was 4 months, compared with 9 months for patients diagnosed after 30 September 1987 (P = 0.001). The relative hazard of death for patients with CMV retinitis who were initially treated with foscarnet was not significantly reduced compared to those initially treated with ganciclovir. Even after controlling for age at time of CMV diagnosis, time from index AIDS diagnosis, hemoglobin, absolute lymphocyte count, absolute neutrophil count and concurrent zidovudine therapy, the relative hazard for foscarnet-treated patients compared with ganciclovir-treated patients was 1.0 (95% confidence interval, 0.5-1.8).

Retinal detachments in patients with cytomegalovirus retinitis.

Retinal detachments are a recognized complication of cytomegalovirus retinitis. In a series of 145 patients with cytomegalovirus retinitis, 38 (26%) developed retinal detachments. The cumulative probability of a retinal detachment 1 year after the diagnosis of cytomegalovirus retinitis was 50%. Detachments were associated with increased size of the cytomegalovirus lesion and with lesions extending anteriorly up to the ora serrata. Treatment with an anticytomegalovirus drug appeared to delay the time to detachment. Median survival time after the diagnosis of retinal detachment was 9 months. In our study, vitrectomy with silicone oil as the initial surgical procedure was the most effective approach. Successful anatomic reattachment of the retina was achieved in 70% of patients undergoing this procedure. Despite the success of surgery, the visual outcome was disappointing, with only 20% of eyes achieving a final visual acuity of 5/200 or better.

Cytomegalovirus retinitis and acquired immunodeficiency syndrome.

In a series of 157 patients with acquired immunodeficiency syndrome (AIDS), 46 (29%) developed cytomegalovirus (CMV) retinitis. In five patients, CMV retinitis was the initial AIDS-defining opportunistic infection (11% of patients with CMV retinitis and 3% of patients with AIDS). Retinal detachments developed in seven patients (15%) and in four were present before the institution of ganciclovir therapy. Bilateral CMV retinitis was present in 35% of patients at presentation and subsequently developed in nine (60%) of 15 patients while not being treated with ganciclovir. Conversely, none of 18 patients with unilateral disease developed bilateral disease while receiving ganciclovir. Of patients treated with ganciclovir for their CMV retinitis, 81% had a response to the drug, and 61% achieved a complete response, resulting in a nonprogressive and inactive scar. Patients who achieved a complete response with ganciclovir had a significantly longer survival than those who did not, suggesting greater immune compromise in those patients who failed to respond to ganciclovir.

Congenital retinitis in the rat following maternal exposure to lymphocytic choriomeningitis virus.

A combined clinical and histopathological study of the eyes of the offspring of females inoculated with LCMV shows that about two-thirds of the pups develop some degree of retinal inflammation. This may range from a mild, subclinical reaction to an overt retinitis characterized clinically by demonstrable inflammatory and degenerative changes. Histopathologically, the latter condition presents the picture of an inflammatory reaction with extensive loss of photoreceptors and retinal neurons in general, macrophagic invasion, mild microcystoid degeneration and total or subtotal retinal detachment. This vertically-transmitted disease does not show the relentless progression and hemorrhagic tendency characteristic of the retinitis which occurs after direct viral inoculation. However, in the most severe cases the final outcome is the same, namely severe retinal impairment subsequent to widespread loss of photoreceptors. In the presence of consistently negative virological data, the hypothesis is proposed that this retinitis could be the result of a vertically-transmitted autoimmune disease.