Summary: Despite largely being ignored, chronic Epstein-Barr is recognized as life threatening. But we don't have a middle diagnosis, hindering treatment.
Adolesc Med State Art Rev. 2012 Aug;23(2):277-84.
An adolescent evaluated for chronic fatigue: does she have a sleep disorder?
Gonzalez M1, Fisher M.
A 13-year-old girl presented to our Division of Adolescent Medicine for evaluation of ongoing fatigue. She and her mother reported that 15 months earlier, when the patient was in the 7th grade, she started to be tired and "sleep all the time." They do not remember her being ill at that time; a test for mononucleosis was reportedly positive, but Epstein-Barr virus titers showed "old disease." She remained fatigued throughout 7th grade but attended school without falling asleep; however, she was too tired to do her work and had to attend summer school. The patient received home instruction in 8th grade and reported that on weekdays she slept from 1 AM to 11 AM with a nap from 4 to 8 PM and on weekends she slept from 3 AM to 1 PM without a nap.
Zhonghua Er Ke Za Zhi. 2009 Sep;47(9):682-6.
[Clinical characteristics and follow-up of 12 cases with severe chronic active Epstein-Barr virus infection].
[Article in Chinese]
Song HM1, Wu XY, Wang W, Xing Y, Li F, Qiu JJ, Xiao J, Wei M.
There are two different types of chronic active Epstein-Barr virus (CAEBV) infection: chronic EBV (CEBV) having persistent infectious mononucleosis (IM)-like illness with relatively good prognosis, and severe CAEBV (SCAEBV)infection that has rather severe manifestations and generally poor prognosis with many life-threatening complications, such as EBV-associated malignant lymphoma and hemophagocytic syndrome (HPS). The purpose of this study was to clarify the clinical and prognostic characteristics in 12 cases with SCAEBV infection.
Data of 12 cases with SCAEBV infection were analyzed retrospectively, which included clinical and auxiliary examination, pathological data, especially EB virus (EBV)-antibodies and DNA in peripheral blood mononuclear cells (PBMC) and infected tissue, and follow-up information.
Of the 12 cases, 7 were male and 5 were female. The age at the onset of diseases ranged from 35 months to 14 years (median, 11 years). The major manifestations were fever (100%), splenomegaly (91.7%), hepatomegaly (83.3%), lymphadenopathy (75.0%), and others, including skin rash, development retardation, jaundice, ascites, pulmonary hypertension, oral ulcer, cholecystitis and pleural effusion. The abnormalities of auxiliary examination were as follows: elevated LDH level (91.7%), liver dysfunction (83.3%), anemia (75.0%), leukopenia (58.3%), neutropenia (50.0%), thrombocytopenia (25.0%) and abnormal chest X-ray findings. At the time of onset, 58.3% of the patients had an IM-like illness. In all of the 12 cases, EBV serologic tests revealed high IgG antibody levels against EB viral capsid antigen (VCA). The patients often had positive IgM and IgA antibodies against VCA (33.3% and 66.7%) as well. Elevated IgG antibody level to early antigen (EA) (100.0%), occasionally positive IgA antibody (40.0%) were also seen. The mean load of EBV-DNA detected by real-time polymerase chain reaction (PCR) in the PBMC was (8.12 x 10(6), median)copies/ml. Four of 12 cases presented a poor clinical course, two of whom died from EBV-associated HPS, 1 from severe multiple pathogens infection, and 1 from multiple organ failure. In addition, 1 case developed Hodgkin's T cell lymphoma and another case showed hepatopulmonary syndrome in 2 years after splenectomy.
The clinical feature of SCAEBV infection varied exceedingly. EBV-DNA load in PBMC of SCAEBV infected patients was significantly increased. More attention should be paid to the disease because of its severe complications, poor prognosis and high mortality.
Am J Gastroenterol. 1986 Sep;81(9):808-11.
Exudative ascites complicating infectious mononucleosis.
Marano AR, Lanse SB, Garsten JJ, Thornton GF, Antopol SC, Gannon D.
A case of Epstein-Barr virus mononucleosis with the unusual complication of exudative ascites is presented. The patient was a 22-yr-old man with the typical symptoms and physical findings of hepatitis secondary to infectious mononucleosis. Extensive evaluation including liver biopsy, failed to show another cause for the patient's ascites. The ascites and hepatitis disappeared with resolution of the acute mononucleosis infection. He is well 12 months after this illness with no evidence for chronic liver disease. This case adds to the list of causes for exudative ascites associated with acute hepatitis.
Wiad Lek. 2009;62(4):235-42.
[Chronic active Epstein-Barr virus infection in an adult].
[Article in Polish]
A chronic active Epstein-Barr virus infection (CAEBV) following infectious mononucleosis in a 58 years old woman is reported. The disease lasted for one year, and in spite of an intensive search for its cause, was diagnosed only at the 8th months since its onset. A low frequency of CAEBV in caucasians and patient's age were likely responsible for the belated diagnosis. The disease presented with a high, intermittent fever, general lymphoadenopathy, splenomegalia, hypoalbuminemia, polyclonal gamma globulinemia and malaise. Starting from the 6th month, i.e. before the diagnosis was established, a high dose oral therapy with methylprednisolone was introduced. The improvement was significant but the disease recurred after drug withdrawal. Nevertheless its course was milder. At the 8th month since the disease onset elevated antibody to viral capsid antigen (VCA) together with antibody to early antigen (EA) and nuclear antigen (EBNA) were still found in patient's blood. DNA of EBV was detected by PCR in patient's blood and saliva. The patient recovered completely after one year, and as of today i.e. June 2009, is feeling well. A likely cause of the successful steroid therapy is discussed. A review part of the article describes etiopathogenesis, complications, occurrence and treatment of CAEBV, as well as its relation to various lymphoproliferation disorders.
Curr Treat Options Neurol. 2012 Oct;14(5):464-73. doi: 10.1007/s11940-012-0189-2.
Sleep dysfunction in multiple system atrophy.
Ferini-Strambi L1, Marelli S.
Sleep disorders in multiple system atrophy (MSA) are common manifestation and include reduced and fragmented sleep, excessive daytime sleepiness, REM sleep behaviour disorder (RBD), and sleep-disordered breathing. Of these, RBD is the most common (affecting 90 %-100 % of patients with MSA) and is regarded as a red flag for MSA. RBD, as well as stridor during sleep, may be the initial manifestation of the disease, occurring several years before the waking motor and dysautonomic onset. Sleep disorders occur in both MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C). Treatment strategies in patients with MSA presenting difficulties in initiating and maintaining sleep need to be highly individualized. Clonazepam has been found to be successful in treating RBD symptoms at the dose of 0.25 to 2.0 mg given approximately 30 min before bedtime. In case of comorbid obstructive sleep apnea, zopiclone (at the dose from 3.75 to 7.5 mg each night) or melatonin (with a recommended dose of 3 to 12 mg at bedtime) may be alternative treatments. An increased survival in MSA patients with stridor may be obtained both with continuous positive airway pressure (CPAP) and tracheostomy. Since tracheostomy is an invasive surgical procedure, not easily accepted by the patient, CPAP therapy should be considered first. However, tracheostomy is first indicated when stridor is present during wakefulness because of the high risk of respiratory failure and death. In MSA, obstructive sleep apnea (OSA) occurs more frequently than central sleep apnea, ranging from 15 % to 37 % of the cases. CPAP is an effective treatment for eliminating obstructive sleep apnea in MSA patients, even if the adaptation to the device may be difficult in advanced cases.