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Acute disseminated encephalomyelitis presenting as fever of unknown origin: case report
© Di Costanzo et al; licensee BioMed Central Ltd. 2011
Received: 31 March 2011
Accepted: 10 November 2011
Published: 10 November 2011
Fever of unknown origin (FUO) can be defined as a body temperature higher than 38.3°C on several occasions over more than 3 weeks, the diagnosis of which remains uncertain after 1 week of evaluation. Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system with a wide range of clinical manifestations. The highest incidence of ADEM is observed during childhood and it usually occurs following a viral or bacterial infection or, more rarely, following a vaccination, or without a preceding cause.
Here, we describe an atypical case of ADEM that initially manifested as several weeks of FUO in a fifteen years old boy.
This case report suggests a new possible syndromic association between ADEM and FUO, which should be considered in the clinical examination of patients with FUO, especially in the presence of also modest neurologic or neuropsychiatric symptoms.
Fever of unknown origin (FUO) can be defined as a body temperature higher than 38.3°C on several occasions over more than 3 weeks, the diagnosis of which remains uncertain after 1 week of evaluation . Evaluation of FUO in children is complex, because of a wide range of possible etiologies. The most common causes of FUO in childhood are viral infections, while in older patients frequent causes are inflammatory illnesses (rheumatic diseases, vasculitides, polymyalgia rheumatic, sarcoidosis), infections and neoplasms [1, 2]. Acute disseminated encephalomyelitis (ADEM) is uncommon inflammatory demyelinating disease of the central nervous system (CNS) with a wide range of clinical manifestations. The highest incidence of ADEM is observed during childhood and it usually occurs following a viral or bacterial infection or, more rarely, following a vaccination, or without a preceding cause . Onset of the disorder is sudden. According to the classic definition, ADEM is a monophasic disease, but it can also present a relapsing course, being described as "recurrent" if the affected districts are always the same or "multiphasic" if there is dissemination in space and time of the lesions . Irritability and lethargy are common first signs of ADEM. Fever and headache are reported about in half of patients. Fever in ADEM is frequently associated with the development of neurologic symptoms, which can occur after hours or weeks from the onset of illness. The most common neurologic symptoms are visual field deficits; language disturbances; mental status abnormalities ranging from irritability and lethargy to coma; psychiatric changes which include depression, personality changes and psychosis. Meningeal signs are reported in children with severe disease. Weakness, which may be hemiparetic or generalized and symmetric, is more commonly detected than sensory defects. Other reported symptoms are cranial nerve palsies, generalized or focal seizures and ataxia [5–8]. Here we describe an atypical case of ADEM presenting as FUO.
Laboratory data: complete and differential blood count, inflammatory indexes (erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum protein electrophoresis (SPEP), assay of immunoglobulins).
White blood cells (× 103/μL)
Red blood cells (× 106/μL)
Platelets (× 103/μL)
Total protein (g/dl)
Alpha 1 (g/dL)
Alpha 2 (g/dL)
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the CNS characterized by a widespread demyelization that predominantly involves the white matter of the brain and spinal cord. The condition is usually precipitated by a viral infection or vaccination  and is more frequent in children. Today the exact incidence of ADEM is little known. In one study of children with ADEM living in San Diego County, California, the incidence was estimated to be at least 0.4/100.000/year , but in the last decade ADEM has been increasingly diagnosed as more magnetic resonance imaging studies are performed on patients with acute encephalopathy. Based on its immune-mediated etiology, ADEM is commonly treated with high-dose steroids. Factors influencing treatment efficacy and possible alternative options for steroid resistant cases remain unclear. In a portion of patients who fail to respond to steroid therapy, intravenous immunoglobulin has been used with some benefit. Unsolved issues regard clinical predictors to best select therapy for groups of patients. Plasmapheresis and cytostatic drugs are alternative treatment options in patients who do not respond to steroid and/or to intravenous immunoglobulins . An acute encephalopathy with multifocal neurologic signs and deficits is usually the first clinical manifestation, but ADEM has a wide range of presenting features. We described an atypical case of ADEM that initially manifested as several weeks of FUO. A successful diagnosis of the underlying disease required an intensive and rational diagnostic evaluation of the wide spectrum of possible etiologies of FUO. This case report suggests a new possible syndromic association between ADEM and FUO, which should be considered in the clinical examination of patients with FUO, especially in the presence of also modest neurologic or neuropsychiatric symptoms.
Written informed consent was obtained from the parents of the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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