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Acute Disseminated Encephalomyelitis (ADEM): Imaging Revisited
Roman A, Anzolin E, Bianchini L,
Introduction: Acute Disseminated Encephalomyelitis (ADEM) is defined as an inflammatory autoimune disease of the central nervous system (CNS) typically presenting as a mono phase syndrome.1 It is a rare syndrome (0.4-0.8:100,000/year) that comprises generally in infancy, adolescents, and young adults.2, 3 Usually it presents posteriorly to an infectious disease or vaccination,4 varying from initial neurologic signs and symptoms multi focally, including motor deficits, sensorial and in some cases, affecting the brainstem with catastrophic consequences.5,6,7
Case Illustration: A female patient, caucasian, 30 years of age, presented with visual compromise, bilaterally, of sudden onset one week prior to medical admittance. Reports moderate, retro-orbital headache, with no further signs or symptoms. Previously a healthy young patient, in regular use of oral contraceptives, with no additional rowsk factors for CNS diseases, although a report of superior airway viral infection was stated. On physical examination, only visual disturbance, with visual acuity of 20/100, without visual field variations on confrontation, as no motor or sensitive deficits were further observed. Chest radiography and thorax and abdomen tomography were negative for any pathologic findings. on MRI, multiple intra-axial lesions were observed, hyper intense on T1WI (Figure 1B and Figure 2A), as well as on T2WI (Figure 1A and Figure 2B), without perilesional apparent edema, and no compression of the adjacent structures, corroborating that no mass effect was evident on the imagining. On laboratorial exams, discrete elevation of VSG and PCR. Cerebrospinal Fluid (CFS) demonstrated discrete elevation of proteins and absence of neoplastic cells. Pulse steroid therapy was administered, with methilprednisolone, during five consecutive days, in the dosage of 1000mg/day. MRI was repeated after termination of pulse therapy, without apparent alterations from prior MRI. Visual symptoms regressed, with acuity improving to a 20/40.
Discussion: It is considered that in Acute Disseminated Encephalomyelitis (ADEM) an autoimmune response directed to myelin basic protein occurs , triggered by infection or immunization, resulting in a cascade reaction. T cells directed to microbial epitopes may recognize amino acid sequences of the myelin such as antigens and trigger an attack aimed at CNS structures. The inflammatory response associated with ADEM is often translated by fever, headache, vomiting and encephalopathy in the clinical picture. Moreover, demyelination may also be observed in the optic nerve, optic neuritis culminating - commonly bilateral - and consequent reduction in visual acuity. The diagnosis of ADEM is considered in patients with acute multifocal neurological signs and is confirmed by the presence of demyelinating lesions on magnetic resonance imaging (MRI) . Furthermore, it is imperative to distinguish between different imaging findings, such as pseudotumoral dem yelinating encephalitis that are often critical, as also multiple sclerosis, Balo's concentric sclerosis and Schelden , and also to rule-out the possibility of central nervous system lymphoma , which carry similar imaging findings . The differential diagnosis with multiple sclerosis is the main hypothesis and lies in the fact that ADEM usually follows a viral prodromal illness, with fever, causing a generalized disorder of the central nervous system. In addition, in MRI, ADEM usually manifests itself with a greater number of lesions than multiple sclerosis and the onset is also generally monophonic and sudden . In the case illustrated above , the clinical course began with bilateral blurred vision, suggestive of optic neuritis. Viral infection of the upper respiratory trac t approximately 15 days prior to optic presentation was most probably the triggering factor of the disease. The patient was then hospitalized for further investigation with MRI, which revealed at least six intraxia l lesions, hyperintense on T1 WI and T2 WI and without clear perilesional edema. A CT-S can of the chest and abdomen were analyzed to rule-out diagnosis of primary neoplastic findings that could lead to brain metastases , which did not demonstrate any lesions in theses images . After pulse therapy with corticosteroids, repeated MRI to rule out the diagnosis of lymphoma, which could demonstrate the diminished volume of the prior found lesions, strongly corroborating thus the diagnosis of ADEM.
Conclusion: As a inflammatory autoimune disease, Acute Disseminated Encephalopathy (ADEM) usually presents with a mono phasic presentation, which responds well to corticosteroid therapy. The findings on MRI are generally those of other pseudotumoral demyelinating diseases, varying from a few hyper-intense lesions on T1WI and T2WI, to a great number of CNS lesions, without prominent mass effect and perilesional edema, corroborating the nature of these pseudo-tumoral diseases. It is believed that these demyelinating diseases respond well to corticosteroid therapy, especially in the early onset cases, and the later diagnosed diseases may have a favorable prognostic with the adjunct therapy of intravenous immunoglobulin and plasmapheresis in selected cases.
A - Axial T2WI demonstrating multiple intra-axial hyperintense lesions, with no perilesional edema or CNS structure compression.
B - Axial T1WI intra-axial periventricular hyperintense lesions, with no apparent mass effect.
A - Sagital T1WI corpus calosum hyperintense multiple lesions, with no mass effect or perilesional edema.
B - Sagital T2WI fat suppression intra-axial multiple lesions, compromising inter-hemispheric white fibers, without mass effect or perilesional edema.
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