scholarly journals Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis?

2005 ◽  
Vol 90 (6) ◽  
pp. 636-639 ◽  
Author(s):  
R C Dale
Children ◽  
2019 ◽  
Vol 6 (6) ◽  
pp. 75 ◽  
Author(s):  
Maria Milagros Galardi ◽  
Cristina Gaudioso ◽  
Saumel Ahmadi ◽  
Emily Evans ◽  
Laura Gilbert ◽  
...  

The differential diagnosis of pediatric multiple sclerosis (MS) can be broad and pose diagnostic challenges, particularly at initial presentation. Among demyelinating entities, neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibodies (MOG-ab) associated disorders, and acute disseminated encephalomyelitis (ADEM) are now well-known as unique disease processes and yet continue to overlap with MS in regards to clinical presentation and imaging. In non-inflammatory entities, such as metabolic disorders and leukodystrophies, an erroneous diagnosis of MS can be made even while applying appropriate diagnostic criteria. Knowing the epidemiology, typical clinical presentation, diagnostic criteria, and ancillary test results in each disease, can aid in making the correct diagnosis by contrasting these features with those of pediatric MS. Determining the correct diagnosis early, allows for efficient and effective treatment as well as appropriate prognostication.


Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis

Acute disseminated encephalomyelitis (ADEM), a fulminant episode of demyelinating disease, which can be confused with a first episode of multiple sclerosis (MS), can present with transiently severe neurological deficits requiring hospitalization and support care. In this chapter, we review the important differential diagnostic and therapeutic considerations and how best to differentiate ADEM from an initial presentation of MS.


2009 ◽  
Vol 15 (12) ◽  
pp. 1534-1536 ◽  
Author(s):  
E. Mawhinney ◽  
M. Watt ◽  
GV McDonnell

Brainstem lesions are rarely associated with neurogenic pulmonary oedema (NPO) in multiple sclerosis and other disorders. The exact mechanism for this is unknown. We describe a case of a 15-year-old boy who presented with transient cardiomyopathy and severe acute pulmonary oedema. Several days after his initial presentation he developed an ataxic syndrome with limb, truncal and gait ataxia and nystagmus on primary gaze. Investigations confirmed acute disseminated encephalomyelitis (ADEM). For the first time, we describe a case of transient cardiomyopathy and NPO as the initial manifestation of ADEM.


2017 ◽  
Vol 16 (03) ◽  
pp. 164-170
Author(s):  
Rachel Gottlieb-Smith ◽  
Amy Waldman

AbstractAcquired demyelinating syndromes (ADS) present with acute or subacute monofocal or polyfocal neurologic deficits localizing to the central nervous system. The clinical features of distinct ADS have been carefully characterized including optic neuritis, transverse myelitis, and acute disseminated encephalomyelitis. These disorders may all be monophasic disorders. Alternatively, optic neuritis, partial transverse myelitis, and acute disseminated encephalomyelitis may be first presentations of a relapsing or polyphasic neuroinflammatory disorder, such as multiple sclerosis or neuromyelitis optica. The clinical features of these disorders and the differential diagnosis are discussed in this article.


2014 ◽  
Author(s):  
J William Lindsey

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS. This review contains 3 highly rendered figures, 7 tables, and 82 references.


2015 ◽  
Author(s):  
J William Lindsey

Multiple sclerosis (MS) is a relatively common cause of neurologic symptoms and disability in young adults. The distinguishing pathologic features of MS are loss of myelin and inflammation in the central nervous system (CNS). The myelin sheath is essential for rapid conduction of nerve signals along large-diameter axons. Oligodendrocytes produce and maintain myelin in the CNS, and Schwann cells produce and maintain myelin in the peripheral nerves. In addition to MS, there are a number of related disorders causing demyelination, inflammation, or both in the CNS. This chapter discusses MS and related disorders, including neuromyelitis optica, optic neuritis, acute disseminated encephalomyelitis, transverse myelitis, Behçet syndrome, neurosarcoidosis, inherited demyelinating diseases (leukodystrophies, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and virus-induced demyelination (progressive multifocal leukoencephalopathy, subacute sclerosing panencephalitis). The section on MS covers epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, and prognosis. Figures include organization of the microenvironment of larger-diameter axons, typical magnetic resonance imaging findings in MS and neuromyelitis optica, postgadolinium images of the cervical spine in MS, and an approach to treatment of relapsing-remitting MS. Tables list MS and related disorders, distribution of neurologic deficits at the onset of MS, differential diagnosis of MS, disease-modifying therapies for relapsing-remitting MS, and selected leukodystrophies, as well as diagnostic criteria and selected symptomatic therapies for MS.   This chapter contains 3 highly rendered figures, 7 tables, 82 references, 1 teaching slide set, and 5 MCQs.


Author(s):  
Siddharthan Chandran ◽  
Alastair Compston

Clinicians suspect demyelination when episodes reflecting damage to white matter tracts within the central nervous system occur in young adults. The paucity of specific biological markers of discrete demyelinating syndromes places an emphasis on clinical phenotype—temporal and spatial patterns—when classifying demyelinating disorders. The diagnosis of multiple sclerosis, the most common demyelinating disorder, becomes probable when these symptoms and signs recur, involving different parts of the brain and spinal cord. Other important demyelinating diseases include post-infectious neurological disorders (acute disseminated encephalomyelitis), demyelination resulting from metabolic derangements (central pontine myelinosis), and inherited leucodystrophies that may present in children or in adults. Accepting differences in mechanism, presentation, and treatment, two observations can usefully be made when classifying demyelinating disorders. These are the presence or absence of inflammation, and the extent of focal vs. diffuse demyelination. Multiple sclerosis is prototypic for the former, whereas dysmyelinating disorders, such as leucodystrophies are representative of the latter....


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