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Inflammatory Disorders of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Acute disseminated encephalomyelitis (ADEM)1 is an acute inflammatory demyelinating disease of the brain and spinal cord characterized by widespread perivascular inflammation and demyelination and caused by an autoimmune attack on the brain, most commonly as a reaction to a viral infection. Early studies emphasized the importance of activated autoreactive T cells that recognize myelin-specific proteins; more recently, the role of antibodies to myelin–oligodendrocyte glycoprotein (MOG) has come to the fore. The disease is one of immunoregulatory failure rather than immunosuppression.
Autoimmune disorders that can be mistaken for viral illness
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Maxwell Greene, Eric Lancaster
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory demyelinating disorder of the CNS that is usually precipitated by viral infection or vaccination, and preferentially affects children under the age of five [4]. The typical presentation is that of rapid onset encephalopathy with diverse neurologic signs over hours to days [4]. Impairment of consciousness is the rule during the acute phase. Lesions are prominent on MRI, usually enhance, and usually resolve over months. The disease is mostly monophasic and rarely recurrent, and is thought to be distinct from multiple sclerosis. The natural history is gradual improvement over weeks with the majority experiencing full resolution, but some patients may have lasting deficits that can include behavioral deficits. ADEM is a known phenomenon post-rubella, post-varicella [4] and post-measles infection [3].
Test Paper 7
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system that typically follows a febrile infection or vaccination. The disorder is immunologically mediated because of an autoimmune reaction to myelin. Peak incidence is in children aged 3–10 years. Typically, ADEM manifests as multifocal lesions mimicking MS. However, clinical and radiologic criteria distinguish between ADEM and MS.
Oligoclonal bands: clinical utility and interpretation cues
Published in Critical Reviews in Clinical Laboratory Sciences, 2022
Sara Carta, Diana Ferraro, Sergio Ferrari, Chiara Briani, Sara Mariotto
Myelin oligodendrocytes glycoprotein antibody-associated disorder (MOGAD) refers to a distinct group of recently identified inflammatory CNS diseases. Onset is usually characterized by optic neuritis, myelitis, brainstem symptoms, encephalitis, or a combination of these syndromes in adults, while children present most commonly with acute disseminated encephalomyelitis (ADEM). MOG-antibody presence is usually detected in serum but paired CSF positivity is quite common (41%) [92,93]. In addition, the presence of CSF-restricted MOG-antibodies in patients with a compatible phenotype has been recently reported, suggesting that in some cases the disease might be driven by intrathecal antibody-producing cells [92,94,95]. In this condition, CSF-restricted OCBs appear to be even less common than in AQP4-positive NMOSD (5–12.3%) [10–13]. In addition, recent studies have shown that the frequency of pleocytosis and OCBs varies according to the clinical phenotype, with brain, brainstem, and spinal cord damage associated with a higher frequency of OCBs compared to optic nerve involvement [12,13]. According to the available data, the presence of MOG-Abs in serum and/or CSF does not correlate with CSF-restricted OCBs, but future studies focusing on this topic are needed [94].
Rapidly progressive dementia in a nonagenarian with acute disseminated encephalomyelitis
Published in Acta Clinica Belgica, 2022
Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is an autoimmune demyelinating disease of the central nervous system. ADEM is more frequent in children and is rarely seen in adults [1,2]. It has been reported in adults 18 to 82 years with median age ranges from 33 to 41 [3–6, 2]. A preceding infection or vaccination are common triggers and cause an inflammatory reaction in the brain and spinal cord [6,7]. The onset of encephalopathy and polyfocal neurologic symptoms is acute and often rapidly progressive. Most patients present with motor deficits including cranial nerves. Sensory deficits are frequent and brainstem involvement is common, including oculomotor deficits and dysarthria. Additional signs and symptoms may include malaise, headache, meningism, ataxia, aphasia, nystagmus, extrapyramidal movement disorders, urinary retention and seizures [3,4,6,7]. Even optic neuritis and increased intracranial pressure may be diagnosed in patients with ADEM [3,4,6,7]
Proceedings of the 43nd Annual Upper Midwest Neuro-Ophthalmology Group Meeting, July 23, 2021 and Second Virtual Upper Midwest Neuro-Ophthalmology Group Meeting
Published in Neuro-Ophthalmology, 2021
Deena Tajfirouz, Casey Judge, John J. Chen, Collin McClelland
Next, Ashwini Kini MD, University of Kentucky, presented a case of a 46-year-old man with no prior medical history who presented with binocular horizontal diplopia and right arm weakness and ataxia following an upper respiratory infection. MRI of the brain demonstrated numerous T2 hyperintensities, some enhancing, in the deep and subcortical white matter including the subcortical U-fibres, brainstem, bilateral basal ganglia and left thalamus. Lumbar puncture was unremarkable and he was treated with three days of high dose intravenous steroids for a presumptive diagnosis of acute disseminated encephalomyelitis (ADEM). Despite steroid treatment, he worsened clinically and returned with a new left cranial nerve VI palsy. Further history and laboratory evaluation revealed that patient had previously undiagnosed human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) with CD4 count of 5. Two additional lumbar punctures failed to show laboratory evidence of John Cunningham (JC) virus. He was started on anti-retroviral therapy but continued to decline, developing quadriparesis and dysphagia with an inability to manage secretions. Brain biopsy was consistent with progressive multifocal leukoencephalopathy (PML) noting JC virus positivity in glial cells. The patient unfortunately succumbed to critical illness. This case highlights that while polymerase chain reaction (PCR) is generally very sensitive for JC virus central nervous system infection, its sensitivity can be altered by AIDS and other immunosuppressed states. Thus, if a high degree of suspicion remains for PML despite negative lumbar puncture, brain biopsy may be necessary to confirm the diagnosis.