Acute disseminated encephalomyelitis: current knowledge and open questions

Steiner, Israel; Kennedy, Peter G. E.

doi:10.1007/s13365-015-0353-z

Cited by 45 publications

(53 citation statements)

References 49 publications

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“…Acute disseminated encephalomyelitis (ADEM) is most commonly thought to be a post-viral, post-vaccination autoimmune phenomenon [1][2][3]. We present a unique case of ADEM following acute appendicitis which was potentially secondary to a bacterial infection.…”

Section: Discussionmentioning

confidence: 98%

“…Based on these theorized mechanisms, numerous case reports and a number of uncontrolled studies have determined that immunosuppression is the mainstay of ADEM treatment [3]. Several studies have demonstrated benefit with high dose IV methylprednisolone treatment for three to five days, followed by an oral taper over six weeks [3].…”

Section: Discussionmentioning

confidence: 99%

“…Several studies have demonstrated benefit with high dose IV methylprednisolone treatment for three to five days, followed by an oral taper over six weeks [3]. Along with the initial steroid treatment, empiric antimicrobial cover is recommended until infectious causes have been ruled out [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…Along with the initial steroid treatment, empiric antimicrobial cover is recommended until infectious causes have been ruled out [1,3]. In patients showing a poor response or failure of high dose glucocorticoids, IVIG therapy for five to seven days has been recommended [3]. Current estimates show that approximately half of patients with ADEM that fail a trail of high dose glucocorticoids will respond to IVIG [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…This autoimmune phenomenon occurs in genetically susceptible individuals, resulting in a rapid inflammatory demyelination of the brain and spinal cord [1,2]. ADEM is typically a monophasic illness, preceded by a viral illness of vaccination days to weeks prior to presentation [3]. Whilst the peak incidence of ADEM is in childhood, there are an increasing number of cases reported in adults and the elderly [4].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Adult Onset Acute Disseminated Encephalomyelitis Following Appendicitis: A Case Report

Ld¹,

Lk²,

Df³

et al. 2016

J Neurol Neurosci

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Acute disseminated encephalomyelitis (ADEM) is a postinfectious autoimmune demyelinating disease of the central nervous system. ADEM typically presents with non-specific symptoms including nausea, vomiting, fever and headache. This then progresses to neurological deficits such as motor weakness, altered sensorium and often significant morbidity or mortality. Typically the infectious insult is thought to be a transient viral infection or vaccination. We describe a case of ADEM in a 50 yearold woman who presented to a rural emergency department fifteen days after surgery to remove a gangrenous appendix. She presented febrile and with signs of encephalopathy. Initial computed tomography (CT) findings showed a left parietal lesion with mass effect, and there was a moderate neutrophilia and lymphocytosis. Empirical therapy for a viral or bacterial cerebral infection was commenced including dexamethasone.After transfer to a tertiary referral center, and after five days of investigation and treatment and LP was performed and the diagnosis of ADEM was reached. Given the lack of response to steroids a course of intravenous immunoglobulin (IVIG) was commenced. Our patient responded extremely well on IVIG, showing substantial recovery within 24 hours. ADEM is a rare condition but is poorly understood in comparison to other diseases of similar incidence. Currently there is insufficient pathophysiological understanding and primary evidence to guide the use of treatment options even when the diagnosis is reached in a timely manner. Further case series or small trials are eagerly awaited. They may allow us to better formulate a treatment approach that can reduce the morbidity associated with even the best outcomes of our current therapies.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%