Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management

Many important central nervous system (CNS) syndromes can develop following microbial infections. The most severe forms of post-infectious encephalitis include acute disseminated encephalomyelitis (ADEM), acute hemorrhagic leukoencephalitis and Bickerstaff's brainstem encephalitis. ADEM is an inflammatory demyelinating disorder of the CNS. It typically follows a minor infection with a 2-30 days latency period and is thought to be immune-mediated. It is clinically characterized by the acute onset of focal neurological signs and encephalopathy. Patients can require intensive care unit admission because of coma, seizures or tetraplegia. Cerebrospinal fluid analysis usually shows lymphocytic pleocytosis but, unlike viral or bacterial encephalitis, no evidence of direct CNS infection is found. There are no biologic markers of the disease and cerebral magnetic resonance imaging is essential to diagnosis, detecting diffuse or multifocal asymmetrical lesions throughout the white matter on T2- and FLAIR-weighted sequences. High-dose intravenous steroids are accepted as first-line therapy and beneficial effects of plasma exchanges and intravenous immunoglobulins have also been reported. Outcome of ADEM is usually favorable but recurrent or multiphasic forms have been described.

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“…Surgical decompression may be required to reduce raised intracranial pressure refractory to medical management. 35…”

Section: Initial Management Of Patients General Supportive Measuresmentioning

confidence: 99%

Post-infectious encephalitis in adults: Diagnosis and management

Sonneville

Klein

Broucker

et al. 2009

Journal of Infection

111

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“…It is relatively simple to perform and is not associated with significant risks or complications. The effectiveness of decompressive craniectomy with hyperacute forms of ADEM has been reported [7,[17][18][19]. It is particularly effective in acute neurological deterioration associated with significant mass effect and brain herniation, particularly in patients with compromised or at risk renal function, in whom the use of osmotic diurectics or other measures to control intracranial pressure may be limited.…”

Section: Discussionmentioning

confidence: 99%

“…A prodrome of fever, headache, nausea, and vomiting just prior to neurologic decline is usually seen, but in approximately 20-30% of patients, this may be absent [1,[5][6][7][8]20]. Presentation of ADEM varies depending on location of the lesions; Motor weakness, sensory loss, cranial nerve palsies, optic neuritis with visual loss, speech deficits, spinal cord or pyramidal signs, and mental status changes have been reported, and symptoms evolve over a period of hours to days [1,20].…”

Section: Introductionmentioning

confidence: 99%

Fulminant acute disseminated encephalomyelitis in renal transplant patient treated by decompressive craniectomy: a case report

Spencer¹,

Vela‐Duarte²,

Sandrian³

et al. 2014

Neurosci Discov

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Background: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease of the central nervous system that most commonly afflicts children and young adults and may be rarely seen in renal transplant patients. Most cases resolve with medical management; however, in some instances, the initial episode may evolve into a fulminant illness with malignant cerebral edema and elevated intracranial pressure that can result in a herniation syndrome with possible fatal consequences. Immediate relief of intracranial pressure is a priority and medical therapies alone may not suffice or may be precluded by compromised renal function. Case description: A case is presented of fulminant ADEM in a pediatric renal transplant patient associated with intracranial hypertension and an acute herniation syndrome with rapid neurological decline that was effectively treated with a decompressive craniectomy. The procedure was life-saving and resulted in a favorable outcome without inflicting any additional morbidity. Conclusion: Decompressive craniectomy is an effective way to rapidly reduce intracranial pressure in patients with malignant cerebral edema associated with fulminant ADEM and rapid neurological decline.

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“…Le traitement repose sur la corticothérapie systémique fortes doses, éventuellement en association avec les immunoglobulines polyvalentes ou les échanges plasmatiques. Certaines observations soulignent l'intérêt d'un traitement précoce agressif associant corticoïdes, immunosuppresseurs et éventuellement décompression chirurgicale [11,29,[32][33][34].…”

Section: Biopsie Cérébraleunclassified

Encéphalomyélite aiguë disséminée et encéphalites post-infectieuses graves

Sonneville

Wolff

2007

Réanimation

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L'encéphalomyélite aiguë disséminée, acute disseminated encephalomyelitis, (ADEM), est une maladie inflammatoire démyélinisante du système nerveux central (SNC). Également appelée encéphalite post-infectieuse, elle est liée à un mécanisme auto-immun et s'installe typiquement dans les suites d'une infection après un intervalle libre de deux à 30 jours. L'ADEM est caractérisée cliniquement par un tableau d'encéphalopathie aiguë avec signes neurologiques multifocaux. Les patients peuvent nécessiter une admission en réanimation du fait de troubles de la conscience, de crises convulsives ou d'une tétraplégie. L'analyse du liquide céphalorachidien peut montrer une méningite lymphocytaire, mais on ne retrouve pas d'infection évolutive du SNC. Il n'existe pas de marqueur spécifique de la maladie et l'imagerie par résonance magnétique cérébrale est essentielle au diagnostic, permettant de mettre en évidence des lésions multifocales de la substance blanche du SNC sur les séquences T2 et FLAIR. Le traitement de l'ADEM est basé sur les corticoïdes fortes doses, éventuellement associés aux immunoglobulines polyvalentes ou aux échanges plasmatiques. Le pronostic est généralement favorable sous traitement, des récurrences peuvent néanmoins survenir dans l'évolution.

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Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management

Cited by 23 publications

References 14 publications

Post-infectious encephalitis in adults: Diagnosis and management

Post-infectious encephalitis in adults: Diagnosis and management

Fulminant acute disseminated encephalomyelitis in renal transplant patient treated by decompressive craniectomy: a case report

Encéphalomyélite aiguë disséminée et encéphalites post-infectieuses graves

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