Akutni diseminirani encefalomijelitis

master's thesis

• Title (english): Acute disseminated encephalomyelitis
• Author: Kristina Bukal
• Mentor: Ivana Zadro (mentor)
• Committee member: Ervina Bilić (predsjednik povjerenstva)
• Committee member: Mario Habek (član povjerenstva)
• Committee member: Ivana Zadro (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Neurology); Zagreb
• Defense date and country: 2020-05-28, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Neurology

Abstract

Akutni diseminirani encefalomijelitis (ADEM) je imunološki posredovana, upalna demijelinizacijska bolest središnjeg živčanog sustava s pretežitim zahvaćanjem bijele tvari. Češći je u djece te uobičajeno nastaje nakon infekcije ili cijepljenja. Bolest je karakterizirana nespecifičnom kliničkom prezentacijom koja uključuje nagli nastup encefalopatije i multifokalnih neuroloških simptoma. Uobičajeno je monofazičnog tijeka, no može se pojaviti i u rekurentnom ili multifazičnom obliku kada se treba uzeti u obzir diferencijalna dijagnoza multiple skleroze. Akutni hemoragijski leukoencefalitis (AHL) fulminantna je varijanta ADEM-a karakterizirana brzom progresijom bolesti te razvojem teškog poremećaja stanja svijesti, a uobičajeno dovodi do fatalnog ishoda. Zbog nepostojanja patognomoničnih bioloških markera i velike sličnosti s drugim upalnim demijelinizacijskim poremećajima (MS, NMO) konačnu dijagnozu teško je postaviti. Dijagnoza se temelji na anamnezi, kliničkom statusu, MR-u središnjeg živčanog sustava te analizi cerebrospinalne tekućine. Nalaz magnetske rezonancije pokazuje multiple demijelinizacijske lezije najčešće smještene u subkortikalnoj i centralnoj bijeloj tvari SŽS-a, a ponekad i u sivoj tvari. Analiza likvora može pokazati blagu do umjerenu pleocitozu, povišene proteine te najčešće negativne oligoklonske vrpce. Često je potrebno produljeno kliničko i neuroslikovno praćenje bolesnika kako bi se potvrdila dijagnoza. U liječenju se najčešće koriste visoke doze intravenskih kortikosteroida dok se u težim oblicima bolesti primjenjuju intravenski imunoglobulini (IVIg) ili plazmafereza. Većina bolesnika u potpunosti se oporavi, a kod nekih zaostaju neurološki ispadi.

Abstract (english)

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated, inflammatory demyelinating disease of the central nervous system with predominant white matter involvement. It is more common in children and usually occurs after infection or vaccination. Disease is characterized by a non-specific clinical presentation which includes sudden onset of encephalopathy and multifocal neurological symptoms. It usually has a monophasic course, but it can also occur in recurrent or multiphasic form when the differential diagnosis of multiple sclerosis needs to be considered. Acute hemorrhagic leukoencephalitis (AHL) is a fulminant ADEM variant characterized by rapid disease progression and severely impaired consciousness, usually leading to fatal outcome. Due to the absence of pathognomonic biological markers and great similarity to other inflammatory demyelinating disorders (MS, NMO), a definitive diagnosis is difficult to establish. Diagnosis is based on medical history, clinical status, MRI of the central nervous system and cerebrospinal fluid analysis. Magnetic resonance image shows multiple demyelinating lesions most commonly located in the subcortical and central white matter of the CNS and sometimes in the gray matter. CSF analysis may show a mild to moderate pleocytosis, elevated protein and usually negative oligoclonal bands. Often, prolonged clinical and neuroimaging follow-up of the patient is required to confirm the diagnosis. High dose intravenous corticosteroids are most commonly used in treatment, whereas in more severe forms of the disease, intravenous immunoglobulins (IVIg) or plasmapheresis are used. Most patients recover completely, but some are left with neurological deficits.

• Keywords: akutni diseminirani encefalomijelitis; upalna demijelinizacijska bolest; multipla skleroza
• Keywords (english): acute disseminated encephalomyelitis; inflammatory demyelinating disease; multiple sclerosis
• Language: croatian
• URN:NBN: urn:nbn:hr:105:882553
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2022-06-08 08:27:25