Search

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated monophasic inflammatory demyelinating disorder of the central nervous system which poses a diagnostic challenge. We report on six cases of different etiologies that mimicked the clinical and radiologic findings of ADEM. The cases were collected from four different reference hospitals in Turkey. The same radiologist from the Akdeniz University Faculty of Medicine examined the magnetic resonance images of all patients. Three (50%) patients had antecedent infections. Initial symptoms of the patients were as follows: fever in 50%, altered consciousness in 33.3% and convulsions in 16.7% of patients. Neurologic examination showed long tract signs in 83.3%, ataxia in 50% and altered consciousness in 50% of patients. Cerebrospinal fluid examination revealed lymphocytic pleocytosis only in case 6. Four patients received steroid pulse therapy and one of these initially underwent intravenous immunoglobulin therapy. The patients’ definitive diagnoses were as follows: paraspinal neuroblastoma-associated paraneoplastic syndrome; histiocytic sarcoma; mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes; and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in one patient each, while two patients had hemophagocytic syndrome. The present case series demonstrated difficulties in diagnosing ADEM while revealing extremely rare disorders that mimic ADEM radiologically and clinically., Akutni diseminirani encefalomijelitis (ADEM) je immuno posredovana monofazna upalna demijelinizacijska bolest središnjega živčanog sustava koja je dijagnostički vrlo zahtjevna. Prikazuje se šest slučajeva različite etiologije gdje su klinički i radiološki nalazi oponašali ADEM. Slučajevi su prikupljeni iz četiri različite referentne bolnice u Turskoj. Slikovne prikaze dobivene magnetskom rezonancom u svih bolesnika pregledao je isti radiolog s Medicinskog fakulteta Sveučilišta u Akdenizu. Troje (50%) bolesnika imalo je prethodnu infekciju. Početni simptomi bili su groznica u 50%, poremećaj svijesti u 33,3% te konvulzije u 16,7% bolesnika. Neurološki pregled pokazao je znakove oštećenja srednjeg ili gornjeg dijela leđne moždine (long tract signs) u 83,3%, ataksiju u 50% te poremećaj svijesti u 50% bolesnika. Pregled likvora otkrio je limfocitnu pleocitozu samo u slučaju br. 6. Četiri bolesnika primilo je pulsnu steroidnu terapiju, a jedan od njih je prvotno bio na terapiji intravenskim globulinom. U bolesnika su postavljene sljedeće konačne dijagnoze: paraneoplastični sindrom udružen s paraspinalnim neuroblastomom; histiocitni sarkom; mitohondrijska miopatija, encefalopatija, laktična acidoza i epizode slične moždanom udaru (MELAS); i cerebralna autosomna dominantna arteriopatija sa subkortikalnim infarktima i leukoencefalopatijom (CADASIL) u po jednog bolesnika, te hemofagocitni sindrom u dvoje bolesnika. Ovaj niz slučajeva ukazuje na teškoće u dijagnosticiranju ADEM-a i istodobno pokazuje iznimno rijetke bolesti koje radiološki i klinički oponašaju ADEM.