Neuobičajeni klinički slučajevi koji oponašaju akutni diseminirani encefalomijelitis

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

A late-onset seizure in a child due to intracranial needle

Placing of sewing needles in the brain through the anterior fontanel is a rare entity. There are very few cases reported in literature. Most of them were asymptomatic, but some of them presented with seizure. We report here a 14-year-old boy, who was admitted to the Pediatric Neurology Department with a history of generalized tonic-clonic seizures due to sewing needle located in the frontal lobe

Interleukin-1β secretion in hippocampal sclerosis patients with mesial temporal lobe epilepsy

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a common medically intractable epilepsy syndrome. Although pathogenesis of HS still remains highly controversial, genetics may play a role as a predisposing factor. Previous evidence in a Japanese population revealed that the homozygotes for allele T at position −511 of the interleukin (IL)-1β gene promoter region (IL-1β-511 T/T) confers susceptibility to the development of HS. However, whether this polymorphism has an effect on IL-1β levels in MTLE-HS patients was not demonstrated. This study aimed to analyze the distribution of this particular polymorphism in a group of Turkish HS patients and correlate the polymorphism with IL-1β secretion from the lymphocytes, thus revealing a functional role for IL-1β in the etiopathogenesis of HS. A single base pair polymorphism at position −511 in the promoter region of the IL-1β gene was analyzed. The spontaneous and 1 ng/mL lipopolysaccharide-stimulated production of IL-1β by peripheral blood mononuclear cells after 4 and 24 h of incubation were measured by ELISA method. The heterozygous type (−511 C/T) was the most common genotype. There was no difference in frequency of allele −511 T between patients and controls. Analysis of IL-1β levels, genotype and allele distributions showed no significant difference among the groups (P>0.05). Nevertheless, it was seen that patients who carry a T allele at position -511 of the IL-1β gene had increased IL-1β levels. T-allele carriage may be important. Only IL-1β secretion from the lymphocytes has been assessed in this study. Considering the importance of IL-1β in the etiopathogenesis of HS, further studies are needed to evaluate locally produced IL-1β levels

Unusal Cinical Cases That Mimic Acute Disseminated Encephalomyelitis

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