Acute Necrotizing Encephalopathy Of Childhood Research Articles

Clinical characteristics and prognostic analysis of acute necrotizing encephalopathy of childhood: a retrospective study at a single center in China over 3 years.

Acute Necrotizing Encephalopathy of Childhood (ANEC) is a rare, fulminant neurological disease in children with unknown mechanisms and etiology. This study summarized the clinical characteristics, treatment, and prognosis of ANEC through a retrospective analysis, providing insights into the ANEC early diagnosis and prognosis assessment. Clinical data of children diagnosed with ANEC at the Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University from July 1, 2020, to June 30, 2023, were retrospectively analyzed. There were 25 cases, 14 males and 11 females, with a median age of 3 years. Hospital admissions were mainly in the winter (14/25, 56%) and spring (9/25, 36%). All patients presented with varying degrees of fever and altered consciousness, with 92% (23/25) experiencing high body temperatures (>39.1°C) and 88% (22/25) having a Glasgow coma scale (GCS) score of ≤8. Seizures were observed in 88% (22/25) of patients. Laboratory findings indicated 100% B lymphocyte activation (14/14), and 78% (14/18) of patients showed cytokine storm (interleukin (IL)-6, IL-8, IL-10, interferon (IFN)-α). Neuroimaging showed symmetrical thalamus involvement, commonly involving basal ganglia and brainstem regions. Viral infection (23/24, 96%) was the predominant etiological finding, with 42% (10/24) of cases due to SARS-CoV-2 infection and 42% (10/24) to influenza A virus infection. Multi-organ dysfunction occurred in 68% (17/25) of patients, and 52% (13/25) died. Correlation analysis revealed the death group exhibited higher proportion of male, lower GCS scores, higher IL-6 level and a greater likelihood of associated brainstem impairment (p < 0.05). ANEC is more prevalent in the winter and spring, and its etiology may be associated with B lymphocyte activation and cytokine storm following viral infections. Clinical manifestations lack specific features, with fever, consciousness disturbances, and seizures being the main presentations, particularly in cases of high fever and hyperpyrexia. ANEC progresses rapidly and has a high mortality rate. The child's gender, GCS score, IL-6 levels, and the presence of brainstem involvement can serve as important risk factors for assessing the risk of mortality.

A rare case of acute necrotising encephalitis: a case report

Acute necrotising encephalopathy of childhood (ANEC) is a rare brain disorder that occurs most commonly after viral infection, predominantly seen in countries like Japan and Taiwan. The goal of this article is to discuss a case of ANEC and highlight the need for early intervention and treatment, as the prognosis is usually poor. Diagnosis is based on clinical signs and radiological imaging- which shows the involvement of multiple parts of brain matter. There are no standardised guidelines for management, however, most literature supports emphasis on infection control, steroids for neuroprotection, and immunotherapy. Prompt intervention can prevent rapid neurological decline- which is seen in many cases.

Distinct Etiologies of RANBP2-negative Acute Necrotizing Encephalopathy of Childhood (P10-9.008)

<h3>Objective:</h3> We sought to determine the etiology of <i>RANBP2</i>-negative pediatric patients presenting with acute necrotizing encephalopathy of childhood (ANEC). <h3>Background:</h3> ANEC is a rare childhood syndrome associated with fulminant encephalopathy and characteristic neuroimaging typically precipitated by acute infection and often associated with heterozygous mutation in <i>RANBP2</i>. Additional genes implicated in ANEC include <i>CPT2, MC1DN1</i> and <i>ILAE4</i>, yet many patients who meet clinical criteria for ANEC do not have mutations in any known genes and the cause of these cases has remained cryptic. <h3>Design/Methods:</h3> We performed a multicenter trio-based whole exome analysis of 12 <i>RANBP2</i>-negative pediatric patients diagnosed with ANEC using our established pipeline and filtering criteria. Whole exome sequencing was performed using a TruSeq exome capture kit and run on an Illumina NovaSeq 6000. Fastq files were analyzed using our established GATK-based pipeline and filtered for rare variants using our published approach. We prioritized rare candidate genes to determine plausible candidates based on the existing literature. <h3>Results:</h3> 32 candidate genes survived filtering and were considered in our analysis. We identified three high-confidence candidate genes. The first was <i>SCN1A</i>, segregating a heterozygous <i>de novo</i> (p.F1765Cfs*28). SCN1A encodes a voltage-gated sodium channel subunit. Mutations in SCN1A are well-known causes of Dravet Syndrome and other forms of epilepsy, but have also been associated with a fulminant encephalopathy phenotype independent of seizure burden. The second was <i>TNFAIP3</i> (heterozygous <i>de novo</i> p.R728C), previously shown to lead to an autoinflammatory Behcet-like syndrome. The third was <i>PRF1</i> (homozygous p.R225W), associated with a hemophagocytic lymphocystiocytosis-like phenotype with encephalopathy, ataxia, deep white matter lesions, and increased intracranial pressure. <h3>Conclusions:</h3> Our study indicates that ANEC is genetically heterogeneous and highlights three additional genes that should be considered in individuals presenting with ANEC. Our results also showcase how monogenic conditions can impact immune system regulation and lead to neuroimmunologic disorders. <b>Disclosure:</b> Dr. Vaidyanathan has nothing to disclose. Somayeh Bakhtiari has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Xilis Inc. Somayeh Bakhtiari has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant (Bioinformatics) with Phoenix Childrens Hospital. Dr. Appavu has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Natus. The institution of Dr. Appavu has received research support from American Heart Association. The institution of Dr. Appavu has received research support from United States Department of Defense. Derek Neilson has nothing to disclose. The institution of Dr. Kruer has received research support from NIH NINDs. Dr. Kruer has received research support from Cure CP. Dr. Kruer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant with NHRSA.

Clinical, Laboratory, and Radiological Findings in Acute Necrotising Encephalopathy of Childhood: A Case Series

Acute Necrotising Encephalopathy of Childhood (ANEC) is a fatal disease diagnosed on basis of clinical and typical Magnetic Resonance Imaging (MRI) findings. The prognosis of this encephalopathy is generally poor, with fewer than 10% of cases recovering completely. Patients with a favourable outcome had reversible neuroimaging findings. Long-term sequelae commonly include neurologic deficits such as spasticity, epilepsy, and involuntary movements. In this case series, five cases of ANEC were reported. The children included in this series ranged in age from nine months to 16 years. All the children were female, and their presenting symptoms included seizures (n=5), altered sensorium (n=5), features of raised intracranial pressure (n=5), and posturing (n=1). Neuroimaging revealed characteristic lesions in the thalamus with varied involvement of other parts of the brain in all cases (n=5). Cerebrospinal Fluid (CSF) analysis was normal in the majority of children. Four out of five cases survived and responded to early steroids, intravenous immunoglobulin, physiotherapy, and rehabilitation. One child had complete recovery, with three children experiencing disabilities and one child succumbing to the disease.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

Acute Necrotizing Encephalopathy of Childhood (ANEC): A Case Report

Acute necrotizing encephalopathy (ANE) is an infrequent disease in childhood with Specific clinical complications. To date, the highest incidence of this disease has been reported in East Asian children. The clinical features of the disease include convulsions, frequent vomiting, and coma. Most of these patients die within days or cause severe neurological complications. The disease is characterized by symmetric and necrotic multifunctional lesions in the thalami and brainstem. We reported a 5-year-old girl infant from Besat Hospital, Sanandaj, Kurdistan, located in west of Iran.

Outcomes in influenza and RANBP2 mutation‐associated acute necrotizing encephalopathy of childhood

To evaluate clinical and imaging features in patients with acute necrotizing encephalopathy of childhood (ANEC) to identify predictors of RANBP2 mutations, influenza association, and long-term outcomes. A retrospective chart review in patients with ANEC (2012-2020) seen at a tertiary pediatric center was performed. Children were included if they had acute inflammatory lesions in the basal ganglia and pons. Variables included presenting features, imaging characteristics, RANBP2 gene testing, nasopharyngeal swab findings, therapies, and long-term outcomes. Twenty patients were included (average age at presentation 3y 6mo, interquartile range3y 7mo, SD2y 8mo; 14 females, six males). Three of the 20 experienced recurrences; one of the 20 died. Ten patients were influenza positive. Seven patients were RANBP2 mutation positive. A higher likelihood of hemorrhage was observed in patients who were influenza positive compared to influenza negative (p=0.048). Patients with influenza had a higher degree of thalamic hemorrhage (2, p=0.035) and greater extent of diffusion restriction (3, p=0.035) in semiquantitive analysis. Children with ANEC who are positive for influenza are more likely to have hemorrhage and greater thalamic swelling. RANBP2 status was predictive of relapse but not predictive of overall outcome.

Clinical, laboratory, radiologic profile, and outcome in acute necrotizing encephalopathy of childhood (ANEC) – A case series

Acute necrotizing encephalopathy of childhood (ANEC) is a potentially devastating illness characterized by fever, acute encephalopathy, and bilateral thalamic lesions. This case series is a retrospective review of clinical, laboratory, neuroimaging data of 19 children diagnosed with ANEC over 9 years at a single-center from south India. The median age was 84 months (interquartile range 25.5–120 months). All children had acute febrile encephalopathy, 12 had seizures and hypotension each, 8 had vomiting, and 5 had abnormal posturing. The liver transaminases were raised almost all children. All had bilateral thalamic lesions followed by lesions in cerebral white-matter, brain stem, and cerebellar white matter. Microbial etiology was established in 9 patients (7 dengue, 1 H1N1, 1 influenza A). Neurodeficits at discharge were seen in 63% and at follow-up in 33.3% cases. Neurodeficits at follow-up were seen in all cases with age &lt;48 months, and only 11.1% with age &gt;48 months age (P = 0.018).

Plasma exchange therapy for acute necrotizing encephalopathy of childhood.

ABSTRACTImportanceAcute necrotizing encephalopathy (ANE) is a rare disease with high mortality. Plasma exchange (PLEX) has recently been reported to treat ANE of childhood (ANEC), but its efficacy is uncertain.ObjectiveThis study aimed to investigate the effectiveness of PLEX on ANEC.MethodsA retrospective study was conducted in four pediatric intensive care units from December 2014 to December 2020. All patients who were diagnosed with ANEC were included; however, these patients were excluded if their length of stay was less than 24 h. Participants were classified into PLEX and non‐PLEX groups.ResultsTwenty‐nine patients with ANEC were identified, 10 in the PLEX group and 19 in the non‐PLEX group. In the PLEX group, C‐reactive protein, procalcitonin, alanine aminotransferase, and aspartate aminotransaminase levels were significantly lower after 3 days of treatment than before treatment (13.1 vs. 8.0, P = 0.043; 9.8 vs. 1.5, P = 0.028; 133.4 vs. 31.9, P = 0.028; 282.4 vs. 50.5, P = 0.046, respectively). Nine patients (31.0%, 9/29) died at discharge, and a significantly difference was found between the PLEX group and non‐PLEX group [0 vs. 47.4% (9/19), P = 0.011]. The median follow‐up period was 27 months, and three patients were lost to follow‐up. Thirteen patients (50.0%, 13/26) died at the last follow‐up, comprising three (33.3%, 3/9) in the PLEX group and ten (58.8%, 10/17) in the non‐PLEX group, but there was no significant difference between the two groups (P = 0.411). Three patients (10.3%, 3/29) fully recovered.InterpretationPLEX may reduce serum C‐reactive protein and procalcitonin levels and improve liver function in the short term. PLEX may improve the prognosis of ANEC, and further studies are needed.

Rehabilitation outcomes in Acute Necrotizing Encephalopathy of Childhood – A Retrospective Study

Aims & Objectives: To assess the outcome of early physiotherapy and rehabilitation in children with AcuteNecrotizing Encephalopathy of Childhood (ANEC).Methods: Retrospective review of 10 infants & children with ANEC at Department of Paediatrics,S.N.Medical College & HSK Hospital, Bagalkot from January 2013 to December 2019. ANEC wassuspected based on clinical and radiological characteristics and diagnosis was made based on diagnosticcriteria proposed by Mizuguchi et al. Clinical and radiological (MRI brain characteristics) findings andresponse to standard therapy, early physiotherapy and rehabilitation were assessed in all cases. All caseswere followed for evaluation of neurodevelopmental outcome.Results: The age ranged from 6 months to 11 years (7 female, 3 male). All cases had precedent viralillnesses and had fever, coryza, diarrhoea. The initial neurological symptoms included altered sensorium(n=3), seizures and status epilepticus (n=7), focal neurological signs, gait disturbances (n=2) and diplopia(n=1). MRI brain revealed characteristic thalamus involvement with varied involvement of midbrain, pons,medulla (n=10). 9 out of 10 cases survived, responded to standard medical therapy , early physiotherapy andrehabilitation. 6 children had complete recovery with minimal disability in 3 cases.Conclusions: Early detection and appropriate treatment improves outcome in ANEC. Physiotherapy helpsin remarkable improvement in the regain of tone, reflexes and movements of limbs

Acute necrotizing encephalopathy of childhood: Not always devastating

Acute necrotizing encephalopathy of childhood (ANEC) is an uncommon cause of acute onset febrile encephalopathy, which progresses rapidly and has poor outcomes. We reported a 4-year-old female who presented with acute febrile encephalopathy, raised intracranial pressure (ICP), and respiratory failure. The brain imaging was suggestive of ANEC. The treatment included antibiotics, antivirals, mechanical ventilation, measures to lower ICP, intravenous immunoglobulin, and supportive care in the Pediatric intensive care unit with which she made a remarkable recovery.

Expanding the Phenotype of Neurological Manifestations of Dengue Fever

Background: Neurological manifestations following dengue fever are increasingly reported and include encephalopathy, encephalitis, neuro-ophthalmic manifestations or Guillain–Barre syndrome-like presentation. Herein, we expand the clinical spectrum of this neurological repertoire by describing two patients presenting with acute necrotizing encephalopathy of childhood (ANEC) following dengue fever. Clinical Description: Both patients presented with features of acute febrile encephalopathy following an acute febrile illness that proved to be dengue fever. Laboratory investigations revealed thrombocytopenia (patient 1), transaminitis and normal findings on cerebrospinal fluid analysis (patient 1 and 2). Both the patients tested positive for NS1 antigen and dengue serology. Neuroimaging revealed bilateral symmetrical thalamic lesions with cerebellar, midbrain, and cortical white matter lesions suggestive of ANEC. Management: Patient 1 received supportive treatment while patient 2 received pulse methylprednisolone therapy along with supportive treatment. Both the patients had complete clinic-radiological recovery. Conclusion: ANEC following dengue fever may have a relatively benign course, besides the fulminant course that has been described earlier in a single case.

Encefalopatía necrotizante aguda asociada a influenza A

La encefalopatía necrotizante aguda (ENA) es una patología rara, caracterizada por compromiso de conciencia y presencia de múltiples lesiones encefálicas simétricas localizadas principalmente en tálamo. Se asocia a alta letalidad e importantes secuelas.Objetivo: Describir el caso de un paciente escolar con ENA asociada a influenza-A con evolución favorable.Caso Clínico: Paciente de 6 años de edad, con historia de 3 días de evolución de síntomas respiratorios altos asociados a fiebre (39 °C). Veinticuatro horas previo a la consulta destacaba compromiso de conciencia cualicuantitativo. Se realizó punción lumbar con proteinorraquia leve. En resonancia magnética (RM) se identificó focos de restricción a la difusión bilaterales de distribución simétrica, talámicos, en cuerpos mamilares, periacueductales, de tegmento pontino, hipocampales y en ambas cápsulas externas, asociado a componente hemorrágico y edema vasogénico, sugerente de ENA. Recibió tratamiento empírico con metilprednisolona y oseltamivir. Posteriormente, se recibió resultado positivo para virus influenzaAH1. Dado diagnóstico, se decidió administrar inmunoglobulina, evolucionando lento pero favorablemente. Al alta levemente bradipsíquico, con disminución de agudeza visual, lenguaje espontáneo y marcha con apoyo. A los 6 meses de seguimiento presentaba lenguaje y marcha normales, persistiendo alteración visual a derecha.Conclusión: Nuestro paciente presentó una ENA cuyo diagnóstico y manejo oportunos se asociaron a una favorable evolución neurológica en el largo plazo. Si bien la ENA es una patología infrecuente, la morbimortalidad asociada es altísima, por lo que resulta de gran importancia tener un alto grado de sospecha, a fin de solicitar estudio imagenológico dirigido, buscar causas infecciosas relacionadas e iniciar un manejo oportuno.

Acute necrotizing encephalopathy of childhood: an under-recognized and diverse clinico-radiological syndrome

Background: With advances in neuroimaging, acute necrotizing encephalopathy of childhood (ANEC) is being recognized as an acute encephalopathy with a characteristic rapidly deteriorating neurological course with poor neuro-developmental outcomes with high mortality and morbidity rates. Indian data of ANEC is primitive and hence this study was planned. The objective of the study was to evaluate the clinical, radiological characteristics and its outcomes in children with ANEC.Methods: Retrospective review of 10 infants and children with ANEC at Department of Paediatrics, S.N. Medical College and HSK Hospital, Bagalkot from January 2013 to December 2019. ANEC was suspected based on clinical and radiological characteristics and diagnosis was made based on diagnostic criteria proposed by Mizuguchi et al. Clinical and radiological (Magnetic resonance imaging (MRI) brain characteristics) findings and response to early pulse dose steroid therapy and supportive treatment were assessed in all cases. All cases were followed for evaluation of neurodevelopmental outcome and response to physiotherapy was noted in all the cases.Results: Total 10 cases were analysed (age ranged from 6 months to 11 years). Sex distribution male to female was 3:7. All cases had precedent viral illnesses and had fever, coryza, diarrhoea. The initial neurological symptoms included seizures and status epilepticus (n=7), altered sensorium (n=3), focal neurological signs , gait disturbances (n=2) and diplopia (n=1). MRI brain revealed characteristic thalamus involvement with varied involvement of midbrain, pons, medulla (n=10). Other findings were cavitation (n=5), haemorrhage (n=4), minimal residual lesions (n=4), cerebral atrophy (n=1), normal brain study (n=5) on follow-up. 9 out of 10 cases survived, responded to early physiotherapy and rehabilitation. 6 children had complete recovery with minimal disability in 3 cases.Conclusions: ANEC is a clinico-radiological syndrome. Early detection and appropriate treatment improves outcome in ANEC.

Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia.

Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving the thalami as well as the supra and infra-tentorial areas. Treatment modalities are not well-established; empirical treatment with antibiotics and antiviral agents is the initial step, followed by steroids and immunoglobulin, as well as supportive care. Patients with ANEC have a variable prognosis, but mortality is very high.Methods: A retrospective chart review of patients diagnosed with ANEC in five tertiary centers from January 2015 to October 2018 was performed. Clinical and radiological findings, as well as the therapeutic approach and outcomes, were described.Results: Twelve children were included ranging in age from 10 months to 6 years. All patients presented with preceding febrile illness, altered level of consciousness, and seizure. Radiological features showed abnormal signals in the thalami, and five patients (41.7%) had brainstem involvement. All patients received empirical treatment with antibiotics and antiviral agents. Ten patients (83.3%) received intravenous immunoglobulin (IVIG) and IV Methylprednisolone therapy. Outcomes were variable ranging from good outcomes with minimal neurological deficits to poor outcomes and death in 25% of cases.Conclusion: ANEC is a rare fulminant disease in children. The treatment is challenging. Early interventions with the use of IVIG and IV Methylprednisolone may change the outcome; however, further studies are needed to establish a consensus guideline for the management.

Imaging diagnosis of acute necrotizing encephalopathy of childhood

Objective To analyze the imaging features of acute necrotizing encephalopathy of childhood (ANEC), and try to investigate its potential clinical value. Methods The clinical and imaging findings of 22 children from Wuhan Children′s Hospital diagnosed with ANEC were retrospective analyzed, from January 2013 to October 2018. All children were presented with hyperpyrexia and rapidly developed into rapid neurological deterioration after prodromic infection. In the initial imaging examination, all patients underwent head MRI, and 6 cases underwent additional head CT. During MRI follow-up, 4 cases were lost, 6 cases were followed up only once ( 14 days).The presence of hemorrhage and encephalomalacia in thalamus, brainstem, white matter and basal ganglia was carefully investigated throughout the follow-up. Results For the imaging manifestations of ANEC, bilateral thalamus were involved in all children. Other symmetrical lesions included white matter (14 cases), basal ganglia (15 cases), brainstem (16 cases), cerebellum (9 cases), corpus callosum (2 cases) and hippocampus (1 case). There were 3 children with asymmetric lesions, which were found in white matter (2 cases) and cerebellum (1 case).In the acute phase, the most typical head MRI showed tricolor pattern(high signal intensity in the center with surrounding low-signal, and hyperintense signals in the periphery of thalamus) or bicolor pattern(low signal in the central thalamus with surrounding hyperintense signals) of the thalamus on the apparent diffusion coefficient (ADC) imaging. Hemorrhage and encephalomalacia on MRI may suggest poor clinical outcome. Conclusions ANEC is a rapid progressive encephalopathy with typical imaging features. Hemorrhage and encephalomalacia on MRI may be associated with poor prognosis. Key words: Brain diseases; Child; Magnetic resonance imaging; Thalamus

3 Cases of Acute necrotizing encephalopathy,is this an epidemic or just an incidental?

Acute necrotizing encephalopathy of childhood (ANEC) is a disease, characterized by a respiratory or gastrointestinal infection, accompanied with fever, rapid alteration of consciousness, and seizures. The clinical characteristics of ANEC include acute encephalopathy following a viral infection, seizure, altered consciousness, and absence of cerebrospinal fluid (CSF) pleocytosis, with an occasional increase in the level of proteins. This disease is almost exclusively seen in previously healthy infants and children from East Asia. Serial magnetic resonance imaging (MRI) examinations have demonstrated symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. ANEC has a poor prognosis with high morbidity and mortality rates. Herein, we present three cases of ANEC, who were referred to Bu-Ali Hospital of Ardabil, Iran during two weeks. Report of these three cases promoted the idea of an epidemic. The purpose of this case series was to raise the issue that ANEC may occur as an epidemic.

Acute necrotizing encephalopathy of childhood secondary to influenza A H1N1 virus

Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rare but distinctive type of encephalopathy predominantly involving infants and young children. This case report describes the characteristic neuroimaging findings which can aid in early diagnosis of ANEC. Case Presentation: An 11-year-old boy presented with a 2-day history of fever and flu-like illness and was given symptomatic treatment. He subsequently deteriorated on day 3 of illness with generalized tonic-clonic seizure and impaired consciousness. Magnetic resonance imaging done revealed abnormal signal intensities at bilateral thalami, brainstem, and cerebellum, which are suggestive of ANEC, correlating with his clinical presentation. Treatment was started immediately, but the patient had a very poor recovery. Conclusion: ANEC is a very rare encephalopathy and has very high mortality and morbidity with severe neurologic sequelae. Early diagnosis is important so that appropriate management can be provided.

Acute necrotizing encephalopathy of childhood (ANEC)- Trilaminar sign on MRI

Acute necrotizing encephalopathy of childhood (ANEC)- Trilaminar sign on MRI

Acute necrotizing encephalopathy as a H1N1 complication: A rare case

Acute necrotizing encephalopathy of childhood (ANEC) is a rare type of encephalopathy seen generally in previously healthy children or infants of East Asian population. The pathology generally considered to be triggered by infectious diseases, mainly by viral agents. In this case report a previously healthy, 2-year –old, male children diagnosed as ANEC, occuring secondarily to H1N1 infection is presented. On MR images hyperintensity is present at bilateral thalamus and brainstem on T2WI and FLAIR. On gradient echo sequences, hemorrage is also present at bilateral thalamic region. ANEC does not have a specific symptomor typical neurological sign. It is important to remember that the typical lesions are generally seen in the gray matter of bilateral thalami. It is reported that administration of steroids within first 24 hours just after ANEC diagnosis was related to a better prognosis in patients without brain stem involvement. Early diagnosis mostly depends on knowing the imaging characteristics of ANEC and being aware of this rare entitiy.