BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare, but potentially life threatening neurological condition in children. This study aimed to investigate its clinical spectrum, diagnostic and therapeutic dilemma, and prognosis. METHODS: Twelve children with ANE were included in the study. The diagnosis was made by clinical and radiological characteristics from January 1999 to December 2017 and their clinical data were retrospectively analyzed. RESULTS: A total of 12 children aged 6 to 93 months at onset (5 male: 7 female) were evaluated. The etiology was found in 4 of them (influenza A, H1N1; coxsackie A 16; herpes simplex virus; and RANBP2 gene/mycoplasma). The most common initial presentations were seizures (67%) and altered mental status (58%). The majority of the subjects showed elevation of aspartate aminotransferase/alanine aminotransferase with normal ammonia and increased cerebrospinal fluid protein without pleocytosis. Magnetic resonance imaging revealed increased T2 signal density in bilateral thalami in all patients, but the majority of the subjects (67%) also had lesions in other areas including tegmentum and white matter. Despite the aggressive immunomodulatory treatments, the long-term outcome was variable. One child and two sisters with genetic predisposition passed away. CONCLUSION: ANE is a distinctive type of acute encephalopathy with diverse clinical spectrum. Even though the diagnostic criteria are available, they might not be watertight. In addition, treatment options are still limited. Further studies for better outcome are needed.
Ammonia ; Aspartic Acid ; Brain Diseases ; Cerebrospinal Fluid ; Child ; Diagnosis ; Genetic Predisposition to Disease ; Humans ; Leukocytosis ; Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies ; Seizures ; Siblings ; Simplexvirus ; White Matter

Acute hemorrhagic encephalomyelitis (AHEM) is a severe form of encephalitis characterized by fulminant clinical course and presence of hemorrhagic necrosis of the white matter. Mycoplasma pneumoniae (M. pneumoniae) have severe central nervous syndrome complication with encephalitis as that most common pediatric manifestations, but have been extremely rare report with AHME. A 10-year-old boy was referred to emergency room because of drowsy mental status, weakness of left side extremities and truncal ataxia. His deep tendon reflexes were hyperactive, neck stiffness sign and Babinski sign were both positive. Motor power were decreased on the both left upper and lower extremities. The sequences of T2-weighted and gradient recalled echo (GRE) showed hyper-intense lesions on multifocal white natter areas with hemorrhagic signal. Cerebrospinal fluid (CSF) analysis showed the pleocytosis with neutrophil dominant. The results of deoxyribonucleic acid (DNA) test and culture of M. pneumoniae were reported negative in CSF, but immunoglobulin M (IgM) was positive in blood. He received intravenous high dose corticosteroid and macrolide. After discharge, his neurologic function gradually returned to normal including sitting and standing without support. We reported the previously healthy boy with M. pneumonia related AHEM. The early diagnosis with brain MRI and the aggressive immunosuppressive treatment may be beneficial for recovery.
Ataxia ; Brain ; Cerebrospinal Fluid ; Child ; DNA ; Early Diagnosis ; Emergency Service, Hospital ; Encephalitis ; Extremities ; Humans ; Immunoglobulin M ; Leukocytosis ; Leukoencephalitis, Acute Hemorrhagic* ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Mycoplasma pneumoniae* ; Mycoplasma* ; Neck ; Necrosis ; Neutrophils ; Pneumonia ; Pneumonia, Mycoplasma* ; Reflex, Babinski ; Reflex, Stretch ; White Matter

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Ankle ; Cerebrospinal Fluid ; Cough ; Demyelinating Diseases* ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated ; Extremities ; Female ; Fever ; Guillain-Barre Syndrome ; Humans ; Knee ; Korea ; Leukocytosis ; Lymphocytes ; Magnetic Resonance Imaging ; Nervous System Diseases ; Neural Conduction ; Neuroimaging ; Peripheral Nervous System* ; Pons ; Reflex, Stretch ; Spinal Cord

PURPOSE: To explore clinical features and neurologic complications of pediatric enteroviral meningitis, and to evaluate risk factors according to the presence of cerebrospinal fluid (CSF) pleocytosis and neurologic complications. METHODS: Retrospective review of medical records of patients aged between 1 month and 18 years who were found positive for CSF enterovirus reverse transcription polymerase chain reaction in Dankook University Hospital from March 2009 to February 2015. RESULTS: A total of 151 children was enrolled in the study. Ninety four patients were males and the age at diagnosis was 40.9±47.0 months. Most common season of admission was summer (n=99, 65.6%). Most frequent presenting symptom was fever followed by poor oral intake, decreased activity, and headache. Fifty five (36.4%) patients had no CSF pleocytosis. Complications were observed in 4 (2.6%). Mean blood white blood cell, CSF protein levels were higher, and CSF glucose levels were lower in group with pleocytosis (P < 0.05). There were more seizure development and mental changes, higher peak body temperature, longer fever duration, and longer duration from symptom onset to CSF tapping in group with complications (P < 0.05). All were boys and mean age at admission was younger in group with complications, without statistically significant difference. CONCLUSION: Relatively high proportion of children with enteroviral meningitis showed no pleocytosis. Complications such as meningoencephalitis, refractory status epilepticus, or recurrent seizure rarely occurred, although most had good clinical outcomes.
Body Temperature ; Cerebrospinal Fluid ; Child ; Diagnosis ; Enterovirus ; Fever ; Glucose ; Headache ; Humans ; Leukocytes ; Leukocytosis ; Male ; Medical Records ; Meningitis* ; Meningoencephalitis ; Polymerase Chain Reaction ; Retrospective Studies ; Reverse Transcription ; Risk Factors ; Seasons ; Seizures ; Status Epilepticus

Infective endocarditis (IE) caused by methicillin-resistant Staphylococcus aureus (MRSA) has become a worldwide concern. We present a case of a 12-year-old child with IE of the native mitral valve due to MRSA infection after an invasive dental procedure. Based on the clinical symptoms and the presence of cerebrospinal fluid pleocytosis, the patient was initially diagnosed with presumed bacterial meningitis and treated with empiric antibiotics. On the third day of hospitalization, MRSA was cultured from the initial blood samples and vegetation was observed on the mitral valve during an echocardiogram, findings which are compatible with a diagnosis of IE. The revised guidelines for antibiotic prophylaxis for the prevention of IE advise that IE prophylaxis for dental procedures is reasonable only for patients with underlying cardiac conditions, who are at the highest risk of adverse outcomes from IE. However, in this case, the patient had no high risk factors indicative of IE prophylaxis, except for mitral valve prolapse. She had no recurrence of IE over a follow-up period of 12 months.
Anti-Bacterial Agents ; Antibiotic Prophylaxis ; Cerebrospinal Fluid ; Child ; Diagnosis ; Endocarditis* ; Follow-Up Studies ; Hospitalization ; Humans ; Leukocytosis ; Meningitis* ; Meningitis, Bacterial ; Methicillin Resistance* ; Methicillin-Resistant Staphylococcus aureus* ; Mitral Valve ; Mitral Valve Prolapse ; Recurrence ; Risk Factors

PURPOSE: Meningitis is among the most common infections affecting the central nervous system. It can be difficult to determine the exact pathogen responsible for the infection and patients are often treated with empiric antibiotics. This study was conducted to identify the most common clinical characteristics of enteroviral meningitis in children and evaluate the diagnostic efficacy of reverse transcriptase-polymerase chain reaction (RT-PCR) for early detection of an enterovirus. METHODS: We analyzed the medical records of children admitted to Korea University Medical Center and diagnosed with meningitis on the basis of cerebrospinal fluid (CSF) analysis and RT-PCR from CSF and other samples from January 2010 to August 2013. RESULTS: A total of 333 patients were enrolled and classified into four groups based on diagnosis: enteroviral meningitis (n=110), bacterial meningitis (n=23), other viral meningitis (n=36), and unknown etiology (n=164). Patients with bacterial meningitis were younger than those in the other groups (P<0.001). Pleocytosis in CSF was similar across all groups. Of patients in the enteroviral meningitis group, 92.7% were diagnosed based on RT-PCR findings. Mean length of hospital stay for patients with enteroviral meningitis was 6.08 days, which was significantly shorter than that for patients with meningitis of bacterial etiology (19.73 days, P<0.001). CONCLUSION: Diagnosis of enteroviral meningitis before viral culture results are available is possible using RT-PCR. Accurate diagnosis reduces the length of hospital stay and helps to avoid unnecessary empiric antibiotic treatment.
Academic Medical Centers ; Anti-Bacterial Agents ; Central Nervous System ; Cerebrospinal Fluid ; Child ; Diagnosis ; Early Diagnosis* ; Enterovirus ; Humans ; Korea ; Length of Stay ; Leukocytosis ; Medical Records ; Meningitis* ; Meningitis, Bacterial ; Meningitis, Viral ; Reverse Transcriptase Polymerase Chain Reaction

OBJECTIVE: Enteroviruses are major causes of aseptic meningitis in children. This study aimed to describe the clinical manifestations of enteroviral meningitis according to the presence of cerebrospinal fluid (CSF) pleocytosis, and to investigate the factors influencing the CSF pleocytosis in children with this condition. METHODS: Eighty children with enteroviral meningitis treated at Soonchunhyang University Hospitals in Seoul and Bucheon between July 2012 and August 2013 were enrolled. The patients were diagnosed by reverse transcription-polymerase chain reaction (RT-PCR), and the clinical variables were compared according to the presence of CSF pleocytosis. RESULTS: Of the 80 patients, 54 (67.5%) and 26 (32.5%) patients had and did not have CSF pleocytosis, respectively. Forty-eight (60%) patients were male, and the median age was 63 months (range, 2 to 192 months). Seventy-six (95%), 67 (83.7%), 51 (63.7), and 2 (2.5%) patients presented with fever, headache, vomiting, and seizure, respectively. Increased CSF protein and pressure were associated with CSF pleocytosis. However, age, peripheral white blood cell count, C-reactive protein, CSF glucose, CSF/serum glucose ratio, and onset-puncture time interval were not associated with the presence of CSF pleocytosis. CONCLUSION: This study demonstrated a high proportion of non-pleocytic enteroviral meningitis in children, and identified several clinical manifestations that were associated with CSF pleocytosis. The findings of this study may help us better understand the characteristics of the disease and facilitate early diagnosis and treatment of enteroviral meningitis. During the outbreak seasons of enteroviral meningitis, the importance of continuous surveillance of enteroviruses and rapid RT-PCR testing should be emphasized.
C-Reactive Protein ; Cerebrospinal Fluid* ; Child* ; Early Diagnosis ; Enterovirus ; Fever ; Glucose ; Gyeonggi-do ; Headache ; Hospitals, University ; Humans ; Leukocyte Count ; Leukocytosis* ; Male ; Meningitis* ; Meningitis, Aseptic ; Pediatrics ; Seasons ; Seizures ; Seoul ; Vomiting

PURPOSE: The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and has been evaluated in several studies. In the present study, we examined the usefulness of BMS in South Korean patients. METHODS: This study included 1,063 patients with CSF pleocytosis aged between 2 months and 18 years. The BMS was calculated for all patients, and the sensitivity and negative predictive value (NPV) of the test were evaluated. RESULTS: Of 1,063 patients, 1,059 (99.6%) had aseptic meningitis (AM). Only four patients (0.4%) had BM. The majority of patients (98%) had a BMS of < or =1, indicating a diagnosis of AM. The BMS was 0 in 635 patients (60%) and 1 in 405 patients (38%). All four BM patients had a BMS of > or =4. CONCLUSION: To our knowledge, this is the first study to investigate the diagnostic strength of the BMS in South Korea. In our study, the BMS showed 100% sensitivity and 100% NPV. Therefore, we believe that the BMS is a good clinical prediction rule to identify children with CSF pleocytosis who are at a risk of BM.
Anti-Bacterial Agents ; Cerebrospinal Fluid* ; Child* ; Decision Support Techniques ; Diagnosis ; Humans ; Korea ; Leukocytosis* ; Meningitis, Aseptic ; Meningitis, Bacterial* ; Vaccines, Conjugate

Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.
Biopsy* ; Bone Marrow ; Brain Diseases ; Brain* ; Central Nervous System ; Cerebrospinal Fluid ; Diagnosis ; Fever ; Humans ; Jaundice ; Leukocytosis ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pathology ; Splenomegaly

PURPOSE: The aim of this study was to evaluate the difference of clinical features among patients with enteroviral mengitis according to the presence of cerebrospinal fluid (CSF) pleocytosis. METHODS: This was a retrospective analysis of the patients' data diagnosed with enteroviral meningitis by CSF reverse transcriptase polymerase chain reaction (RT-PCR). We reviewed the medical records of children younger than 16 years who visited Korea University Ansan Hospital and Guro Hospital for meningitis or encephalitis between March 2013 and August 2013. Clinical and laboratory variables were compared with regard to the presence of CSF pleocytosis. RESULTS: Among 49 patients with enteroviral meningitis, eight of 49 (16.3%) did not have pleocytosis. The enteroviral meningitis patients without pleocytosis had lower CSF protein level than patients with pleocytosis (18.9+/-4.3 vs 34.9+/-18.3 mg/dL; P=0.002). Age, duration of hospital stay, severity of clinical symptom, peripheral white cell blood counts, absolute neutrophil counts and CSF/serum glucose ratio were not different between two groups. CONCLUSION: Enteroviral meningitis could manifest without pleocytosis. We therefore think that CSF RT-PCR is helpful for the diagnosis, especially in suspected cases, which leads to shorter hospital stay and minimal use of antibiotics.
Anti-Bacterial Agents ; Cerebrospinal Fluid ; Child ; Diagnosis ; Encephalitis ; Enterovirus ; Glucose ; Gyeonggi-do ; Humans ; Korea ; Length of Stay ; Leukocytosis* ; Medical Records ; Meningitis* ; Neutrophils ; Retrospective Studies ; Reverse Transcriptase Polymerase Chain Reaction