Antibodies, Viral ; blood ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Fever ; Humans ; Leukoencephalitis, Acute Hemorrhagic ; diagnosis ; therapy ; Male ; Prognosis

OBJECTIVE: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors. MATERIALS AND METHODS: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain. RESULTS: This encephalopathy predominantly affected the bilateral thalami (n=14), pons (n=12), and midbrain (n=10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n=12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n=6) to severe (n=6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015). CONCLUSION: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.
Brain/pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Korea ; Leukoencephalitis, Acute Hemorrhagic/complications/*pathology ; *Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies

Brain ; pathology ; Demyelinating Diseases ; complications ; diagnosis ; pathology ; Encephalomyelitis ; complications ; diagnosis ; pathology ; Fatal Outcome ; Humans ; Leukoencephalitis, Acute Hemorrhagic ; complications ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Male ; Young Adult

Herpes simplex virus type I (HSV-I) causes an acute necrotizing encephalitis that selectively affects tempioral and frontal lobes. The sequelae and mortality of herpes simplex encephalitis(HSE) may be reduced by available antiviral therapy and therefore early diagnosis of HSE is essential. We have assessed the potential of brain perfusion scintigraphy using Tc-99m-HMPAO single photon emission computed tomoglaphy(SPECT) in HSE. Nine Tc-99m-HMPAO SPECTs were performed in eight patients with clinically suspected herpes simplex encephalitis. The examinations were made between 18 days and 10 months after onset of encephalitic symptoms The SPECT images in seven patients revealed decreased accumulation ol radioactivity in the affected temporal, frontal or parietal lobes. In a 30 yr-old female patient, two SPECT images were obtained on 18th day after the onset of symptoms and 3 months later. Her initial SPECT showed increased accumulation of radioactivity in the affected temporal and inferior frontal lobes. 3 month later follow-up SPECT image revealed the decreased radioactivity in the affected temporal, inferior frontal, and inferior parietal lobes. We found that the SPECT done at early stage of HSE may show increased cerebral perfusion due to inflammatory or other pathologic mechanism and the later stage SPECT may show decreased cerebral perfusion due to decreased cerebral metabolism caused by neuronal death We suggest that Tc-99m-HMPAO SPECT may support the clinical diagnosis of HSE.
Brain ; Diagnosis ; Early Diagnosis ; Encephalitis, Herpes Simplex ; Female ; Follow-Up Studies ; Frontal Lobe ; Herpes Simplex ; Humans ; Leukoencephalitis, Acute Hemorrhagic ; Metabolism ; Mortality ; Neurons ; Parietal Lobe ; Perfusion ; Perfusion Imaging ; Radioactivity ; Simplexvirus ; Tomography, Emission-Computed, Single-Photon*

This study was conducted to investigate the etiology, the clinical characteristics and prognosis of acute necrotizing encephalopathy (ANE) in Korean children. Six children (1 yr to 7 yr) patients with ANE were enrolled. They were diagnosed by clinical and radiological characteristics and their clinical data were retrospectively analyzed. In a search of clinically plausible causes, brain MRI in all patients, mitochondrial DNA studies for mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) and myoclonus epilepsy and ragged red fibers (MERRF) in four patients, and genomic typing on HLA DRB/HLA DQB genes in three patients were performed. All had precedent illnesses and the main initial symptoms included mental change (83%), seizures (50%), and focal deficits (50%). MRI revealed increased T2 signal density in the bilateral thalami and/or the brainstem in all patients. Mitochodrial DNA studies for MELAS and MERRF were negative in those children and HLA-DRB1*1401, HLA-DRB3*0202, and HLA-DQB1*0502 seemed to be significant. A high dose steroid was given to all patients, which seemed to be partly effective except for 2 patients. In conclusion, ANE is relatively rare, but can result in serious neurological complication in children. Early detection and appropriate treatment may lead to a better neurological outcome.
Child ; Child, Preschool ; Female ; HLA-DQ Antigens/metabolism ; HLA-DQ beta-Chains ; HLA-DR Antigens/metabolism ; HLA-DRB1 Chains ; HLA-DRB3 Chains ; Humans ; Infant ; Korea ; Leukoencephalitis, Acute Hemorrhagic/diagnosis/etiology/*pathology/*physiopathology ; MELAS Syndrome/pathology/physiopathology ; MERRF Syndrome/pathology/physiopathology ; Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.
Administration, Intravenous ; Axons ; Central Nervous System ; Cerebellum ; Child ; Demyelinating Diseases ; Diffusion Tensor Imaging* ; Diffusion* ; Emergency Service, Hospital ; Female ; Follow-Up Studies* ; Humans ; Immunoglobulins ; Infant ; Leukoencephalitis, Acute Hemorrhagic* ; Magnetic Resonance Imaging ; Methylprednisolone ; Mortality ; Neurologic Manifestations ; Parents ; Pupil ; Rare Diseases ; Reflex, Stretch ; Rehabilitation ; Seizures ; Seizures, Febrile ; Stupor ; Survivors ; Thalamus ; White Matter

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.
Administration, Intravenous ; Axons ; Central Nervous System ; Cerebellum ; Child ; Demyelinating Diseases ; Diffusion Tensor Imaging* ; Diffusion* ; Emergency Service, Hospital ; Female ; Follow-Up Studies* ; Humans ; Immunoglobulins ; Infant ; Leukoencephalitis, Acute Hemorrhagic* ; Magnetic Resonance Imaging ; Methylprednisolone ; Mortality ; Neurologic Manifestations ; Parents ; Pupil ; Rare Diseases ; Reflex, Stretch ; Rehabilitation ; Seizures ; Seizures, Febrile ; Stupor ; Survivors ; Thalamus ; 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