Objective: To summarize the clinical and imaging features of linear scleroderma en coup de saber (LSCS) with central nervous system involvement in children. Methods: The clinical data(clinical manifestations and imaging features) of 6 children diagnosed with LSCS with central nervous system involvement who were admitted to Beijing Children's Hospital Affiliated to Capital Medical University from May 2019 to November 2021 were retrospectively analyzed. Results: The 6 patients were all female, aged 6.8 (3.3, 11.0) years at the time of diagnosis, and aged 3.0 (1.7, 4.1) years at the time of discovery of facial skin lesions. Facial skin lesions appeared before neurological symptoms in 5 cases, and neurological symptoms appeared 2 months before skin lesions in 1 case. All the patients had "sword wound" skin lesions on the forehead with alopecia. Neurological manifestations included epileptic seizures in 6 cases, focal neurological defects in 5 cases, and headaches in 2 cases. The intracranial lesions were all ipsilateral to the skin lesions. The magnetic resonance imaging (MRI) of 6 cases showed abnormal signals mainly involving white matter in 1 hemisphere, and 3 cases showed local encephalomalacia. The scattered low signal was observed in 5 cases on susceptibility weighted imaging. Localized brain parenchyma or leptomeninges enhancement was seen on Gadolinium-enhanced sequences in 5 cases. Scattered foci of calcification on the affected side were seen on cranial CT in 4 cases. Skin biopsy was performed in 2 cases. Part of the lesion of the brain was removed in 1 case, and the pathological findings suggested small vasculitis, which was consistent with skin pathological changes. All patients received symptomatic treatment with antiepileptic drugs. Oral prednisone combined with methotrexate was given in 4 cases, and 1 case was given oral prednisone only. One case was presumed to be in the resting stage of the disease due to significant cerebral atrophy in half of the brain, and only antiepileptic drugs were added. The patients were followed up for 6-36 months. The skin lesions of scleroderma and alopecia did not progress in 5 cases, and hemifacial atrophy was developed in 1 case, which was considered to be combined with Parry-Romberg syndrome. The seizures were controlled in 4 cases. One case had reduced seizure frequency but left hemiplegia. One patient still had intractable epilepsy and paroxysmal headache. Conclusions: LSCS with central nervous system involvement is more common in girls, with seizures and neurological defects as the main manifestations. Intracranial lesions are mostly ipsilateral to the skin lesions. Cerebral microbleeds, calcification, and encephalomalacia foci are common, and the pathological changes in skin and intracranial lesions are consistent with small-vessel vasculitis. Prednisone combined with methotrexate treatment has shown some efficacy, but some children remain with refractory epilepsy and neurological deficit symptoms.
Child ; Humans ; Female ; Scleroderma, Localized ; Anticonvulsants ; Methotrexate ; Prednisone ; Retrospective Studies ; Seizures ; Drug Resistant Epilepsy ; Calcinosis ; Alopecia ; Brain ; Encephalomalacia ; Headache

Herpes simplex virus (HSV) is a common pathogen, that causes a broad spectrum of diseases, ranging from minor skin infections to severe encephalitis and widespread infections. Acute retinal necrosis (ARN), one of the most serious manifestations of HSV infection, is defined as a rapidly progressing necrotizing retinopathy that presents discrete areas of circumferential retinal necrosis, along with signs of uveitis, vitreitis, and retinal vasculitis. We encountered a case of a female infant, born at 33 weeks of gestation with a body weight at birth of 2,080 g, who had ARN and encephalomalacia due to HSV infection. ARN associated with HSV infection should be suspected when nonspecific retinal exudates are observed in neonates, especially preterm infants.
Body Weight ; Encephalitis ; Encephalomalacia ; Exudates and Transudates ; Female ; Herpes Simplex ; Herpesvirus 2, Human ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Necrosis ; Parturition ; Pregnancy ; Retinal Necrosis Syndrome, Acute ; Retinal Vasculitis ; Retinaldehyde ; Simplexvirus ; Skin ; Uveitis

The magnetic resonance imaging findings of multicystic encephalomalacia are featured by bilateral frontal large cystic lesion with corpus callosum involvement,evident heterogeneous enhancement of the lesion margin,ring hyperintensity on diffusion weighted imaging,and high choline peak and low N-acetylaspartate peak of the enhanced lesion margin on magnetic resonance spectroscopy.This article reports a case of multicystic encephalomalacia.
Corpus Callosum ; Encephalomalacia ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy

PURPOSE: The aim of this study was to characterize the brain ultrasonographic findings of late-onset circulatory dysfunction (LCD) due to adrenal insufficiency (AI) in preterm infants. METHODS: Among the 257 preterm infants born at <33 weeks of gestation between December 2009 and February 2014 at our institution, 35 preterm infants were diagnosed with AI. Brain ultrasonographic findings were retrospectively analyzed before and after LCD in 14 preterm infants, after exclusion of the other 21 infants with AI due to the following causes: death (n=2), early AI (n=5), sepsis (n=1), and patent ductus arteriosus (n=13). RESULTS: Fourteen of 257 infants (5.4%) were diagnosed with LCD due to AI. The age at LCD was a median of 18.5 days (range, 9 to 32 days). The last ultrasonographic findings before LCD occurred showed grade 1 periventricular echogenicity (PVE) in all 14 patients and germinal matrix hemorrhage (GMH) with focal cystic change in one patient. Ultrasonographic findings after LCD demonstrated no significant change in grade 1 PVE and no new lesions in eight (57%), grade 1 PVE with newly appearing GMH in three (21%), and increased PVE in three (21%) infants. Five infants (36%) showed new development (n=4) or increased size (n=1) of GMH. Two of three infants (14%) with increased PVE developed cystic periventricular leukomalacia (PVL) and rapid progression to macrocystic encephalomalacia. CONCLUSION: LCD due to AI may be associated with the late development of GMH, increased PVE after LCD, and cystic PVL with rapid progression to macrocystic encephalomalacia.
Adrenal Insufficiency* ; Brain* ; Ductus Arteriosus, Patent ; Encephalomalacia ; Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Leukomalacia, Periventricular ; Pregnancy ; Retrospective Studies ; Sepsis ; Ultrasonography

BACKGROUND AND PURPOSE: Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection. METHODS: We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery. RESULTS: The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussen's syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussen's encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities. CONCLUSIONS: The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.
Child ; Chungcheongnam-do ; Cohort Studies ; Electroencephalography ; Encephalitis ; Encephalomalacia ; Epilepsy* ; Follow-Up Studies ; Hemispherectomy ; Humans ; Infant ; Infant, Newborn ; Infarction ; Paresis ; Patient Selection ; Quality of Life ; Retrospective Studies ; Seizures ; Spasms, Infantile ; Sturge-Weber Syndrome ; Walking

OBJECTIVE: Chronic subdural hematoma (CSDH) is relatively common in neurosurgical field. However not all patients develop CSDH after minor head trauma. In this study, we evaluate the risk factors of post-traumatic CSDH. METHODS: Two-hundred and seventy-seven patients were enrolled and analyzed in this study from January 2012 to December 2013. Of those, 20 participants had minor head trauma developed CSDH afterward. We also included 257 patients with minor head trauma who did not develop CSDH during the same follow-up period as the control group. We investigated the risk factors related to the development of CSDH after minor head trauma. RESULTS: Old age (p=0.014), preexisting diabetes mellitus (p=0.010), hypertension (p=0.026), history of cerebral infarction (p=0.035), antiplatelet agents (p=0.000), acute subdural hematoma in the convexity (p=0.000), encephalomalacia (p=0.029), and long distance between skull and brain parenchyma (p=0.000) were significantly correlated with the development of CSDH after trauma. Multivariate analysis revealed that only the maximum distance between the skull and the cerebral parenchyma was the independent risk factor for the occurrence of CSDH (hazard ratio 2.55, p=0.000). CONCLUSION: We should consider the possibility of developing CSDH in the post-traumatic patients with the identified risk factors.
Brain ; Cerebral Infarction ; Craniocerebral Trauma* ; Diabetes Mellitus ; Encephalomalacia ; Follow-Up Studies ; Hematoma, Subdural, Acute ; Hematoma, Subdural, Chronic* ; Humans ; Hypertension ; Multivariate Analysis ; Platelet Aggregation Inhibitors ; Risk Factors ; Skull

PURPOSE: Encephalomalacia is one of the major causes of symptomatic epilepsy. However, no previous study has examined the correlation between encephalomalacia and epilepsy in children. In this study, we aimed to quantify the prevalence, clinical features, and risk factors of epilepsy associated with encephalomalacia. METHODS: We performed a retrospective review of the medical records of 95 patients who were diagnosed with encephalomalacia by neuroimaging techniques at Pusan National University Children's Hospital between November 2008 and July 2013. Patients were classified into two groups: epilepsy (Group A) and non-epilepsy (Group B). We compared the demographics, underlying causes, and distribution of encephalomalacic lesions of these two groups. RESULTS: Groups A and B comprised 35 (36.8%) and 60 (63.1%) patients, respectively. Compared to Group B, Group A showed shorter mean gestational period (35.99+/-4.63 vs. 38.09+/-3.70, P=0.02), lower birth weight (2.60+/-0.94 vs. 3.02+/-0.64, P=0.02), and earlier onset of encephalomalacia (2.74+/-3.52 vs. 5.60+/-5.96, P=0.01). In comparing the underlying cause of encephalomalacia, the occurrence of epilepsy was lower after cerebrovascular disease (P<0.01), but trended towards a higher incidence after a central nervous system infection (P=0.09). Multifocal encephalomalacic lesions were significantly higher in Group A (P=0.04). CONCLUSION: The risk factors for epilepsy associated with encephalomalacia are early gestational age, low birth weight, early onset of encephalomalacia, and multifocal encephalomalacic lesions. It may be necessary for clinicians to search for these risk factors, and make a particularly close observation on these patients.
Birth Weight ; Busan ; Central Nervous System Infections ; Child* ; Demography ; Encephalomalacia* ; Epilepsy* ; Gestational Age ; Humans ; Incidence ; Infant, Low Birth Weight ; Infant, Newborn ; Medical Records ; Neuroimaging ; Prevalence ; Retrospective Studies ; Risk Factors

We present a case of tacrolimus-induced encephalopathy after successful kidney transplantation. An 11-year-old girl presented with sudden onset of neurologic symptoms, hypertension, and psychiatric symptoms, with normal kidney function, after kidney transplantation. The symptoms improved after cessation of tacrolimus. Magnetic resonance imaging (MRI) showed acute infarction of the middle cerebral artery (MCA) territory in the right frontal lobe. Three days later, she had normal mental function and maintained normal blood pressure with left hemiparesis. Follow-up MRI was performed on D19, showing new infarct lesions at both cerebral hemispheres. Ten days later, MRI showed further improvement, but brain single photon emission computed tomography (SPECT) showed mild reduction of uptake in both the anterior cingulate gyrus and the left thalamus. One month after onset of symptoms, angiography showed complete resolution of stenosis. However, presenting as a mild fine motor disability of both hands and mild dysarthria, what had been atrophy at both centrum semiovale at 4 months now showed progression to encephalomalacia. There are two points of interest in this case. First, encephalopathy occurred after administration of tacrolimus and improved after discontinuation of the drug. Second, the development of right-side hemiplegia could not be explained by conventional MRI; but through diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) of white matter tract, visualization was possible.
Angiography ; Atrophy ; Blood Pressure ; Brain ; Cerebrum ; Child ; Constriction, Pathologic ; Diffusion ; Diffusion Tensor Imaging ; Dysarthria ; Encephalomalacia ; Follow-Up Studies ; Frontal Lobe ; Gyrus Cinguli ; Hand ; Hemiplegia ; Humans ; Hypertension ; Infarction ; Kidney ; Kidney Transplantation ; Magnetic Resonance Imaging ; Middle Cerebral Artery ; Neurologic Manifestations ; Paresis ; Tacrolimus ; Thalamus ; Tomography, Emission-Computed, Single-Photon

The authors present a rare case of severe vasospasm following the rupture of arteriovenous fistula. On initial CT scan, hematoma in the corpus callosum and left inferior frontal region with surrounding cerebromalacia and all ventricles without apparent subarachnoid hemorrhage were seen. Angiograms showed arterivenous fistula but did not show cerebral vasospasm. Thirteen days after admission the neurological state of patient suddenly deteriorated and bilateral motor weaknesses developed. Following angiograms revealed severe narrowing on the supraclinoid portion of bilateral internal carotid arteries, bilateral anterior cerebral arteries and bilateral middle cerebral arteries. Transluminal angioplasty and intra-arterial papaverine infusion were performed. The patient remained stable with moderate neurologic deficits.
Angioplasty ; Anterior Cerebral Artery ; Arteriovenous Fistula ; Carotid Artery, Internal ; Corpus Callosum ; Encephalomalacia ; Fistula ; Hematoma ; Hemorrhage ; Humans ; Middle Cerebral Artery ; Neurologic Manifestations ; Papaverine ; Rupture ; Subarachnoid Hemorrhage ; Vasospasm, Intracranial

Infections associated with Enterococcus durans have been rare in humans until now. E. durans is a frequent component of the intestinal flora of several domestic animal species and may cause septicemia and encephalomalacia in chicks. We report a case of ventriculoperitoneal shunt infection caused by vancomycin-resistant E. durans in a 17-year-old woman with Dandy-walker syndrome. To the best of our knowledge, this is the first human case of ventriculitis caused by vancomycin-resistant E. durans.
Adolescent ; Animals, Domestic ; Dandy-Walker Syndrome ; Encephalomalacia ; Enterococcus ; Female ; Humans ; Sepsis ; Vancomycin Resistance ; Ventriculoperitoneal Shunt