Treatment of an adult case with Japanese encephalitis / 中华实验和临床病毒学杂志

Objective@#The features of clinical, laboratory and radiologic examinations of an adult patient with Japanese encephalitis (JE) were analyzed to understand the differences in clinical characteristics between adult and children patients, and to investigate the method of etiological tests.@*Methods@#The clinical, laboratory tests and radiographic data of an adult case with JE requiring hospitalization were analyzed retrospectively and the related literature was reviewed.@*Results@#The clinical presentation of patients with JE was nonspecific, the patient presented with fever, consciousness and cognitive impairment, convulsion and meningeal irritation. Its main test indicator is IgM antibody of Japanese encephalitis virus(JEV) in acute cerebrospinal fluid and serum specimens.Craniocerebral MRI is an important auxiliary examination for JE, the common sites involved are thalamus, basal ganglia and cerebral cortex, of which thalamus is almost 100% affected.@*Conclusions@#The clinical presentation of patients with JE was nonspecific. Clear diagnosis should be accompanied by the combination of epidemiology, laboratory tests and specific antibodies detection. Neural damages commonly occur in thalamus.

The Correlation between ADHD and Brain MRI Findings in Children with Epilepsy / 대한소아신경학회지

PURPOSE: We analyze the brain magnetic resonance imaging (MRI) findings of children with epilepsy and concomitant attention deficit hyperactivity disorder (ADHD) to investigate the correlation between brain MRI and ADHD, and to determine whether abnormal MRI finding can be a risk factor for the development of ADHD. METHODS: A total of 55 patients (36 male, 19 female) were diagnosed as ADHD in children with epilepsy at the pediatric neurology department of Seoul St. Mary hospital from March, 2009 to December, 2013. The records of these patients were retrospectively reviewed. RESULTS: 29 patients (52.7%) had normal MRI findings, and 26 patients (47.3%) had abnormal MRI findings. The inattention type of ADHD (96.5%) was the largest type in a group of normal brain MRI findings, but the combined type (53.8%) and the inattention type (46.2%) occupied the majority in a group of abnormal MRI findings. The score of symptom in inattention was 7.44/9 in a group of normal MRI findings, while 8.2/9 in a group of abnormal MRI findings. And the score of symptom in hyperactivity was 2.93/9 in a group of normal MRI findings, while it was 4.8/9 in a group of abnormal MRI findings (P < 0.001). 7 patients (12.7%) revealed hippocampal sclerosis out of 26 abnormal MRI findings, and they had significantly higher scores of ADHD symptoms (8.57/9, 4.8/9) compared to normal group (7.83/9, 4.23/9). CONCLUSION: Abnormal MRI findings in children with epilepsy are not only closely related to ADHD but may also be associated with the severity of ADHD.

Value of Repeat Brain MRI in Children with Focal Epilepsy and Negative Findings on Initial MRI

OBJECTIVE: To evaluate the value of repeat brain magnetic resonance imaging (MRI) in identifying potential epileptogenic lesions in children with initial MRI-negative focal epilepsy. MATERIALS AND METHODS: Our Institutional Review Board approved this retrospective study and waived the requirement for informed consent. During a 15-year period, 257 children (148 boys and 109 girls) with initial MRI-negative focal epilepsy were included. After re-evaluating both initial and repeat MRIs, positive results at repeat MRI were classified into potential epileptogenic lesions (malformation of cortical development and hippocampal sclerosis) and other abnormalities. Contributing factors for improved lesion conspicuity of the initially overlooked potential epileptogenic lesions were analyzed and classified into lesion factors and imaging factors. RESULTS: Repeat MRI was positive in 21% (55/257) and negative in 79% cases (202/257). Of the positive results, potential epileptogenic lesions comprised 49% (27/55) and other abnormalities comprised 11% of the cases (28/257). Potential epileptogenic lesions included focal cortical dysplasia (n = 11), hippocampal sclerosis (n = 10), polymicrogyria (n = 2), heterotopic gray matter (n = 2), microlissencephaly (n = 1), and cortical tumor (n = 1). Of these, seven patients underwent surgical resection. Contributing factors for new diagnoses were classified as imaging factors alone (n = 6), lesion factors alone (n = 2), both (n = 18), and neither (n = 1). CONCLUSION: Repeat MRI revealed positive results in 21% of the children with initial MRI-negative focal epilepsy, with 50% of the positive results considered as potential epileptogenic lesions. Enhanced MRI techniques or considering the chronological changes of lesions on MRI may improve the diagnostic yield for identification of potential epileptogenic lesions on repeat MRI.

The Effectiveness of Brain MRI in Children and Adolescents with Headache

PURPOSE: In this study, the authors classified the pediatric headache patients according to the presence of associated symptoms or signs, and the duration of headache. The purpose of this study is to evaluate the clinical usefulness of brain MRI in pediatric headache. METHODS: Total 1859 patients diagnosed with headache, migraine, other headache syndromes were recruited in the patients at the age of 4–14 years who visited Inje University Ilsan Paik Hospital between 2012 and 2016. 216 patients who met the exclusion criteria were excluded. Of total 1643 patients, 304 patients had brain MRI, and the brain MRI results were retrospectively analyzed. RESULTS: The mean age of 304 patients who had brain MRI was 9.4 years. Among the 316 brain MRI results of total 304 patients, the most common finding was normal in 58.5%, followed by incidental findings (23.4%) and pathologic findings (18.0%). The patients group with associated symptoms or signs with headache showed less normal findings, more incidental and pathologic findings than those without associated symptoms or signs. The MRI findings of two groups showed significant difference (P=0.049). The patients group with less than 3 months of headache showed less normal findings, more incidental and pathologic findings than those with more than 3 months of headache. The MRI findings of the two groups showed significant difference (P=0.001). CONCLUSIONS: Brain MRI should be performed with careful history taking and neurologic evaluation to rule out the underlying intracranial diseases especially in case of headaches with associated symptoms or signs.

Clinical Features and Neurodevelopmental Outcomes of Infants with Congenital Cytomegalovirus Infection: Single NICU Experience

PURPOSE: To assess the clinical features, hearing loss and neurodevelopmental outcomes of infants with congenital cytomegalovirus (CMV) infection and to discover significant brain magnetic resonance imaging (MRI) findings that predict poor neurodevelopmental outcomes. METHODS: The medical records of 31 infants who were diagnosed with congenital CMV infection at the Neonatal Intensive Care Unit of Asan Medical Center between 2002 and 2012 after CMV was isolated from their urine within the second week of their life were retrospectively reviewed. The long-term neurodevelopmental outcomes of the infants were monitored using the Bayley Scale of Infant Development II, the Korean Infants' Development Screening Test and the Wechsler Scale of Intelligence, as appropriate. RESULTS: The infants' mean gestational age was 36.3+/-3.2 weeks and their mean birth weight was 2,395+/-715 g. Microcephaly were detected in three of them (9.7%), and petechiae and hepatosplenomegaly were detected in one (3.2%). Ten infants (32.2%) and nine infants (29.0%) showed preterm and intrauterine growth retardation, respectively. Of the 27 infants with whom long-term follow-up was possible, six (22.2%) showed developmental disabilities. The brain MRI findings, which included ventriculomegaly, periventricular calcification, polymicrogyria, microcephaly and cerebellar hypoplasia, were correlated with the poor neurodevelopmental outcomes, but no correlation was found between the presence of periventricular cysts and that of white matter disease. Of the infants who survived, six (20.0%) had sensorineural hearing loss at the median age of 30.6 months. CONCLUSION: Congenital cytomegalovirus infection is one of the most frequent causes of intrauterine viral fetal infection and need to be distinguished if congenital infection is suspected. On the follow up observation, the hearing loss was observed in 20% of the patients. A long term neurological observation is required for the patients who had unusual impression on MRI.

Brain Stem Hypoplasia Associated with Cri-du-Chat Syndrome

Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

Does the ADC Map have Additional Clinical Significance Compared to the DWI in the Brain Infarction?

PURPOSE: To re-evaluate additional clinical significance of the apparent diffusion coefficient (ADC) map in the inference of infarction stage, authors studied the evolution patterns of the DWI and the ADC map of the brain infarction. MATERIALS AND METHODS: In 127 patients with cerebral infarctions, including follow-up checks, 199 studies were performed. They were classified as hourly (117 studies)-, daily (108 studies)-, weekly (62 studies)-based groups. The signal intensity (SI) was measured at the core of the infarction and contralateral area with ROI of 0.3 cm2 or more on the images of the DWI and the ADC map, and calculated the ratios of SI and ADC value of the infarction area / contralateral normal area, and compared the patterns of the change according to the evolution. RESULTS: Infarction was detected as early as 1 hour after the attack, and the ratio of SI in the DWI became over than 2 after 12 hours, which showed a plateau until the 6th day. Thereafter, it decreased slowly to 1 on the 30th day, and changed to lower SI than the surrounding brain. The ratio in the ADC map became 0.46 in 24 hours after the attack, and increased slowly to 1 in the 15th day. Thereafter, it became a higher value than the surrounding brain. Overall, the ratio in the ADC map changed earlier than in the DWI, and the ratio curves showed inverse pattern each other according to the evolution of the infarction. CONCLUSION: The evolution patterns of infarction on the ADC map showed an inverse curve of DWI curve, which means that the ADC value is accurately predictable from DWI, and the ADC map joined with the DWI seems helpful in the determination of subacute infarction between 15 to 30 days.

Idiopathic Trigeminal Neuropathy with Brain MRI Abnormalities

No abstract available.

Hippocampal Atrophy Identified by Volumetric Measurement in Intractable Epilepsy Cases

BACKGROUND: Recurrent seizures result in brain damage, but it is usually gradual, minimal, and difficult to observe by visual inspection of magnetic resonance images (MRIs). It is well known that hippocampal structure is vulnerable to seizure-associated brain damage. We measured the hippocampal volume in patients with epilepsy to evaluate the degree of damage to the hippocampus. METHODS: We recruited 33 patients with epilepsy and 21 healthy subjects from January 2007 to December 2008. We subclassified the patients into two groups: (1) 14 patients with intractable epilepsy and (2) 19 patients with drug-responsive epilepsy. In each group, the volumes of the left and right hippocampus were measured by manual drawing on brain MRIs. We compared the hippocampal volume in intractable epilepsy, drug-responsive epilepsy, and healthy subjects. The compounding effect of hippocampal sclerosis was ruled out by excluding eight patients with hippocampal sclerosis; we then compared the hippocampal volume in the two groups with epilepsy. RESULTS: The volume of the bilateral hippocampus on brain MRIs was smaller in patients with intractable epilepsy than in those with drug-responsive epilepsy and healthy subjects (left, p<0.004; right, p<0.03). After excluding the patients with hippocampal sclerosis by visual inspection, the hippocampal volumes were also found to be smaller in patients with intractable epilepsy than in those with drug-responsive epilepsy (left, p<0.04; right, p<0.05). CONCLUSIONS: While there is no definitive abnormality of the hippocampus on visual inspection of brain MRIs, we determined the degree of hippocampal atrophy and volume loss in patients with intractable epilepsy. Hippocampal volumetry will be helpful for the assessment of brain damage in patients with intractable epilepsy.

Myotonic Dystrophy Type 1 With Brain MRI Lesions Involving White Matters in Anterior Temporal Pole and Insula

No abstract available.

Risk Factors and Prognosis for Periventricular Leukomalacia According to Neuroimage in Preterm Infants

PURPOSE: The aim of this study was to determine the risk factors, clinical characteristics and prognosis for the development of periventricular leukomalacia (PVL) in preterm infants according to the extent and site of the PVL. METHODS: The medical records of infants (under 36 weeks of gestational age) delivered from January 1999 to December 2008 were reviewed. Twenty-five preterm infants with were PVL were diagnosed by brain magnetic resonance imaging (MRI) and an addition 50 preterm infants with no brain lesions were enrolled in this study. The perinatal and neonatal risk factors for the development of PVL was determine in these infants. Mental and Psychomotor Developmental Indices (MDI, PDI) were assessed by a clinical psychologist using the Bayley Scales of Infant Development II. We compared the differences of the clinical characteristics and prognosis according to brain MRI findings. RESULTS: Maternal fever, young maternal age, extended oxygen use, hypotension within the first week of birth, use of inotropics within the first week of birth, and respiratory distress syndrome were the risk factors associated with PVL (P<0.05). In the multivariate analysis, maternal fever and extended oxygen use were statistically significant independent risk factors (P<0.05). The mean MDI and PDI scores of the PVL group (74.4+/-27.8 and 58.0+/-17.7) were significantly lower than those of the control group (103.5+/-8.9 and 101.7+/-16.1, P<0.05). CONCLUSION: Maternal fever and extended oxygen use were independent risk factors for PVL. We should pay attention to infants who had the risk factors and follow them up closely by brain imaging study and Bayley Scales of Infant Development II.

Occipital Lobe Simple Partial Epilepsy Presenting as Visual Episodes Associated with Non-Ketotic Hyperglycemia / 대한간질학회지

Non-ketotic hyperglycemia (NKH) is recognized both as a direct cause of a precipitating factor of many types of epileptic seizure, including simple partial motor, complex partial as well as reflex motor seizure, choreoathetosis and ballismus. Its association with isolated visual alteration is less known. A 74-year-old diabetic woman with visual complaints manifested as flashing colorful lights (red, yellow and blue) in both visual field with progressive increase in frequency. Among the laboratory tests, NKH of 508mg/dL stood out. Slow waves from the right occipital region maximum at O2 associated with visual symptom were recorded in EEG. Brain MRI showed subcortical T2WI and FLAIR hypointensities in the right occipital lobe with scanty enhancement. Patient was treated with hydration and insulin and her symptoms disappeared after hyperglycemia was corrected. We stress that isolated visual episodes may be initial manifestation of occipital lobe simple partial seizure associated with NKH.

A Case of Heidenhain Variant of Sporadic Creutzfeldt-Jakob Disease

PURPOSE: To report a case of the Heidenhain variant of sporadic Creutzfeldt-Jakob disease (CJD), predominantly characterized by visual impairment at onset. CASE SUMMARY: History-taking, ophthalmologic examination, neurologic examination, cerebrospinal fluid examination including 14-3-3 protein analysis, and brain MRI were performed in a 48-year-old man with progressive visual loss and a visual field defect. These symptoms were accompanied by visual illusion and macropsia. Neurologic examination revealed relatively rapidly progressing cognitive impairment, ataxia, aphasia, and myoclonus. The 14-3-3 protein was detectable in otherwise normal CSF samples. The diffusion weighted brain MRI showed increased signal intensity in both occipital lobes, the basal ganglia, the temporal and frontal lobes. He was clinically diagnosed as having a Heidenhain variant of sporadic CJD. CONCLUSIONS: In a patient with a rapidly progressive visual loss, visual field defects, visual illusion, and neurologic abnormalities including progressive dementia, ataxia, aphasia, and myoclonus, the Heidenhain variant of CJD should be considered. Because prions, a cause of CJD, exhibit unusual resistance to conventional chemical and physical decontamination methods, it is necessary to have an appropriate management scheme to prevent the spread of infection.

A Case of Cerebral Erdheim-Chester Disease With Progressive Cerebellar Syndrome

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. Cerebellar involvement is rare in this syndrome. We report a 37-year-old woman with slowly progressive cerebellar ataxia, dysmetria of limbs, nystagmus, and dysarthria, bilateral painful axillary masses, and generalized arthralgia. Brain MRI revealed cerebellar atrophy with focal lesions in the pons, middle cerebellar peduncle, and the cerebellum. She underwent incisional biopsy of her axillary masses which showed findings consistent with ECD. An MRI of her lower extremities revealed lesions in the diaphyses, metaphyses, and epiphyses of the proximal tibia and distal femur bilaterally. This is a rare case of cerebral ECD with progressive cerebellar syndrome associated with cerebellar atrophy.

Current opinion of cerebral palsy / 대한산부인과학회지

Despite improvement in perinatal practice during the past several decades, the incidence of cerebral palsy has remained essentially unchanged. The cause of cerebral palsy is thought to be multifactorial, including prematurity, inflammation, genetic cause and environmental factors. Although evidences suggest that 70-80% of cerebral palsy is due to prenatal factors and birth asphyxia plays a relatively minor role (<10%), development of cerebral palsy is frequently attributed to the obstetric misstep. Therefore, it is of critical importance to keeping in touch with recent trend and advances regarding cerebral palsy. In this background, this review was mainly focused on the articles published from Jan 2006 to June 2007, excluding the orthopedic and rehabilitational aspects. The subjects are arbitrary divided into the following four categories; 1) recent epidemiologic studies of cerebral palsy, 2) recent evidences of antenatal risk factors, 3) cerebral palsy and placental pathology, 4) role of MRI in diagnosis of cerebral palsy.

Use of the Putamen/Caudate Volume Ratio for Early Differentiation between Parkinsonian Variant of Multiple System Atrophy and Parkinson Disease

BACKGROUND AND PURPOSE: Neuropathological studies have demonstrated that multiple system atrophy (MSA) produces selective atrophy of the putamen with sparing of the caudate nucleus, while both structures are spared in idiopathic Parkinson's disease (PD). In this study we evaluated the clinical efficacy of using putaminal atrophy in brain MRI to differentiate MSA and PD. METHODS: We measured the putamen/caudate volume ratio on brain MRI in 24 patients with MSA and 21 patients with PD. Two clinicians who were blinded to the patients' diagnoses and to each other's assessments measured the volume ratio using a computer program. RESULTS: The measured volume ratios of the two investigators were highly correlated (r=0.72, p<0.0001). The volume ratio was significantly lower in MSA (1.29+/-0.28) than PD (1.91+/-0.29, p<0.0001). Setting an arbitrary cutoff ratio of 1.6 resulted in about 90% of patients with MSA falling into the group with a lower ratio, whereas more than 80% of patients with PD belonged to the other group. CONCLUSIONS: The present results demonstrate that putaminal atrophy in MSA as measured on brain MRI represents an effective tool for differentiating MSA from PD.

Two Cases of Metronidazole-Induced Neurotoxicity Lacking of Clinico-Radiological Correlation

Metronidazole is an antimicrobial agent widely used for the treatment of trichomoniasis, giardiasis, amebiasis, and anaerobic bacterial infections. It has been reported that metronidazole-induced neurotoxicity is associated with responsible lesions on brain MRI. We report 2 patients with metronidazole-induced neurotoxicity, who had no responsible lesions on brain MRI for their neurological deficits. The pathogenic mechanisms are discussed.

Right Superior Oblique Paralysis associated with Idopathic Hypertropic Cranial Pachymeningitis

PURPOSE: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disase, which causes chronic progressive inflammation and thickening of the basal dura mater. We report a case of IHCP associated with superior oblique paralysis and present a review of the literature. METHODS: An 8-year-old boy presented with binocular diplopia and left side head tilting. Suspecting right superior oblique muscle paralysis, an alternating prism test, head tilt test, fundus examinations, neurologic examination, and brain MRI were performed. RESULTS: The brain MRI revealed abnormal enhancement of the right tentorium in the course of the right fourth cranial nerve, leading to a diagnosis of IHCP with paralytic strabismus and the patient was treated with systemic steroid therapy. CONCLUSIONS: We report a case of IHCP with right superior oblique paralysis. Patients with recent onset paralytic strabismus require appropriate neurolgic and neuroimaging examinations.

MR Imaging Findings of Recurred Dermatofibrosarcoma Protuberans of the Scalp: A Case Report

A 48-year-old man presented with a dermatofibrosarcoma protuberans (DFSP) of the scalp associated with local recurrence. Axial T1- and T2-weighted images demonstrated a well-circumscribed hypointense and intermediate hyperintense mass in the skin and subcutaneous layer of the scalp, respectively. Contrast-enhanced T1-weighted images showed the strongly enhanced mass invasion to the skin, subcutaneous layer and adjacent galeal layer. Scalp DFSP is very uncommon but is an aggressive tumor, so MR imaging diagnosis of the extent of the lesion to underlying structures, and initial wide local resection is important to prevent recurrence.

Merosin Deficient Congenital Muscular Dystrophy in Korea

PURPOSES:Congenital muscular dystrophies(CMDs) are an autosomal recessive and heterogeneous disorders. The classic forms of CMD are subclassified into two major categories:merosin positive and deficient. Merosin deficient congenital muscualr dystrophy (MDCMD) is rare in Asia and it has never been reported especially in Korea. So, we summarized the clinical features with neuroimaging findings of the patients, who were diagnosed as MDCMD, for the first time in Korea. METHODS: Twenty three patients were diagnosed as CMD in Seoul National University Children's Hospital over 3 years(2001-2004). Among them, four patients with MDCMD were proven by merosin immunohistochemical staining. We reviewed their clinical, pathologic features, EMG/NCS findings and brain MRIs. RESULTS: Among 23 patients with CMD, 4 patients(17.4%) were MDCMD. All of them were presented at birth or early infancy with hypotonia, muscle weakness and joint contracture. They all could not walk and had myopathic faces, developmental delay, poor weight gain and scoliosis. EMG/NCS showed myopathic motor unit action potential (MUP) and decreased compound motor unit action potential(CMAP). Merosin deficiency was demonstrated in muscle or skin tissues. All of them had diffuse or focal high signal intensity lesions of white matter in brain MR T2WI. However, they showed neither mental retardation nor seizure though one of them had right occipital polymicrogyria. CONCLUSION: We reported 4 children with MDCMD for the first time in Korea. The prevalence in Korea might be lower than in Europe but probably higher than in Japan. If CMD patients have sustained delayed motor milestone with normal intelligence, myopathic face, decreased CMAP and myopathic MUP in EMG/NCS, MDCMD should be suspected and further diagnostic work up such as brain MR and merosin immunohistochemistry will be needed.