A 68-year-old man presented with a three-week history of rapidly progressive dementia, gait ataxia and myoclonus. Subsequent electroencephalography showed periodic sharp wave complexes, and cerebrospinal fluid assay revealed the presence of a 14-3-3 protein. A probable diagnosis of sporadic Creutzfeldt-Jakob disease was made, which was further supported by magnetic resonance (MR) imaging of the brain showing asymmetric signal abnormality in the cerebral cortices and basal ganglia. The aetiology, clinical features, diagnostic criteria, various MR imaging patterns and radiologic differential diagnosis of sporadic Creutzfeldt-Jakob disease are discussed in this article.
Aged ; Brain ; pathology ; Cerebral Cortex ; Cerebrospinal Fluid ; metabolism ; Creutzfeldt-Jakob Syndrome ; diagnostic imaging ; Dementia ; physiopathology ; Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging ; Electroencephalography ; Humans ; Hypoxia-Ischemia, Brain ; diagnostic imaging ; Male ; Prion Diseases ; physiopathology

OBJECTIVETo investigate the current status of the application of H-magnetic resonance spectroscopy (H-MRS) in neonates with hypoxic-ischemic encephalopathy (HIE), and to describe the trend of research in the field.

METHODSPubMed, EMBASE, and Web of Science were searched for English articles published up to January 10, 2018, with the combination of key words and MeSH terms. The articles were screened according to inclusion and exclusion criteria. Excel 2016, Bicomb 2.0, and VOSviewer1.6.6 were used to analyze the key words, to perform a cluster analysis of hot words, and to plot the knowledge map.

RESULTSA total of 66 articles were included, and 27 high-frequency key words were extracted. The results showed that H-MRS was mainly used in four directions of the clinical practice and scientific research on HIE. In clinical practice, H-MRS attracted wide attention as a clinical examination for HIE and a tool for prognostic evaluation; in scientific research, H-MRS was used in animal experiments and studies associated with mild hypothermia therapy.

CONCLUSIONSAs an auxiliary means of magnetic resonance imaging, H-MRS plays an important role in investigating the pathogenesis of neonatal HIE, improving existing therapies, and evaluating the prognosis of neonates with HIE.

Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; diagnostic imaging ; Infant, Newborn ; Infant, Newborn, Diseases ; diagnosis ; diagnostic imaging ; Magnetic Resonance Imaging ; methods ; Male

OBJECTIVETo establish a neonatal Tibet minipig model of hypoxic-ischemic encephalopathy and evaluate the magnetic resonance imaging (MRI) manifestations and pathological findings.

METHODSSix neonatal (1-3 days old) Tibet minipigs were randomized into model group (n=4) and control group (n=2). In model group, hypoxic-ischemic encephalopathy was induced by surgical ligation of the bilateral carotid artery followedimmediately by hypoxic exposure in a hypoxia chamber for 1 h. ESWAN was performed at 2 h, 24 h, 3 days and 5 days after induction of HIE or at 2 h after sham surgery in the control animals to evaluate the brain damage. Conventional MRI scans (T2FLAIR, T2WI, and DWI) were also performed at 24 h after the modeling.

RESULTSIn the neostriatum, values of T(2)*-weighted MRI increased and reached the peak level at 3 days post-injury (P<0.05). Subcortical white matter T(2)* values reached the peak level at 24 h (P<0.05). Neostriatum R(2)* values were at the lowest level at 3 days (P<0.05). Magnitude values were significantly increased after the model establishment (P<0.05). DWI showed multiple mild focal high signals in the bifrontal subcortical white matter and bilateral neostriatum; T2FLAIR showed slightly increased signal; T2WI showed no obvious abnormalities. SWI showed dilated medulla veins adjacent to the bilateral lateral ventricles and basal ganglia. In the early stage of HIE, brain pathologies were characterized mainly by edema and venous congestion with occasional focal necrosis and hemosiderin deposition.

CONCLUSIONESWAN sequence is capable of detecting bleeding and brain edema, and T(2)*, R(2)*, and magnitude values can be used to estimate the changes of brain damage following HIE.

Animals ; Disease Models, Animal ; Hypoxia-Ischemia, Brain ; diagnosis ; pathology ; Magnetic Resonance Imaging ; Swine ; Swine, Miniature ; Tibet

BACKGROUNDMild hypoxic-ischemic encephalopathy (HIE) injury is becoming the major type in neonatal brain diseases. The aim of this study was to assess brain maturation in mild HIE neonatal brains using total maturation score (TMS) based on conventional magnetic resonance imaging (MRI).

METHODSTotally, 45 neonates with clinically mild HIE and 45 matched control neonates were enrolled. Gestated age, birth weight, age after birth and postmenstrual age at magnetic resonance (MR) scan were homogenous in the two groups. According to MR findings, mild HIE neonates were divided into three subgroups: Pattern I, neonates with normal MR appearance; Pattern II, preterm neonates with abnormal MR appearance; Pattern III, full-term neonates with abnormal MR appearance. TMS and its parameters, progressive myelination (M), cortical infolding (C), involution of germinal matrix tissue (G), and glial cell migration bands (B), were employed to assess brain maturation and compare difference between HIE and control groups.

RESULTSThe mean of TMS was significantly lower in mild HIE group than it in the control group (mean ± standard deviation [SD] 11.62 ± 1.53 vs. 12.36 ± 1.26, P < 0.001). In four parameters of TMS scores, the M and C scores were significantly lower in mild HIE group. Of the three patterns of mild HIE, Pattern I (10 cases) showed no significant difference of TMS compared with control neonates, while Pattern II (22 cases), III (13 cases) all had significantly decreased TMS than control neonates (mean ± SD 10.56 ± 0.93 vs. 11.48 ± 0.55, P < 0.05; 12.59 ± 1.28 vs. 13.25 ± 1.29, P < 0.05). It was M, C, and GM scores that significantly decreased in Pattern II, while for Pattern III, only C score significantly decreased.

CONCLUSIONSThe TMS system, based on conventional MRI, is an effective method to detect delayed brain maturation in clinically mild HIE. The conventional MRI can reveal the different retardations in subtle structures and development processes among the different patterns of mild HIE.

Brain ; pathology ; Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; Infant, Newborn ; Magnetic Resonance Imaging ; methods ; Male

OBJECTIVETo investigate the clinical and imaging characteristics of acute hypoxic-ischemic brain injury (HIBI) due to perinatal sentinel events in neonates.

METHODSForty-six neonates with acute HIBI who were admitted between January 2004 and May 2013, and who had a history of major cardiopulmonary resuscitation, were enrolled in the study. They were classified into full-term and preterm infants to analyze the clinical and imaging characteristics.

RESULTSAmong full-term infants, the incidence rates of white matter injury, cortical injury, basal ganglia /thalamic injury, and brain stem injury were 95%, 90%, 75%, and 65%, respectively; among preterm infants, the incidence rates of white matter injury, cortical injury, basal ganglia/thalamic injury, and brain stem injury were 73%, 23%, 19%, and 15%, respectively. Compared with full-term infants, preterm infants had a significantly lower incidence of gray matter injury in the cortex, basal ganglia/thalamus, and brain stem (P<0.05). About 46% of all subjects had multiple organ dysfunction. The 20 full-term infants with HIBI had typical clinical manifestations; 19 (95%) of them had moderate or severe neonatal encephalopathy, with mixed lesions on magnetic resonance imaging (MRI), and moderate or severe basal ganglia/thalamic injury was found in 68% of these patients. Multiple organ dysfunction, various abnormal neurological manifestations, and arterial blood pH less than 7.1 were closely related to moderate or severe brain injury.

CONCLUSIONSWhite matter injury is the most common type of HIBI. Gray matter injury can be found in preterm infants, but the incidence is lower than that in full-term infants. Moderate or severe neonatal encephalopathy is mainly manifested as basal ganglia/thalamic injury on MRI. Evaluation of multiple organ dysfunction and abnormal neurological manifestations and early blood gas analysis are very important for the diagnosis of neonatal HIBI.

Acute Disease ; Brain ; pathology ; Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; etiology ; pathology ; Infant, Newborn ; Magnetic Resonance Imaging ; Male

BACKGROUND: Ocular dipping (OD), or inverse ocular bobbing, consists of slow, spontaneous downward eye movements with rapid return to the primary position. It has been mainly reported following hypoxic-ischemic encephalopathy, but has also been described in association with other types of diffuse or multifocal encephalopathies and structural brainstem damage. CASE REPORT: We report the case of a previously asymptomatic 66-year-old woman who presented with confusion, recent memory disturbances, and abnormal involuntary movements, followed by a coma. Abnormal spontaneous vertical eye movements consistent with OD developed from the fourth day after admission, and the patient died 20 days later. The pathological examination of the brain confirmed the diagnosis of Creutzfeldt-Jakob disease. CONCLUSIONS: The precise location of damage causing OD is unknown. In contrast to ocular bobbing, OD has no localizing value itself, but structural brainstem damage is likely when it appears combined with other spontaneous vertical eye movements.
Aged ; Brain ; Brain Stem ; Coma ; Creutzfeldt-Jakob Syndrome* ; Diagnosis ; Dyskinesias ; Eye Movements ; Female ; Humans ; Hypoxia-Ischemia, Brain ; Memory

OBJECTIVECerebral infarction (CI) is one of severe diseases of central nervous system in neonates, and some infants with CI could have poor prognosis in the long term. This study aimed to analyze the clinical data and prognosis of all neonatal cases with cerebral infarction in recent years and to help future clinical work.

METHODTotally 58 neonatal cases with CI admitted to NICU of the hospital from January 1999 to December 2010 were included in this study. We analyzed all clinical data and prognosis by retrospective analysis.

RESULTSFifty-two term babies and six preterm babies were included. There were altogether 51 cases with asphyxia and 7 with hemorrhagic cerebral infarction. Perinatal hypoxia-ischemia was the most common high-risk factor and it accounted for 46.6%. Seizure was the most frequent initial symptom and the most common clinical manifestation (accounted for 77.6%), and it was followed by intermittent cyanosis, apnea and lethargy. Cerebral CT scan and magnetic resonance imaging were major methods to help to make the diagnosis and they also had close relation with prognosis. Diffusion weighted imaging was very helpful to diagnose infarction in early stage. Left middle cerebral artery was the most common artery to be involved. Supportive therapy and symptomatic treatment were the main methods in the acute stage of neonatal cerebral infarction. Those babies with poor prognosis mostly had large infarction involving cerebral hemisphere, thalamus and basal ganglia.

CONCLUSIONSNeonatal cerebral infarction was a severe brain injury affecting long tern nervous system prognosis. Perinatal hypoxia was the most common high-risk factor and seizure was the most frequent initial symptom. Diffusion weighted imaging was valuable to diagnose infarction in early stage. Most of infants with poor prognosis had large infarction involving hemisphere, thalamus and basal ganglia. Early diagnosis with brain imaging would be helpful for rehabilitation therapy and improving prognosis.

Brain ; diagnostic imaging ; pathology ; Cerebral Infarction ; diagnosis ; etiology ; pathology ; therapy ; Cerebral Palsy ; etiology ; Humans ; Hypoxia-Ischemia, Brain ; complications ; Incidence ; Infant ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Prognosis ; Radiography ; Retrospective Studies ; Risk Factors ; Seizures ; etiology ; Ultrasonography, Doppler, Color

OBJECTIVESTo evaluate the diagnostic value of arterial spin labeling (ASL) technology in newborns with hypoxic ischemic encephalopathy (HIE).

METHODSeven full-term newborn infants without any history of asphyxia and other nervous system diseases were selected as the control and 33 full-term newborn infants were assigned into HIE group. The patients in HIE group were further divided into three subgroups (19 cases of mild, 6 cases of moderate and 8 cases of severe HIE) based on their clinical diagnosis. The control group and HIE group were examined with GE Signa EXCITE HD 3.0T superconducting MRI scanner with a head phase array coil. Both groups were scanned with conventional axial MRI (T1FLAIR, T2WI and T2FLAIR), 1HMRS (PRESS sequence) and ASL (FAIR). Original images of 1HMRS and ASL were processed by Functool software of ADW 4.3 workstation. ASL perfusion images were observed and the signal intensity values of the region of interest (bilateral gray, white matter and basal ganglia) of the two groups were quantitatively measured, and mean value were calculated and compared between groups. Statistical analysis was performed with SPSS 13.0 software, and statistically significant difference was set at P < 0.05.

RESULTThe perfusion images of two groups were obtained perfectly. The signal intensity values of bilateral gray, white matter and basal ganglia of control group were 125.34 ± 11.76, 73.42 ± 11.67 and 173.65 ± 15.49, respectively and there was a statistically significant difference between the different areas. The signal intensity values of bilateral gray, white matter and basal ganglia of HIE group were 153.47 ± 11.72, 71.35 ± 10.37 and 217.13 ± 12.51, respectively. There was a statistically significant difference (P < 0.05) in the average signal intensity value of gray matter and basal ganglia, but there were no statistically significant difference (P > 0.05) in white matter between the two groups.

CONCLUSIONASL Perfusion technique can assess HIE comprehensively and accurately. Furthermore, it can evaluate the brain damage of hypoxic ischemia. The results provide a strong basis for clinical treatment.

Case-Control Studies ; Electron Spin Resonance Spectroscopy ; Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; Infant, Newborn ; Male ; Spin Labels

OBJECTIVETo study the diagnostic value of the signal intensity on T1-weighted images of MRI and proton magnetic resonance spectroscopy (1H MRS) for neonatal hypoxic-ischemic encephalopathy (HIE).

METHODSThirty full-term neonates with HIE admitted into the Department of Neonatology of the First Affiliated Hospital of Medical College, Xi'an Jiaotong University between January, 2007 and December 2009 were enrolled. Ten normal neonates born at the same period served as control group. Cerebral MRI and 1H MRS examinations were performed within 15 days after birth.

RESULTSIn the HIE group, the signal intensity of the posterolateral lentiform nucleus was higher than or equal to that of the posterior limb of internal capsule, but in the control group, the results were opposite, namely, the signal intensity of the postero-lateral lentiform nucleus was lower than that of the posterior limb of internal capsule. The ratios of lactic acid/creatinine and glutamate/creatinine in the basal ganglia and the frontal lobe shown by 1H MRS increased significantly in the HIE group compared with controls (P<0.05 or 0.01). The differences of the signal intensity between the posterolateral lentiform nucleus and the posterior limb of internal capsule were positively correlated with the ratios of lactic acid/creatinine and glutamate/creatinine shown by 1H MRS (P<0.05).

CONCLUSIONSThe comparison of the signal intensity between the posterolateral lentiform nucleus and the posterior limb of internal capsule on T1-weighted images of the cerebral MRI is valuable for the diagnosis of neonatal HIE and the accuracy of diagnosis can be improved when combined with 1H MRS.

Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; Infant, Newborn ; Magnetic Resonance Imaging ; methods ; Magnetic Resonance Spectroscopy ; methods ; Male

OBJECTIVETo elucidate that diffusion weighted imaging (DWI) can be used to predict the injured regions of neonatal brain with hypoxic-ischemic encephalopathy (HIE) in the early phase of injury, and to measure the apparent diffusion coefficient (ADC) values in the multiple regions of the brain.

METHODThe participants in this study were twenty-six infants with HIE from neonatology ward hospitalized between July 2006 and July 2009. Nineteen patients had severe HIE, and seven had moderate HIE. DWI and conventional magnetic resonance imaging (MRI) were performed for each case within the first 72 hrs. The ADC values of eight regions of interest (ROIs) were measured in ten cases with severe HIE (ADC values group). ROIs included posterior limb of internal capsule (PLIC), ventrolateral thalami, basal ganglia, perirolandic cortex, occipital cortex, centrum semiovale, brainstem, and frontal white matter. Twelve neonates were enrolled as the control subjects.

RESULTSDuring the first 72 hrs, the conventional MRI of 26 patients showed subarachnoid hemorrhage in 5, subdural hemorrhage in 2, and mild high signal intensity in the cortex of only one patient. In the 19 cases with severe HIE, abnormal signal intensities were seen in ventrolateral thalami and perirolandic cortex of 17 patients (89%), and the remaining 2 infants showed abnormal cortex and subcortical white matter. In 7 cases with moderate HIE, 4 had abnormal signal intensity in the cortex and subcortical white matter, 2 had abnormal periventricular white matter, and only one showed abnormal signal intensity in the ventrolateral thalami and perirolandic cortex. In the ADC values group, the average ADC values of posterior limb of internal capsule (PLIC), ventrolateral thalami, basal ganglia, perirolandic cortex, occipital cortex, centrum semiovale, brainstem, and frontal white matter respectively were 0.68 (0.56 - 0.88), 0.73 ± 0.13, 0.67 ± 0.11, 0.78 ± 0.22, 0.90 ± 0.16, 0.87 ± 0.21, 0.73 ± 0.19, 1.32 ± 0.22 × 10(-3) mm(2)/S. In the control group, the average ADC values of posterior limb of internal capsule (PLIC), ventrolateral thalami, basal ganglia, perirolandic cortex, occipital cortex, centrum semiovale, brainstem, and frontal white matter respectively were 0.96 (0.95 - 1.02), 1.02 ± 0.90, 1.15 ± 0.99, 1.08 ± 0.07, 1.09 ± 0.08, 1.39 ± 0.20, 0.96 ± 0.05, 1.58 ± 0.18× 10(-3) mm(2)/S. There was statistically significant difference in the average ADC values between each of 8 ROIs of infants with HIE and healthy neonates (P < 0.01).

CONCLUSIONIn the first days after birth, the major injured regions of severe HIE were ventrolateral thalami and perirolandic cortex, the minor injured regions were cortex and subcortical white matter. Multiple regions of moderate HIE were injured, including cortex with subcortical white matter, periventricular white matter, and ventrolateral thalami with perirolandic cortex. The ADC values of the regions with abnormal signal intensity decreased, also some regions with the normal signal intensity.

Brain ; pathology ; Diffusion Magnetic Resonance Imaging ; methods ; Female ; Humans ; Hypoxia-Ischemia, Brain ; diagnosis ; Infant, Newborn ; Male