Objective: To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD) in children. Methods: For the case series study, 21 children with AESD from Peking University First Hospital, Provincial Children's Hospital Affiliated to Anhui Medical University, Children's Hospital of Fudan University, and Shanxi Children's Hospital who were diagnosed and treated from October 2021 to July 2023 were selected. Clinical data were collected to summarize their clinical information, imaging, and laboratory tests, as well as treatment and prognostic characteristics. Descriptive statistical analysis was applicated. Results: Of the 21 cases with AESD, 11 were males and 10 were females, with the age of onset of 2 years and 6 months (1 year and 7 months, 3 years and 6 months). Of the 21 cases, 18 were typical cases with biphasic seizures. All typical cases had early seizures within 24 hours before or after fever onset. Among them, 16 cases had generalized seizures, 2 cases had focal seizures, and 7 cases reached the status epilepticus. Of the 21 cases, 3 atypical cases had late seizures in biphasic only. The late seizures in the 21 cases occurred on days 3 to 9. The types of late seizures included focal seizures in 12 cases, generalized seizures in 6 cases, and both focal and generalized seizures in 3 cases. Diffusion-weighted imaging (DWI) test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named "bright tree sign" in all cases. The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease. Among 21 cases, 20 had been misdiagnosed as autoimmune encephalitis, central nervous system infection, febrile convulsions, posterior reversible encephalopathy syndrome, acute disseminated encephalomyelitis, and hemiconvulsion-hemiplegia-epilepsy syndrome. All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect. By July 2023, 18 cases were under follow-up. Among them, 17 cases were left with varying degrees of neurologic sequelae, including 11 cases with post-encephalopathic epilepsy; 1 recovered completely. Conclusions: AESD is characterized by biphasic seizures clinically and "bright tree sign" on DWI images. Symptomatic and supportive treatments are recommended. The immunotherapy is ineffective. The prognosis of AESD is poor, with a high incidence of neurological sequelae and a low mortality.
Male ; Female ; Child ; Humans ; Infant ; Child, Preschool ; Posterior Leukoencephalopathy Syndrome/complications* ; Seizures/etiology* ; Brain Diseases/diagnostic imaging* ; Status Epilepticus ; Seizures, Febrile/diagnostic imaging*

Humans ; Pseudotumor Cerebri/therapy* ; Intracranial Hypertension/therapy*

OBJECTIVES: Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical imaging syndrome. The causes of RPLS are complex and diverse, the pathogenesis is not yet clear. The onset is urgent and the onset age span is large, ranging from children to the elderly. The clinical symptoms of RPLS have no significant specificity, which can be manifested as headache, blurred vision, disturbance of consciousness or seizures. Clinicians have little knowledge on the disease, which may lead to misdiagnosis or missed diagnosis. This study aims to analyze and summarize the MRI changes and clinical characteristics regarding RPLS patients, so as to provide basis for rapid diagnosis and timely intervention for this disease. METHODS: The clinical data and complete imaging data of 77 patients with RPLS diagnosed in Xiangya Hospital of Central South University from January 2012 to March 2021 were retrospectively collected. The main image data include T1 weighted imaging (T1WI), T2 weighted imaging (T2WI), T2 liquid attenuation inversion recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b value=1 000×10-6 mm2/s), and apparent diffusion coefficient (ADC). The case group included 63 patients who underwent DWI examination, and 71 normal controls matched in age and sex. The characteristics of patients' magnetic resonance signals and the ADC value of 19 regions of interest (ROI) were analyzed. The differences in bilateral ADC value in the case group, the difference of ADC value between the case group and the normal control group, and the difference of ADC value in the case group before and after treatment were compared. RESULTS: Compared with the normal control group, the ADC value of the right frontal lobe, bilateral parietal lobe, bilateral thalamus, bilateral head of caudate nucleus, left lenticular nucleus, right internal capsule, bilateral temporal lobe and pons in the case group were significantly higher (all P<0.01). There was no significant difference in ADC value of bilateral sides of the case group and before and after treatment in the case group (all P>0.01). The lesions of RPLS were widely distributed and multiple, usually high signal in the posterior parieto temporo occipital lobe or pons of the brain, and involved the cortex and subcortical white matter. Most of them were bilateral, but not completely symmetrical. CONCLUSIONS The imaging manifestations of RPLS and the occurrence and development of clinical symptoms are basically synchronous. The imaging manifestations are specific. Magnetic resonance imaging can show the range of involvement of RPLS. ADC value can provide information on the severity of the disease and predict the prognosis. There are few reversible diseases. It is very important to fully understand and timely diagnose the disease.
Child ; Humans ; Aged ; Posterior Leukoencephalopathy Syndrome/etiology* ; Retrospective Studies ; Magnetic Resonance Imaging/methods* ; Brain/pathology* ; Diffusion Magnetic Resonance Imaging

Humans ; Cranial Sinuses/surgery* ; Intracranial Hypertension/etiology*

Humans ; Intracranial Hemorrhage, Hypertensive ; East Asian People ; Hypertension ; Cerebral Hemorrhage

PURPOSE: Hypertonic fluids such as mannitol and half-molar sodium lactate are given to treat intracranial hypertension in patients with severe traumatic brain injury (TBI). In this study, sodium lactate was compared to mannitol in patients with TBI to investigate the efficacy in reducing intracranial pressure (ICP). METHODS: This study was a systematic review with literature research on articles published in any year in the databases of PubMed, ScienceDirect, Asian Journal of Neurosurgery, and Cochrane Central Register of Controlled Trials. The keywords were "half-molar sodium lactate", "mannitol", "cerebral edema or brain swelling", and "severe traumatic brain injury". The inclusion criteria were (1) studies published in English, (2) randomized control trials or retrospective/prospective studies on TBI patients, and (3) therapies including half-molar sodium lactate and mannitol and (4) sufficient data such as mean difference (MD) and risk ratio (RR). Data analysis was conducted using Review Manager 5.3. RESULTS: From 1499 studies, a total of 8 studies were eligible. Mannitol group reduced ICP of 0.65 times (MD 0.65; p = 0.64) and improved cerebral perfusion pressure of 0.61 times (MD 0.61; p = 0.88), better than the half-molar group of sodium lactate. But the half-molar group of sodium lactate maintained the mean arterial pressure level of 0.86 times, better than the mannitol group (MD 0.86; p = 0.09). CONCLUSION Half-molar sodium lactate is as effective as mannitol in reducing ICP in the early phase of brain injury, superior over mannitol in an extended period. It is able to prevent intracranial hypertension and give better brain tissue perfusion as well as more stable hemodynamics. Blood osmolarity is a concern as it increases serum sodium.
Brain Edema ; Brain Injuries, Traumatic/drug therapy* ; Diuretics, Osmotic/therapeutic use* ; Humans ; Intracranial Hypertension/etiology* ; Intracranial Pressure ; Mannitol/therapeutic use* ; Prospective Studies ; Retrospective Studies ; Saline Solution, Hypertonic ; Sodium Lactate

OBJECTIVES: Idiopathic intracranial hypertension (IIH) is a syndrome that excludes secondary causes such as intracranial space-occupying lesion, hydrocephalus, cerebrovascular disease, and hypoxic ischemic encephalopathy. If not be treated promptly and effectively, IIH can cause severe, permanent vision disability and intractable, disabling headache. This study aims to explore the clinical and image features for IIH, to help clinicians to understand this disease, increase the diagnose rate, and improve the outcomes of patients. METHODS: We retrospectively analyzed 15 cases of IIH that were admitted to Xiangya Hospital, Central South University, during January 2015 to September 2020. The diagnosis of IIH was based on the updated modified Dandy criteria. We analyzed clinical data of patients and did statistical analysis, including age, gender, height, weight, medical history, physical examination, auxiliary examination, treatment and outcome. RESULTS: There were 10 females and 5 males. Female patients were 22 to 42 years old with median age of 39.5. Male patients were 27 to 52 years old with the median age of 44.0. The BMI was 24.14-34.17 (28.71±2.97) kg/m CONCLUSIONS IIH primarily affects women of childbearing age who are overweight. The major hazard of IIH is the severe and permanent visual loss. Typical image signs have high specificity in IIH diagnosis. Prompt diagnosis and effective treatment are significantly important to improve the outcomes of patients.
Adult ; Anemia, Iron-Deficiency ; Female ; Humans ; Intracranial Hypertension ; Male ; Middle Aged ; Pseudotumor Cerebri/diagnostic imaging* ; Retrospective Studies ; Ventriculoperitoneal Shunt ; Young Adult

Idiopathic intracranial hypertension,also known as pseudotumor cerebri,is a syndrome characterized by raised intracranial pressure of unknown cause.These patients present normal neuroimaging and cerebrospinal fluid analysis while increased intracranial pressure and associated symptoms and signs.Delay of treatment can cause severe visual impairment.There are some new understandings of this disease,and we will review the pathogenesis,diagnosis,and treatment of idiopathic intracranial hypertension.
Humans ; Intracranial Hypertension ; Neuroimaging ; Pseudotumor Cerebri/therapy*

Posterior reversible encephalopathy syndrome (PRES) is a newly described adverse effect possibly associated with gonadotropin-releasing hormone (GnRH) agonist therapy. We report a case of PRES after 2 doses of depot GnRH agonists in a 44-year-old woman with a huge myoma uteri and iron-deficiency anemia. Brain magnetic resonance imaging showed high signal lesions in both occipital lobes on fluid-attenuated inversion-recovery (FLAIR) images, compatible with PRES. After treatment with anticonvulsant, she recovered both radiographically and clinically. The association between PRES and GnRH agonist use is still enigmatic, and thus should be further clarified.
Adult ; Anemia, Iron-Deficiency ; Brain ; Brain Diseases ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Leuprolide ; Magnetic Resonance Imaging ; Myoma ; Occipital Lobe ; Posterior Leukoencephalopathy Syndrome* ; Uterus

PURPOSE: Cerebral small vessel disease (SVD) is known to be associated with ischemic stroke, intracerebral hemorrhage (ICH), and cognitive impairment. In this retrospective observational study, we explored SVD markers on MRI relevant to spontaneous ICH. MATERIALS AND METHODS: The ICH group consisted of 150 consecutive patients with a first primary parenchymal ICH, and the control group consisted of 271 age- and sex-matched individuals who underwent brain MRI in a health care center. We compared cerebral microbleeds (CMBs), white matter hyperintensities (WMHs), enlarged perivascular space (EPVS), and lacunae in the ICH and control groups. RESULTS: A total of 1278 CMB lesions were identified in 121 of the 150 patients in the ICH group (80.6%), while 77 CMB lesions were found in 32 of the 271 individuals in the control group (11.8%). WMH and EPVS were more severe and lacunae were more frequent in the ICH patients than in the control group. When receiver operating characteristic (ROC) curves were plotted, number of CMBs most significantly predicted ICH. All imaging markers were significantly associated with ICH in every age group. The location of CMBs coincided with the location of ICH, and ICH volume correlated with CMB count. CONCLUSION: All MRI markers for SVD were worse in ICH patients than in healthy controls, and these markers were prominent even in young ICH patients. Lacunae, WMH, EPVS, and CMB should be considered as factors related with spontaneous ICH.
Brain ; Cerebral Amyloid Angiopathy ; Cerebral Hemorrhage ; Cerebral Small Vessel Diseases ; Cognition Disorders ; Delivery of Health Care ; Humans ; Hypertension ; Intracranial Hemorrhages ; Magnetic Resonance Imaging ; Observational Study ; Retrospective Studies ; ROC Curve ; Stroke ; White Matter