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Idiopathic intracranial hypertension (IIH) is a rare condition characterized by increased intracranial pressure, with an unknown cause. However, the pathophysiology of antibiotic-induced IIH remains unclear. The clinical symptoms include headache, visual disturbances, and vomiting. The diagnosis is confirmed by an elevated intracranial pressure (ICP) with normal CSF study and cerebral imaging. Management includes discontinuing the offending antibiotic and reducing ICP with medications such as acetazolamide or diuretics. Therefore, surgical intervention may be necessary in severe cases. In this article, we report the case of a 19-year-old patient, admitted with symptoms of intracranial hypertension syndrome, occurring three days after receiving antibiotics (gentamicin, penicillin). Physical examination revealed bilateral optic disc edema. Cerebral magnetic resonance imaging (MRI) revealed indirect signs of intracranial hypertension. The CSF pressure measurement was approximately 290 mmHg, while CSF and other laboratory blood tests were normal. The patient received methylprednisolone bolus and topiramate (50 mg/day). A month later, the clinical outcome showed regression of headaches and regression of the papilledema.

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APP duplication is a rare genetic cause of Alzheimer disease and cerebral amyloid angiopathy (CAA). We aimed to evaluate the phenotypes of APP duplications carriers.
Clinical, radiological, and neuropathological features of 43 APP duplication carriers from 24 French families were retrospectively analyzed, and MRI features and cerebrospinal fluid (CSF) biomarkers were compared to 40 APP-negative CAA controls.
Major neurocognitive disorders were found in 90.2% symptomatic APP duplication carriers, with prominent behavioral impairment in 9.7%. Symptomatic intracerebral hemorrhages were reported in 29.2% and seizures in 51.2%. CSF Aβ42 levels were abnormal in 18/19 patients and 14/19 patients fulfilled MRI radiological criteria for CAA, while only 5 displayed no hemorrhagic features. We found no correlation between CAA radiological signs and duplication size. Compared to CAA controls, APP duplication carriers showed less disseminated cortical superficial siderosis (0% vs 37.5%, p = 0.004 adjusted for the delay between symptoms onset and MRI). Deep microbleeds were found in two APP duplication carriers. In addition to neurofibrillary tangles and senile plaques, CAA was diffuse and severe with thickening of leptomeningeal vessels in all 9 autopsies. Lewy bodies were found in substantia nigra, locus coeruleus, and cortical structures of 2/9 patients, and one presented vascular amyloid deposits in basal ganglia.
Phenotypes associated with APP duplications were heterogeneous with different clinical presentations including dementia, hemorrhage, and seizure and different radiological presentations, even within families. No apparent correlation with duplication size was found. Amyloid burden was severe and widely extended to cerebral vessels as suggested by hemorrhagic features on MRI and neuropathological data, making APP duplication an interesting model of CAA.

暂无摘要。

Due to high survival rates, long-term sequelae, especially neurotoxicity, need to be considered in childhood acute leukemias. In this retrospective analysis of morphologic changes of the brain in children treated for acute leukemias, we included 94 patients (77 ALL, 17 AML; 51 male, 43 female; median age: 5 years) from a single center. We analyzed 170 cranial MRI scans (T2, FLAIR axial) for morphologic alterations of the brain and variations of the ventricular width (GDAH). In addition, the corresponding literature was reviewed. More than 50% of all patients showed cerebral pathomorphologies (CP). They were seen more often in children with ALL (55.8%), ≤ 6 years of age (60.8%), in relapse (58.8%) or after CNS irradiation (75.0%) and included white matter changes, brain atrophy, sinus vein thrombosis and ischemic events. GDAH significantly enlarged mainly in children up to 6 years, with relapse, high-risk leukemias or ALL patients. However, GDAH can normalize again. The number of intrathecal Methotrexate applications (≤12 vs. >12) showed no correlation to morphologic alterations besides a significant increase in GDAH (−0.3 vs. 0.9 mm) between the first and last follow-up MRI in ALL patients receiving >12 ith. MTX applications. The role of ith. MTX on CP needs to be further investigated and correlated to the neurocognitive outcome of children with acute leukemias.

OBJECTIVE: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare manifestation related to CAA, thought to be more severe. We aimed to compare the clinical and radiological outcomes of CAA-ri and non-inflammatory CAA.
METHODS: We retrospectively included all patients with CAA-ri from 13 French centers. We constituted a sex- and age-matched control cohort with non-inflammatory CAA and similar disease duration. Survival, autonomy and cognitive evolution were compared after logistic regression. Cerebral microbleeds (CMB), intracerebral hemorrhage, cortical superficial siderosis and hippocampal atrophy were analyzed as well as CSF biomarker profile and APOE genotype when available. Outcomes were compared using Kaplan-Meier curves and log-rank tests.
RESULTS: Data from 48 CAA-ri patients including 28 already reported and 20 new patients were analyzed. Over a mean of 3.1 years, 11 patients died (22.9%) and 18 (37.5%) relapsed. CAA-ri patients were more frequently institutionalized than non-inflammatory CAA patients (30% vs 8.3%, p < 0.001); mortality rates remained similar. MMSE and modified Rankin scale scores showed greater severity in CAA-ri at last follow-up. MRI showed a higher number of CMB at baseline and last follow-up in CAA-ri (p < 0.001 and p = 0.004, respectively). CSF showed lower baseline levels of Aß42 in CAA-ri than non-inflammatory CAA (373.3 pg/ml vs 490.8 pg/ml, p = 0.05). CAA-ri patients more likely carried at least one APOE ε4 allele (76% vs 37.5%, adjusted p = 0.05) particularly as homozygous status (56% vs 6.2%, p < 0.001).
CONCLUSIONS: CAA-ri appears to be more severe than non-inflammatory CAA with a significant loss of autonomy and global higher amyloid burden, shown by more CMB and a distinct CSF profile. This burden may be partially promoted by ε4 allele.

BACKGROUND: Cerebral sinovenous thrombosis (CSVT) is a rare disease with potential catastrophic consequences. However, neonatal brain damage after venous injury and long-term neurologic outcomes have been poorly investigated. Some found an association between site and number of sinus occlusions, severity of lesions, clinical presentation and the neurodevelopmental outcome.
METHODS: We describe the case of a term newborn girl with multiple CSVT who presented with clonic seizures and who received early treatment with heparin. MRI scans showed a progressive recanalization of deep venous system, and only minor cerebral lesions were present at 3 months of life. Neurocognitive outcome was normal at 12 months of life.
CONCLUSIONS: This case demonstrates that multiple CSVT presenting with severe seizures does not necessarily underlie major cerebral lesions or lead to severely abnormal neurodevelopmental outcome.

BACKGROUND: Brain Magnetic Resonance Imaging (MRI) examination of cerebral small vessel disease (CSVD) may help screen vascular cognitive impairment. A recently estimated CSVD score system was suggested to capture the overall CSVD burden. The study aimed to detect the association between systemic evaluation score of cerebral vascular imaging parameters with cognitive functions.
METHODS: This was a cross-sectional study in community settings. From October 2017 to September 2018, elder (≧60) residents were recruited through on-site visit in 6 communities from Shanghai, China. The participants underwent brain MRI, carotid ultrasound, laboratory tests of blood and urine samples. Cognitive function was evaluated using Mini-Mental State Examination (MMSE). MRI score of CSVD was calculated according to the 2012 standard for the evaluation of statistical changes in imaging.
RESULTS: Total 171 subjects completed survey and examinations. There were 55 participants diagnosed with cognitive impairment, with a total percentage of 32.2%. Participants with and without cognitive impairment showed significant differences in age, BMI and education level. Cognitive impaired participant had more disease history/comorbidity of hypertension and chronic renal insufficiency, higher level of creatinine, as well as lower level of full blood count (FBC) and alanine aminotransferase (ALT). A significant difference was detected in CSVD score between participants with and without cognitive impairment. Results of linear regression analysis showed significant negative correlations between MMSE score and both left and right carotid artery peak systolic velocity (PSV), however the CSVD score was only borderline (P = 0.0566) positively correlated with MMSE. Multivariate linear correlation analysis including all collected risk factor data showed that left carotid artery PSV score was among the independent negative correlated factors of MMSE. Multivariate binary logistic analysis showed that age, education and history of hypertension were the only statistically associated factors of cognitive impairment.
CONCLUSIONS: The current study identified high prevalence of cognitive impairment in a Chinese community. In addition, correlations between cerebral vascular disease imaging status and cognitive functions were confirmed although the sample size limited the possibility of screening cognitive impairment with imaging technique.

UNASSIGNED: Cerebral microbleeds (CMB) are associated with intracerebral haemorrhage. Therefore they may represent a concern if anticoagulant and/or antiplatelet therapy is needed. The aim of this study was to determine the prevalence of CMB in patients with acute myocardial infarction (AMI), and to follow their progression at 3 months under dual antiplatelet therapy (DAPT).
UNASSIGNED: This prospective study included patients aged over 60 hospitalized in intensive cardiac care unit in our city for AMI. These patients underwent a first brain Magnetic resonance imaging (MRI) within 72 h of admission, that was repeated 3 months.
UNASSIGNED: 108 patients were included between November 2016 and December 2018. The prevalence of CMB was 21.3%, with a female predominance of 65.2% vs 32.1% (p = 0.004). Diabetes is significantly associated with the presence of CMB, 45.5% vs 21.2% (p = 0.021). Patients with at least one acute CMB had higher haemorrhagic risk as evaluated with CRUSADE score (40.5 ± 13.6 vs 31.2 ± 14.8 (p = 0.004).Multivariate analysis showed that only female sex was associated with the presence of a CMB on the initial MRI. On repeated MRI, an increase in CMB was observed in 6% of patients.Our results suggest that discharge treatment with anticoagulant in combination with antiplatelet therapy may be an independent predictor of early progression of CMB.
UNASSIGNED: Our study confirms the high prevalence of CMB in patients over 60 years with AMI. The association of anticoagulant with DAPT, 3 months after stenting, may be an independent factor of CMB progression.