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We report the case of an 18-year old male with a history of asymmetric weakness and amyotrophy of distal upper extremities, suggestive of Hirayama disease. Magnetic resonance imaging (MRI) of the cervical spine was obtained both in flexion and neutral position. Flexion MRI showed forward displacement of the dura and subsequent cord compression, with associated marked enlargement and postcontrast enhancement of posterior epidural plexus. These findings are pathognomonic of the disorder. On neutral MRI abnormalities may be subtle: in our case, they included loss of physiological lordosis, asymmetric atrophy and increased T2 signal intensity of the lower anterior cervical cord. The ability to identify abnormalities on neutral MRI however is even more important in that it allows the radiologist to include a flexion sequence in the MRI examination, if not specifically requested by the referring physician, and in cases in which the suspicion of the disorder has not been raised.

Cluster headaches are characterized by painful unilateral periorbital attacks accompanied by dysautonomic symptoms. Although cluster headaches are classified as a primary headache disorder, a few cases of inflammatory, vascular or neoplastic conditions have been reported to cause or mimic cluster headaches. This raises the question as to the possible causal relationship between symptoms and an underlying structural lesion that may potentially interfere with the trigemino-autonomic reflex. We hereby report the first pediatric case of a brainstem cavernoma causing cluster headache-like symptoms. Of note, the child experienced pain relief after surgery. With regards to the pathophysiology, the observed ptosis observed in our child during the painful crises might be explained by mass effect of the cavernorma on the cisternal portion of the third cranial nerve. In addition, chronic inflammation of mesencephalic and motor nuclei of the trigeminal nerve by thalamo-pedoncular edema surrounding the lesion may have participated to painful pathways. This case illustrates that cluster headaches may occasionally be secondary to an underlying lesion. This advocates the need for MRI screening, especially as neurosurgical intervention may offer significant symptomatic relief and an improvement in the quality of life of these patients.

Brain metastasis (BM) is a frequent complication of systemic cancer usually associated with poor prognosis. Survival depends on numerous factors, which complicates prognosis and treatment. It has been suggested that BM growing from previously dormant disseminated tumour cells (DTCs) may exhibit a milder phenotype than BM derived from continuously progressing metastatic cells; however, to the best of our knowledge, the prognosis of patients presenting with BM from dormant DTCs is unknown. The present study retrospectively compared survival data, collected from a single neurosurgical centre, between patients presenting with BM from previously dormant DTCs and patients with non-dormant BM. A total of 262 medical records were reviewed. In the univariate Cox regression analysis, the median survival of the dormant BM group was statistically longer than that of the non-dormant group (P=0.048); a trend towards a longer survival persisted after correcting for age, presence of breast cancer and treatment options (P=0.057), which are all factors known to influence outcome. The improved outcome of these patients could be considered in models for prognostication. Moreover, the development of therapies able to eradicate dormant DTCs could provide a new promising strategy to prolong the survival of patients with a favourable prognosis.

Background: The central vein sign (CVS) is an imaging biomarker able to differentiate multiple sclerosis (MS) from other conditions causing similar appearance lesions on magnetic resonance imaging (MRI), including cerebral small vessel disease (CSVD). However, the impact of vascular risk factors (VRFs) for CSVD on the percentage of CVS positive (CVS+) lesions in MS has never been evaluated. Objective: To investigate the association between different VRFs and the percentage of CVS+ lesions in MS. Methods: In 50 MS patients, 3T brain MRIs (including high-resolution 3-dimensional T2*-weighted images) were analyzed for the presence of the CVS and MRI markers of CSVD. A backward stepwise regression model was used to predict the combined predictive effect of VRF (i.e. age, hypertension, diabetes, obesity, ever-smoking, and hypercholesterolemia) and MRI markers of CSVD on the CVS. Results: The median frequency of CVS+ lesions was 71% (range: 35%–100%). In univariate analysis, age (p < 0.0001), hypertension (p < 0.001), diabetes (p < 0.01), obesity (p < 0.01), smoking (p < 0.05), and the presence of enlarged-perivascular-spaces on MRI (p < 0.005) were all associated with a lower percentage of CVS+ lesions. The stepwise regression model showed that age and arterial hypertension were both associated with the percentage of CVS+ lesions in MS (adjusted R2 = 0.46; p < 0.0001 and p = 0.01, respectively). Conclusion: The proportion of CVS+ lesions significantly decreases in older and hypertensive MS patients. Although this study was conducted in patients with an already established MS diagnosis, the diagnostic yield of the previously proposed 35% CVS proportion-based diagnostic threshold appears to be not affected. Overall these results suggest that the presence of VRF for CSVD should be taken into account during the CVS assessment., SCOPUS: ar.j, info:eu-repo/semantics/published

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is considered to have potential neuro-invasiveness that might lead to acute brain disorders or contribute to respiratory distress in patients with coronavirus disease 2019 (COVID-19). This study investigates the occurrence of structural brain abnormalities in non-survivors of COVID-19 in a virtopsy framework., info:eu-repo/semantics/published

Acute hyperammonemic encephalopathy is a rare but life-threatening condition that might complicate liver disease as well as non-hepatic conditions. It can lead to coma and death, secondary to brain edema and intracranial hypertension. We present three cases of acute hyperammonemic encephalopathy of different etiologies and the observed brain MRI findings. Symmetrical extensive cortical signal abnormalities, typically involving the insular and cingulate cortices, often showing restricted diffusion, are commonly described. These specific imaging features should be recognized by the radiologist since prompt treatment of the condition is paramount., SCOPUS: ar.j, info:eu-repo/semantics/published

Summary Background and purpose Cerebral sinovenous thrombosis (CSVT) represents an increasingly recognized cause of pediatric stroke. Our purpose was to assess gender and age differences in the etiology, clinical presentation, and imaging features of CSVT in neonates and older children. Methods Subjects aged newborn to 18 years diagnosed with CSVT at the Lille university hospital between 2011 and 2014 were included. Results Eleven neonates and 16 non-neonates constituted the study population. The incidence of CSVT was significantly higher in male newborns. Clinical presentation did not vary significantly between the groups. Risk factors were age-dependent, with acute systemic illnesses significantly predominating in neonates (54%), whereas local infections, prothrombotic conditions, and trauma were more common in older children (36, 27, and 27% respectively). No predisposing factor could be identified in 36% of the neonates as compared to less than 5% of the non-neonates. Thrombosis of the deep venous structures was documented in 73% of the neonates whereas involvement of the superficial sinuses was significantly more frequent in the non-neonates group. Venous infarctions and extraparenchymal hemorrhages were significantly more frequent in the neonates group. Conclusion Male patients are at higher risk for CSVT than females. In neonates, involvement of the deep venous structures is significantly more common. Brain parenchymal and extraparenchymal changes occur more frequently in this age group than in older children.

Myelopathies have multiple causes and broad differential diagnoses, including demyelinating, metabolic, vascular and neoplastic disorders, often with distinctive imaging manifestations. Compressive myelopathy, especially of degenerative and neoplastic origin, is the most common cause of myelopathy, followed by inflammatory disorders such as multiple sclerosis, acute disseminating encephalomyelitis, neuromyelitis optica, and transverse myelitis of other etiologies. An accurate and early diagnosis will guide the treatment and will provide information about the prognosis of the patient. The aim of this review is to illustrate the magnetic resonance imaging features of different etiologies of myelopathy.

The aim of emergency imaging is to detect treatable lesions before secondary neurological damage occurs. CT plays a primary role in the acute setting of head trauma, allowing accurate detection of lesions requiring immediate neurosurgical treatment. CT is also accurate in detecting secondary injuries and is therefore essential in follow-up. This review discusses the main characteristics of primary and secondary brain injuries.

OBJECTIVE—Corrected QT (QTc) prolongation is predictive of cardiovascular mortality in both the general and diabetic populations. As part of the EURODIAB Prospective Complication Study, we have assessed the 7-year incidence and risk factors of prolonged QTc in people with type 1 diabetes. RESEARCH DESIGN AND METHODS—A total of 1,415 type 1 diabetic subjects, who had normal QTc at baseline, were reanalyzed after the 7-year follow-up period. QTc >0.44 s was considered abnormally prolonged. RESULTS—Cumulative incidence of prolonged QTc was 18.7%, which is twofold higher in women than in men (24.5 vs. 13.9%, P < 0.0001). At the baseline examination, incident cases were older and less physically active than nonincident cases, had higher mean values of systolic blood pressure and HDL cholesterol, and had higher frequencies of hypertension, coronary heart disease, and distal symmetrical polyneuropathy. In multivariate logistic regression analyses, female sex and higher values of A1C and systolic blood pressure were associated with the risk of prolonged QTc, whereas physical activity and BMI within the range of 21.5–23.2 kg/m2 were protective factors. In women, association with modifiable factors, particularly BMI, was stronger than in men. CONCLUSIONS—In type 1 diabetic subjects from the EURODIAB cohort, female sex, A1C, and systolic blood pressure are predictive of prolonged QTc, whereas physical activity and BMI within the range of 21.5–23.2 kg/m2 play a protective role. These findings are clinically relevant, as they may help to identify subjects at higher risk for prolonged QTc, as well as provide potential targets for risk-lowering strategies.

Chediak-Higashi syndrome is a rare inherited metabolic disorder characterized by partial oculocutaneous albinism, immunodeficiency, and neurological dysfunction. We present the brain magnetic resonance imaging (MRI) and MR spectroscopy (MRS) findings obtained during the accelerated phase of the disorder in an 8-year-old. The brain MRI manifestations at recurrences 15 months and 24 months later are reported as well.

Levels of serum heat shock protein 27 (sHsp27) have been studied in numerous cancer types, but their potential relevance in patients with hepatocellular carcinoma (HCC) is undetermined. Our aim was to compare sHsp27 levels in patients with HCC and HCC-free controls. Specifically, we recruited 71 patients with HCC (80 % with early tumour), 80 patients with chronic liver disease (59 with liver cirrhosis and 21 with chronic active hepatitis) and 42 healthy subjects. sHsp27 was measured by immunoenzymatic assay. Results showed that sHsp27 levels were significantly (p

Primary central nervous system (CNS) lymphoma (PCNSL) accounts for approximately 3% of all primary CNS tumors. Congenital or acquired immunodeficiency is the only established risk factor for PCNSL. Rates decreased slightly in the mid-1990s, concordantly with the decreasing rates of AIDS. However, the incidence has been increasing in the elderly immunocompetent population, and this trend seems to be independent of improvements in diagnostic techniques, and of overall trends in the incidence of brain tumors and systemic lymphomas. This study presents our experience with the imaging features of PCNSL. Computed tomography (CT) and magnetic resonance imaging (MRI) findings were reviewed in a series of 38 cases of pathologically proven PCNSL. The incidence rate of PCNSL was higher in men than in women (58% versus 42%). Mean age at presentation was 63 years; 120 lesions were demonstrated in the 38 patients, with a 53% frequency of tumor multiplicity. Both CT and MR mainly showed solitary or multiple well-defined round or oval-shaped mass lesions, typically hyperdense on unenhanced CT scans, iso to hypointense on T2 MR weighted images. These lesions also showed an increased signal intensity on diffusion-weighted images. Virtually all lesions enhanced after intravenous administration of contrastmedium. On (1)H-magnetic resonance spectroscopy ((1)H-MRS) most lesions presented increased Cho/Cr, Cho/NAA and lactate/Cr ratios when compared to normal gray matter. No changes in the imaging presentation have occurred over the past two decades, apart from lesions now being smaller at diagnosis. Our imaging findings are in agreement with the existing literature data and with the reported increasing trend of multifocal tumors. Our epidemiologic results add value to the existing evidence of increasing incidence rates among the immunocompetent elderly population.

“Laboratory investigations revealed leukopenia [white blood cells: 3,500 to 10,500/mm3 (normal values: 3,500 to 10,500/ mm3), polymorphonucleates: 1,500 cells/mm3 (normal values: ≥ 1,500 cells/mm3)], anemia [hemoglobin: 13.5 to 17.5 g/dL], and a relative decrease of thrombocytes [platelets: 169,000/mm3 (normal values: between between 150 and 400/ mm3]. Polymerase-chain-reaction tests failed to reveal evidence of herpes simplex virus, enterovirus, cytomegalovirus, varicella-zoster virus, Epstein-Barr virus or adenovirus. Cerebrospinal fluid analysis showed 24 nucleated cells consisting with lymphocytes [normal values: (×10-6/L)