Ischemic stroke on SARS-CoV2 vasculitis in a healthy young girl.

Background and Aims: In France, we noted the fifth wave of SARS-CoV2 pandemic, characterized by presence of Omicron variant. This variant is very contagious, but less often aggressive, especially in pediatric population. Methods: We report a case of a 10-year-old girl, previously healthy, not yet vaccinated for SARS-CoV2, presented to our emergency department for left hemiparesis associated with headache and vomiting, without any signs of respiratory tract infection. Results: Cerebral CT and MRI showed an ischemic stroke of right sylvian artery. Magnetic resonance angiography performed upon resurgence of new symptoms was in favor of vasculitis on the right internal carotid and right sylvian artery. PCR SARS-CoV2 was positive for Omicron variant. She fully recovered after few days and was treated with acetylsalicylic acid and intravenous corticosteroids. Conclusion: We report this case to raise awareness on the possible complications related to SARS-CoV2 infection and we highly recommend vaccination in this age group.

Morphological features in juvenile Huntington disease associated with cerebellar atrophy - magnetic resonance imaging morphometric analysis.

BACKGROUND: The imaging features of Huntington disease are well known in adults, unlike in juvenile-onset Huntington disease. OBJECTIVE: To conduct a morphometric magnetic resonance imaging (MRI) analysis in three juvenile Huntington disease patients (ages 2, 4 and 6 years old) to determine whether quantitative cerebral and cerebellar morphological metrics may provide diagnostically interesting patterns of cerebellar and cerebellar atrophy. MATERIALS AND METHODS: We report the cases of three siblings with extremely early presentations of juvenile Huntington disease associated with dramatic expansions of the morbid paternal allele from 43 to more than 100 CAG trinucleotide repeats. Automatic segmentation of MRI images of the cerebrum and cerebellum was performed and volumes of cerebral substructures and cerebellar lobules of juvenile Huntington disease patients were compared to those of 30 normal gender- and age-matched controls. Juvenile Huntington disease segmented volumes were compared to those of age-matched controls by using a z-score. RESULTS: Three cerebral substructures (caudate nucleus, putamen and globus pallidus) demonstrated a reduction in size of more than three standard deviations from the normal mean although it was not salient in one of them at clinical reading and was not diagnosed. The size of cerebellum lobules, cerebellum grey matter and cerebellum cortex was reduced by more than two standard deviations in the three patients. The cerebellar atrophy was predominant in the posterior lobe. CONCLUSION: Our study sheds light on atrophic cerebral and cerebellar structures in juvenile Huntington disease. Automatic segmentations of the cerebellum provide patterns that may be of diagnostic interest in this disease.

Catatonia and Autoimmune Conditions in Children and Adolescents: Should We Consider a Therapeutic Challenge?

OBJECTIVE: Catatonia as a result of autoimmune conditions offers new therapeutic opportunities for patients that child and adolescent psychiatrists should consider. However, the diagnosis is sometimes challenging when an autoimmune signature is not identified. METHODS: In this study, we aim to summarize seven cases from a 20-year series of 84 youths with catatonia, including three cases that represented a diagnostic challenge because of the absence of positive autoimmune testing. RESULTS: Immunosuppressive/modulatory treatment improved catatonic and psychotic features in all cases. CONCLUSION: To facilitate treatment decision-making, we propose a causality assessment score and a treatment algorithm, which may help clinicians consider whether an autoimmune condition is associated with catatonia.

MRI and suspected acute pyelonephritis in children: comparison of diffusion-weighted imaging with gadolinium-enhanced T1-weighted imaging.

OBJECTIVES: To evaluate the performance of diffusion-weighted imaging (DWI) against the reference standard of gadolinium-enhanced T1-weighted imaging (Gd-T1-WI) in children. METHODS: Thirty-nine consecutive patients (mean age 5.7 years) with suspected acute pyelonephritis underwent magnetic resonance imaging (MRI) including DWI and (the reference standard) Gd-T1-WI. Each study was read in double-blinded fashion by two radiologists. Each kidney was graded as normal or abnormal. Sensitivity and specificity of DWI were computed. Agreement between sequences and interobserver reproducibility were calculated (Cohen κ statistic and the McNemar tests). RESULTS: Thirty-two kidneys (41 %) had hypo-enhancing areas on Gd-T1-W images. The sensitivity and specificity of DWI were 100 % (32/32) and 93.5 % (43/46). DWI demonstrated excellent agreement (κ = 0.92,) with Gd-T1-W, with no significant difference (P = 0.25) in detection of abnormal lesions. Interobserver reproducibility was excellent with DWI (κ = 0.79). CONCLUSION: DWI enabled similar detection of abnormal areas to Gd-T1-WI and may provide an injection-free means of evaluation of acute pyelonephritis. KEY POINTS: • Diffusion weighted magnetic resonance imaging (DWI) can confirm acute pyelonepritis. • DWI provided comparable results to gadolinium enhanced T1-W MRI in acute pyelonepritis. • Contrast medium injection could be avoided for diagnosing acute pyelonephritis by MRI. • MRI with T2-WI and DWI provide a fast and comprehensive diagnostic tool.

Exhaled nitric oxide fractions are well correlated with clinical control in recurrent infantile wheeze treated with inhaled corticosteroids.

Fractional exhaled nitric oxide (FeNO) is a non-invasive marker of bronchial inflammation in asthma. However, the interest of FeNO measurement remained limited in infantile wheeze. The aim of this prospective study was to evaluate the feasibility and reproducibility of FeNO off-line measurement in very young children with recurrent wheeze and to assess whether clinical control of infantile wheeze correlates with FeNO levels. Two exhalation samples were collected in mylar balloon during quite tidal breathing. FeNO measurements were performed off-line by a NO analyzer. The participating patients were aged ≤36 months, wheezes had started before the age of 24 months, and they were receiving maintenance treatment with inhaled corticosteroids for at least 3 months duration. The studied population comprised of 40 uncontrolled infants with persistent wheezy respiratory symptoms, median age 14.5 months, and 40 with optimal controlled infantile wheeze, median age 14 months. The reproducibility was excellent (r = 0.95; p < 0.0001). There was a significant difference in FeNO levels between the groups of persistent wheeze and well-controlled infants: 19.8 (2.5-99.3) ppb vs. 7.7 (0.6-29.5) ppb, p < 0.0001. At a FeNO level >15 ppb, the predictive values for uncontrolled disease were as follows: positive predictive value = 65%, negative predictive value = 90%. FeN0 levels were not increased by atopy or passive tobacco. Off-line assessment of FeNO is feasible, reproducible, and well accepted in wheezy very young children. Optimal clinical control of infantile wheeze appeared to be associated with the control of bronchial inflammation when evaluated by FeNO measurements.

Bolus methylprednisolone efficacy for uncontrolled exacerbation of cystic fibrosis in children.

We present here the clinical course of 4 children with cystic fibrosis, deltaF508/deltaF508, who were admitted with severe respiratory distress and in whom no improvement was obtained by intensive antibiotic therapy and systemic corticosteroids. Chest computed-tomography scans showed hyperinflation and atelectasis. The severity of these exacerbations was explained neither by visible mucus plugging nor by allergic bronchopulmonary aspergillosis. We hypothesized that these clinical features were related to a severe inflammatory process in small airways. Therefore, a high-dose short course of methylprednisolone (1 g/1.73 m(2) per day for 3 days) was given; all the patients' conditions were dramatically improved, and the therapy was safe. To our knowledge, this is the first reported use of bolus methylprednisolone in the treatment of uncontrolled pulmonary exacerbation in children with cystic fibrosis.