Autoantibody status, neuroradiological and clinical findings in children with acute cerebellitis.

BACKGROUND: Acute cerebellitis (AC) in children and adolescents is an inflammatory disease of the cerebellum due to viral or bacterial infections but also autoimmune-mediated processes. OBJECTIVE: To investigate the frequency of autoantibodies in serum and CSF as well as the neuroradiological features in children with AC. MATERIAL AND METHODS: Children presenting with symptoms suggestive of AC defined as acute/subacute onset of cerebellar symptoms and MRI evidence of cerebellar inflammation or additional CSF pleocytosis, positive oligoclonal bands (OCBs), and/or presence of autoantibodies in case of negative cerebellar MRI. Children fulfilling the above-mentioned criteria and a complete data set including clinical presentation, CSF studies, testing for neuronal/cerebellar and MOG antibodies as well as MRI scans performed at disease onset were eligible for this retrospective multicenter study. RESULTS: 36 patients fulfilled the inclusion criteria for AC (f:m = 14:22, median age 5.5 years). Ataxia was the most common cerebellar symptom present in 30/36 (83 %) in addition to dysmetria (15/36) or dysarthria (13/36). A substantial number of children (21/36) also had signs of encephalitis such as somnolence or seizures. In 10/36 (28 %) children the following autoantibodies (abs) were found: MOG-abs (n = 5) in serum, GFAPα-abs (n = 1) in CSF, GlyR-abs (n = 1) in CSF, mGluR1-abs (n = 1) in CSF and serum. In two further children, antibodies were detected only in serum (GlyR-abs, n = 1; GFAPα-abs, n = 1). MRI signal alterations in cerebellum were found in 30/36 children (83 %). Additional supra- and/or infratentorial lesions were present in 12/36 children, including all five children with MOG-abs. Outcome after a median follow-up of 3 months (range: 1 a 75) was favorable with an mRS ≤2 in 24/36 (67 %) after therapy. Antibody (ab)-positive children were significantly more likely to have a better outcome than ab-negative children (p = .022). CONCLUSION: In nearly 30 % of children in our study with AC, a range of abs was found, underscoring that autoantibody testing in serum and CSF should be included in the work-up of a child with suspected AC. The detection of MOG-abs in AC does expand the MOGAD spectrum.

N-Methyl-D-Aspartate Receptor Encephalitis with Psychiatric Symptoms and an Ovarian Teratoma Detected by MRI in a 17-Year-Old Girl.

N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare antibody-mediated autoimmune encephalitis often associated with an ovarian teratoma in adolescent females. Here we present a 17-year-old girl with only and unusual psychiatric symptoms as part of her NMDAR encephalitis in combination with a very small ovarian teratoma suspected by magnetic resonance (MR) imaging and finally histologically confirmed. We further review the literature of NMDAR encephalitis in combination with an ovarian teratoma and discuss the recommended radiological workup in children with a suspected ovarian tumor.

[Comparative ultrasound visualization of clinically relevant structures for evaluating the infant hip joint utilizing trapezoidal vs. parallel transducers]. / Vergleichende Darstellung beurteilungsrelevanter Strukturen der kindlichen Hüfte mittels paralleler und trapezförmiger Ultraschallabbildung.

PURPOSE: Sonographic evaluation of the infant hip joint according to the method of Graf has proven to be an important pediatric investigative instrument. Our goal was to investigate quantitatively whether (and in what ways) the clinically relevant infant hip joint structures visualize differently when utilizing trapezoidal as opposed to linear transducers. Our approach was both theoretical via a mathematical model and practical with in-vivo measurements in neonates. MATERIALS AND METHODS: In a prospective study: 1. theoretical and computed analyses were performed for both linear and trapezoidal transducers regarding their respective accuracy for demonstrating the anatomic geometry of the infant hip, assuming not only correctly centered transducer positioning but also cases with off-centered displacement in the cranial or caudal direction; 2. both hip joints in 97 infants were examined by experienced investigators with comparison of the results for parallel vs. trapezoidal transducers. RESULTS: Theoretical mathematical error analysis reveals no intrinsic systemic deviations between trapezoidal vs. parallel transducers in US scanning of the infant hip and furthermore no inherent disadvantages in the trapezoidal technique. Even when off-center transducer alignments of 1.5 cm are employed in the mathematical models, there is no significant relative distortion of the required anatomic structures when comparing the characteristics of both transducers. The practical in-vivo data from our 97 neonates confirmed the theoretical considerations. CONCLUSION: No loss of accuracy or other negative factors are evident when trapezoidal transducers are used to visualize the infant hip joint in comparison with the customary parallel technique. There are no significantly measurable differences between the two approaches.