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Objective: Our understanding of IgG4‐RD and pachymeningitis has grown substantially, but the optimal approach for diagnosis, management, and long‐term outcomes is still an area of uncertainty. Methods: HUVAC is a database for IgG4‐RD patients, this database was retrospectively evaluated for pachymeningeal disease. Demographic, clinical, serological, imaging, histopathological data, and treatment details were re‐interpreted in patients with pachymeningitis. Results: Among 97 patients with IgG4‐RD, 6 (6.2%) had pachymeningitis. None of these patients had extracranial features, and also, in most of the patients, serum IgG4 levels were normal. Tentorium cerebelli and transverse sinus dura were the most commonly involved in the posterior fossa. During 18 months of median follow‐up on steroid+‐rituximab, none of them relapsed as pachymeningitis. Conclusion: Our patients were mainly older males with sole neurological involvement. Non‐specific headache was the most common manifestation, and serum IgG4 levels were not useful for diagnosis. Typical radiology and tentorial thickening should suggest IgG4‐RD and prompt an early biopsy. Moreover, accompanying hypophysitis could also be a clue. With steroids+ rituximab treatment, no relapse related to meningeal involvement was seen in long‐term follow‐up. [ABSTRACT FROM AUTHOR]

Claude syndrome is a rare midbrain stroke syndrome characterized by ipsilateral third cranial nerve palsy and contralateral hemiataxia. So far, only a few cases have been reported in childhood. We present two children with Claude syndrome at 9 and 15 years of age. The typical clinical picture was consistent with brain magnetic resonance imaging findings. A thorough investigation regarding the underlying etiology revealed no definite diagnosis but clues suggestive of probable neuro-Behcet disease. Awareness of pediatric neurologists on arterial ischemic stroke has been increasing over the past decades, enabling timely diagnosis and appropriate management of rare childhood cases with midbrain stroke. [ABSTRACT FROM AUTHOR]

Objective: Our aim is to describe an unusual pattern of craniocervical pneumatization resulting in a spontaneous fracture in a patient, who presented with tinnitus. Methods: Temporal CT was ordered in a patient with tinnitus lasting over a year. His audiogram was normal. No history of previous trauma, recent flight, diving, or weight lifting was present. Further questioning revealed his habitual Valsalva maneuvers to relieve symptoms of nasal congestion. Results: Temporal CT showed abnormal pneumatization of the occipital bone and C1 vertebra. A definite cortical defect resulted with the presence of air around the neighboring soft tissues. The emphysema was most prominent in the suboccipital fossa but it was also seen in the epidural space of the spinal canal. Conclusion: Abnormal pneumatization of the skull base and cervical vertebra can be symptomatic due to its tendency to fracture. The presumed cause of pneumatization in this patient is habitual Valsalva maneuvers. [ABSTRACT FROM AUTHOR]

PURPOSE lthough clinical ophthalmologic findings have been reported, no study documented magnetic resonance imaging (MRI) findings in mucopolysaccharidosis (MPS) type VI. The aim of this study was to determine the ophthalmologic imaging findings of MPS type VI in the pediatric age group retrospectively. METHODS Brain MRIs of 10 patients with MPS type VI and 49 healthy children were evaluated independently by two pediatric radiologists for the following characteristics: globe volume, ocular wall thickness, and optic nerve sheath diameter for each orbit. The means of the measurement of each group were compared by using an independent t-test. Agreement and bias between reviewers were assessed by intra-class correlation coefficients (ICC). RESULTS A total of 59 children [32 girls (54.23%), 27 boys (45.77%); age range, 4-16 years; mean age, 10.37 ± 3.73 years] were included in the study. Statistical analysis revealed smaller eyeballs and thicker ocular walls of patients with MPS type VI (P < .001 and P < .001, respectively). However, there was no statistically significant difference in terms of optic nerve sheath diameter between the two groups (P=.648). CONCLUSION Patients with MPS type VI displayed reduced globe volumes and increased ocular wall thicknesses compared to the healthy children. Therefore, we recommend that ophthalmologic imaging findings might prove to be an auxiliary tool in the diagnosis of MPS patients. [ABSTRACT FROM AUTHOR]

Purpose: To compare the inter-center cranial computed tomography (CT) acquisition rates, CT findings, CT related radiation dose, and variability of CT acquisition parameters for neurologic events among patients with implantable cardioverter-defibrillator (ICD) or left ventricular assist device (LVAD).Methods: A total of 224 patients [ICD group (n = 155) and LVAD group (n = 69)] who had at least one cranial CT scan were enrolled from three medical centers. The variability and effect of the number, indication, and findings of cranial CT scans as well as CT acquisition parameters including tube potential, tube current, tube rotation time (TI), slice collimation (cSL), and spiral or sequential scanning techniques on CT dose index volume (CTDIvol), total dose length product (DLP) were analyzed.Results: The mean DLP value of Center A and mean CTDIvol values of Center A and C were significantly lower than Center B (p < 0.001). The mean CTDIvol and DLP values in the ICD group were substantially lower than the LVAD group (p<0.001). The most potent parameters causing the changes in CTDIvol and DLP were kV, mAs values, and CT scanning technique as sequential or spiral according to multivariate linear regression analysis.Conclusion: Cranial CT acquisition parameters and radiation doses vary significantly between centers, which necessitates optimization of cranial CT protocols to overcome the cumulative radiation dose burden in patients with neurologic events. [ABSTRACT FROM AUTHOR]