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Mahmoud H Ayesh Haj Yousef,1 Ziad Audat,2 Duha M Al-Shorafat,3 Sohaib Al-Khatib,4 Ammar K Daoud1 1Department of Internal Medicine, Faculty of Medicine, King Abdullah University Hospital, Jordan University of Science and Technology, Irbid, Jordan; 2Department of Special Surgery, Faculty of Medicine, King Abdullah University Hospital, Jordan University of Science and Technology, Irbid, Jordan; 3Department of Neurology, Faculty of Medicine, King Abdullah University Hospital, Jordan University of Science and Technology, Irbid, Jordan; 4Department of Pathology and Microbiology, Faculty of Medicine, King Abdullah University Hospital, Jordan University of Science and Technology, Irbid, JordanCorrespondence: Mahmoud H Ayesh Haj Yousef, Department of Internal Medicine, Faculty of Medicine, Jordan University of Science and Technology, P.O. Box 3030, Irbid, 22110, Jordan, Email ayeshmahmoud@hotmail.comBackground: Primary diffuse large B-cell lymphoma of the bone (PB-DLBCL) is a rare type of extra-nodal lymphoma. This study aimed to examine the clinical characteristics, outcomes, treatment modalities and risk of central nervous system relapse (CNSR) among adult Jordanian patients with PB-DLBCL.Methods: This retrospective study included patients aged > 16 years who were diagnosed with PB-DLBCL and treated at our hospital between 2002 and 2021. Clinical characteristics, treatment modalities, outcomes and CNSR events were extracted from the hospital’s data system and analysed. Patients were categorised into unifocal (UF) and multifocal (MF) PB-DLBCL groups according to the number of bone sites involved. The involvement of only one site was defined as UF, whereas the involvement of two or more sites was defined as MF.Results: In total, 12 patients were diagnosed with PB-DLBCL. Their median age was 47.5 years (range, 17– 80 years). The male:female ratio was 1:1. There were eight patients in the UF PB-DLBCL group and four in the MF PB-DLBCL group. All patients received treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone. In the UF PB-DLBCL group, the male:female ratio was 5:3, the median age was 41 years, and the follow-up duration was 9– 135 (mean, 83.3) months. In the MF PB-DLBCL group, the male:female ratio was 1:3, the median age was 51.5 years, and the survival time was 3– 11 (mean, 7) months. Three patients with vertebral UF PB-DLBCL underwent early vertebroplasty without complications. The most common site involved was the vertebral column. Most patients with UF PB-DLBCL achieved complete remission (CR), whereas no patients with MF PB-DLBCL achieved CR.Conclusion: PB-DLBCL is rare in adult Jordanian patients. UF PB-DLBCL is more common than MF PB-DLBCL. Patients with UF PB-DLBCL had a good prognosis. Patients with MF PB-DLBCL had a high international prognostic index score, risk of CNSR and short survival time.Keywords: primary diffuse large B-cell lymphoma of the bone, WHO classification of primary bone lymphoma, unifocal, multifocal

Dopa-responsive dystonia (DRD) is caused by an impaired dopamine biosynthesis due to a GTP-cyclohydrolase-1 (GCH1) deficiency, resulting in a combination of dystonia and parkinsonism. However, the effect of GCH1 mutations and levodopa treatment on motor control beyond simple movements, such as timing, action preparation and feedback processing, have not been investigated so far. In an active time estimation task with trial-by-trial feedback, participants indicated a target interval (1200 ms) by a motor response. We compared 12 patients tested (in fixed order) under their current levodopa medication ("ON") and after levodopa withdrawal ("OFF") to matched healthy controls (HC), measured twice to control for repetition effects. We assessed time estimation accuracy, trial-to-trial adjustment, as well as task- and feedback-related pupil-linked arousal responses. Patients showed comparable time estimation accuracy ON medication as HC but reduced performance OFF medication. Task-related pupil responses showed the reverse pattern. Trial-to-trial adjustments of response times were reduced in DRD, particularly OFF medication. Our results indicate differential alterations of time estimation accuracy and task-related arousal dynamics in DRD patients as a function of dopaminergic medication state. A medication-independent alteration of task repetition effects in DRD cannot be ruled out with certainty but is discussed as less likely., (© 2022. The Author(s).)

Introduction: GCH1 mutations have been linked to decreased striatal dopamine and development of dopa-responsive dystonia (DRD) and Parkinsonism. Sensory and sensorimotor integration impairments have been documented in various forms of dystonia. DRD patients with confirmed GCH1 mutations have demonstrated normal short-latency afferent inhibition (SAI), a measure of sensorimotor inhibition, under chronic dopaminergic replacement therapy (DRT), but reduced inhibition after a single l-dopa dose following 24 h withdrawal. Studies have revealed normal SAI in other forms of dystonia but reductions with DRT in Parkinson's disease. Longitudinal changes in sensorimotor inhibition are unknown., Methods: We analyzed sensorimotor inhibition using two different measures: SAI and somatosensory-motor inhibition using dual-site transcranial magnetic stimulation (ds-TMS). SAI was measured using digit stimulation 25 ms prior to contralateral primary motor cortex (M1) TMS. DS-TMS was measured using TMS over the somatosensory cortex 1 or 2.5 ms prior to ipsilateral M1 stimulation. A total of 20 GCH1 mutation carriers and 20 age-matched controls were included in the study. SAI and ds-TMS were evaluated in GCH1 mutation carriers both OFF and ON DRT compared to controls. Furthermore, longitudinal changes of SAI were examined in a subset of the same individuals that were measured ∼five years earlier., Results: Neither SAI nor ds-TMS were significantly different in GCH1 mutation carriers relative to controls. No effects of DRT on SAI or ds-TMS were seen but SAI decreased over time in mutation carriers OFF DRT., Conclusion: Our longitudinal results suggest changes in SAI that could be associated with plasticity changes in sensorimotor networks., (Copyright © 2021. Published by Elsevier Ltd.)

Objective: The objective of this study was to assess the 12-week efficacy and safety of oral glycopyrrolate for moderate-to-severe sialorrhea in Parkinson's disease (PD)., Background: Chronic moderate-to-severe sialorrhea has a negative impact on quality of life in PD. There is no robust evidence for oral treatments for sialorrhea longer than 1 week., Methods: This was a 12-week, double-blinded, placebo-controlled, parallel phase II study in patients with PD and Movement Disorder Society-Unified Parkinson's Disease Rating Scale item 2.2 > 2. The intervention was glycopyrrolate up to 4.5 mg/d; the primary outcome was sialorrhea related-disability (Radboud Oral Motor Inventory for Parkinson's Disease-Saliva). We used an intention-to-treat analysis. A P < 0.05 was deemed significant., Results: We recruited 28 patients (age, 71.1 ± 6.9 years; PD duration, 11.4 ± 7.2 years; Radboud Oral Motor Inventory for Parkinson's Disease-Saliva, 22.4 ± 5.7). Glycopyrrolate was superior to placebo at 12 weeks in the Radboud Oral Motor Inventory for Parkinson's Disease-Saliva (between-group difference, 5.3; 95% confidence interval, 1.0-9.6). Dry mouth was the most common adverse event (glycopyrrolate, n = 6; placebo, n = 2)., Conclusions: The results support the efficacy of glycopyrrolate to treat sialorrhea-related disability up to 12 weeks and contribute to addressing unmet nonmotor care needs in PD. © 2020 International Parkinson and Movement Disorder Society., (© 2020 International Parkinson and Movement Disorder Society.)