Search

This is a pre-copyediting, author-produced PDF of an article accepted for publication in The Journal of Rheumatology following peer review. The definitive publisher-authenticated version Glerup, M., Stoustrup, P., Matzen, L.H., Rypdal, V., Nordal, E., Frid, P. ... Klit Pedersen, T. (2020). Longterm Outcomes of Temporomandibular Joints in Juvenile Idiopathic Arthritis: 17 Years of Followup of a Nordic Juvenile Idiopathic Arthritis Cohort is available online at: https://doi.org/10.3899/jrheum.190231 in The Journal of Rheumatology, 47(5), 730-738: https://www.jrheum.org/. Objective - To determine the prevalence of orofacial symptoms, dysfunctions, and deformities of the temporomandibular joint (TMJ) in juvenile idiopathic arthritis (JIA) 17 years after disease onset. Methods - Drawn from a prospective, population-based Nordic JIA cohort with disease onset from 1997 to 2000, 420 consecutive cases were eligible for orofacial evaluation of TMJ involvement. The followup visit included demographic data, a standardized clinical orofacial examination, and full-face cone-beam computed tomography (CBCT). For comparison, 200 age-matched healthy controls were used. Results - Of 420 eligible participants with JIA, 265 (63%) were included (mean age 23.5 ± 4.2 yrs) and completed a standardized clinical orofacial examination. Of these, 245 had a full-face CBCT performed. At least 1 orofacial symptom was reported by 33%. Compared to controls, the JIA group significantly more often reported TMJ pain, TMJ morning stiffness, and limitation on chewing. Further, among participants reporting complaints, the number of symptoms was also higher in JIA. The mean maximal incisal opening was lower in the JIA group (p < 0.001), and TMJ pain on palpation was more frequent. Condylar deformities and/or erosions were observed in 61% as assessed by CBCT, showing bilateral changes in about 70%. Risk factors of condylar deformities were orofacial dysfunction or biologic treatment; enthesitis-related arthritis was protective. Conclusion - This study of the longterm consequences of TMJ involvement in a population-based JIA cohort reports persistence of comprehensive symptoms, dysfunctions, and damage of the TMJ into adulthood. We suggest interdisciplinary followup of JIA patients also in adulthood.

We describe four pediatric patients with ulcerative colitis and cerebral sinovenous thrombosis and review the pediatric and adult literature on the treatment of sinovenous thrombosis. All of our patients had headache as the initial complaint with onset during an ulcerative colitis flare. Evaluation for hypercoagulable states revealed heterozygosity for prothrombin gene mutation and increased homocysteine level in one patient and mild elevation of anticardiolipin antibodies in two patients. Treatment in the acute period included thrombolysis, heparin, and low-molecular-weight heparin. Chronic therapy included warfarin, low-molecular-weight heparin, and aspirin. Peripheral vein thrombosis occurred in two patients while not on antiplatelet or anticoagulation therapy. Neurologic outcome was positive in this series without complications of therapy, suggesting that aggressive therapy should be considered. Although anticoagulation therapy of sinovenous thrombosis is controversial, particularly in the context of intestinal hemorrhage, it can be beneficial given the possibility of an ongoing hypercoagulable state. (J Child Neurol 2002;17:479-482).

Activated protein C resistance caused by an Arg506Gln mutation in the factor V gene (factor V Leiden mutation) is the most common cause of familial thrombosis. This mutation is associated with arterial and venous thromboembolic disease in neonates, infants, and children, but is not a significant risk factor for ischemic stroke in adults. We report on 3 babies with different neonatal cerebrovascular disorders including ischemic infarction and hemorrhagic stroke who are heterozygous for factor V Leiden mutation. One infant had multiple thrombi in the fetal placental vasculature. This is the first reported association between hemiplegic cerebral palsy, placental thrombosis, and factor V Leiden mutation. We suspect that activated protein C resistance may be an important cause of in utero cerebrovascular disease and hemiplegic cerebral palsy.

To describe trends in cerebral palsy (CP) prevalence, severity, and associated impairments among 139 Icelandic children (65 males, 74 females) born from 1990 to 1996 (period one) and 1997 to 2003 (period two).A population-based study using systematically collected data on motor functioning and associated impairments of children with CP. Mean age at assessment was 5 years 5 months (SD 7.68 mo) in period one and 5 years 5 months (SD 10.44 mo) in period two. Infants with postneonatal CP were excluded.Prevalence of CP per 1000 live births was 2.2 in period one and 2.3 in period two (p=0.862); it decreased from 1.5 to 0.9 for children born at term, was stable for preterm births, but increased from 33.7 to 114.6 for very preterm births (p=0.002). Concurrently, neonatal and infant mortality rates decreased in Iceland. The proportion of children born preterm increased over time (p=0.002), whereas improvements in gross motor function assessed with the Gross Motor Function Classification System were confined to term births (p=0.009). The proportion of children with diplegia increased, accompanied by a decrease in the proportion with quadriplegia (p=0.047). Furthermore, among term births there was a significant reduction over time in the proportion of children with epilepsy (p=0.030) and in the proportion with two or more associated impairments (p=0.030).Although CP prevalence remained stable over 14 years, we observed a decrease in prevalence and severity of the disability among term births.

There are only two reported cases of unilateral posterior cervical spinal cord infarction as a result of vertebral artery dissection. We describe the first reported case of bilateral posterior spinal cord infarction caused by vertebral artery dissection.