Αρχειοθήκη ιστολογίου

Τετάρτη 25 Οκτωβρίου 2017

Similarities and Differences Between Migraine in Children and Adults: Presentation, Disability, and Response to Treatment

Abstract

Purpose of Review

This review presents findings from investigations of migraine in children and adults. Similarities and differences in the presentation, related consequences, and treatments between children and adults are reviewed.

Recent Findings

Significant similarities exist in the presentation, disability, and treatments for migraine between children and adults. Despite such similarities, many adult migraine treatments adapted for use in children are not rigorously tested prior to becoming a part of routine care in youth. Existing research suggests that not all approaches are equally effective across age groups. Specifically, psychological treatments are shown to be somewhat less effective in adults than in children. Pharmacological interventions found to be statistically significant relative to placebo in adults may not be as effective in children and have the potential to present more risk than benefit when used in youth. The placebo effect in both children and adults is robust and is need of further study. Better understanding of treatment mechanisms for all interventions across the age spectrum is needed.

Summary

Although migraine treatments determined to be effective for adults are frequently adapted for use in children with little evaluation prior to implementation, existing research suggests that this approach may not be the best practice. Adaptation of adult pharmacological treatment for use in youth may present a particular risk in comparison to benefits gained. Because of the known efficacy of psychological treatments, such as cognitive behavioral therapy, more universal use of these interventions should be considered, either as first-line treatment or in combination with pill-based therapies.



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An Acquired Form of Dandy-Walker Malformation with Enveloping Hemosiderin Deposits

Dandy-Walker malformation (DWM) is a posterior fossa anomaly characterized by hypoplasia and upward rotation of the cerebellar vermis and cystic dilation of the fourth ventricle. The cyst of DWM rarely extends posteriorly to almost completely fill the entire posterior fossa, which mimics primary cerebellar agenesis, a cerebellar porencephalic cyst, and an arachnoid cyst due to the lack of clarity of the thin cystic wall. A 10-month-old female born at 23 weeks' gestation with cerebellar hemorrhage in the neonatal period was admitted to our hospital with dysphagia and side-to-side head bobbing. The detection of hemosiderin deposits enveloping the cyst wall by T2 star-weighted angiography (SWAN) was useful for the differential diagnosis of an acquired form of DWM from primary cerebellar agenesis. Cyst fenestration successfully improved dysphagia and head bobbing. A pathological specimen of the perforated cyst consisted of collagen fibers with hemosiderin deposits but lacked congenital cyst components. In infants with posterior fossa cysts, SWAN will be useful for a differential diagnosis between DWM and primary cerebellar agenesis.

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Familial acanthosis nigricans with p.K650T FGFR3 mutation

Abstract

Acanthosis nigricans (AN) is a pigmentary skin disorder, which may present in association with clinical disorders such as obesity and malignancy. Occasionally, this unique skin manifestation is seen in alliance with several skeletal disorders, such Crouzon syndrome, achondroplasia and hypochondroplasia (HCH). These orthopedic disorders are known to have genetic changes in FGFR3. Recently, AN was reported in HCH with p.K650T mutation in FGFR3, and to date, there are only three reports, comprising 18 cases, describing AN harboring this specific gene mutation. Herein, we detail three new cases of AN with p.K650T FGFR3 mutation, and review the 21 known cases.



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Regional incidences of adult T-cell leukemia/lymphoma with cutaneous involvement in Japan

Abstract

Between 2008 and 2015, 462 newly-diagnosed adult T-cell leukemia/lymphoma (ATLL) patients with cutaneous involvement were found from the nationwide registry for Japanese patients with cutaneous lymphoma, of which 391 were selected for the study. They ranged in age from 28 to 93 years (median, 69 years), and included 215 men and 176 women (male : female ratio = 1.2). The 391 patients comprised 193 (50%) with smoldering type, 52 (13%) with chronic type, 44 (11%) with lymphoma type and 102 (26%) with acute type. The total number of patients in Kyushu/Okinawa was 8.8-times higher than that in Kanto, which was set as the reference value, while the estimated prevalence of human T-lymphotropic virus 1 (HTLV-1) carriers in Kyushu/Okinawa has been reported to be only 2.5-times higher than that in Kanto. In this study, the annual incidence of ATLL per 100 000 residents in Kyushu/Okinawa was 32-times higher than that in Kanto. Our results indicated the higher incidence rate of ATLL in the endemic area than those in the non-endemic areas in Japan, compared with the regional differences of HTLV-1 prevalence determined by serological HTLV-1 screening for blood donors. In addition, this analysis revealed that regional differences of mycosis fungoides/Sézary syndrome incidence rates were very small compared with those of ATLL.



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Novel KRT9 missense mutation in a Japanese case of epidermolytic palmoplantar keratoderma



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Dramatic effect of hydroxychloroquine on lupus alopecia

Abstract

Lupus alopecia is usually difficult to treat. We report a case of a 40-year-old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later.



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Successful treatment of relapsed folliculotropic mycosis fungoides with mogamulizumab followed by intensity-modulated radiotherapy



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