1517 The Clinical Features and Outcomes of Moyamoya Disease in a Medical Center in Taiwan

Background:Polymyositis (PM) and dermatomyositis (DM) are autoimmune inflammatory diseases characterized by proximal myositis. Dysphagia has been reported to develop in 35 to 62% of PM/DM patients and known as poor prognosis factor.Objectives:The purpose of this study is to determine the clinical characteristics of PM/DM patients who present with deglutition disorder.Methods:Consecutive patients with PM/DM who visited National Hospital Organization Tokyo Medical Center between April 2010 and January 2021 are included in this study. We compared clinical features between the patients with and without dysphagia. The diagnosis of dysphagia was based on videofluorography swallow study, and dysphagia requiring gastrostomy was defined as severe dysphagia. The clinical characteristics compared in this study were following: age of onset, levels of serum creatine kinase (CK) and lactate dehydrogenase(LDH), sense of dysphagia, manual muscle test (MMT) score, and complication of malignancy or interstitial pneumonia.Results:A total of 73 patients with PM/DM were identified. Among them, 12 patients were diagnosed with dysphagia, and 5 patients developed severe dysphagia. Patients with dysphagia had the following characteristics compared to patients without dysphagia: higher levels of serum LDH (833.7 ± 500.1 U/L vs 471.9 ± 321.0 U/L, p = 0.0088), higher levels of serum CK at initial examination (6070.3 ± 7184.8 IU/L vs 1534.7 ± 2978.8 IU/L, p = 0.0086) and more frequent sense of dysphagia (90.9% vs 10.6%, p< 0.0001), lower MMT score(3.18 ± 1.07 vs 4.31 ± 0.75, p = 0.0017). In addition to those, patients with severe dysphagia presented older age of onset (mean age 69.4 ± 12.0 vs 51.7 ± 14.8, p = 0.014), more frequent complication of malignancy (80.0% vs 14.8%, p= 0.0048) and less frequent complication of interstitial pneumonia (0.0% vs 55.5%, p= 0.023).Conclusion:These results indicate that dysphagia develops frequently in PM/DM patients with higher levels of serum LDH or CK, sense of dysphagia and low MMT score. Among them, patients with elderly onset or malignancy are at risk for sever dysphagia, and should be treated carefully.Disclosure of Interests:None declared.

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Clinical Features of Unilateral Moyamoya Disease

Neurologia medico-chirurgica ◽

10.2176/nmc.50.378 ◽

2010 ◽

Vol 50 (5) ◽

pp. 378-385 ◽

Cited By ~ 13

Author(s):

Kentaro HAYASHI ◽

Kazuhiko SUYAMA ◽

Izumi NAGATA

Keyword(s):

Clinical Features ◽

Moyamoya Disease ◽

Unilateral Moyamoya Disease

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The Clinical Features of Geriatric Ocular Trauma in a Regional Emergency Medical Center

Journal of the Korean Geriatrics Society ◽

10.4235/jkgs.2013.17.4.192 ◽

2013 ◽

Vol 17 (4) ◽

pp. 192-197

Author(s):

Jae Ho Jang ◽

Seung Woo Lee

Keyword(s):

Clinical Features ◽

Ocular Trauma ◽

Medical Center ◽

Emergency Medical

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Clinical Features of Moyamoya Disease: An Overview

Moyamoya Disease Update ◽

10.1007/978-4-431-99703-0_16 ◽

2010 ◽

pp. 107-109 ◽

Cited By ~ 1

Author(s):

Yong-Seung Hwang

Keyword(s):

Clinical Features ◽

Moyamoya Disease

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Clinical Features of Moyamoya Disease in the United States

Stroke ◽

10.1161/01.str.29.7.1347 ◽

1998 ◽

Vol 29 (7) ◽

pp. 1347-1351 ◽

Cited By ~ 174

Author(s):

David Chiu ◽

Peter Shedden ◽

Patti Bratina ◽

James C. Grotta

Keyword(s):

United States ◽

Clinical Features ◽

Moyamoya Disease ◽

The United States

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Clinical features of familial juvenile cases of moyamoya disease: analysis of patients treated in a single institute over a 28-year period

Journal of Neurosurgery Pediatrics ◽

10.3171/2013.4.peds12420 ◽

2013 ◽

Vol 12 (2) ◽

pp. 175-180 ◽

Cited By ~ 3

Author(s):

Maki Mukawa ◽

Tadashi Nariai ◽

Yoshiharu Matsushima ◽

Kikuo Ohno

Keyword(s):

Clinical Features ◽

Moyamoya Disease ◽

Mr Angiography ◽

Posterior Cerebral Artery ◽

Clinical Course ◽

Intelligence Scale ◽

Familial Cases ◽

Sporadic Cases ◽

Tokyo Medical ◽

Disease Analysis

Object The authors compared the clinical features between familial and sporadic cases of moyamoya disease (MMD) by retrospectively analyzing data on patients with MMD registered in the database of Tokyo Medical and Dental University over a period of 28 years. Methods In total, 383 patients with hospital records at Tokyo Medical and Dental University from 1980 to 2007 were registered into the database. The data on all of these patients were retrospectively reviewed to clarify the occurrence of familial cases. Clinical features of child or adolescent patients (< 20 years of age) with MMD were compared between familial and sporadic cases in a subgroup of patients who were registered after 1995, initially diagnosed using MR angiography, and assessed using an intelligence scale. Results Familial occurrence was observed in 59 patients (15.4%) in 40 pedigrees. The clinical features of juvenile patients were analyzed in 124 patients, 22 (17.7%) of whom had familial histories. In comparison with the sporadic cases, patients with familial histories were significantly younger at onset (4.7 vs 6.6 years old), had significantly more cortical infarction (59.1% vs 25.5%), and had significantly more stenoocclusive lesions in the posterior cerebral artery (45.4% vs 24.5%). The rate of patients with intellectual disturbance (intelligence quotient < 75) was significantly larger in the familial cases (47.4%) than in the sporadic cases (17.8%). Conclusions This survey of the clinical features of familial MMD suggests that patients with familial MMD had a more serious clinical course in childhood than the sporadic MMD cases.

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Clinical features, surgical treatment, and outcome of intracranial aneurysms associated with moyamoya disease

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2020.09.006 ◽

2020 ◽

Vol 80 ◽

pp. 274-279

Author(s):

Peicong Ge ◽

Xun Ye ◽

Qian Zhang ◽

Xingju Liu ◽

Xiaofeng Deng ◽

...

Keyword(s):

Surgical Treatment ◽

Clinical Features ◽

Moyamoya Disease ◽

Intracranial Aneurysms

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Prevalence, clinical features and treatment pattern of patients with concurrent diagnoses of rheumatoid arthritis and psoriatic disease: results of a 14-year retrospective study in a tertiary referral center

Therapeutic Advances in Chronic Disease ◽

10.1177/2040622319847900 ◽

2019 ◽

Vol 10 ◽

pp. 204062231984790

Author(s):

Kai-Lung Chen ◽

Hsien-Yi Chiu ◽

Jui-Hsiang Lin ◽

Jian-De Ye ◽

Yi-Hsuan Cho ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Clinical Features ◽

Age Of Onset ◽

Medical Center ◽

Skin Lesions ◽

Tertiary Referral ◽

Female Ratio ◽

Tertiary Referral Center ◽

Referral Center ◽

Psoriatic Disease

Background: Multiple comorbidities, including rheumatoid arthritis (RA), have been reported to be associated with psoriasis. Objective: This study aimed to determine the prevalence and the clinical features of RA among patients with psoriasis in a tertiary referral center. Methods: Between January 2000 and December 2013, all patients coded with psoriatic disease (ICD-9 CM 696.0 OR ICD-9 CM696.1) and RA (ICD-9 CM 714.0) in a tertiary medical center were enrolled. Results: There were 10,844 patients and 9073 patients with psoriatic disease and RA identified by diagnostic codes, respectively. Among patients with psoriasis, 111 patients had claim-based diagnosis of RA (1.02%). By reviewing medical records and telephone interview or clinic visits, 25 of the 111 patients (0.23%) was identified unequivocally as having concurrent RA. Among them, 17 (68%) were female and 16 (64%) patients developed arthritis prior to the onset of psoriasis with a mean lag of 6.3 years (1–19 years); 8 (32%) had psoriasis skin lesions prior to the onset of arthritis with a mean lag of 6.9 years (3–20 years); 1 (4%) had skin lesions and arthritis in the same time; 17 (68%) patients also fulfilled the CASPAR classification criteria for psoriatic arthritis. The mean age of onset for arthritis was 49.6 years old. Conclusions: The prevalence of RA in psoriasis might be overestimated in some previous studies using claimed database. Patients with concurrent RA and psoriasis showed a comparable age of onset and male to female ratio, but had more axial involvements compared to patients without psoriasis.

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