scholarly journals Why It Is Not Always Anxiety: A Tough Diagnosis of Stiff Person Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Carmen Elena Cervantes ◽  
Hsien Lee Lau ◽  
Tina Ataian Binazir ◽  
Keith O. O’Brien ◽  
Jonathan S. Cross
Keyword(s):  
Anxiety Disorder ◽  
Hospital Admissions ◽  
Beta Blockers ◽  
Panic Attacks ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Gamma Aminobutyric Acid ◽  
Magnetic Resonance Imaging Mri ◽  
Electroencephalogram Eeg

Anxiety disorder is a commonly used diagnosis that may mask underlying conditions. Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive rigidity and painful muscle spasms affecting axial and lower extremity musculature. These episodes can be triggered by sudden movement, noise, or emotional stress, which may present as a psychiatric condition. We report the case of a 30-year-old female who presented with recurrent panic attacks with multiple prior hospital admissions for anxiety, rigidity, and difficulty in walking. Previous electroencephalogram (EEG) and brain and cervical spine magnetic resonance imaging (MRI) were unremarkable. She was empirically treated with diazepam and beta-blockers for SPS, which was confirmed by positive glutamic acid decarboxylase (GAD) antibodies. The patient’s symptoms became refractory to benzodiazepines and required steroids with intravenous immunoglobulin (IVIG). Her rigidity subsequently responded to plasmapheresis. In SPS, antibodies in the cerebrospinal fluid (CSF) most commonly target the GAD antigen on gamma-aminobutyric acid (GABA) neurons. The goal of treatment is to ameliorate symptoms and improve quality of life. Our case of SPS was masked as generalized anxiety disorder for at least six years since onset of symptoms. The criteria for both diagnoses may overlap as seen in this patient.

scholarly journals Cerebellar Ataxia Followed by Stiff Person Syndrome in a Patient with Anti-GAD Antibodies

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sinali O. Seneviratne ◽  
Katherine A. Buzzard ◽  
Belinda Cruse ◽  
Mastura Monif
Keyword(s):  
Cerebellar Ataxia ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Gamma Aminobutyric Acid ◽  
Neurological Manifestations ◽  
Rare Occurrence ◽  
Gad Antibodies ◽  
Long Term Follow Up

Anti-GAD antibody syndrome is a result of the production of antibodies against glutamic acid decarboxylase (GAD), the main enzyme responsible for the production of gamma-aminobutyric acid (GABA). Several neurological manifestations including cerebellar ataxia and stiff person syndrome have been reported in association with anti-GAD antibodies. In this paper, we present a case of a young woman with anti-GAD antibodies who initially presented with cerebellar ataxia followed by stiff person syndrome three and a half years later. Having both cerebellar ataxia and stiff person syndrome is a rare occurrence in anti-GAD antibody syndrome. We emphasise the importance of long-term follow-up of patients with anti-GAD antibody syndrome, as delayed neurological manifestations can occur.

Stiff-person syndrome with acute recurrent peripheral vertigo: possible evidence of gamma aminobutyric acid as a neurotransmitter in the vestibular periphery

2007 ◽  
Vol 122 (6) ◽  
pp. 636-638 ◽  
Author(s):  
R Teggi ◽  
L O Piccioni ◽  
G Martino ◽  
C Bellini ◽  
M Bussi
Keyword(s):  
Glutamic Acid ◽  
Glutamic Acid Decarboxylase ◽  
Aminobutyric Acid ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Gamma Aminobutyric Acid ◽  
Vestibular Damage ◽  
Peripheral Vertigo

AbstractObjective:We report a case of a 58-year-old man suffering from stiff-person syndrome and recurrent peripheral vertigo.Method:A case report and a review of the recent literature on stiff-person syndrome are presented.Results:The patient presented with recurrent episodes of vertigo with a pure peripheral pattern and with concomitant episodes of burning muscle pain, muscle twitching, weight gain and fatigue, worsening with tension or stress that also occurred in periods without vertigo. Cochlear examinations only showed presbyacusis-like hearing loss. The diagnosis of stiff-person syndrome was made with electromyographic examination and from findings in the blood and cerebrospinal fluid of high titres of anti-glutamic acid decarboxylase (GAD67) autoantibodies. In a two-year follow-up period, therapy for stiff-person syndrome abolished episodes of both stiffness and vertigo.Conclusion:As far as we know, no other clinical case of acute vestibular damage with a possible correlation with anti-glutamic acid decarboxylase antibodies has been described. Peripheral vertigo possibly related to a lack of gamma aminobutyric acid underlines a possible role of gamma aminobutyric acid as a neurotransmitter in the peripheral vestibular system.

scholarly journals Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Susanne Buechner ◽  
Igor Florio ◽  
Loredana Capone
Keyword(s):  
Neurological Disorder ◽  
Clinical Manifestations ◽  
Case Series ◽  
Acid Decarboxylase ◽  
Stiff Person Syndrome ◽  
Gamma Aminobutyric Acid ◽  
Immunomodulatory Agents ◽  
Recurrent Vomiting ◽  
Significant Disability ◽  
Gaba Transmission

Background. Stiff person syndrome (SPS) is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. SPS can be classified into classic SPS, paraneoplastic SPS, and SPS variants. Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase (GAD) are observed. Similarly, paraneoplastic SPS is usually linked to anti-amphiphysin antibodies. Treatment is based on drugs enhancing gamma-aminobutyric acid (GABA) transmission and immunomodulatory agents.Case Series. Patient 1 is a 45-year-old male affected by the classic SPS, Patient 2 is a 73-year-old male affected by paraneoplastic SPS, and Patient 3 is a 68-year-old male affected by the stiff limb syndrome, a SPS variant where symptoms are confined to the limbs. Symptoms, diagnostic findings, and clinical course were extremely variable in the three patients, and treatment was often unsatisfactory and not well tolerated, thus reducing patient compliance. Clinical manifestations also included some unusual features such as recurrent vomiting and progressive dysarthria.Conclusions. SPS is a rare disorder that causes significant disability. Because of its extensive clinical variability, a multitask and personalized treatment is indicated. A clearer understanding of uncommon clinical features and better-tolerated therapeutic strategies are still needed.

scholarly journals Stiff-Person Syndrome: Seeing Past Comorbidities to Reach the Correct Diagnosis

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Jared Hicken ◽  
Daniel Ramirez ◽  
Mark Rigby ◽  
Aram Minasian
Keyword(s):  
Correct Diagnosis ◽  
Acid Decarboxylase ◽  
Muscle Rigidity ◽  
African American Female ◽  
Stiff Person Syndrome ◽  
Gamma Aminobutyric Acid ◽  
Axial Muscle ◽  
History Of ◽  
The Central Nervous System ◽  
Psychotic Features

Stiff-person syndrome (SPS) is a rare disorder seen in approximately one in one million people. Although it is rare, the symptoms and findings of a typical case should paint a clear clinical picture for those who are familiar with the disease. The primary findings in SPS include progressive axial muscle rigidity as well as muscle spasms. These symptoms most commonly occur in the setting of antibodies against Glutamic Acid Decarboxylase (GAD), the rate-limiting enzyme in the production of Gamma-Aminobutyric Acid (GABA), which is the primary inhibitory enzyme in the central nervous system. Here, we report the case of a 65-year-old African-American female with a past medical history of hypothyroidism, anxiety, and depression with psychotic features who presented with axial muscle rigidity and lactic acidosis. She had been symptomatic for several months and reported extensive workups performed at two previous hospitals without a definitive diagnosis. A complete neurological and musculoskeletal investigation yielded no positive findings except for the presence of GAD antibodies. The patient was treated with diazepam, tizanidine, and Intravenous Immunoglobulin (IVIG) with significant improvement, thus solidifying the diagnosis of SPS, a rare autoimmune and/or paraneoplastic syndrome.

RESULTS OF ENDOVASCULAR MYOCARDIAL REVASCULARIZATION AND QUALITY OF LIFE IN SUCH PATIENTS AGED UNDER 40

2020 ◽  
pp. 44-57
Author(s):  
F.Z. Abdullaev ◽  
F.E. Abbasov ◽  
N.M. Babaev ◽  
L.S. Shikhieva ◽  
G.A. Amrakhova
Keyword(s):  
Quality Of Life ◽  
Myocardial Infarction ◽  
Coronary Artery ◽  
Stable Angina ◽  
Myocardial Revascularization ◽  
Overweight And Obesity ◽  
Panic Attacks ◽  
Recurrent Myocardial Infarction ◽  
Stent Diameter

The aim of the paper is to correlate the results of endovascular myocardial revascularization (EMR) in patients under 40 years of age with acute coronary syndrome (ACS) / stable angina, primary EMR age ≤35/36–40 and stent diameter. Materials and Methods. The authors examined 208 patients (24–40 years of age) with coronary artery disease: 157 patients (75.5 %) aged 36–40; 51 patients (24.5 %) aged ≤35. All in all, 165 (79.3 %) patients underwent EMR; 32 patients (15.4 %) underwent coronary artery bypass grafting; 11 patients (5.3 %) refused medical intervention. EMR results and the quality of life were studied in 126 patients (76.4 %) at 10–107 months of observation (62.5±2.6 months). Results. 84 patients with ACS (50.9 %) and 81 patients with stable angina (49.1 %) underwent EMR. There were no any peri-procedural complications. The authors observed 0 % hospital and 30-day mortality. The survival rate of patients for the period up to 9 years was 99.2 %; long-term mortality was 0.8 %; the constancy of results up to 1, 2 and 3 years of follow-up was 90.5 %, 84.1 % and 81.7 %, respectively, and up to 5–9 years it was 79.4 %. An active lifestyle was verified in 74.6 % of patients; a sparing lifestyle was observed in 25.4 %; 86.5 %, of patients returned to work; transient sexual disorders were found in 28.6% of patients. 83.3 % and 27.8 % of patients kept to drug therapy and dietary regimen. 34.1 % and 23.8 % of patients kept smoking and taking power drinks; overweight and obesity were observed in 23.8 % and 19 % of patients. Recurrent myocardial infarction (MI) / angina was verified in 23 % of patients; 20.6 % of patients underwent second EMR. 23 % of patients got depressed due to recurrent myocardial infarction / angina, while 18.3 % of patients were down due to the quarantine (COVID-19); 6.3 % suffered from panic attacks, for fear of recurrent anginous pain. Conclusion. Predictors of recurrent EMR and stability of results were: 1) ACS; 2) patients’ age (≤35); 3) history of MI, ejection fraction ≤35–40 %, diabetes; 4) stent diameter (<28 mm); 5) smoking and taking power drinks. The leading predictor was the aggressiveness of atherosclerosis and diabetes in persons under 40, causing: a) early stent dysfunction; b) native and “protected” coronary vessels damage in case of intact stents patency. Keywords: ischemic heart disease, young patients, risk profile, semiotics of coronary arteries, endovascular myocardial revascularization, quality of life. Цель. Корреляция результатов эндоваскулярных реваскуляризаций миокарда (ЭВРМ) у больных моложе 40 лет с острым коронарным синдромом (ОКС) / стабильной стенокардией, возрастом при первичной ЭВРМ ≤35 лет / 36–40 лет и диаметром стентов. Материалы и методы. Обследовано 208 больных ИБС в возрасте 24–40 лет: 157 (75,5 %) чел. – в возрасте 36–40 лет, 51 (24,5 %) – ≤35 лет. ЭВРМ выполнена 165 (79,3 %) больным, коронарное шунтирование – 32 (15,4 %), 11 (5,3 %) пациентов отказались от вмешательства. Исходы ЭВРМ и качество жизни изучены у 126 (76,4 %) больных на сроках 10–107 мес. (62,5±2,6 мес.) наблюдения. Результаты. ЭВРМ выполнена 84 (50,9 %) больным ОКС и 81 (49,1 %) больному стабильной стенокардией. Перипроцедурных осложнений не выявлено. Госпитальная и 30-дневная летальность составила 0 %. Выживаемость пациентов на сроках до 9 лет – 99,2 %; отдаленная летальность – 0,8 %; сохранность результатов до 1, 2 и 3 лет наблюдения составила соответственно 90,5, 84,1 и 81,7 %, до 5–9 лет – 79,4 %. Активный образ жизни верифицирован у 74,6 % обследованных, щадящий – у 25,4 %, возврат к работе – у 86,5 %, транзиторные сексуальные нарушения – у 28,6 %. Медикаментозную терапию и диетический режим питания соблюдали 83,3 и 27,8 % пациентов. Продолжали курить и принимать энергетические напитки 34,1 и 23,8 % обследованных; избыточный вес и ожирение сохранялись у 23,8 и 19 %. Рецидив инфаркта миокарда (ИМ) / стенокардии отмечен у 23 % пациентов; повторные ЭВРМ выполнены 20,6 % больным. Депрессию из-за рецидива ИМ / стенокардии отмечали 23 % пациентов, ввиду карантина (COVID-19) – 18,3 %; 6,3 % страдали паническими атаками, боясь повторения ангинозных болей. Выводы. Предикторами повторных ЭВРМ и стабильности результатов являются: 1) ОКС; 2) возраст ≤35 лет; 3) анамнез ИМ, фракция выброса ≤35–40 %, диабет; 4) диаметр стентов <28 мм; 5) курение и прием энергетических напитков. Ведущий предиктор – агрессивность атеросклероза и диабета у лиц не старше 40 лет, обусловливающих: а) раннюю дисфункцию стентов; б) при сохранной проходимости стентов – поражение нативных и «защищенных» коронарных сосудов. Ключевые слова: ишемическая болезнь сердца, молодые больные, профиль риска, семиотика коронарных артерий, эндоваскулярная реваскуляризация миокарда, качество жизни.

scholarly journals Beta-blocker therapy in patients with COPD: a systematic literature review and meta-analysis with multiple treatment comparison

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Claudia Gulea ◽  
Rosita Zakeri ◽  
Vanessa Alderman ◽  
Alexander Morgan ◽  
Jack Ross ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Beta Blocker ◽  
Observational Studies ◽  
Beta Blockers ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Life Outcomes ◽  
Blocker Therapy ◽  
Beta Blocker Therapy

Abstract Background Beta-blockers are associated with reduced mortality in patients with cardiovascular disease but are often under prescribed in those with concomitant COPD, due to concerns regarding respiratory side-effects. We investigated the effects of beta-blockers on outcomes in patients with COPD and explored within-class differences between different agents. Methods We searched the Cochrane Central Register of Controlled Trials, Embase, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and Medline for observational studies and randomized controlled trials (RCTs) investigating the effects of beta-blocker exposure versus no exposure or placebo, in patients with COPD, with and without cardiovascular indications. A meta-analysis was performed to assess the association of beta-blocker therapy with acute exacerbations of COPD (AECOPD), and a network meta-analysis was conducted to investigate the effects of individual beta-blockers on FEV1. Mortality, all-cause hospitalization, and quality of life outcomes were narratively synthesized. Results We included 23 observational studies and 14 RCTs. In pooled observational data, beta-blocker therapy was associated with an overall reduced risk of AECOPD versus no therapy (HR 0.77, 95%CI 0.70 to 0.85). Among individual beta-blockers, only propranolol was associated with a relative reduction in FEV1 versus placebo, among 199 patients evaluated in RCTs. Narrative syntheses on mortality, all-cause hospitalization and quality of life outcomes indicated a high degree of heterogeneity in study design and patient characteristics but suggested no detrimental effects of beta-blocker therapy on these outcomes. Conclusion The class effect of beta-blockers remains generally positive in patients with COPD. Reduced rates of AECOPD, mortality, and improved quality of life were identified in observational studies, while propranolol was the only agent associated with a deterioration of lung function in RCTs.

scholarly journals Why Do Patients Leave against Medical Advice? Reasons, Consequences, Prevention, and Interventions

Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 111
Author(s):  
Asseel Albayati ◽  
Steven Douedi ◽  
Abbas Alshami ◽  
Mohammad A. Hossain ◽  
Shuvendu Sen ◽  
...  
Keyword(s):  
Hospital Admissions ◽  
Medical Advice ◽  
Preventive Strategies ◽  
Against Medical Advice ◽  
Cause Analysis ◽  
Persistent Problem ◽  
Root Cause ◽  
Discharge Against Medical Advice ◽  
Leaving Against Medical Advice

Background: A patient decides to leave the hospital against medical advice. Is this an erratic eccentric behavior of the patient, or a gap in the quality of care provided by the hospital? With a significant and increasing prevalence of up to 1–2% of all hospital admissions, leaving against medical advice affects both the patient and the healthcare provider. We hereby explore this persistent problem in the healthcare system. We searched Medline and PubMed within the last 10 years, using the keywords “discharge against medical advice,” “DAMA,” “leave against medical advice,” and “AMA.” We retrospectively reviewed 49 articles in our project. Ishikawa fishbone root cause analysis (RCA) was employed to explore reasons for leaving against medical advice (AMA). This report presents the results of the RCA and highlights the consequences of discharge against medical advice (DAMA). In addition, the article explores preventive strategies, as well as interventions to ameliorate leaving AMA.

scholarly journals A Taxonomic Review of Patient Complaints in Adult Hospital Medicine

2021 ◽  
Vol 8 ◽  
pp. 237437352110073
Author(s):  
Richard M Elias ◽  
Karen M Fischer ◽  
Mustaqeem A Siddiqui ◽  
Trevor Coons ◽  
Cindy A Meyerhofer ◽  
...  
Keyword(s):  
Hospital Admissions ◽  
Hospital Medicine ◽  
Patient Complaints ◽  
Period Data ◽  
The Mean ◽  
Key Features ◽  
Person Characteristics ◽  
The Relationship ◽  
Adult Hospital

Previous studies show that patient complaints can identify gaps in quality of care, but it is difficult to identify trends without categorization. We conducted a review of complaints relating to admissions on hospital internal medicine (HIM) services over a 26-month period. Data were collected on person characteristics and key features of the complaint. The complaints were also categorized into a previously published taxonomy. Seventy-six unsolicited complaints were identified, (3.5 per 1000 hospital admissions). Complaints were more likely on resident services. The mean duration between encounter and complaint was 18 days, and it took an average of 12 days to resolve the complaint. Most patients (59%) had a complaint in the Relationship domain. Thirty-nine percent of complaints mentioned a specific clinician. When a clinician was mentioned, complaints regarding communication and humaneness predominated (68%). The results indicate that the efforts to reduce patient complaints in HIM should focus on the Relationships domain.

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