scholarly journals Chloroquine and Hydroxychloroquine Myopathy: Clinical Spectrum and Treatment Outcomes

2021 ◽  
Vol 11 ◽  
Author(s):  
Elie Naddaf ◽  
Pritikanta Paul ◽  
Omar F. AbouEzzeddine
Keyword(s):  
Treatment Outcomes ◽  
Muscle Biopsy ◽  
Cumulative Dose ◽  
Clinical Phenotype ◽  
Wide Spectrum ◽  
Dose Effect ◽  
Limb Weakness ◽  
Marked Weight ◽  
Cardiac Evaluation ◽  
Cardiac Muscles

Chloroquine (CQ) and hydroxychloroquine (HCQ) have been associated with muscle toxicity, mostly described as a proximal myopathy with evidence of lysosomal dysfunction on muscle biopsy. In this retrospective study, we aimed to define the clinical phenotype, laboratory features, and treatment outcomes of CQ/HCQ myopathy, as well as the safety profile of these drugs. We identified 13 patients seen between 2000 and 2019, with a median age at presentation of 66 years (range 53–89); 11 were females. At onset of symptoms, patients were on CQ or HCQ for a minimum of 6 months and up to 21 years. Diagnosis was often delayed by a median of 6 months (range 3–48). At presentation, 13 patients reported limb weakness, with five requiring assistance in walking. Ten reported dysphagia, often severe, resulting in marked weight loss or aspiration pneumonia. Nine reported respiratory symptoms, which were multifactorial in four, and four reported severe neck weakness. Myopathy clinical phenotype showed predominant involvement of one or more of the following: proximal limb muscle weakness (12 patients), dysphagia (9), axial weakness (4), and respiratory failure (5). Eleven patients had a cardiac evaluation showing prolonged QT interval in 10 and CQ/HCQ cardiomyopathy (CMP) in four. Ten out of 12 patients markedly improved after discontinuing the medication, but most were left with some residual weakness. Eleven patients had a muscle biopsy showing a myopathy with rimmed vacuoles and marked acid phosphatase reactivity. Nine had elevated creatine kinase level up to 1,199 U/L. Twelve patients had an electromyography (EMG), which showed myopathic motor unit potentials with fibrillation potentials in 11 and myotonic discharges in 3. Higher cumulative dose and longer exposure duration were associated with more severe disability and more common cardiac and swallow involvement, indicating a cumulative dose effect. Herein, we demonstrate that long-term exposure to CQ and HCQ may result in a myopathy with a wide spectrum of clinical presentation and predilection for swallowing, respiratory, and cardiac muscles, often with marked associated morbidity. Once accurately diagnosed and the drug is discontinued, patients usually improve but often fail to return to baseline.

THUR 219 Myopathic manifestations in haematological conditions

2018 ◽  
Vol 89 (10) ◽  
pp. A31.3-A31 ◽  
Author(s):  
Vivekanandam Vinojini ◽  
Devine H ◽  
Holton J ◽  
Jaunmuktane Z ◽  
Turner C ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplant ◽  
Muscle Biopsy ◽  
Plasma Cells ◽  
Late Onset ◽  
Autologous Bone Marrow ◽  
Neurological Complications ◽  
Marrow Transplant ◽  
Limb Weakness ◽  
Allogenic Bone

Neurological complications of haematological malignancies are wide ranging but isolated myopathy as a presenting complaint is rare.Cases: 63 year-old lady with progressive proximal upper then lower limb weakness over 7 years to wheelchair-dependence and hoist-transfers. Muscle biopsy revealed nemalin rods, HIV negative, IgG kappa paraprotein=6 g/L. Late-onset nemalin rod myopathy was diagnosed; function is stable 6/12 post-autologous bone marrow transplant.56 year-old lady with limb-girdle weakness leading to immobility over 2 years and IgG lambda MGUS. Re-examination of muscle biopsy revealed amyloid deposition, SAP scan negative for systemic AL amyloid. Treated with velcade and dexamethasone but died from amyloid cardiomyopathy 6/12 later.73 year-old man with painless deltoid weakness. Muscle biopsy revealed plasma cells infiltrate and lightchain deposition with matched lightchain restriction in bone marrow, IgG kappa paraprotein=8 g/L. Cyclosporin and dexamethasone were recommended for infiltrative multifocal, myopathic plasmacytoma.28 year-old female with pain, fatigue and pelvic-girdle weakness was diagnosed with haemophagocytic lymphohistiocytosis without systemic lymphoma or connective tissue disease. Muscle biopsy demonstrated T-cell and macrophage infiltration. Muscle symptoms are responding to steroids, mycophenolate, cyclosporine and etopiside. An allogenic bone marrow transplant is planned.We describe four cases of progressive myopathic weakness as the primary manifestation of an underlying haematological disorder.

CMR—basic principles

2021 ◽  
pp. 67-78
Author(s):  
Jan Bogaert ◽  
Rolf Symons ◽  
Jeremy Wright
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Tissue Characterization ◽  
Wide Spectrum ◽  
Pulse Sequences ◽  
Resonance Imaging ◽  
Cardiac Evaluation ◽  
One Stop ◽  
Magnetic Resonance Imaging Mri ◽  
Single Modality

Cardiac magnetic resonance imaging (CMR) has rapidly evolved to become the modality of choice in the evaluation of a wide spectrum of cardiovascular disorders. This is mostly related to its multiparametric approach where the different features such as morphology (including deep tissue characterization), function, perfusion can be non-invasively studied using a series of (different) pulse sequences. Therefore, CMR may provide a ‘one stop shop’ approach to cardiac patients offering a complete cardiac evaluation by a single modality without the use of ionizing radiation. However, to maximize its clinical use, not all pulse sequences should be used in every patient. Moreover, minimizing time spent in the magnetic resonance imaging (MRI) machine is desirable in critically ill, unstable patients. Therefore, experienced cardiac imagers from radiology and cardiology should be present to provide on-site real-time assessment of the images and to determine which pulse sequences are necessary each patient. In this fashion, a complete CMR exam should be obtainable in less than 30 minutes for the vast majority of patients. The aim of this chapter is to describe the physics and practical aspects of CMR and then explore the available pulse sequences, so that the clinical utility of CMR can be maximized.

scholarly journals Computed Tomographic Evaluation of Posterior Fossa Lesions

2013 ◽  
Vol 3 (1) ◽  
pp. 53-58
Author(s):  
Mukesh Kumar Gupta ◽  
RK Rauniyar ◽  
N Bhatta ◽  
S Raja ◽  
K Ahmad
Keyword(s):  
Posterior Fossa ◽  
Wide Spectrum ◽  
Cranial Computed Tomography ◽  
Limb Weakness ◽  
Neck Stiffness ◽  
Cerebellar Haemorrhage ◽  
Cross Sectional ◽  
Presenting Symptoms ◽  
Cerebellar Abscess ◽  
Study Results

Background: Various neoplastic and nonneoplastic lesions can involve posterior cranial fossa. Posterior fossa lesions are potentially fatal since they may result in compression of the brainstem. The objective of this study was to analyze the spectrum of posterior fossa lesions and their clinical presentations. Methods: A prospective cross sectional study was conducted over a period of one year. Thirty patients with clinical features of posterior fossa pathology, referred for cranial computed tomography, were evaluated in the study. Results: Posterior fossa lesions found in this study were cerebellar abscess- 4(13.3%), arachnoid cyst- 4(13.3%), brainstem glioma- 3(10%), cerebellar astrocytoma- 3(10%), acute cerebellar infarct- 2(6.7%), cerebellar metastasis- 2(6.7%), acoustic neuroma- 2(6.7%), meningioma- 2(6.7%), medulloblastoma- 2(6.7%), ependymoma- 1(3.3%), Dandy-Walker malformation- 1(3.3%), 4th ventricular bleed- 1(3.3%), brainstem haemorrhage- 1(3.3%), cerebellar haemorrhage- 1(3.3%), and neurocysticercosis- 1(3.3%). The maximum numbers of cases (23.3%) with posterior fossa lesions were in their first decade of life. Features of raised intracranial pressure (i.e. headache, nausea & vomiting) were the commonest presenting symptoms. Other symptoms included altered sensorium, ataxia, fever, limb weakness, vertigo, seizure, hearing loss, neck stiffness, slurring of voice, ear discharge, tinnitus, loss of consciousness and macrocrania in decreasing order of frequency. Conclusion: There is wide spectrum of the lesions in posterior fossa in children and adults in this part of Nepal, requiring prompt diagnosis and intervention, and CT has a pivotal role in the management. Nepalese Journal of Radiology / Vol.3 / No.1 / Issue 4 / Jan-June, 2013 / 53.58 DOI: http://dx.doi.org/10.3126/njr.v3i1.8796

scholarly journals From Routine to Research Laboratory: Strategies for the Diagnosis of Congenital Fibrinogen Disorders

2020 ◽  
Vol 40 (04) ◽  
pp. 460-466
Author(s):  
Alessandro Casini
Keyword(s):  
Fibrinogen Level ◽  
Clinical Course ◽  
Clinical Phenotype ◽  
Wide Spectrum ◽  
Fibrin Clot ◽  
Optimal Management ◽  
Coagulation Assays ◽  
Patient Diagnosis ◽  
Work Up ◽  
Routine Coagulation

AbstractCongenital fibrinogen disorders (CFDs) encompass a heterogeneous group of fibrinogen defects with a wide spectrum of biological and clinical features. An accurate diagnosis is thus essential to assure the optimal management for the patient. Diagnosis involves a multistep approach starting with routine coagulation assays and assessment of functional and antigenic fibrinogen followed by identification of the molecular anomaly. However, the diagnosis of CFD can be challenging as the sensitivity and specificity of coagulation assays depend on the fibrinogen level as well as on the fibrinogen variant. In addition, patients suffering from CFD have a heterogeneous clinical course which is often unpredictable by routine coagulation assays. To better determine the patient's clinical phenotype, global hemostasis assays and an assessment of the fibrin clot properties are performed in research laboratories. In this review, we summarize the fibrinogen work-up highlighting some common pitfalls and provide an update of the research on CFD.

Previous, current, and cumulative dose effect of waterpipe smoking on LDL and total cholesterol

2019 ◽  
Vol 26 (8) ◽  
pp. 8194-8201 ◽  
Author(s):  
Souheil Hallit ◽  
Rabih Hallit ◽  
Chadia Haddad ◽  
Lara Youssef ◽  
Marouan Zoghbi ◽  
...  
Keyword(s):  
Total Cholesterol ◽  
Cumulative Dose ◽  
Dose Effect ◽  
Waterpipe Smoking

scholarly journals Case Report: Post-Chikungunya–Associated Myeloneuropathy

2021 ◽  
Author(s):  
Ameya Patwardhan ◽  
Atchayaram Nalini ◽  
Priyanka Priyadarshini Baishya ◽  
Karthik Kulanthaivelu ◽  
Haripriya Krishnareddy ◽  
...  
Keyword(s):  
Early Recognition ◽  
Wide Spectrum ◽  
Rare Complication ◽  
Neurological Complications ◽  
Immunoglobulin M ◽  
Sensory Level ◽  
Normal Brain ◽  
Mri Findings ◽  
Limb Weakness ◽  
Hyperintense Signal

Chikungunya virus (CHIKV) is an arbovirus endemic to South Asia with frequent outbreaks. A wide spectrum of neurological complications has been described in Chikungunya infections. Myeloneuropathy is a rare complication seen in Chikungunya and is proposed to have an underlying immune mediated pathogenesis. We report a case of a 45-year-old man presenting to the emergency services with acute onset of quadriparesis, breathlessness, urinary retention, profound pain, and sensory disturbances 6 weeks after the onset of high-grade fever and arthralgia. On examination, the patient had Medical Research Council grade 1 flaccid quadriparesis with prominent wasting and areflexia with distinct sensory level at T4. Immunoglobulin M CHIKV antibodies were positive, tested twice at a 1-week interval. He had notable magnetic resonance imaging (MRI) findings in the form of patchy T2 hyperintensities involving the entire length of the cervical and thoracic cord with normal brain imaging and extensive short tau inversion recovery hyperintense signal changes on muscle MRI. He was treated with five cycles of plasmapheresis and intravenous methylprednisolone followed by oral steroids for 8 weeks. At 20-week follow-up, the patient had improvement in upper limb weakness, but paraparesis persisted. The case highlights the presence of unusual MRI findings and also the importance of early recognition of after infective neurological complications, and prompt treatment with immunomodulation may be beneficial.

The Polyneuropathy of Critical Illness

1994 ◽  
Vol 9 (3) ◽  
pp. 132-138 ◽  
Author(s):  
Charles F. Bolton
Keyword(s):  
Multiple Organ Failure ◽  
Organ Failure ◽  
Muscle Biopsy ◽  
Correct Diagnosis ◽  
Clinical Signs ◽  
Multiple Organ ◽  
Neuromuscular Blocking ◽  
Disuse Atrophy ◽  
Limb Weakness ◽  
Electrophysiological Studies

Polyneuropathy occurs in 70% of patients who have sepsis and multiple organ failure. It presents as limb weakness and difficulty in weaning from the ventilator. Clinical signs are often unreliable, and electrophysiological studies are necessary to establish the diagnosis. These studies show a primary axonal degeneration of predominantly motor fibers. Creatinine phosphokinase levels are normal or mildly elevated. Muscle biopsy shows predominantly denervation atrophy. Recovery occurs, except in the most severe cases, if sepsis and multiple organ failure can be successfully treated. Other neuromuscular conditions that may be associated with sepsis are disuse atrophy and, rarely, acute myositis or pyomyositis. Measurements of creatinine phosphokinase levels and muscle biopsy, in addition to electrophysiological studies, are necessary to establish the correct diagnosis in these conditions. Recent reports in the literature implicate use of neuromuscular blocking agents or steroids as a possible further mechanism for causing either an axonal polyneuropathy or a primary myopathy; however, the precise role of these drugs in this type of toxicity has not been established. Nonetheless, comprehensive investigation of these patients is worthwhile to aid respiratory and rehabilitation management and to establish a long-term prognosis.

Cumulative dose-effect curves in a conflict test with incremental shock

1982 ◽  
Vol 78 (2) ◽  
pp. 195-196 ◽  
Author(s):  
James L. Howard ◽  
Kenneth W. Rohrbach ◽  
Gerald T. Pollard
Keyword(s):  
Cumulative Dose ◽  
Dose Effect ◽  
Conflict Test
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