Reasons For Performing Study: Improved understanding of the epidemiology of atypical myopathy (AM) will help to define the environmental factors that permit or support the causal agent(s) to exert toxicity.
Objectives: This European survey of AM aimed to describe spatiotemporal distribution, survival, clinical signs, circumstances in which AM develops and its different expressions between countries and over time.
Methods: The spatiotemporal distribution, history and clinical features of AM cases reported to the Atypical Myopathy Alert Group from 2006 to 2009 were described. Comparisons of data from the most severely affected countries and from the large outbreaks were made with Fisher's exact and Welch's tests with Bonferroni correction.
Results: Of 600 suspected cases, 354 met the diagnostic criteria for confirmed or highly probable AM. The largest outbreaks occurred during the autumns of 2006 and 2009 in Belgium, France and Germany. For the first time, donkeys, zebras and old horses were affected, and clinical signs such as gastrointestinal impaction, diarrhoea, penile prolapse, buccal ulceration and renal dysfunction were observed. Affected horses spent >6 h/day on pastures that almost always contained or were surrounded by trees. The latency period was estimated at up to 4 days. Overall survival rate was 26%. Although differences between countries in affected breeds, body condition, horse management and pasture characteristics were recognised, the common presenting clinical signs and mortality were similar between countries.
Conclusions And Potential Relevance: This study describes new data on case details, history and clinical course of AM that is of preventive, diagnostic and therapeutic value. However, the true impact of the findings of this study on the development of or severity of AM should be tested with case-control studies.
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http://dx.doi.org/10.1111/j.2042-3306.2012.00556.x | DOI Listing |
Muscle Nerve
January 2025
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Introduction: Mixed connective tissue disease (MCTD) patients often have myositis, however, myopathological and clinical data for MCTD are limited. Recent reports have shown that the pathology of MCTD myositis resembles that of immune-mediated necrotizing myopathy (IMNM), whereas earlier reports described perifascicular atrophy or inflammatory infiltrates predominantly in the perivascular region in MCTD myositis. We aim to describe the clinical and myopathological features of MCTD myositis.
View Article and Find Full Text PDFActa Orthop Belg
December 2024
Atypical mycobacteria can cause rare and atypical infections of the hand. We report the case of an immunocompetent 46-year-old male initially presenting with thumb felon and progressively developing symptoms of carpal tunnel syndrome, tenosynovitis of multiple fingers and a sporotrichoid lymphocutaneous infection causing chronic cutaneous lesions all over the body. We would like to highlight the diagnostic and therapeutic difficulties of these atypical infections, which mimic other conditions and can cause a lot of morbidity.
View Article and Find Full Text PDFEquine Vet J
January 2025
Comparative Neuromuscular Diseases Laboratory, Department of Clinical Science and Services, The Royal Veterinary College, London, UK.
Background: Sycamore tree-derived hypoglycin A (HGA) toxin causes atypical myopathy (AM), an acute, equine pasture-associated rhabdomyolysis but incidence fluctuates.
Objectives: Investigate whether tree or environmental factors influence HGA concentration in sycamore material and are associated with AM relative risk.
Study Design: Retrospective and experimental prospective study.
Nat Med
January 2025
Huntington's Disease Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, London, UK.
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease with the age at which characteristic symptoms manifest strongly influenced by inherited HTT CAG length. Somatic CAG expansion occurs throughout life and understanding the impact of somatic expansion on neurodegeneration is key to developing therapeutic targets. In 57 HD gene expanded (HDGE) individuals, ~23 years before their predicted clinical motor diagnosis, no significant decline in clinical, cognitive or neuropsychiatric function was observed over 4.
View Article and Find Full Text PDFPhysiother Res Int
January 2025
Department of Health Sciences, Ribeirão Preto Medical School, University of São Paulo, São Paulo, Brazil.
Background And Purpose: Children with myopathies often experience muscle weakness in their lower limbs. However, the upper limbs are also affected and, at the same time, play a key role in daily living activities as well as in transfers and assisted mobility using auxiliary devices. The objective was to assess the performance of the elbow flexor and extensor muscles through static and dynamic contractions in children with myopathies and in their typical peers.
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