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Fulltext Transcriptomic profiling of skeletal muscle from the Ts1Cje mouse model of Down syndrome suggests dysregulation of trisomic genes associated with neuromuscular junction signaling, oxidative stress and chronic inflammation

Leong, Melody Pui Yee, Usman Bala, Lim, Chai Ling, Rozita Rosli, Cheah, Pike-See, Ling, King-Hwa

Neuroscience Research Notes, 2018;1(1):21-41.
MyJurnal

Ts1Cje is a mouse model of Down syndrome (DS) with partial triplication of chromosome 16, which encompasses a high number of human chromosome 21 (HSA21) orthologous genes. The mouse model exhibits muscle weakness resembling hypotonia in DS individuals. The effect of extra gene dosages on muscle weakness or hypotonia in Ts1Cje and DS individuals remains unknown. To identify molecular dysregulation of the skeletal muscle, we compared the transcriptomic signatures of soleus and extensor digitorum longus (EDL) muscles between the adult Ts1Cje and disomic littermates. A total of 166 and 262 differentially expressed protein-coding genes (DEGs) were identified in the soleus and EDL muscles, respectively. The partial trisomy of MMU16 in Ts1Cje mice has a greater effect on gene expression in EDL. Top-down clustering analysis of all DEGs for represented functional ontologies revealed 5 functional clusters in soleus associated with signal transduction, development of reproductive system, nucleic acid biosynthesis, protein modification and metabolism as well as regulation of gene expression. On the other hand, only 3 functional clusters were observed for EDL namely neuron and cell development, protein modification and metabolic processes as well as ion transport. A total of 11 selected DEGs were validated using qPCR (disomic DEGs: Mansc1; trisomic DEGs: Itsn1, Rcan1, Synj1, Donson, Dyrk1a, Ifnar1, Ifnar2, Runx1, Sod1 and Tmem50b). The validated DEGs were implicated in neuromuscular junction signalling (Itsn1, Syn1), oxidative stress (Sod1, Runx1) and chronic inflammation processes (Runx1, Rcan1, Ifnar1, Ifnar2). Other validated DEGs have not been well-documented as involved in the skeletal muscle development or function, thus serve as interesting novel candidates for future investigations. To our knowledge, the study was the first attempt to determine the transcriptomic profiles of both soleus and EDL muscles in Ts1Cje mice. It provides new insights on the possible disrupted molecular pathways associated with hypotonia in DS individuals.

Matched MeSH terms: Muscle Weakness
Spectra of central nervous system melioidosis

Muthusamy KA, Waran V, Puthucheary SD

J Clin Neurosci, 2007 Dec;14(12):1213-5.
PMID: 17964168

Burkholderia pseudomallei infection of the central nervous system (CNS) is rare with less than 50 cases reported over the last 30 years. The retrospective melioidosis study at University Malaya Medical Centre has documented three cases of CNS melioidosis out of more than 160 cases of melioidosis since 1978. There were two patients with brain abscess and one with spinal epidural abscess. The predisposing factors were: one patient was an aboriginal farmer and the other two were diabetic. Their age ranged from 17 to 45 years. Prominent neurological features were limb weakness, cranial nerve palsy (6th and 7th) and visual disturbance. CT brain scan and MRI spine showed abscess formation, subdural collection, and spinal epidural collection, osteomyelitis of vertebra and occipital bone and also sagital sinus thrombosis. All these patients underwent surgical drainage leading to bacteriological diagnosis as well as appropriate long-term antibiotic therapy. All had good recovery at 6 months after completion of treatment.

Matched MeSH terms: Muscle Weakness/etiology
Fulltext SLE/ polymyositis overlap syndrome

Seng, Wee Cheo, Rosdina Zamrud Ahmad Akbar, Tee, Tat Khoo, Kuo, Zhau Teo, Carwen Siaw, Qin, Jian Low

Borneo Journal of Medical Sciences, 2020;14(3):33-38.
MyJurnal

Inflammatory myopathies (IM) is a rare inflammatory muscle disorder, which can be broadly divided into 5 subgroups. The accurate diagnosis of subtype of IM can be challenging due to a diverse presentation of the disease. On the other hand, skeletal muscle complication is common in patients with systemic lupus erythematosus (SLE) in the form of myalgia or myopathy. Inflammatory myopathy is a rare association of SLE and the diagnosis and treatment can be quite challenging. A 43-year-old lady with underlying systemic lupus erythematosus (SLE), presented with subacute onset progressively worsening muscle weakness involving upper limbs and lower limbs. Neurological examination showed findings consistent with proximal myopathy, with proximal power of 3/5 and distal power of 4/5. She has elevated creatinine kinase, ALT and AST level. Her myositis-specific autoantibodies were positive for anti-Ku antibodies. Her electromyography showed evidence of active myopathy of the upper and lower limb. Here, we would like to report a case of polymyositis in a patient with SLE.

Matched MeSH terms: Muscle Weakness
Fulltext Remission of recalcitrant dermatomyositis following a chiropractic adjustment

Chu ECP, Lo FS, Bhaumik A

J Family Med Prim Care, 2019 Nov;8(11):3742-3744.
PMID: 31803683 DOI: 10.4103/jfmpc.jfmpc_839_19

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by progressive muscle weakness and pathognomonic skin eruptions. Systemic corticosteroid with or without an immunosuppressive agent is the current treatment of choice in most cases. Cutaneous disease in DM is often refractory and can become the most challenging component to manage effectively. Here, we report a case of recalcitrant DM in a 66-year-old female who sought chiropractic attention for recent episodes of pain and paresthesia in the neck and exacerbation of joint pain. As expected, the musculoskeletal complaints including neck pain, peripheral arthralgia, and muscle weakness that resolved within 1 month after starting treatment. Unexpectedly, dramatic remission of the characteristic skin rashes occurred concurrently. The underlying therapeutic mechanisms of chiropractic remain elusive. This case highlights the importance of family physicians becoming familiar with diagnosing the condition and using a multidisciplinary team approach to treat recalcitrant DM.

Matched MeSH terms: Muscle Weakness
Prolonged Exercise Test in Patients With History of Thyrotoxicosis

Tan HT, Tan CY, Teong CS, Ratnasingam J, Goh KJ

J Clin Neurophysiol, 2020 Aug 05.
PMID: 32773648 DOI: 10.1097/WNP.0000000000000766

PURPOSE: Thyrotoxic periodic paralysis is characterized by recurrent episodes of reversible, severe proximal muscle weakness associated with hypokalemia and hyperthyroidism. Prolonged exercise test is an easy, noninvasive method of demonstrating abnormal muscle membrane excitability in periodic paralyses. Although abnormal in thyrotoxic periodic paralysis patients, the effects thyroid hormone levels in non-thyrotoxic periodic paralysis thyrotoxicosis patients have not been well studied. The study aims to evaluate thyrotoxicosis patients (regardless of thyrotoxic periodic paralysis history) with prolonged exercise test and correlate it with their thyroid status.
METHODS: This is a prospective, cross-sectional study of consecutive thyrotoxicosis patients seen at the endocrine clinic of a tertiary medical center. Thyroid status was determined biochemically before prolonged exercise test. Compound muscle action potential (CMAP) amplitudes postexercise were compared against pre-exercise amplitudes and recorded as percentage of mean baseline CMAP amplitude. Comparisons of time-dependent postexercise CMAP amplitudes and mean CMAP amplitude decrement were made between hyperthyroid and nonhyperthyroid groups.
RESULTS: Seventy-four patients were recruited, 23 (31%) men, 30 (41%) Chinese, and the mean age was 48.5 ± 16.8 years. Of 74 patients, 32 (43%) were hyperthyroid and 42 (57%) were nonhyperthyroid viz. euthyroid and hypothyroid. Time-dependent CMAP amplitudes from 10 to 45 minutes after exercise were significantly lower in hyperthyroid patients compared with nonhyperthyroid patients (P < 0.01). Mean CMAP amplitude decrement postexercise was significantly greater in hyperthyroid than nonhyperthyroid patients (23.4% ± 11.4% vs. 17.3% ± 10.5%; P = 0.02).
CONCLUSIONS: Compound muscle action potential amplitude declines on prolonged exercise test were significantly greater in hyperthyroid patients compared with nonhyperthyroid patients. Muscle membrane excitability is highly influenced by thyroid hormone level. Thyrotoxic periodic paralysis occurs from increased levels of thyroid hormone activity in susceptible patients.

Matched MeSH terms: Muscle Weakness
Patient with statin-associated immune-mediated necrotizing myopathy presenting with subcutaneous edema, persistent bulbar weakness and absent anti-HMGCR

Ong SG, Ding HJ

Int J Rheum Dis, 2017 Dec;20(12):2175-2178.
PMID: 26692320 DOI: 10.1111/1756-185X.12814
Matched MeSH terms: Muscle Weakness
Fulltext Oncogenic osteomalacia, you say? better start looking then - a case report

Vijay, A.P., Tan, A.T.B., Suhaida, A.M., Chan, S.P.

JUMMEC, 2010;13(1):63-68.
MyJurnal

Tumour-induced or oncogenic osteomalacia (OOM) is a rare paraneoplastic syndrome characterized by bone pain and muscle weakness. A biochemical profile consisting of normocalcaemia, hypophosphataemia, phosphaturia, increased serum alkaline phosphatase and inappropriately low serum levels of 1, 25-dihydroxyvitamin-D is diagnostic. OOM is usually caused by an osseous or soft-tissue tumour of mesenchymal origin that secretes phosphaturic substances leading to increased urinary phosphate wasting. These tumours are small and slow growing. The diagnosis continues to be easily missed and when eventually made, localization of the tumour can be difficult. We describe the case of a young man who presented with severe generalized pain associated with muscle weakness. He was extensively investigated and eventually diagnosed to have OOM 3 years after initial presentation. Specialized investigations were necessary to localize the offending tumour.

Matched MeSH terms: Muscle Weakness
Fulltext Occupational risk factors for carpal tunnel syndrome among nurses in medical

Anuar Ithnin, Kong, Dinnee, Venkataraman, Saraswathy

Int J Public Health Res, 2012;2(2):137-143.
MyJurnal

Carpal tunnel syndrome (CTS) is a hand disorder which indicates the presence of symptoms such as pain, numbness, and muscle weakness among the patient. CTS is an occupational related disorder which can occur in any profession. However, it can be prevented and managed. The aims of the research were to determine the prevalence of acquiring CTS among nurses who worked in the wards and occupational risk factors involving the upper limbs during nursing tasks performance. The specific aims were to determine the relationship between the prevalence of acquiring CTS and individual factors (age, gender, race, educational level, duration of work and medical history), relationship between the prevalence of acquiring CTS and occupational risk factors in nursing tasks. Nurses profession was chosen as they are performing multitask involving upper limbs especially the wrist joints. Boston Carpal Tunnel Syndrome Questionnaire (BCTQ) was used to determine the level of severity in CTS. Occupational risk factors were assessed by using the Rapid Upper Limb Assessment (RULA). This research was a cross sectional mode which was carried out at a government university medical centre from November through December 2010. Eighty nurses were involved in the research. The respondents were required to fill in the socio-demographic information sheet. Those having CTS were required to fill in the BCTQ. Assessments were performed by observing of the job activity through RULA. The results showed that the prevalence for nurses acquiring CTS is 7.5%. The RULA assessment also indicated that the risk factor was in the highest level with a score of 7. No relationship was shown between the prevalence of CTS and race, gender, educational level and medical history. Significant relationship was indicated by the prevalence of CTS and occupational risk factors. In conclusion, a significant prevalence of CTS related to age of more than 30 years old, Malay races compared to Indian, working experience of more than 10 years and respondents with right hand dominant. Occupational risk factors also indicated among the active nurses. Therefore, it is important for us to modify the work environment, work flow, work methodology and ergonomic factors in order to prevent the nurses from acquiring CTS. Furthermore, education about the condition of CTS should be implemented and reinforced especially among the higher risk nurses.

Matched MeSH terms: Muscle Weakness
Myopathic dropped head syndrome: a syndrome of mixed aetiology

Goh KJ, Wong KT, Tan CT

J Clin Neurosci, 2000 Jul;7(4):334-6.
PMID: 10938615

We report two patients with myopathic dropped head syndrome, a rare and interesting neuromuscular syndrome characterised by a predominant weakness of the neck extensor muscles. The first patient, a middle aged Chinese man, presented with progressive weakness of neck extension but his clinical course later stabilised despite a lack of response to corticosteroids. Muscle biopsy revealed a necrotising myopathy with no evidence of inflammation. This patient supports the existence of an idiopathic restricted non-inflammatory myopathy, a so called isolated neck extensor myopathy syndrome which is recognised to pursue a less progressive, more benign course. Our second patient had histopathological evidence for polymyositis; there was a favourable response to steroids. Our cases underscore the fact that there may be a spectrum of pathological processes associated with the myopathic dropped head syndrome ranging from non-inflammatory muscle necrosis to a full blown inflammatory myositis.

Matched MeSH terms: Muscle Weakness/etiology*; Muscle Weakness/pathology*; Muscle Weakness/physiopathology
Maximal respiratory pressures in adult Chinese, Malays and Indians

Johan A, Chan CC, Chia HP, Chan OY, Wang YT

Eur Respir J, 1997 Dec;10(12):2825-8.
PMID: 9493668

Maximal static inspiratory and expiratory mouth pressures (PI,max and PE,max, respectively) enable the noninvasive measurement of global respiratory muscle strength. The aim of this study was primarily to obtain normal values of PI,max and PE,max for adult Chinese, Malays and Indians and, secondarily, to study their effect on lung volumes in these subjects. Four hundred and fifty two healthy subjects (221 Chinese, 111 Malays, 120 Indians) were recruited. Measurements of PI,max from residual volume (RV), PE,max from total lung capacity (TLC) and forced vital capacity (FVC) were obtained in the seated position. There were significant ethnic differences in PI,max and PE,max measurements obtained in males, and FVC measurements in both males and females. Chinese males had higher PI,max values (mean (+/-SD) 88.7+/-32.5 cmH2O) and higher PE,max values (113.4+/-41.5) than Malay males (PI,max 74.0+/-22.7 cmH2O, PE,max 94.7+/-23.4 cmH2O). Chinese males had higher PE,max than Indian males (PI,max = 83.7+/-30.0 cmH2O, PE,max 98.4+/-29.2 cmH2O). There were no significant differences among Chinese females (PI,max 53.6+/-2.3 cmH2O, PE,max 68.3+/-24.0 cmH2O), Malay females (PI,max 50.7+/-18.3 cmH2O, PE,max 63.6+/-21.6 cmH2O) and Indian females (PI,max 50.0+/-15.2 cmH2O, PE,max 60.7+/-20.4 cmH2O). In both sexes, the Chinese had a higher FVC compared with Malays and Indians. After adjusting for age, height and weight, race was still a determinant for PE,max in males, and FVC in both sexes. The FVC only correlated weakly with PI,max and PE,max in both sexes. Ethnic differences in respiratory muscle strength, and lung volumes, occur among Asians. However, respiratory muscle strength does not explain the differences in lung volumes in healthy Asian subjects.

Matched MeSH terms: Muscle Weakness/ethnology*
Loss of muscle strength, mass (sarcopenia), and quality (specific force) and its relationship with functional limitation and physical disability: the Concord Health and Ageing in Men Project

Hairi NN, Cumming RG, Naganathan V, Handelsman DJ, Le Couteur DG, Creasey H, et al.

J Am Geriatr Soc, 2010 Nov;58(11):2055-62.
PMID: 21054284 DOI: 10.1111/j.1532-5415.2010.03145.x

To determine the association between loss of muscle strength, mass, and quality and functional limitation and physical disability in older men.

Matched MeSH terms: Muscle Weakness/physiopathology*
Fulltext Levels of Mercury in Fish-Eating Children, With and Without Amalgam Restoration

Padmakumar V, Premkala Raveendran K, Abdulla AM, Ganapathy S, Sainudeen S, Nasim VS, et al.

J Pharm Bioallied Sci, 2019 May;11(Suppl 2):S397-S401.
PMID: 31198376 DOI: 10.4103/JPBS.JPBS_44_19

Background: Mercury is a naturally occurring metal that exists in three forms: elemental (metallic), inorganic, and organic mercury. Amalgam, which is an alloy of inorganic mercury, is used as a restorative material in dentistry. Organic mercury gets ingested in the body mainly by the consumption of seafood. Mercury is also stated to cause various adverse health effects such as gastrointestinal disturbances, dermatitis, muscle weakness, and neurological disorders. In recent years, the use of amalgam has become a controversy stating the various adverse effects of mercury. Hence, the study was conducted to determine and compare the variation in levels of organic and inorganic mercury in fish-eating children before and after placement of amalgam restoration.
Materials and Methods: Seventy-five subjects, 42 males (56%) and 35 females (44%) of age group ranging 7-13 years, living in South Canara district of Karnataka, India, were selected as a part of the study. Hair and urine samples were collected for estimation of organic and inorganic levels of mercury, respectively. Informed consent was collected from all the participating subjects.
Results: On comparison between organic and inorganic mercury levels during the study period, the concentration of organic mercury in hair samples was greater irrespective of amalgam restorations present (1.172 and 0.085, respectively; P < 0.001).
Conclusion: Thus inorganic levels of mercury do not seem to pose a threat as much as the organic levels observed in hair, which remain fairly constant for a longer period of time. Hence in a coastal region where this study was undertaken and fish being a staple food, the risk could probably be attributed to more of an organic toxicity than an inorganic one. Thus amalgam is relatively safe to be practiced and the controversy against it should be reevaluated.

Matched MeSH terms: Muscle Weakness
Late clinical and magnetic resonance imaging follow up of Nipah virus infection

Lim CC, Lee WL, Leo YS, Lee KE, Chan KP, Ling AE, et al.

J Neurol Neurosurg Psychiatry, 2003 Jan;74(1):131-3.
PMID: 12486285

The Nipah virus is a newly identified paramyxovirus responsible for an outbreak of fatal encephalitis in Malaysia and Singapore. This paper reports the follow up clinical and magnetic resonance imaging findings in 22 affected subjects. Of 13 patients with encephalitis, one died, one was lost to follow up, and seven recovered. Among the four remaining patients, one had residual sixth nerve palsy, another suffered from severe clinical depression, and a third patient had evidence of retinal artery occlusion. One patient with delayed onset Horner syndrome had a single lesion in the cervical spinal cord. The brain magnetic resonance findings were stable or improved in nine patients over 18 months of follow up. Among a second group of nine asymptomatic seropositive abattoir workers, magnetic resonance examination in seven subjects revealed discrete small lesions in the brain; similar to those detected in encephalitis patients. These findings suggest that in addition to encephalitis, the newly discovered Nipah virus affects the spinal cord and the retina. Late clinical and radiological findings can occur in Nipah virus infections as with other paramyxoviruses.

Matched MeSH terms: Muscle Weakness/epidemiology
Juvenile acid maltase deficiency

Loi, H.D.K., Wong, K.T., Choo, K.E.

Malaysian Journal of Paediatrics and Child Health, 2005;14(1):58-63.
MyJurnal

An eight-year-old Chinese girl presented with a slowly progressive generalized muscle weakness and wasting, complicated by respiratory failure. She had many hospital admissions requiring ventilator support. Eventually tracheostomy tube was inserted. Initial investigations failed to elicit a diagnosis but a muscle biopsy and histological study confirmed the diagnosis of juvenile acid maltase deficiency.

Matched MeSH terms: Muscle Weakness
Fulltext Isolated Hypophosphataemia Mimicking Cerebrovascular Accident

Hanizah Ngadiron, Razrim Rahim, Firdaus Hayati, Nornazirah Azizan, Affirul Chairil Ariffin

Borneo Journal of Medical Sciences, 2019;13(1):29-32.
MyJurnal

Hypophosphataemia occurs in an abnormally low serum phosphate level. Three main mechanisms are postulated: decreased intestinal absorption, increased renal excretion, and extracellular shifts to intracellular compartments. It is potentially a fatal disease if not intervene. The management is merely treating the underlying disorder, giving phosphate supplement and requiring close biochemical monitoring. The incidence of symptomatic isolated hypophosphataemia is extremely rare. In this case report, a 33-year-old man presented with three days history of dysphagia, inability to complete sentences and generalized muscle weakness. He developed blurred vision especially upon exposure to bright light. He had a history of single parathyroidectomy for parathyroid adenoma 2 years ago. Physical examinations were unremarkable. Laboratory investigations were normal except for phosphate level of 0.30 mmol/L. Intravenous KH2PO4 with a dosage of 10 mmol was administered in slow bolus in 3 hours. His symptoms resolved slowly after correction. Although isolated hypophosphataemia is rare but need to recognize the symptoms and signs of hypophosphataemia and treat accordingly.

Matched MeSH terms: Muscle Weakness
Investigating the performance of an amplitude-independent algorithm for detecting the hand muscle activity of stroke survivors

Hameed HK, Wan Hasan WZ, Shafie S, Ahmad SA, Jaafar H, Inche Mat LN

J Med Eng Technol, 2020 Apr;44(3):139-148.
PMID: 32396756 DOI: 10.1080/03091902.2020.1753838

To make robotic hand devices controlled by surface electromyography (sEMG) signals feasible and practical tools for assisting patients with hand impairments, the problems that prevent these devices from being widely used have to be overcome. The most significant problem is the involuntary amplitude variation of the sEMG signals due to the movement of electrodes during forearm motion. Moreover, for patients who have had a stroke or another neurological disease, the muscle activity of the impaired hand is weak and has a low signal-to-noise ratio (SNR). Thus, muscle activity detection methods intended for controlling robotic hand devices should not depend mainly on the amplitude characteristics of the sEMG signal in the detection process, and they need to be more reliable for sEMG signals that have a low SNR. Since amplitude-independent muscle activity detection methods meet these requirements, this paper investigates the performance of such a method on people who have had a stroke in terms of the detection of weak muscle activity and resistance to false alarms caused by the involuntary amplitude variation of sEMG signals; these two parameters are very important for achieving the reliable control of robotic hand devices intended for people with disabilities. A comparison between the performance of an amplitude-independent muscle activity detection algorithm and three amplitude-dependent algorithms was conducted by using sEMG signals recorded from six hemiparesis stroke survivors and from six healthy subjects. The results showed that the amplitude-independent algorithm performed better in terms of detecting weak muscle activity and resisting false alarms.

Matched MeSH terms: Muscle Weakness/physiopathology*
Intensive exercise and a patient in acute phase of polymyositis

Hejazi SM, Engkasan JP, Qomi MS

J Back Musculoskelet Rehabil, 2012;25(4):231-4.
PMID: 23220804 DOI: 10.3233/BMR-2012-0340

BACKGROUND AND OBJECTIVE: Polymyositis (PM) is an idiopathic inflammatory myopathy manifested by proximal limb muscles weakness, elevated creatinin kinase, electromyography changes, and muscle inflammation in biopsy. We report an instance of intensive rehabilitation therapy in a patient with clinically active polymyositis.
CASE REPORT: A 19-year-old female patient, diagnosed with 'electromyography and biopsy proven' polymyositis for 5 years, suffered from worsening limbs weakness and dysphagia. In her history, she had upper and lower limbs weakness accompanied by dysphagia which was further complicated by right bronchial aspiration 9 months ago. A four-week trial of intensive training and exercise rehabilitation, concurrently accompanied by medications was prescribed for this patient. At the end of therapy she achieved significant improvement in muscle strength, activities of daily living, and ambulation without any disease exacerbation.
CONCLUSION: We concluded that short-term intensive training and exercise may lead to improvements in patients with PM, without causing a progress in the disease. Due to the rarity of PM and difficulty of conducting well-controlled studies to examine the risks and benefits of exercise in these patients, further research is necessary to investigate benefits of exercise training in active phase of disease.

Matched MeSH terms: Muscle Weakness/physiopathology; Muscle Weakness/rehabilitation; Muscle Weakness/therapy
Hypokalemic periodic paralysis due to Graves disease

Drakaki A, Habib M, Sweeney AT

Am J Med, 2009 Dec;122(12):e5-6.
PMID: 19958876 DOI: 10.1016/j.amjmed.2009.06.016

Hypokalemic thyrotoxic periodic paralysis is a potentially life-threatening complication of hyperthyroidism, defined by 3 characteristic features: thyrotoxicosis, hypokalemia, and acute painless muscle weakness. In this case, a 25-year-old Malaysian man presented with acute, painless lower extremity weakness immediately after a meal. His associated symptoms included palpitations, tremor, and anxiety. He also reported a 30-pound unintentional weight loss over the previous 18 months, dyspnea on exertion, and insomnia.

Matched MeSH terms: Muscle Weakness/etiology
Fulltext Gestational age at time of in utero lipopolysaccharide exposure influences the severity of inflammation-induced diaphragm weakness in lambs

Karisnan K, Mahzabin T, Bakker AJ, Song Y, Noble PB, Pillow JJ, et al.

Am J Physiol Regul Integr Comp Physiol, 2018 04 01;314(4):R523-R532.
PMID: 29212808 DOI: 10.1152/ajpregu.00150.2017

The preterm diaphragm is functionally immature compared with its term counterpart. In utero inflammation further exacerbates preterm diaphragm dysfunction. We hypothesized that preterm lambs are more vulnerable to in utero inflammation-induced diaphragm dysfunction compared with term lambs. Pregnant ewes received intra-amniotic (IA) injections of saline or 10 mg lipopolysaccharide (LPS) 2 or 7 days before delivery at 121 days (preterm) or ∼145 days (term) of gestation. Diaphragm contractile function was assessed in vitro. Plasma cytokines, diaphragm myosin heavy chain (MHC) isoforms, and oxidative stress were evaluated. Maximum diaphragm force in preterm control lambs was significantly lower (22%) than in term control lambs ( P < 0.001). Despite similar inflammatory cytokine responses to in utero LPS exposure, diaphragm function in preterm and term lambs was affected differentially. In term lambs, maximum force after a 2-day LPS exposure was significantly lower than in controls (by ~20%, P < 0.05). In preterm lambs, maximum forces after 2-day and 7-day LPS exposures were significantly lower than in controls (by ~30%, P < 0.05). Peak twitch force after LPS exposure was significantly lower in preterm than in controls, but not in term lambs. In term lambs, LPS exposure increased the proportion of MHC-I fibers, increased twitch contraction times, and increased fatigue resistance relative to controls. In preterm diaphragm, the cross-sectional area of embryonic MHC fibers was significantly lower after 7-day versus 2-day LPS exposures. We conclude that preterm lambs are more vulnerable to IA LPS-induced diaphragm dysfunction than term lambs. In utero inflammation exacerbates diaphragm dysfunction and may increase susceptibility to postnatal respiratory failure.

Matched MeSH terms: Muscle Weakness/blood; Muscle Weakness/chemically induced*; Muscle Weakness/physiopathology
Fulltext Four nucleotide deletions of exon 47 in Dystrophin gene: a case report of a Kelantanese Duchenne Muscular Dystrophy patient

Fatimah Azman, Rose Adzrianee Adnan, Norhafizah Che Abdul Razak, Nazihah Mohd Yunus, Sarina Sulong, Rozita Abdullah, et al.

Journal of Biomedical and Clinical Sciences, 2017;2(202):11-13.
MyJurnal

Muscular dystrophy is a group of diseases that result in progressive muscle weakness and atrophy. Duchenne Muscular Dystrophy (DMD) is classified as dystrophinopathy and is an X-linked recessive disease. It is caused by alterations in the dystrophin gene at Xp21.2 encoding 79 exons [1]. It is characterised by progressive muscle wasting that begins at 3 to 5 years, delay in motor development and eventually wheelchair confinement followed by premature death at about 30 years from cardiac or respiratory complications [2]. Genetic etiology of cases of DMD in Malaysia are still scarcely reported. Here, we report the genetic cause in the case of an 11-year-old Kelantanese Malay boy who has progressive muscle weakness since 5 years old. He has difficulty in getting up from sitting and supine position also in climbing up stairs until 1st floor. He has a strong family history of DMD and musculoskeletal problems. His younger brother was diagnosed with DMD by molecular analysis and his maternal uncle died at the age of 16 with musculoskeletal problems but was never investigated. Physical examination revealed no dysmorphic features, positive Gower sign with absent tounge fasciculation. On neurological examination, tendon reflexes and muscle tone for limbs were normal. Muscle power for bilateral upper limbs were normal, however, bilateral lower limbs showed slight reduction in muscle power with calf hypertrophy.

Matched MeSH terms: Muscle Weakness

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