OBJECTIVETo evaluate the therapeutic efficacy of hemoperfusion in the treatment of intermediate myasthenia syndrome (IMS) following acute organophosphate poisoning (AOPP).

METHODSEighty cases of IMS following AOPP, who were admitted to the Emergency Department of our hospital from 2006 to 2011 and had complete clinical records, were divided into HP treatment group (n = 36) and non-HP (NHP) treatment group (n = 44). The therapeutic efficacy of HP was evaluated by comparing the clinical data of the two groups.

RESULTSThe HP treatment group showed significantly increased serum cholinesterase activity at 24h and 72 h after admission (P < 0.05), while the NHP treatment group showed significantly increased serum cholinesterase activity at 72 h after admission (P < 0.05). The serum cholinesterase activity in the HP treatment group was significantly higher than that in the NHP treatment group at 24 h after admission (P < 0.05). Compared with the NHP treatment group, the HP treatment group had significantly decreased total atropine dose, time of ventilatory assistance, length of ICU stay, recovery time from coma, incidence of pulmonary infection, and mortality due to respiratory failure (P < 0.05). There were no significant differences in the incidence of upper gastrointestinal hemorrhage and total mortality between the two groups (P > 0.05).

CONCLUSIONHemoperfusion is an effective therapy for improving clinical symptoms, shorten the course of disease, reducing complications, and decreasing the mortality due to respiratory failure in the patients with IMS following AOPP.

Cholinesterases ; blood ; Female ; Hemoperfusion ; Humans ; Male ; Muscle Weakness ; etiology ; therapy ; Organophosphate Poisoning ; therapy ; Syndrome ; Treatment Outcome

Amyopathic dermatomyositis is diagnosed when the biopsy-confirmed cutaneous lesions of dermatomyositis are present for longer than 2 years in the absence of muscle weakness, elevated muscle enzymes, and the history of immunosuppressive drug therapy and ingestion of drugs such as hydroxyurea that can produce dermatomyositis-like cutaneous hypersensitivity changes. We report a 36-year-old woman with a 3-year history of typical skin features of dermatomyositis with no evidences of muscle involvement.
Adult ; Dermatomyositis* ; Drug Therapy ; Eating ; Female ; Humans ; Hydroxyurea ; Hypersensitivity ; Muscle Weakness ; Skin

Dermatomyositis is an idiopathic inflammatory myopathy characterized by progressive symmetric proximal muscle weakness and typical cutaneous lesions. Recently the association of adult dermatomyositis and malignancy has generated much attention. A 56-year-old male presented with skin eruptions, proximal muscle weakness, and a neck mass on the right side. Diagnosis of dermatomyositis was established by clinical investigation, muscle enzyme study, electromyogram, and histological findings of the skin and muscle. A computerized tomography scan and histologic finding of right neck mass showed nasopharyngeal carcinoma. The patient was treated with chemotherapy and radiotherapy, and showed partial remission of nasopharyngeal carcinoma, with some improvement of the skin eruptions. But muscle weakness and myalgia were aggravated. He was then treated with systemic steroids (prednisolone) and azathioprine.
Adult ; Azathioprine ; Dermatomyositis* ; Diagnosis ; Drug Therapy ; Humans ; Male ; Middle Aged ; Muscle Weakness ; Myalgia ; Myositis ; Neck ; Radiotherapy ; Skin ; Steroids

Musculoskeletal symptoms, such as muscle weakness, stiffness and pain, are observed frequently in patients with hypothyroidism. In theory, hypothyroidism can cause weakness of the swallowing muscles, but dysphagia associated with hypothyroidism-associated myopathy has not been reported. The present case involved a 51-year-old man who experienced acute onset of severe dysphagia with aspiration pneumonia. A video fluoroscopic swallowing study and fiberoscopic endoscopic evaluation of swallowing revealed pharyngo-laryngeal function impairment. With a prior history of subclinical hypothyroidism and clinical symptoms such as proximal limb weakness, further evaluation involving a hormonal study, electrodiagnostic study, and histopathology assessment revealed myopathy. Hormone replacement therapy was started and the patient recovered within three weeks of treatment and was taking a regular diet. In conclusion, this study suggests that it is necessary to consider further evaluations to determine if hypothyroid myopathy is involved in the case of unknown origin dysphagia accompanied by hypothyroid myopathy.
Deglutition Disorders ; Deglutition* ; Diet ; Extremities ; Hormone Replacement Therapy ; Humans ; Hypothyroidism ; Middle Aged ; Muscle Weakness ; Muscles ; Muscular Diseases* ; Pneumonia, Aspiration

OBJECTIVE: To systematically assess the efficacy of traditional Chinese therapy in the treatment of ICU-acquired weakness (ICU-AW). METHODS: PubMed, Cochrane Library, Embase, Web of Science, CNKI, Wanfang, VIP were retrieved by computer and were used to collect a randomized controlled trials (RCT) of traditional Chinese therapy for ICU-AW. The retrieval time was from databases establishment to December 2021. After 2 researchers independently screened the literature, extracted data and evaluated the risk of bias included in the study, and RevMan 5.4 software was used for Meta-analysis. RESULTS: 334 articles were selected, totally 13 clinical studies and 982 patients were included, including 562 in the trial group and 420 in the control group. Meta-analysis results showed that traditional Chinese therapy could improve clinical efficacy of ICU-AW patients [relative risk (RR) = 1.35, 95% confidence interval (95%CI) was 1.20 to 1.52, P < 0.000 01], improve the muscle strength [Medical Research Council score (MRC score); standardized mean difference (SMD) = 1.00, 95%CI was 0.67 to 1.33, P < 0.000 01], improve daily life ability [modified Barthel index score (MBI score); SMD = 1.67, 95%CI was 1.20 to 2.14, P < 0.000 01], shorten mechanical ventilation time (SMD = -1.47, 95%CI was -1.84 to -1.09, P < 0.000 01), reduce the length of intensive care unit (ICU) stay [mean difference (MD) = -3.28, 95%CI was -3.89 to -2.68, P < 0.000 01], reduce the total hospitalization time (MD = -4.71, 95%CI was -5.90 to -3.53, P < 0.000 01), reduce tumor necrosis factor-α (TNF-α; MD = -4.55, 95%CI was -6.39 to -2.70, P < 0.000 01) and interleukin-6 (IL-6; MD = -5.07, 95%CI was -6.36 to -3.77, P < 0.000 01). There was no obvious advantage in reducing the severity of the disease [acute physiology and chronic health evaluation II (APACHE II; SMD = -0.45, 95%CI was -0.92 to 0.03, P = 0.07). CONCLUSIONS Based on the current research, traditional Chinese therapy can improve the clinical efficacy of ICU-AW, improve muscle strength and daily life ability, shorten mechanical ventilation, the length of ICU stay and total hospitalization time, reduce TNF-α and IL-6. But traditional Chinese therapy can not reduce the overall disease severity.
Humans ; APACHE ; East Asian People ; Intensive Care Units ; Interleukin-6 ; Tumor Necrosis Factor-alpha ; Medicine, Chinese Traditional ; Muscle Weakness/therapy*

Pompe disease is a genetic disorder caused by a deficiency of acid alpha-glucosidase (GAA). Infantile onset Pompe disease is uniformly lethal. Affected infants generally present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, which is rapidly followed by death, usually by the age of one. The late-onset form is characterized less severe symptoms and prognosis. Therapy for Pompe disease is intended to directly address the underlying metabolic defect via intravenous infusions of recombinant human GAA to replace the missing enzyme. We report a case of atypical infantile-onset Pompe disease that presented symptoms in infancy but had less severe clinical manifestations and improved after GAA enzyme replacement (Myozyme(R), Genzyme Co., MA, USA) therapy. It is very important that pediatricians become aware of signs and symptoms of Pompe disease, such as a nasal voice or a waddling gait at an early stage so that these patients can benefit from appropriate GAA replacement therapy as soon as possible.
alpha-Glucosidases ; Cardiomyopathy, Hypertrophic ; Enzyme Replacement Therapy* ; Gait ; Glycogen Storage Disease Type II* ; Humans ; Infant ; Infusions, Intravenous ; Muscle Hypotonia ; Muscle Weakness ; Prognosis ; Voice

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder due to the loss of dystrophin in muscle fiber. The deficiency of dystrophin produces severe progressive muscle degeneration which leads to progressive muscle weakness. Affected patients usually become unambulatory in their early teens, and suffer a respiratory failure before 20 years of age. In an attempt to improve quality of life and extend life span of DMD patients, various treatments have been challenged; corticosteroid trial, rehabilitation, cardiac and pulmonary managements, orthopedic interventions, and nutritional support. However, only corticosteroid therapy and non-invasive ventilation have shown a salutary effect on the clinical course of DMD. Recently, a better understanding of the DMD pathophysiology has provided the scientific basis for new treatment modalities including cell and molecular therapy. Although previous clinical trials have demonstrated the limitation and possibility of new therapies, antisense-mediated exon skipping technology is now emerging as a promising approach to restore dystrophin expression. This article summarizes the current challenges and recommendations of treatment approaches in DMD patients.
Adolescent ; Adrenal Cortex Hormones ; Dystrophin ; Exons ; Genetic Therapy ; Humans ; Muscle Weakness ; Muscles ; Muscular Dystrophy, Duchenne ; Noninvasive Ventilation ; Nutritional Support ; Orthopedics ; Quality of Life ; Respiratory Insufficiency ; Tissue Therapy

Myalgia, muscle weakness, muscle cramping, muscular rigidity and elevated levels of serum creatinine kinase (CK) are very common in patients with hypothyroidism. However, these symptoms are not clinically serious in most cases and are not found in primary care. So far, 5 Cases with hypothyroid myopathy have been reported in the Korean literature. However, it is not reported that this case has been found in primary care or in periodic health examination. We report a case of hypothyroid myopathy that was presented with exercise-induced myalgia. This case was diagnosed as hypothyroid myopathy through abnormal thyroid function test and elevated levels of serum CK and myoglobin in periodic health examination. Muscle symptoms and laboratory abnormality were improved only with thyroid hormone replacement therapy.
Creatine Kinase ; Creatinine ; Hormone Replacement Therapy ; Humans ; Hypothyroidism ; Muscle Cramp ; Muscle Rigidity ; Muscle Weakness ; Muscles ; Muscular Diseases ; Myoglobin ; Myxedema ; Phosphotransferases ; Primary Health Care ; Rhabdomyolysis ; Thyroid Function Tests ; Thyroid Gland

BACKGROUND: Sinus node dysfunction (SND) is caused not only by intrinsic sinus node disease, but also by the extrinsic factors. Among the extrinsic factors, autonomic imbalance is most common. Symptomatic SND usually requires permanent pacemaker therapy. However, the clinical characteristics and patient response to medical therapy for hypervagotonic SND have not been properly clarified. MATERIALS AND METHODS: Thirty two patients (14 men, 18 women, 51 +/- 14 years) with hypervagotonic SND were included in this study, but those patients who had taken calcium antagonists, beta-blockers or other antiarrhythmic drugs were excluded. Hypervagotonic SND was diagnosed if the abnormal electrophysiologic properties of the sinus node were normalized after the administration of atropine (0.04 mg/kg). RESULTS: The presenting arrhythmias were 16 cases of sinus bradycardia (50.0%), 12 of sinus pause (37.5%), 3 of sinoatrial block (9.4%) and 1 of tachy-bradycardia (3.1%). Nine (28.1%) patients had hypertension, 7 (21.9%) smoked, 2 (6.3%) had diabetes mellitus, and 1 (3.1%) had hypercholesterolemia. Among the patients, 3 had no remarkable symptoms, 13 had dizziness, 7 had syncope, 3 had weakness and 6 had shortness of breath. Twenty five (78.1%) patients were treated with theophylline, 1 patient with tachy-bradycardia syndrome was treated with digoxin and propafenone, and 6 (18.8%) were treated with no medication. During the 43 +/- 28 month follow-up, 25 patients remained asymptomatic, but 6 who took no medication developed mild dizziness. One patient needed permanent pacemaker implantation owing to recurrent syncope despite of theophylline treatment. CONCLUSION: These results show that hypervagotonic SND has a benign course and most of the patients can be managed safely without implanting a pacemaker. (Ed note: I like the abstract. It is short and direct, as it should be.)
Dizziness/etiology ; Dyspnea/etiology ; Female ; Humans ; Male ; Middle Aged ; Muscle Weakness/etiology ; Sick Sinus Syndrome/*complications/drug therapy ; Syncope/etiology ; Theophylline/therapeutic use ; Vasodilator Agents/therapeutic use

Cholangiocarcinoma with paraneoplastic dermatomyositis (DM) is extremely rare, and the whole body positron emission tomography-computed tomography (PET-CT) finding of paraneoplastic DM is rarely reported. We report a 66-year-old woman with metastatic cholangiocarcinoma, initially presented with bilateral proximal muscle uptake on PET-CT without clinical muscle symptoms. The initial interpretation of the high muscle uptake was metastasis to the muscles. However, while awaiting for chemotherapy, muscle weakness evolved and rapidly progressed. The level of creatine phosphokinase was significantly elevated. Electromyography revealed moderate myopathy, and a muscle biopsy showed degenerating myofibers with variable sizes. The diagnosis of paraneoplastic dermatomyositis was made. This case highlights that, although rare, paraneoplastic dermatomyositis can be present with cholangiocarcinoma. Also, muscle inflammation can precede the clinical muscle symptoms, and paraneoplastic DM should be considered as a possible differential diagnosis in the assessment of cancer patients who present with abnormal muscle tracer uptake in PET-CT scans.
Aged ; Biopsy ; Cholangiocarcinoma* ; Creatine Kinase ; Dermatomyositis* ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Electromyography ; Electrons* ; Female ; Humans ; Inflammation ; Muscle Weakness ; Muscles ; Muscular Diseases ; Neoplasm Metastasis ; Positron-Emission Tomography