Symptoms Of Myopathy Research Articles

Central Core Myopathy with Mutation of the RYR1 Gene in Combination with Mutation of the COL6A2 Gene and Severe Infantile Myoclonic Epilepsy

Children with central core myopathy (CCD) are diagnosed by the clinical symptom of muscle hypotonia. Primarily, the diagnosis is made by muscle biopsy. The disease is caused by mutation in the RYR1 gene. Dysfunction of the ryanodine receptor results in dysregulation of calcium metabolism in the sarcoplasmic reticulum of muscle cells. Our patient showed typical symptoms of congenital myopathy and dislocation of the hip from birth. The diagnosis of CCD was made by muscle biopsy and confirmed by a gene test which showed mutation in the RYR1 gene. In addition, there was a mutation in the COL6A2 gene, which may encode for congenital muscular dystrophy type Ulrich. At the age of approximately 8 months, the child developed the symptoms of severe infantile myoclonic epilepsy with generalized irregular polyspikes in the electroencephalography (EEG). Seizures could be reduced by treatment with various antiepileptic drugs. However, by introducing valproate and zonisamide, the EEG almost normalized and the convulsions stopped. In literature, there are no data about the combination of the mutation in the RYR1 gene and severe epilepsy. As the CCD is a dysfunction of the intracellular calcium channels, it must be assumed that the epilepsy is probably caused by central nervous system involvement of the channel dysfunction. This essential aspect has to be taken into consideration when deciding on the appropriate antiepileptic drug choice.

Musculoskeletal safety outcomes of patients receiving daptomycin with HMG-CoA reductase inhibitors.

Daptomycin, a cyclic lipopeptide antibiotic, and 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors (statins) are commonly administered in the inpatient setting and are associated with creatine phosphokinase (CPK) elevations, myalgias, and muscle weakness. Safety data for coadministration of daptomycin with statins are limited. To determine the safety of coadministration of daptomycin with statin therapy, a multicenter, retrospective, observational study was performed at 13 institutions in the Southeastern United States. Forty-nine adult patients receiving statins concurrently with daptomycin were compared with 171 patients receiving daptomycin without statin therapy. Detailed information, including treatment indication and duration, infecting pathogen, baseline and subsequent CPK levels, and presence of myalgias or muscle complaints, was collected. Myalgias were noted in 3/49 (6.1%) patients receiving combination therapy compared with 5/171 (2.9%) of patients receiving daptomycin alone (P = 0.38). CPK elevations of >1,000 U/liter occurred in 5/49 (10.2%) patients receiving combination therapy compared to 9/171 (5.3%) patients receiving daptomycin alone (P = 0.32). Two of five patients experiencing CPK elevations of >1,000 U/liter in the combination group had symptoms of myopathy. Three patients (6.1%) discontinued therapy due to CPK elevations with concurrent myalgias in the combination group versus 6 patients (3.5%) in the daptomycin-alone group (P = 0.42). CPK levels and myalgias reversed upon discontinuation of daptomycin therapy. Overall musculoskeletal toxicity was numerically higher in the combination group but this result was not statistically significant. Further prospective study is warranted in a larger population.

SP0090 Steroid myopathy - do molecular mechanisms bring us closer to novel treatment strategies?

Introduction of glucocorticoids in the treatment of inflammatory myopathies and other inflammatory conditions in the 1950s had a major impact on survival and morbidity. However, it was already early recognized...

Problems and possible solutions for therapy with statins.

Despite issues about the value of statins, benefit for high cardiovascular (CV) risk outweighs problems. However, the practitioner must be aware of concerns, be prepared to respond, and justify statin usage. Symptoms of statin-related myopathy are of more concern than stated by pharmaceutical companies. Occurrence of myopathy symptoms, estimated to be up to 10.4%, can decrease statin adherence of high CV risk patients. Dosage modification, or use of pitavastatin, may help the problematic patient. There are concerns that there may be little benefit of statins for primary prevention in women. However, evidence appears to support statin use in women at high CV risk, both in primary and secondary prevention. Abandoning low-density lipoprotein cholesterol (LDL-C) as a valid target is unwarranted; there is much evidence to support "lower is better." The practitioner must be aware of the complicated processes causing atherosclerosis and when to incorporate new approaches to disease management. Tailoring therapy for CV risk, when indicated, may contribute further to LDL-C reduction. Liver inflammation can occur with statins but is of minimal concern; frequently, statins alleviate the problem. Unless liver transaminases are over three times normal, a statin should be prescribed, if indicated. The net effect of statins on cognition appears to be zero-no harm, no benefit. Despite reports of improved cognition, statins should not be prescribed for this. With diabetes mellitus (DM), statins can increase incidence, but the CV benefit far outweighs any risk. Therefore, statins should be prescribed in DM to reduce CV risk. Statins are a major medical contribution when used appropriately.

Clinical, pathological and molecular biological characteristics of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episode in children

To delineate the characteristics of the clinical manifestation, pathology of skeletal muscle and gene mutations of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episode (MELAS) in children. The clinical manifestation, laboratorial data, brain images, muscle pathology and mitochondrial gene mutations were analyzed in 24 patients with MELAS who were diagnosed in Department of Pediatrics, Peking University First Hospital. Their prognosis was evaluated by following up. Symptoms of central nervous system such as stroke-like episodes, vomiting, convulsion and headache were present in all the 24 cases. Nine cases had the symptoms of myopathy. Twenty cases had developmental delay. Short stature, being thin and hairy was very common in these cases. Serum lactate level increased in all the cases, pyruvate increased in 17 cases. Elevated CSF lactate was found in 2 cases. Magnetic resonance imaging (MRI) was performed on 24 cases, out of them 23 were abnormal. The lesions mainly involved cerebral lobes. Occipital lobe was the most common site of lesions. Computed tomography (CT) was performed on 13 cases, low density lesions were present in 10 cases, basal ganglia calcifications in 5 cases. Muscle biopsy was performed on 8 cases, ragged-red fibers (RRF) were found in 4/8 cases, and abnormal accumulation of mitochondria were found in 3/8 cases. The mtDNA gene mutational analysis showed A3243G mutation in these patients. The mutation rates varied from 11.6% to 75.0%. The same mutation were found in 4/5 mothers who had the genetic tests, and the mutation rates of the mothers varied from 15.0% to 23.6%. The clinical information of 11 cases was available through recent following up. Three cases died, the others had some degrees of mental retardation. Children with MELAS had various clinical manifestations. Central nervous system and skeletal muscle were usually involved. Short stature and hypertrichosis were common signs. The prognosis of this disease was disappointing. mtDNA A3243G was the most common mutation in MELAS. Fully understanding the characteristics of its clinical manifestation, laboratory tests, brain image, muscle pathology and molecular features can be helpful to the early diagnosis and treatment.

Metabolomic profiling rationalized pyruvate efficacy in cybrid cells harboring MELAS mitochondrial DNA mutations

Pyruvate treatment was found to alleviate clinical symptoms of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome and is highly promising therapeutic. Using capillary electrophoresis time-of-flight mass spectrometry (CE-TOFMS), we measured time-changes of 161 intracellular and 85 medium metabolites to elucidate metabolic effects of pyruvate treatment on cybrid human 143B osteosarcoma cells harboring normal (2SA) and MELAS mutant (2SD) mitochondria. The results demonstrated dramatic and sustainable effects of pyruvate administration on the energy metabolism of 2SD cells, corroborating pyruvate as a metabolically rational treatment regimen for improving symptoms associated with MELAS and possibly other mitochondrial diseases.

Induction of DPM Changes in Broiler Chickens and Characteristics of Myopathy Symptoms

Abstract The aim of the study was to induce deep pectoral myopathy (DPM) lesions in 42 to 45-day-old broiler chickens of the Ross 308 and Flex genetic lines, kept under standard intensive breeding management conditions applied in Poland, and to assess the degree of myopathy. A total of 110 and 120 carcasses, respectively, were examined. The study method consisted of the exposure of the birds to a stress factor (forced wing flapping) at specific time intervals prior to slaughter and at different durations of the stress factor. As a result of the conducted experiment, DPM symptoms appeared in the examined chickens. The symptoms of the progressing anomaly were divided into four stages. The first stage was characterised by the occurrence of bloody extravasations, stage II was characterised by a pale pink colour of muscles, stage III - greening of the muscle tissue, while stage IV was connected with necrosis and white-grey-green colour of muscles. The application of the forced wing flapping several days before slaughter (1, 3 d) resulted in the incidence of earlier symptoms of myopathy - stages I and II. Stimulation of wing flapping 5, 7, and 14 d before slaughter caused subsequent DPM stages (i.e. stages III and IV). In the group of the youngest birds subjected to the stress factor at 21 d before slaughter, DPM lesions were not found. Moreover, the longer the duration (15-60 s) of the stress factor, the greater the intensity of this phenomenon was observed. Bilateral DPM symptoms occurred more frequently than unilateral symptoms. Recorded results show that increased wing flapping is a significant factor inducing DPM in 42 to 45-day-old broiler chickens. These investigations indicated a possibility to determine the degree of DPM lesions depending on the passage of time from the induction of the anomaly to the slaughter of birds.

Familial mutation in ACTA1 gene - clinical variability

Results: In our family father and daughter possess the same mutation in exon 6 of ACTA1 gene. The father shows only mild symptoms of myopathy with a

Muscular myopathies other than myotonic dystrophy also associated with (CTG) n expansion at the DMPK locus

Objective:Assess triplet repeat expansion (CTG)n at the ‘dystrophia-myotonica protein kinase’ (DMPK) locus in muscular myopathies to elucidate its role in myopathic symptoms and enable genetic counseling and prenatal diagnosis in families.Methods and Results:Individuals with symptoms of myopathy, hypotonia and controls selected randomly from the population were evaluated for triplet repeat expansion of (CTG)n repeats in the 3’untranslated region (UTR) of DMPK gene, the causative mutation in myotonic dystrophy (DM). DNA was isolated from peripheral blood of 40 individuals; they presented symptoms of muscle myopathy (n = 11), muscle hypotonia (n = 4), members of their families (n = 5) and control individuals from random population (n = 20). Molecular analysis of genomic DNA by polymerase chain reaction (PCR) using primers specific for the DMPK gene encompassing the triplet repeat expansion, showed that all controls (n = 20) gave a 2.1 kb band indicating normal triplet repeat number. Three out of 11 cases (two clinically diagnosed DM and one muscular dystrophy) had an expansion of the (CTG)n repeat in the range of 1000-2100 repeats corresponding to the repeat number in cases of severe DM. Other two of these 11 cases, showed a mild expansion of ~ 66 repeats. Three samples, which included two cases of hypotonia and the father of a subject with muscular dystrophy, also gave a similar repeat expansion (~66 repeats).Conclusion:Results suggest a role of (CTG)n expansion at the DMPK locus in unexplained hypotonias and muscular myopathies other than DM. This calls for screening of the triplet repeat expansion at the DMPK locus in cases of idiopathic myopathies and hypotonia.

Fatigue in Colchicine Myopathy: A Study of Transcranial Magnetic Stimulation

Transcranial magnetic stimulation (TMS) is a noninvasive method to assess brain physiology and plasticity. TMS has shown that nervous system excitability may be altered in myopathy, and it presents with motor disinhibition on cortical and subcortical levels. Eight patients who had colchicine myopathy were observed to have fatigue, but they did not have significant weakness. This study investigated whether there was central reorganization to compensate for their muscle strength. TMS was applied to study the central compensative mechanism. The TMS parameters included motor evoked potentials, central conduction time, cortical silent period and intracortical inhibition of paired TMS paradigms. TMS results did not show any significant differences between patient and control groups. Although central reorganization may occur in patients with hereditary myopathy to compensate for muscular strength, our study did not find any change in cortical excitabilities in acquired myopathy due to colchicine. Muscle fatigue may precede weakness as an early symptom of myopathy.

Expanding Phenotype and Clinical Analysis of Tyrosine Hydroxylase Deficiency

This study included 12 Chinese patients with a wide spectrum of phenotypes of tyrosine hydroxylase deficiency. Seven females and 5 males, aged 2.2 to 41 years, had phenotypes ranging from severe type with onset at infancy to mild type with onset after 3 years of age. Patients with the severe type had encephalopathy with poor treatment response or infantile parkinsonism with motor delay. Patients with the less common mild type had dopa-responsive dystonia or a newly recognized predominant symptom of myopathy. Female siblings had more severe phenotypes. The phenotype and treatment outcomes were strongly related to a homovanillic acid level and homovanillic acid/5-hydroxyindolacetic acid ratio of less than 1 in the cerebrospinal fluid. Hyperprolactinemia was found in 50% of the severe cases. Levodopa was the mainstay of treatment, and early addition of selegiline resulted in a remarkable response in some patients. Treatment response for mild-type patients is universally good even with a treatment delay of 10 years after onset of neurological symptoms.

Concomitant administration of simvastatin and danazol associated with fatal rhabdomyolysis

Background: Simvastatin, a 3-hydroxy-3-methylglutarylcoenzyme A reductase inhibitor, is indicated for the treatment of hypercholesterolemia and plays an important role in both the primary and secondary prevention of cardiovascular disease. Danazol is a steroid analogue approved for the treatment of endometriosis, fibrocystic breast disease, and hereditary angioedema. Despite not being licensed, danazol has been used for other off-label indications, such as idiopathic thrombocytopenic purpura (ITP), paroxysmal nocturnal hemoglobinuria, and aplastic anemia. Objective: We report a case of fatal rhabdomyolysis that occurred after concomitant administration of simvastatin and danazol in a patient with ITP. Case summary: An 80-year-old white male (height, 182 cm; weight, 90 kg) presented to the emergency department of the Clinical Hospital Centre Zemun, Belgrade, Serbia, with head injuries after an accidental fall caused by generalized weakness. He denied other complaints, except fatigue, mild pretibial edema, and progressive bilateral leg pain and cramping that began 7 days before. At the time of presentation, he was receiving aspirin 100 mg/d, clopidogrel 75 mg/d, ramipril 2.5 mg/d, pantoprazole 40 mg/d, danazol 600 mg/d, prednisone 60 mg/d, simvastatin 40 mg/d, and longacting insulin 24 IU/d. After the injuries were treated, he was diagnosed with collapse and nasal contusion, and discharged without any changes in his therapy. Two days after initial presentation, the patient was readmitted to the hospital due to nausea, dark urine, and oliguria. All clinical signs (oliguria, dark urine, muscle pain, and tenderness) and laboratory markers (creatine kinase levels ∼100 times the upper limit of normal, along with hyperkalemia, hyperphosphatemia, and hypoalbuminemia) were consistent with severe rhabdomyolysis. Despite intravenous hydration, forced diuresis, and hemodialysis, oliguria persisted and the patient died 6 days after admission. A score of 5 on the Naranjo adverse drug reaction probability scale was consistent with a probable association of rhabdomyolysis and concomitant treatment with simvastatin and danazol in this patient. Conclusions: Statin-induced rhabdomyolysis must be considered whenever muscle or motor symptoms occur, especially when concomitant treatment with known inhibitors of statin metabolism is administered. Patients must be strictly monitored and the statin should be promptly discontinued with the onset of first signs and symptoms of myopathy. Clinicians should be aware of the potentially fatal consequences of both approved and unapproved treatments and be alert for the early detection of toxicity.

Metabolic Myopathies

The aim was to identify additional noninvasive tools allowing to detect and to quantify the metabolic impairment in patients with mitochondrial myopathies (MM) or McArdle's disease (McA). Kinetics of adjustment of pulmonary oxygen uptake (VO2 kinetics) during transitions to constant-load moderate-intensity cycle ergometer exercise were determined on 15 MM, 8 McA, 21 patients with signs and/or symptoms of metabolic myopathy but a negative biopsy ("patient controls"; P-CTRL), and 22 healthy untrained controls (CTRL). VO2 kinetics were slower in MM and in McA versus P-CTRL and CTRL, slower in McA versus MM, and not significantly different between P-CTRL and CTRL. The time constants (tau) of the monoexponential function describing the VO2 kinetics were (X +/- SE) 59.2 +/- 8.5 s in MM, 87.6 +/- 16.4 s in McA, 36.9 +/- 3.1 s in P-CTRL, and 35.4 +/- 1.9 s in CTRL. In a subgroup of the patients (eight MM and seven McA), tau of VO2 kinetics were negatively correlated with two variables determined in a previous study (Grassi B, Marzorati M, Lanfranconi F, et al. Impaired oxygen extraction in metabolic myopathies: detection and quantification by near-infrared spectroscopy. Muscle Nerve. 2007;35:510-20): a) a muscle oxygenation index, obtained by near-infrared spectroscopy, estimating the peak capacity of skeletal muscle fractional O2 extraction; and b) VO2 peak. In MM and McA patients, analysis of pulmonary VO2 kinetics during moderate-intensity exercise allows to identify and to quantify, noninvasively, the impairment of skeletal muscle oxidative metabolism. In these patients, the slower VO2 kinetics can be considered a marker of the impaired exercise tolerance. The present data could be useful for clinicians who need an objective, quantitative, and longitudinal evaluation of the impairment to be used in the follow-up of these patients as well as in the assessment of therapeutic interventions.

Neuroleptic Malignant Syndrome or a Statin Drug Reaction? A Case Report

A 60-year-old woman with a long psychiatric history presented with delirium and mutism. She was febrile, with marked limb rigidity and elevated creatinine kinase (CK) level. Current medications included pericyazine. Current or recent use of dopamine-blocking agents, such as pericyazine, together with a disturbance in conscious state, autonomic dysfunction, and an elevated CK level may be suggestive of neuroleptic malignant syndrome (NMS). The diagnosis was confirmed as NMS, and she was successfully treated with bromocriptine. Eight years later, she represents with symptoms suggesting recurrence of NMS including elevated CK level and myalgia, however, without limb rigidity. Current medications include quetiapine, lithium, simvastatin, and a recent course of clarithromycin. Macrolide antibiotics such as clarithromycin inhibit the metabolic pathway of statins via the cytochrome CYP450 3A4 hepatic enzyme system and may result in elevated CK level, myopathy, or rhabdomyolysis producing symptoms that may be confused with NMS. Simvastatin was ceased with rapid decrease in CK level and resolution of symptoms. This case highlights the importance of considering other diagnoses in any patient presenting with a disturbance in conscious state, autonomic dysfunction, and an elevated CK level. Particularly in a patient with a history of NMS, a thorough medication history is essential to aid diagnosis and avoid confusion with presenting symptoms and medical history.

Mitochondrial DNA: An Up‐and‐coming Actor in White Adipose Tissue Pathophysiology

Mitochondrial DNA: An Up‐and‐coming Actor in White Adipose Tissue Pathophysiology

Are Physically Active Individuals Taking Statins at Increased Risk for Myopathy?

Statin drugs are generally well tolerated in most patients. A limited number of studies have been published that look at the relationship of skeletal muscle injury in patients who take statin medications and exercise compared with taking statin medications without exercise. To date, the sample size of these studies is small, but there appears to be evidence to show that statin medications may exacerbate exercise-induced skeletal muscle injury. In addition, a study of professional athletes with familial hypercholesterolemia showed that 16 of 22 athletes could not tolerate statin medications. Patients with dyslipidemia who experience muscle pain and discomfort may be less likely to be compliant with their medication and/or physical activity regimens. Educating patients to recognize the signs and symptoms of medication-induced myopathy versus exercise-induced muscle soreness is an important counseling topic for health care providers.

Thyrotoxicosis Complicated with Dysphagia

A 36-year-old man was admitted to our hospital because of general fatigue and dysphagia. He had experienced progression of thyrotoxic symptoms of weight loss, palpitation and sweating for several months. On admission, he showed thyroid storm associated with dysphagia and aspiration pneumonia. Dysphagia was restored completely with 2 months after intensive treatment with anti-thyroid agent, beta-blocker, potassium iodide and glucocorticoid for thyroid storm. Although dysphagia is an uncommon manifestation, we should keep in mind that dysphagia may be one of symptoms of thyrotoxic myopathy.

Genetic predisposition to statin myopathy

Genetic predisposition to statin myopathy is a rapidly expanding area of investigation. This review summarizes the latest information on genetic risk factors associated with statin-induced myopathy. Genetic determinants involved in both pharmacokinetics of statins and metabolic muscle diseases are discussed. Data are provided on the prevalence of statin use in the United States; incidence of associated myopathy; terminology relating to statin myopathy and genetic susceptibility; and common myths surrounding this disorder. Technological advances now make it possible to identify genetic variation in the human genome that reveals disease-causing mutations and single nucleotide polymorphisms associated with disease. More than 30,000 individuals in the United States suffer from severe life-threatening symptoms of statin-induced myopathy that may, in some cases, persist long after the cessation of therapy. Genes of interest include those involved in the pharmacokinetics of the statin response, muscle atrophy, exercise intolerance, pain perception, and mitochondrial energy metabolism. Genetic analysis for variants and disease-causing mutations relevant to statin myopathy will provide predisposition testing for this and other drug-induced disorders. This testing will become an integral part of personalized medicine that will contribute to the safe and informed use of selected drugs and improved compliance.

Cushing's Disease Associated with both Pituitary Microadenoma and Corticotroph Hyperplasia

Herein, we present the case of a 63-year old female patient with initial symptoms of myopathy, hypokaliemia, glucosuria and psychotic symptoms. Laboratory analysis demonstrated elevated plasma levels of ACTH and cortisol. Additionally, urine cortisol excretion was increased approximately 60-fold. MRI imaging revealed a possible pituitary microadenoma. To confirm the diagnosis a bilateral inferior petrosal sinus sampling was performed presenting higher ACTH levels on the right side. However, after surgery cortisol levels did not return to normal range. Histological examination of the tumor revealed a microadenoma. Six days postoperatively, the patient developed several pneumonic infiltrations and fever therefore antibiotic and antifungal therapy was started immediately. In addition aspergillus antigen was elevated. During this septic condition, cortisol levels further increased. The patient died despite optimal intensive care under septical conditions 8 days after surgery. Microbiological analysis identified Aspergillus fumigatus in broncho-alveolar lavage and several organ systems including the heart and brain. Neuropathological autopsy revealed nodular proliferations of corticotropic cells in the pituitary gland that are assumed to be morphological entities between diffuse hyperplasias and adenomas, termed as tumorlets. In single reports, multiple pituitary lesions in patients with Cushing's disease have been demonstrated, but to our knowledge none of these cases presented the combination of an ACTH-producing microadenoma and corticotroph cell hyperplasia in the same patient. Therefore, even after resection of a pituitary microadenoma one should be aware of the possibility of continuously elevated ACTH level being due to multifocal nodular corticotroph hyperplasia which is invisible by neuroradiological examination.

Esophageal Cancer Presenting as a Case of Polymyositis

Purpose: We report a case of polymyositis (PM) associated with esophageal cancer. This presentation is rare Methods: A 63 year old white gentleman presented to the our medical center with symptoms of progressive worsening in his ability to initiate a swallow, difficulty standing up from a sitting position without the use of the upper extremities and difficulty combing his hair. These symptoms were progressive. He reported mild weight loss. He had bilateral symmetrical muscle weakness. His laboratory tests were significant for an elevated creatine kinase and lactate dehydrogenase. Radiological studies assessing swallowing were consistent with transfer dysphagia. Electromyography (EMG) was consistent with a myopathy. Overlap syndromes, myotonic dystrophy, and myasthenia were ruled out. He was given the diagnosis of PM, and an upper endoscopy performed as a part of cancer workup revealed a 6 cm flat exophytic mass in the distal esophagus. Biopsies revealed an adenocarcinoma. It was staged at IIA. The patient was treated with a combination of chemoradiotherapy and surgery. The myopathy symptoms subsided totally as the patient recovered. Results: PM is classified as an idiopathic inflammatory myopathy. Its prevalence rate is estimated at approximately one per 100,000 in the general population. There is a female to male predominance of about 2:1. The peak incidence in adults occurs between the ages of 40 and 50, but individuals of any age may be affected. PM can either be a pure entity (sole presentation) or associated with an overlap syndrome or associated with cancer (paraneoplastic syndrome). Muscle weakness is the most common presenting feature. The onset is usually insidious, with gradual worsening over a period of several months. However, an acute onset has been described. The distribution of weakness is symmetric and proximal. Diagnosis is usually based on the clinical picture, abnormal muscle enzymes, abnormal EMG, the presence of myositis specific antibodies, and a muscle biopsy. PM is associated with an increased incidence of cancer although the precise links among the two entities remain incompletely understood. Cancer of the cervix, lung, ovaries, pancreas, bladder, and stomach account for approximately 70 percent of the cancers associated with inflammatory myopathies. Esophageal cancer is very rarely associated with PM. A possible delay in the diagnosis of esophageal cancer is due to the fact that PM causes weakness of the muscles in the upper esophagus and occasionally the dysphagia caused by the cancer can be attributed to the former. Conclusion: In conclusion, esophageal cancer should be considered in the differential diagnosis in patients presenting with PM as early diagnosis can translate into better survival.