Anesthesiologists have been at the forefront of initiatives addressing perioperative patient safety. As anesthesia has no direct therapeutic benefit, its risk must be minimized. At times the surgery is simple but the patient’s condition complicates anesthetic management, increasing the risk for complications. This report describes the anesthetic management of an adult patient diagnosed with inclusion body myositis (IBM), a rare inflammatory degenerative myopathy, who initially presented with decreased motor function in both lower and upper extremities causing him to be bedbound for two years. Due to the progression of his disease, he eventually developed dysphagia, hence he was scheduled for esophagoscopy, cricopharyngeal Botox injection, and percutaneous endoscopic gastrostomy. As patients with IBM are at risk for exaggerated sensitivity to neuromuscular blockers and respiratory compromise, anesthesia was at the helm of a multidisciplinary team approach. The perioperative management centered on preoperative optimization, prevention of aspiration, avoidance of anesthetics that may trigger malignant hyperthermia, and prevention of postoperative pulmonary complication. The hospital course was uncomplicated and the patient was discharged well after one day. This report emphasizes how improvements in resources, technology, and healthcare delivery, especially in anesthesia, help prevent perioperative adverse events.
Patient Safety ; Myositis, Inclusion Body ; Malignant Hyperthermia

We describes a patient with hypokalemia-induced rhabdomyolysis due to primary aldosteronism (PA), who suffered from slowly progressive muscle weakness after laparoscopic adrenalectomy, and was later diagnosed with coexisting sporadic inclusion body myositis (sIBM). A 54-year-old Asian male presented with severe muscle weakness of both lower extremities. Laboratory findings showed profound hypokalemia, and extreme elevation of the serum creatine phosphokinase levels, suggestive of hypokalemia-induced rhabdomyolysis. Further evaluation strongly suggested PA by an aldosterone-producing adenoma, which was successfully removed surgically. However, muscle weakness slowly progressed one year after the operation and a muscle biopsy demonstrated findings consistent with sIBM. This case is the first report of hypokalemia-induced rhabdomyolysis by PA coexistent with sIBM, to the best of our knowledge.
Adenoma ; Adrenalectomy ; Asian Continental Ancestry Group ; Biopsy ; Creatine Kinase ; Humans ; Hyperaldosteronism* ; Hypokalemia ; Lower Extremity ; Male ; Middle Aged ; Muscle Weakness ; Myositis, Inclusion Body* ; Rhabdomyolysis*

OBJECTIVES: Sporadic inclusion body myositis (s-IBM) is characterized by progressive muscle weakness and inclusion bodies on muscle biopsy and is the most common type of myopathy in patients over 50 years old. However, it is not only under-diagnosed but also frequently misdiagnosed as polymyositis hence treated with steroids. In the evaluation of progressive weakness in older males, S-IBM should be investigated. This case report and literature review is presented to explore these issues. A discussion on the clinical and laboratory manifestations and histopathological findings are also included.

REPORT OF THE CASE: A 42-year-old male presented with six-year history of a slowly progressive weakness seen as difficulty brushing his teeth, buttoning his shirt, tying his shoelaces, associated with progressive dysphagia. He undergone EMGNCV of all extremities and was given a provisional diagnosis of facioscapulohumeral dystrophy. He was treated with Prednisone 5 mg/tab, 2 tablets twice a week, which he took for 2 years but afforded no relief. The weakness progressed, and he eventually required assistance to walk. Pertinent on general physical examination was severe, diffuse, symmetric muscle atrophy. Neurologic examination was consistent with proximal muscle weakness. Diagnostics showed normal CPK and ESR. Muscle biopsy revealed rimmed vacuoles, mononuclear inflammatory infiltrates, tubulofilaments which suggests a diagnosis of sporadic inclusion body myositis.

CONCLUSION: Though s-IBM is the most common myopathy in patients over 50 years of age, its symptoms can start up to 20 years earlier. Hence in the evaluation of progressive weakness in adult males, s-IBM should be an important diagnostic consideration.

Human ; Male ; Myositis, Inclusion Body ; Muscle Weakness ; Prednisone ; Vacuoles ; Deglutition Disorders ; Polymyositis ; Muscular Dystrophy, Facioscapulohumeral ; Neurologic Examination ; Muscular Atrophy ; Inclusion Bodies ; Biopsy ; Tablets

Several predetermined concentrations of beta-amyloid peptide, (betaA) were administered to the rat cardiac myocyte cultures for three days to determine the effects of betaA. Stainings with congo red and crystal violet were used to evaluate the deposition of betaA in the cardiac myocytes and MTT assay was used to elucidate the cytotoxic effects of betaA by anlaysis of cell viability. Beating rates and morphological changes were investigated with inverted microscope and TEM was used to study the fine structures. Administration of 0.5 microgram/ml of betaA to cardiac myocytes induced the reduction of beating rate, however, it did neither affect the viability nor fine structures. No significant differences in cell viability or fine structures were noted in the experimental groups which were exposed to 5 microgram/ml or higher concentration of betaA. Deposition of betaA was confirmed in the cytoplasm of betaA treated cardiac myocytes with congo red and crystal violet amyloid stains. The viability of cardiac myocytes exposed to betaA was found to be reduced significantly (19%) compared to control cultures with the MTT assay. Cardiac myocytes treated with betaA presented a reduced cytoplasmic area that appeared very condensed under inverted microscope. Mitochondrial abnormalities in betaA treated cardiac myocytes included their significant enlargement, vacuolization, disorganization or paucity of cristae, paracrystalline inclusion, and accumulation of amorphous material in mitochondrial space. Mitochondrial abnormalities were present sometimes in betaA treated cardiac myocytes without disorganization of myofibils or degeneration of other cell organelles. To understand the mechanism involved in amyloid deposit and its role in pathogenesis of the diseases such as Alzheimer and inclusion body myositis (IBM), a need for in vitro model is imperative. This model of betaA treated cultured cardiac myocytes represent a amyloidosis model, and it offers several advantages for future studies of betaA to help elucidate the pathogenesis of amyloid diseases. For example, cardiac myocytes can be easily accessible, and since cardiac myocytes can be cultured for quite a long time, it is possible to study morphological and physiological changes consequent to amyloid deposits.
Amyloid ; Amyloidosis ; Animals ; Cell Survival ; Coloring Agents ; Congo Red ; Cytoplasm ; Gentian Violet ; Myocytes, Cardiac* ; Myositis, Inclusion Body ; Organelles ; Plaque, Amyloid ; Rats

In 1971 inclusion body myositis was reported by Yunis and Samaha. This disease is similar with chronic multiple myositis clinically. Pathologically, inclusion body myositis is characterized by intracytoplasmic vacuole with degenerating fibers and accompanied with inclusion body in internal nucleus and cytoplasm. Since then 240 cases of inclusion body myositis have been reported in the world including 3 cases in Korea. A 27 years-old lady had inclusion body myositis, which show slowly progressive muscular weakness. We confirmed this with clinical symptom, muscle biopsy, and electrophysiologic study. We report the typical manifestation of inclusion body myositis in a 27 years-old lady with the brief review of literature.
Adult ; Biopsy ; Cytoplasm ; Humans ; Inclusion Bodies* ; Korea ; Muscle Weakness ; Myositis, Inclusion Body* ; Polymyositis ; Vacuoles

Myositis caused by mumps virus is very rare. Mumps virus has been suggested as a causative agent of inclusion body myositis, but there has been no definite evidence. We experienced a case of severe myositis associated with mumps virus infection. A 21-year old man was admitted because of myalgia, headache, fever, and chill for 2 months. The cerebrospinal fluid analysis performed at a local clinic showed findings compatible with viral meningitis. His blood chemistry results revealed elevated levels of serum creatine kinase, lactate dehydrogenase, and serum myoglobin. On the 5th day of admission, the patient showed pain and swelling of parotid gland and scrotum. Electromyography was compatible with inflammatory myopathy. Muscle biopsy of his calf muscle revealed necrotizing (leukocytoclastic) vasculitis and multifocal myolysis with multinucleation. We suspected mumps virus infection because of his symptoms of meningitis, epididymo-orchitis and parotitis. Mumps virus was isolated in throat culture. The past medical history of the patient's mumps virus vaccination was unclear. After 2 weeks of supportive treatment, the patient's condition was improved.
Biopsy ; Cerebrospinal Fluid ; Chemistry ; Creatine Kinase ; Electromyography ; Fever ; Headache ; Humans ; L-Lactate Dehydrogenase ; Meningitis ; Meningitis, Viral ; Mumps virus* ; Mumps* ; Myalgia ; Myoglobin ; Myositis* ; Myositis, Inclusion Body ; Parotid Gland ; Parotitis ; Pharynx ; Scrotum ; Vaccination ; Vasculitis ; Young Adult

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

Rigid spine syndrome (RSS) is a childhood onset muscle disorder characterized by: marked limitation of motility of cervical and lumbar spine with severe lordosis, contracture of limb joints, mild and nonprogressive proximal muscle weakness, moderately elevated muscle enzymes, myopathic electromyographic patterns, and histological features of nonspecific myopathies. Here we present a 14-year-old girl with distinctive clinical features of rigid spine syndrome. She developed slowly progressive difficulty on walking because of joint contracture and rigid spine with severe lordosis since 4 years of age. There was mild but generalized muscle weakness. The serum creatine kinase was increased up to 743 IU/ml and the EMG studies showed combined features of myopathy and neuropathy. The muscle biopsy of vastus lateralis revealed the typical findings of rimmed vacuolar myopathy with perivascular inflammatory cell infiltration, which were consistent with the inclusion body myositis.
Adolescent ; Animals ; Biopsy ; Contracture ; Creatine Kinase ; Extremities ; Female ; Humans ; Joints ; Lordosis ; Muscle Weakness ; Muscular Diseases* ; Myositis, Inclusion Body ; Quadriceps Muscle ; Spine* ; Walking

Becker muscular dystrophy is a X-linked recessive disease with the affected gene at locus Xp21, characterized by progressive muscular weakness. Without the definite family history, it has been known that the diagnosis of this disease is almost impossible on clinical grounds alone. We reviewed the muscle pathology of two casses of genetically confirmed Becker muscular dystrophy to know the diagnositc significances of this study. The first case, a 20 year old man, is the classical one with definite family history of X-linked recessive heredity. The muscle pathology of the biceps showed dystrophic muscular changes, including increased internal nuclei, marked variation of fiber sizes and mild endomysial fibrosis. The dystrophin stain of the muscle was also confirmative for the diagnosis. The second case was a 32 year old man who has been biopsied his left vastus lateralis 5 years before this genetic diagnosis. This case is a sporadic one without the family history. The diagnosis at the time of muscle biopsy was limb-girdle muscular dystorphy or inclusion body myositis because of the typical rimmed vacuoles and marked variation of fiber sizes. The dystophin stain was not available at that time. Our conclusion is that the molecular genetic study and/or dystrophin protein test of muscle biopsy should be done in every clinically suspected patient, including limb-girdle muscular dystorphy, inclusion body myositis or rimmed vacuolar myopathies.
Adult ; Biopsy ; Diagnosis ; Dystrophin ; Fibrosis ; Heredity ; Humans ; Incontinentia Pigmenti* ; Molecular Biology ; Muscle Weakness ; Muscular Diseases ; Muscular Dystrophy, Duchenne ; Myositis, Inclusion Body ; Pathology ; Quadriceps Muscle ; Vacuoles ; Young Adult