Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

OBJECTIVETo study the pathologic changes of the palatopharyngeal muscles with transmission electronmicroscopy (TEM) in patients with obstructive sleep apnea hypopnea syndrome (OSAHS), the role of the above muscle in OSAHS pathogenesis was discussed.

METHODSThirty-eight palatopharyngeal muscle from OSAHS patients receiving uvulopalatopharyngoplasty (UPPP) were collected in in-patient department of Chinese Medical University and five palatal tumor patients receiving resection without snoring were chosen as the control. The palatopharyngeal muscle fiber and the feature of changes in mitochondrial morphology were observed by TEM.

RESULTSThe pathological changes were not observed in the normal control group. The muscle fibers were regularly arranged, and the mitochondrial between muscles were normal. The palatopharyngeal myofibrillar in mild OSAHS group was regularly arranged. The Z lines were straight, and most mitochondria structure were normal. In the moderate group, the myofibrillar was disorganized, and the Z lines were shortened or distorted. The myofibrillar in severe group was disorganized, similar to point-like or flake, and the Z lines and the structures of sarcomeres were disappeared. And organelle were disintegrated and mitochondria were disappeared similar to flocculent. There existed obvious fatty infiltration in the palatopharyngeal muscle. In the control, mild, moderate and severe group, pharyngeal muscle fiber disarrangement of the occurrence rate was 0, 2/10, 8/13, 14/15, the occurrence rate of mitochondrial degeneration was 0, 2/10, 8/13, 14/15, increased with the severity of the ultrastructural changes in the trend of increasing incidence.

CONCLUSIONSThe degree of OSAHS is correlated with the pathological changes of palatopharyngeal muscles. Incidence of myopathy is an important part of OSAHS secondary to chronic intermittent hypoxia in OSAHS and other pathological lesions, but also an important reason for increasing pharyngeal collapse.

Adult ; Humans ; Male ; Microscopy, Electron, Transmission ; Middle Aged ; Mitochondria, Muscle ; pathology ; ultrastructure ; Muscle Fibers, Skeletal ; pathology ; ultrastructure ; Pharyngeal Muscles ; pathology ; ultrastructure ; Sleep Apnea, Obstructive ; physiopathology

OBJECTIVETo explore the efficacy differences in early intervention of different acupuncture and moxibustion methods on gastrocnemius fatigue in rats induced by electrical stimulation.

METHODSFifty male SD rats were randomly divided into a control group, a model group, a hand acupuncture group, an electroacupuncture group and a moxibustion group, 10 rats in each group. Electrical stimulation of the sciatic nerve was given in the control group and gastrocnemius fatigue test was induced by electrical stimulation of the sciatic nerve in the model group after anesthesia without other treatment, but just take 6 times interval stimulation in the control group. The hand acupuncture group, the electroacupuncture group and the moxibustion group were treated with the corresponding acupoints stimulation method respectively for 20 min before gastrocnemius fatigue test, and Dazhui (GV 14) and Zusanli (ST 36) were selected. Immediately after gastrocnemius fatigue test, three or four gastrocnemius tissues at the same site on the right side were quickly taken for making specimen for transmission electron microscope (TEM). The changes of skeletal muscle ultrastructure of myofibrils, mitochondria, sarcoplasmic reticulum, glycogen particles were observed under TEM.

RESULTS(1) Muscle fibers disorder, partial mitochondrial vacuolization and glycogen particles smaller were shown in the model group. (2) No abnormalities were shown in the hand acupuncture group and the moxibustion group with mitochondrial morphology and number, which better than that in the model group, and glycogen particles increased. (3) Abnormal changes in morphology were shown in the electroacupuncture group with part of the muscle fibers derangement, Z line malalignment and a few mitochondria vacuolization.

CONCLUSIONHand acupuncture, electroacupuncture and moxibustion have the different effects on ultrastructure of gastrocoemius in rats. Acupuncture and moxibustion have shown good effects on the prevention and treatment of exercise-induced skeletal muscle cell and organelle damage and delaying exercise-induced fatigue.

Acupuncture Points ; Acupuncture Therapy ; Animals ; Glycogen ; metabolism ; Male ; Mitochondria ; metabolism ; ultrastructure ; Moxibustion ; Muscle, Skeletal ; metabolism ; ultrastructure ; Rats ; Rats, Sprague-Dawley

The effects of morphine HCI on the rat mesenteric mast cells were studied with the electron microscopy. The materials were prepared for electron microscopy by osmium tetroxide fixation and embedding in Epon. The rat mesenteric mast cells showed no distinct morphological changes due to morphine HCl, but the mast cell granlues were changed in various ways. For instance, they formed dusters, showed granular lysis, and an appearance of electron transparency. Frequently, some granules appeared in the extracellular space and the boundary of the granules was not evident. From the results mentioned above, it was suggested that rat mesenteric mast cell granules were affected by morphine HCl in the shape, the granular matrix, and the granular boundaries.
Animal ; Cell Nucleus/ultrastructure ; Cytoplasm/ultrastructure ; Cytoplasmic Granules/drug effects ; Cytoplasmic Granules/ultrastructure ; Golgi Apparatus/ultrastructure ; Male ; Mast Cells/drug effects ; Mast Cells/ultrastructure* ; Mesentery/drug effects ; Mesentery/ultrastructure* ; Microscopy, Electron ; Mitochondria, Muscle/ultrastructure ; Morphine/pharmacology* ; Rats

OBJECTIVE: To study the pathological changes of genioglossus with transmission electron microscope (TEM) in patients with obstructive sleep apnea hypopnea syndrome (OSAHS) dominated by lingual region obstruction, and to explore the role of tongue organizations in the pathogenesis and its clinical significance. METHOD: Thirty-eight cases of genioglossus were collected from the patients received UPPP and partial glossectomy (3060 severe group 15 cases), 6 adult patients without oropharyneal and hypopharyneal obstructive disease received tongue tumor resection or trauma debridement surgery were collected as control group. The features of morphological changes in genioglossus were observed by TEM. RESULT: Under the TEM, in the control group,the muscle fibers of the genioglossus organization arranges regular, mitochondrial shape between muscle was regular; The below 3 kinds of variations existed simultaneously in all genioglossus specimens of all the OSAHS patients. In the mild group, myofibrillar atrophied, arranged sloppily, the gap was increased, localized filaments were edema, connective tissue between muscle bundles was proliferated, mitochondria were swelling, some were spherical, crests were still clear; In the moderate group, myofibrillar obviously atrophied with different diametric sizes and disorderliness, the Z lines were shortened or distorted, part of the myofibrillar ruptured, dissolved or disappeared, the connective tissues between muscle bundles were obviously proliferated, mitochondria were swellen, vacuolar degeneration, crests were vague, shorten and irregulatio; In the severe group, a large number of myofibrillar were fractured, dissolved, disorganized, integrated condensate lumpy, spotty or flake arranged, Z-lines were distorted or disappeared. Mitochondria were sizes, showed vacuolar degeneration, crests were disappeared, some changes were flocculent, mitochondria accumulation phenomenon was visible in some samples. Moreover, with the AHI increased, the occurence ratio of mild changes was decreased while severe changes occurence ratio increased. CONCLUSION The changes of genioglossus and mitochondrial in OSAHS patients is a continuous, progressive process, moverover, with the aggravation of OSAHS, genioglossus histopathological changes had gradually worsening tendency.
Adult ; Glossectomy ; Humans ; Mitochondria ; Muscle, Skeletal ; pathology ; ultrastructure ; Sleep Apnea, Obstructive ; complications ; Tongue ; pathology

This study aimed to investigate the effects of acupuncture intervention on excessive eccentric training-induced changes of perimysial junctional plates (PJPs) domain. Thirty healthy male Wistar rats were randomly assigned to 5 groups: control group, four-week training group, four-week training + 1-week recovery group and four-week training + 1-week acupuncture group. Rats were subjected to continuous excessive eccentric training for 4 weeks (incline -16°, speed 16-20 m/min, 60-90 min/d, 5 day per week), and then were subjected to one-week spontaneous recovery or one-week recovery with acupuncture intervention (a piece of filiform needle for 4 min every day). The PJPs domain changes were observed under transmission electron microscopy, and the perimysial collagen network structural changes were examined by scanning electron microscopy with or without a digestion technique (NaOH). The following results were obtained: (1) Compared with control group, PJPs domain of four-week training group showed excessive shortening of sarcomere (P < 0.001), serious damage of sarcomere structure, and altered mitochondria morphology in intermyofibria and subsarcolemma; 54% degradation of sarcolemma, and increased number of caveolae (P < 0.01); reduced number of PJPs (P < 0.001). (2) In comparison with four-week training group, PJPs domain was slightly changed in four-week training + 1-week recovery group, i.e., partial recovery of sarcomere length and structure (accounting for 85.23% of control group), and recovery of intermyofibrial and subsarcolemmal mitochondria morphology; decreased sarcolemmal degradation (P < 0.001), and increased number of caveolae (P < 0.05); increased PJPs number (P < 0.001). (3) PJPs domain changed in four-week training + 1-week acupuncture group compared with four-week training + 1-week recovery group, which were substantial recovery of sarcomere length (accounting for 94.51% of control group), increased subsarcolemmal mitochondrial fusion (P < 0.001), decreased caveolae number (P < 0.001), and decreased PJPs number (P < 0.001). The results indicated that excessive eccentric training resulted in excessively reduced number of PJPs with altered PJPs domain homeostasis, thus impeding the adaptability to eccentric training. After 1 week of natural recovery, the number of PJPs was excessively increased, hindering muscle damage repair. Acupuncture intervention helped to recover PJPs number and PJPs domain homeostasis, thus significantly relieving overuse injuries.
Acupuncture Therapy ; Animals ; Male ; Microscopy, Electron, Transmission ; Mitochondria ; ultrastructure ; Muscle, Skeletal ; ultrastructure ; Physical Conditioning, Animal ; Random Allocation ; Rats ; Rats, Wistar ; Sarcomeres ; ultrastructure

Although it is well known that the respiratory failure is a major cause of death in most patients with chronic neuromuscular disease, predominant respiratory dysfunction without severe involvement of limb muscles is an unusual complication of mitochondrial myopathy in adult age. We experienced two cases of mitochondrial myopathy with severe involvement of respiratory function and only mild involvement of limb muscles. One is a 16 year old female and another is a 22 year old male. The diagnosis is based on morphologic characteristics of "ragged red fibers" under the light microscope and abnormal mitochondrias on the electron microscope in the muscle biopsy.
Adolescent ; Adult ; Electromyography ; Female ; Human ; *Mitochondria, Muscle/ultrastructure ; Muscular Diseases/*complications ; Respiration, Artificial ; Respiratory Insufficiency/*etiology/therapy

We report a first Korean case of presumably dominantly inherited primary tubular aggregate myopathy in a 19-yr-old man, who presented with slowly progressive proximal muscle stiffness and weakness. In hematoxylin and eosin stain, it showed subsarcolemmal, or central pale basophilic granular vacuoles, which stained red with modified Gomori's trichrome and intensive blue with nicotinamide adenonine dinucleotide-tetrazolium reductase, respectively. Ultrastructurally, aggregates of 60 nm-sized hexagonal tubules were found in both type 1 and type 2 fibers. We briefly review the pathologic findings of the previously reported cases of tubular aggregate myopathy and discuss the possible pathogenesis of this disease. We briefly discuss the possible pathogenesis of sarcoplasmic reticulum and review the ultrastructural characteristics.
Adult ; Biopsy ; Frozen Sections ; Genes, Dominant ; Genes, Recessive ; Human ; Korea ; Male ; Microscopy, Electron ; Microtubules/ultrastructure ; Mitochondria, Muscle/ultrastructure ; Muscle, Skeletal/pathology* ; Myopathies, Structural, Congenital/diagnosis ; Myopathies, Structural, Congenital/genetics ; Myopathies, Structural, Congenital/pathology* ; Pedigree

OBJECTIVETo explore the mechanism of electroacupuncture on improving insulin resistance of rat from aspects of morphology and function of mitochondrial in quadriceps femoris.

METHODSForty-eight 8-week Wistar rats (female and male in half) were randomly divided into a normal group (16 rats, group A), a model control group (16 rats, group B), a model plus electroacupuncture (EA) group (8 rats, group C) and a model plus sham acupoint EA group (8 rats, group D). Group A was given with basic diet while high-fat diet was applied in the group B, group C and group D for 8 weeks to establish model of insulin resistance. After the model establishment, "Guanyuan" (CV 4), "Zhongwan" (CV 12), "Zusanli" (ST 36) and "Fenglong" (ST 30) were selected according to acupoint combination of manifestation-root in the group C, while four points in non-meridian area where 1 to 2 mm next to the acupoints used in group C were selected in the group D. The treatment was given 15 min per time with 1 mA of intensity and 2 Hz in frequency, 5 times per week for totally 8 weeks. The transmission electron microscope was adopted to observe mitochondria structure, and chemical colorimetry was used to test the activity of adenosine triphosphate (ATP) synthase and phosphomolybdic acid colorimetry was applied to measure the content of ATP.

RESULTSAfter the treatment, the body mass was (401.63 +/- 109.81) g in the group B, which was significantly higher than (305.88 +/- 62.72) g in the group A (P < 0.05); morphological structure of mitochondrion was damaged, showing swelling and deformation; the activity of ATP synthase was decreased (P < 0.05) and the content of ATP in tissue of quadriceps femoris was also obviously lowered (P < 0.05). The body mass was (294.13 +/- 53.78) g in the group C, which was significantly lower than that in the group B (P < 0.05); the damaged mitochondrion was restored and merged among each other; the activity of ATP synthase was increased (P < 0.05); the content of ATP in tissue of quadriceps femoris was obviously lifted (P < 0.05). The results in group D were not different from those in group B.

CONCLUSIONThe electroacupuncture with manifestation-root acupoint combination could improve the recovery of damaged structure of mitochondrion and promote the merge among each other, which could enhance oxidizing capacity, lower body mass and improve synthetic rate of ATP.

Acupuncture Points ; Adenosine Triphosphate ; biosynthesis ; Animals ; Diabetes Mellitus, Type 2 ; metabolism ; therapy ; Electroacupuncture ; Female ; Humans ; Insulin ; metabolism ; Insulin Resistance ; Male ; Mitochondria ; enzymology ; metabolism ; ultrastructure ; Mitochondrial Proton-Translocating ATPases ; metabolism ; Quadriceps Muscle ; metabolism ; ultrastructure ; Rats ; Rats, Wistar