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[Two adult cases of nemaline myopathy presenting different clinical symptoms and CT findings].

We report herein two cases of nemaline myopathy which showed peculiar muscle involvement and clinical symptoms. Case 1: A 44-year-old woman had developed gradual woresening of muscle weakness. Neurologically, only flexion of her neck was found to be weak. Her muscular CT revealed mild atrophy of four extremities and the sternocleidomastoid muscles. Histological examinations with Gomori-trichrome staining revealed tiny structures whose form was compatible with nemaline rods. Moreover, electronmicroscopic examination demonstrated the lattice pattern of electron-dense structures, and they also appeared to possess structural continuity with the Z-band. Case 2: A 56-year-old woman visited our department because of neck pulsations. Neurological examinations revealed bilateral hearing disturbance, marked atrophy of neck muscles, muscle weakness in four extremities and hypoactive deep tendon reflexes. She also exhibited steppage and waddling gaits. Her muscular CT demonstrated degenerative processes in the neck muscles(splenius muscles and semispinal muscles), trapezius muscles, para-spinal muscles, deltoid muscles and gluteal muscles. Among them, the para-spinal muscles and extensor muscles of the lower limbs showed marked degeneration and had been partly replaced by fatty tissues. A muscle biopsy was performed, and the presence of nemaline rods was confirmed by Gomori-trichrome staining. Although these two cases could be diagnosed as nemaline myopathy, the clinical symptoms and muscular CT findings were not quite the same. Whether these differences might simply indicate different clinical phases during the disease progression or be of further pathogenic significance still remains unclear. Additionally, since long-term follow-up studies of nemaline myopathy are quite rare, further follow-up examinations of these cases are necessary in order to understand the clinical and pathological alterations of nemaline myopathy.

Adult↗

Functional motor innervation of brachial plexus roots. An intraoperative electrophysiological study.

OBJECTIVE: To study the functional motor innervation of C5-T1 nerve roots of the brachial plexus electrophysiologically. PATIENTS AND METHODS: The electrophysiological properties of normal brachial plexus roots and their functional motor innervation were examined in 15 patients with brachial plexus injuries, while the contralateral healthy side C7 root transfer was operated on. Different nerve roots of the brachial plexus were stimulated and maximum amplitudes were recorded in the following 12 muscles: deltoid, biceps, infraspinatus, brachiaradialis, flexor carpi radialis, triceps, extensor digitorum communis, latissimus dorsi, flexor digitorum profundus, abductor brevis, abductor digiti minimi and flexor carpi ulnaris. RESULTS: Functional motor innervation of brachial plexus roots showed the following results: a. C5 mainly forms the axillary nerve to innervate deltoid muscle; b. C6 mainly constructs the musculocutaneous nerve to innervate biceps muscle; c. C7 mainly forms the radial nerve to innervate triceps muscle; d. C8 mainly forms the median nerve to innervate the flexor digitorum muscle; e. T1 mainly forms the ulnar nerve to innervate the intrinsic muscles of the hand. CONCLUSIONS: The brachial plexus is functionally divided into three groups: C5, 6 innervate shoulder and elbow; C8T1 innervate the hand; and C7 innervates shoulder, elbow, wrist and hand. Theoretically, any division of the nerve root alone may not cause damage to limb function.

Adolescent↗

The hemideltoid muscle flap.

The deltoid muscle is a reliable source of well-vascularized tissue for bulk to obliterate wounds that particularly involve the adjacent glenohumeral joint. A homologue of the gluteus maximus muscle, the deltoid muscle also has two independent dominant vascular pedicles, making this a type IIl muscle flap. Consequently, the muscle can be split vertically into two halves to form hemideltoid flaps, with the undisturbed portion still remaining innervated to preserve its function as a major arm abductor-an important point because this muscle as a whole is not usually considered expendable. Because of its short vascular pedicle, the arc of rotation is limited so that larger defects of the shoulder are covered preferably with more traditional alternatives. Hallock GG. The hemideltoid muscle flap.

Adult↗

Electromyographic analysis of shoulder joint function of the biceps brachii muscle during isometric contraction.

Surface electromyography was performed for both heads of the biceps brachii in 11 healthy men while the muscles were under 30% maximum isometric shoulder flexion and abduction. Elbow related biceps activity was minimized by using a brace locked in neutral forearm rotation. Electromyographic activity was normalized as a percentage of maximal muscle contraction during 24 shoulder motions. Electromyographic activity was detected in all motions examined, suggesting that the biceps muscle acts as a flexor and an abductor of the shoulder. Both heads of the biceps muscle had higher activities during external rotation than during internal rotation for most motions. Activities of both heads increased with arm elevation, but showed little dependence on elbow position. The long head was still active during internal rotation of the shoulder. These findings also suggest that the biceps muscle is a flexor and an abductor of the shoulder, and that the long head can act as a humeral head stabilizer in superior and anterior directions. Muscle fatigue of the biceps and the deltoid muscle also was determined at 30% of maximum isometric flexion. All muscles had significantly decreased mean power frequency and turns count, and increased amplitude and integrated electromyography. The rate of decrease in mean power frequency was larger for the biceps than for the deltoid muscle, and the rate of increase in amplitude was larger for the long head of the biceps than for the short head or for the deltoid muscle. These findings suggest that the long head of the biceps must increase its mechanical output to keep the arm in elevation to a greater extent than do the short head and the deltoid muscle. This may be one of the causes of tendinitis or rupture of the long head.

Adolescent↗

Electromyographic activity of shoulder muscles during unilateral upper extremity proprioceptive neuromuscular facilitation patterns.

Twenty-nine normal adults were tested to measure the electrical activity of the anterior, middle, and posterior portions of the deltoid muscle and sternal portion of the pectoralis major muscle during the performance of four upper extremity diagonal patterns with the elbow straight, flexing, and extending. The patterns were performed through range of motion, with an isometric contraction performed in the shortened range. With the elbow straight, the middle portion of the deltoid muscle was primarily active with shoulder flexion, abduction, and external rotation; the anterior portion of the deltoid muscle with shoulder flexion, adduction, and external rotation; the posterior portion of the deltoid muscle with shoulder extension, abduction, and internal rotation; and the sternal portion of the pectoralis major muscle with shoulder extension, adduction, and internal rotation. The middle and anterior portions of the deltoid muscle exhibited significantly greater activity in their optimal patterns with the elbow strainght, followed by patterns with the elbow flexing and significantly less activity with the elbow extending. The posterior portion of the deltoid muscle in its optimal pattern produced greatest activity with the elbow straight or extending. Findings confirm the patterns considered optimal for monitored portions of these two muscles in normal subjects. Suggestions are made for study of patients who exhibit imbalances of muscle strength.

Adult↗

Cortical representation of proximal and distal arm muscles as assessed by focal transcranial magnetic stimulation.

In 21 healthy volunteers, a mapping of cortical areas from which motor evoked potentials in deltoid muscle and first dorsal interosseal muscle (FDI) could be elicited by focal transcranial magnetic stimulation was performed. For this purpose, magnetic stimuli were applied using a figure-of-eight shaped magnetic coil and a Magstim 200 stimulator at an intensity of 15% above motor threshold for the deltoid muscle. There was a considerable overlap between the cortical areas from which motor responses of the two muscles could be evoked with the FDI represented more laterally than the deltoid muscle. The cortical location from which maximal amplitudes of motor evoked potentials were elicited was 4-8 cm lateral of Cz with the FDI and 2-6 cm lateral of Cz with the deltoid muscle. Calculated centres of cortical points with maximal muscle responses were significantly separated with the FDI represented 5.7 cm lateral of Cz and deltoid muscle 4.3 cm lateral of Cz. The anterior-posterior extension of the cortical maps did not show significant differences between the proximal and distal arm muscle. In no subject an ipsilateral representation of either muscle could be found even when stimulating at maximal intensity.

Adult↗

Shoulder prostheses treating cuff tear arthropathy: a comparative biomechanical study.

UNLABELLED: Painful cuff tear arthropathy (CTA) affects the independence of the elderly. Surgical treatment often consists of joint replacement, the functional outcome of which remains variable. Knowledge of the biomechanical properties of the different prosthetic designs can guide the orthopaedic surgeon in the choice of implant to predict its clinical result. A 3-D computer model of the glenohumeral joint is used to analyse the moment of the deltoid muscle in the scapular plane. A geometrical 3-D ball-and-socket model of the shoulder joint was used to calculate (1) the angle-force relationships, (2) the moment arm of the deltoid muscle and (3) the moment of the deltoid muscle components, for increasing degrees of arm elevation in the scapular plane. In this 3-D model, a clinical thoraco-scapular rhythm analysis was implemented, based on measurements in normal subjects, patients treated with an anatomical prosthesis and patients treated with an inversed delta III prosthesis. These data were compared for 10 different prosthetic treatment options. RESULTS: Muscle angle-force curves show a favourable slope in non-anatomical prosthetic designs, where the centre of rotation of the glenohumeral joint is medialized, the deltoid muscle is elongated and the humeral shaft is lateralized. On the contrary, anatomical prosthetic designs do not perform well in this computer analysis. CONCLUSIONS: From a biomechanical point of view, a shoulder prosthesis which medializes the centre of rotation, lengthens the deltoid muscle and increases the deltoid lever arm, results in a significantly more powerful abduction of the shoulder, despite complete loss of rotator cuff function. RELEVANCE: This study explains why a successful functional outcome can be expected in CTA with a reversed prosthesis.

Arthroplasty, Replacement↗

Different short-term modulation of cortical motor output to distal and proximal upper-limb muscles during painful sensory nerve stimulation.

The pattern of upper-limb muscle activation following painful stimulation has not been clarified in detail. We investigated the short-term inhibitory and excitatory effects of painful electrical digital stimulation on the motoneuron pools of distal and proximal upper-limb muscles. Transcranial magnetic stimulation (TMS) was used as test stimulus, and painful digital nerve stimulation as conditioning stimulus for motor evoked potential (MEP) recordings over the abductor digiti minimi (ADM), abductor pollicis brevis (APB), biceps brachii (BB), and deltoid muscles. Inhibition of the conditioned MEP response was most prominent in the distal muscles, whereas BB and deltoid muscles were only weakly inhibited. The mean MEP response over APB decreased with painful cutaneous stimuli, showing maximum inhibition (by 82%) at interstimulus intervals (ISIs) of 50 ms. Inhibition in the ADM was maximal (49%) but less pronounced at an ISI of 40 ms. The BB and deltoid muscles showed inhibition by 25% and 29%, respectively. Significant facilitation was present in BB and deltoid muscles by 43% and 41% at an ISI of 100 ms, but not in the smaller hand muscles. The observed pattern of upper-limb muscle activation corresponds to the protective withdrawal reflex and the neuronal basis of the observed short-term modulation of motor activity is compatible with a spinal or brainstem pathway.

Adult↗

The posterior deltoid to triceps transfer: a clinical and biomechanical assessment.

Transfer of the posterior deltoid muscle to the triceps insertion for elbow extension provides improved function in patients with C5 and C6 level tetraplegia. We have modified the surgical technique using the tibialis anterior tendon as a graft. The length-tension characteristics and available amplitude of the posterior deltoid muscle were determined with intraoperative electrical stimulation. The excursion of the posterior deltoid muscle was 7.31 cm with a standard deviation of 1.23 cm. Postoperative mean torque measurements of elbow extension power were 36.4 kg cm with a standard deviation of 15 kg cm. All patients had maximal strength between 90 degrees and 120 degrees of elbow flexion. Length-tension curves for the posterior deltoid muscle showed a large range of effective strength and helped confirm optimum tension of the posterior deltoid muscle and proper shoulder and elbow positions at surgery. Clinical results in 10 patients were excellent.

Arm↗

[Articulation disorder as the initial manifestation of facioscapulohumeral muscular dystrophy in childhood].

A five year old boy who had received logopedic treatment for more than two years was seen as an outpatient because of speech retardation. He presented with myopathic face, incomplete closure of both lids and severe weakness of facial muscles, bilateral winging of scapulae and hyperlordosis. Extraocular and pharyngeal muscles were not affected. Motor and sensory nerve fibre conductions and electromyography were within normal limits. CPK was moderately elevated (320 U/I). Muscle biopsy of right deltoid muscle revealed unspecific myopathic changes. The patients brother aged 7 also presented with facial weakness, elevated CPK and neurogenic changes in EMG of deltoid muscle. Both parents were clinically and electrophysiologically unremarkable. Although problems to speak distinctly are usually not the first manifestation, we found in this family facio-scapulo-humeral muscular dystrophy.

Arm↗

Electromyographic validation of basic exercises for physical conditioning programmes. III. Influence of the grip in the capacity of the rowing exercises in determining action potential levels for the deltoid and the pectoralis major muscle.

The electromyographic activity of the shoulder muscles Deltoid--anterior portion (DA) and Pectoralis major--clavicular portion (PMC) was tested on 24 male volunteers using a 2 channel TECA TE4 electromyograph and Hewlett Packard surface electrodes during the execution of three different modalities of rowing exercises comparing middle grip with closed grip. Statistical comparisons showed that in both PMC and DA closed grip determined muscular solicitation higher than middlegrip excepting sitting rowing. The authors present some suggestions to the use of the tested exercises.

Action Potentials↗

[Traction and kinesitherapy in the management of painful syndromes of the cervical spine].

Painful cervical spine syndromes caused by discopathy become ever more frequent. Conservative treatment of cervical discopathy includes mainly traction, kinesitherapy, thermotherapy, massage and electrotherapy. Traction treatment and kinesitherapy are particularly important. The author carried out investigations in 90 cases of cervical discopathy divided into 3 groups of 30 cases in each. In each group cervical traction was applied using Glisson's loop at different traction force and duration of traction. It was observed that three factors were of decisive importance for the end-result of traction: a) a proper position of the patient during the procedure and a proper direction of traction force, b) a proper duration of traction, c) a properly selected weight. Electromyography demonstrated bioelectric activity in the trapezius muscle and deltoid muscle on the affected side before treatment. After application of traction the bioelectric activity of these muscles was significantly reduced at rest. It is concluded that cervical traction and kinesitherapy are effective methods in treatment of cerival spinal painful syndromes.

Cervical Vertebrae↗

Muscle fiber composition in patients with traumatic cord lesion.

Muscle fiber composition and oxidative and glycolytic enzymatic activity have been studied with complete traumatic transection of the spinal cord and spastic paralysis of the lower extremities. Muscle sample were taken by means of needle biopsy from the vastus lateralis, gastrocnemius, and soleus muscles. Biopsies were also taken for comparison from the deltoid muscle. Fibers staining darkly for alkaline stable myofibrillar ATP-ase (type II) dominated or were the only fibers identified in the paralysed muscles. The deltoid muscles of the same patients had a rather even mixture of type I and II fibers. Staining pattern was reversed after acid preincubation (pH 4.3). Mean diameters in the paralysed muscles were reduced for both fiber types. All fibers stained relatively weakly for NADH-diaphorase. Succinyldehydrogenase activity was low and phosphofructokinase activity usually moderately reduced. The findings imply that neuronal influence on the muscular fibers had led to a change in the staining characteristics of the muscle fibers. Such a change migh indicate altered contractile characteristics, though the detailed nature of the observed findings in still unclear.

Adenosine Triphosphatases↗

Pressure pain thresholds and electromyographically defined muscular fatigue induced by a muscular endurance test in normal women.

OBJECTIVE: This study was undertaken to examine the relation between muscular tenderness measured as pressure pain thresholds (PPTs) and electromyographic (EMG) signs of fatigue before and after a local standardized static muscle contraction. DESIGN: Pressure pain thresholds were measured in the shoulder region before, immediately after, and 10 minutes after a standardized static endurance test while monitoring the EMG signs of local muscular fatigue and its recovery. The study did not address local biochemical issues. SETTING: The study was conducted at the Department of Rehabilitation, Lund University Hospital, Lund, Sweden. SUBJECTS: Twenty-five healthy female volunteers without musculoskeletal problems participated in this study. INTERVENTION: A static endurance test was performed, which consisted of a submaximal unilateral activation of the right trapezius and deltoid muscles for as long as possible. OUTCOME MEASURES: Bilateral PPTs over the trapezius and deltoid muscles were measured with an electronic pressure algometer. Established surface EMG parameters of local muscular fatigue were assessed. The Borg Rating of Perceived Exertion scale was used. RESULTS: The average endurance time was 330 seconds. Immediately after the test, significant bilateral increases in the normalized PPTs over both muscles were found, although the increase was more pronounced on the test side: over the right trapezius muscle by 13% (p <0.001), over the right deltoid muscle by 23% (p <0.001), and over the left trapezius and deltoid muscles by 6% (p = 0.04) and (p = 0.009), respectively. These increases persisted 10 minutes after the end of the test. The subjects developed significant signs of fatigue as defined by EMG criteria in both muscles on the right side during the test. The recovery from fatigue was approximately half complete 15 seconds after the end of the test and complete or almost complete 10 minutes thereafter. CONCLUSIONS: Pressure pain thresholds over shoulder muscles remained elevated up to 10 minutes after a unilateral static endurance test. This time course was completely different from that of EMG-defined muscle fatigue, which showed a fast recovery. These findings indicate that the mechanisms of recovery from fatigue and nociception are independent of each other. The bilateral PPT increases might be explained by central antinociceptive mechanisms activated by static muscle work.

Adult↗

Scapular muscle recruitment pattern: electromyographic response of the trapezius muscle to sudden shoulder movement before and after a fatiguing exercise.

STUDY DESIGN: Test-retest reliability study and single-group repeated measures design. OBJECTIVES: To evaluate the muscle latency times of the 3 portions of the trapezius muscle to a sudden arm movement in normal shoulders and to determine if this recruitment pattern is altered as a result of fatigue. BACKGROUND: It has been suggested that shoulder impingement may be related to altered muscle activity and muscle fatigue in the scapular stabilizers. Fatigue-induced changes in latency times of the trapezius might influence scapular stability. METHODS AND MEASURES: Muscle latency times were investigated in 30 healthy shoulders with surface electromyography. Muscle activity was measured in all 3 sections of the trapezius and the middle deltoid muscle during a sudden downward falling movement of the arm. Subsequently the shoulder was fatigued on an isokinetic dynamometer, after which muscle latency time measurement was repeated. RESULTS: ANOVA for repeated measures revealed significant differences in latency times (P < 0.05) among the 4 muscles of interest. Although there were no significant differences among the 3 sections of the trapezius muscle, they all were recruited after the initialization of the deltoid muscle. The recruitment order of the shoulder muscles did not change with muscle fatigue. However, after fatigue, muscle responses were significantly slower in all muscles except for the lower trapezius (P < 0.05). CONCLUSIONS: There is a specific recruitment sequence in the shoulder muscles in response to a sudden arm movement characterized by initial activation of the middle deltoid muscle and followed by simultaneous contraction of all 3 sections of the trapezius. This muscle activation pattern is delayed but not altered with fatigue.

Adolescent↗

[Case of 12-year-old boy with idiopathic recurrent neuralgic amyotrophy].

Here we report a 12-year-old boy with idiopathic neuralgic amyotrophy who had two episodes of shoulder pain followed by shoulder muscle atrophy and weakness at the age of 7 and 11 years, respectively. These symptoms were self-limited and disappeared within 9 months. During the second episode, electromyograph (EMG) revealed neurogenic changes in the deltoid muscle. Muscle imaging showed the right deltoid muscle atrophy with slightly high intensity areas on T1 and T2 weighted images in MRI. Muscle biopsy from the right deltoid muscle revealed neurogenic changes with denervating and reinnervating processes. Neuralgic amyotrophy is characterized by neuralgic pain followed by weakness and atrophy at a unilateral extremity and is usually self-limited. EMG and imaging studies showed focal neurogenic abnormalities, which were confirmed by muscle biopsy. Neuralgic amyotrophy usually occurs in young adults and it is very rare in children.

Brachial Plexus Neuritis↗

Simultaneous vaccination against hepatitis A and B: results of a controlled study.

Hepatitis A and hepatitis B are endemic in many countries and must be considered as serious health risks for large parts of the world population. Simultaneous or combined vaccination against these two diseases would therefore be most advantageous. In order to investigate possible interactions between these vaccines with respect to their tolerability and immunogenicity, we conducted a randomized prospective study comparing single and simultaneous administration of the two vaccines. Three groups of healthy volunteers, each with 55 persons, were included in the study. All were negative for hepatitis A and hepatitis B markers and had normal serum liver enzyme values. Group I received hepatitis A vaccine (720 ELISA units) into the left deltoid muscle, group II received hepatitis B vaccine (20 micrograms) into the right deltoid muscle and group III received hepatitis A vaccine into the left, and hepatitis B vaccine into the right deltoid muscle. Three doses of the vaccines were administered at 0, 1 and 6 months. Local and systemic reactions were monitored by means of questionnaires. Blood samples for determination of antibody to hepatitis A virus (anti-HAV) and antibody to hepatitis B surface antigen (anti-HBs) and of serum SGOT and SGPT levels were drawn at months 0, 1, 2, 6 and 7. There were no serious general and only mild local reactions. The mean serum SGOT and SGPT values remained in the normal range in all groups. The seroconversion rates and mean geometric titres of the anti-HAV and anti-HBs antibodies were similar when the vaccines were administered separately or simultaneously. There were no significant differences between the compared groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Function of the supraspinatus muscle in a dynamic shoulder model].

Active glenohumeral joint motion was simulated in ten cadaveric shoulder specimens with hydrodynamic cylinder forces at the deltoid muscle and at the rotator cuff. Computerized regulation initiated standardized cycles of glenohumeral joint motion in which the isolated effect of the supraspinatus muscle could be studied. The effect of the supraspinatus muscle on the elevation of the glenohumeral joint was measured with an ultrasonic sensor system. Pressures underneath the coracoacromial arch as an indicator of the 'impingement' at the shoulder were recorded with capacitive sensors. Elimination of force of the supraspinatus muscle led to a significant 6% decrease in elevation (p < 0.05). The deltoid muscle was able to reverse this loss of elevation by an increase in force equal to only one third the force lost at the supraspinatus muscle. When no force was applied to the supraspinatus muscle average pressures under the coracoacromial vault decreased by 8% (p > 0.05). In conclusion, the supraspinatus muscle produces less torque and more glenohumeral joint compression than the deltoid muscle. However, the supraspinatus muscle has no significant effect on the centering of the humeral head on the glenoid during elevation.

Acromioclavicular Joint↗