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[The diagnosis of amyotrophic lateral sclerosis supported by motor evoked potential and brain MRI studies].

A 57-year-old man developed severe muscle weakness and atrophy of the upper extremities within a five-month period. Neurological examination revealed severe weakness and atrophy in the scapular muscles and proximal and distal muscles of the upper extremities. Fasciculations were also observed in the various muscles of the upper extremities. There was neither muscle weakness, atrophy nor fasciculation in either his face, neck muscles or lower extremities. He had no pseudobulbar or bulbar signs. Tendon reflexes were mildly hyperactive in the jaw and lower extremities, and normal in the upper extremities. There were no pathological reflexes, spasticity or sensory disturbances. The needle EMG study revealed denervation potentials in all muscles of the upper extremities examined. The nerve conduction study revealed no findings of the conduction block. Cervical spine X-rays revealed the narrowing of the spinal foramens at the left C3/C4 and bilateral C4/C5, C5/C6, and C6/C7 intervertebral levels. In addition, magnetic resonance imaging (MRI) revealed compressions of the cervical cord at C4/C5 and C5/C6 intervertebral levels. These clinical and neuroradiological findings resembled those of the cervical spondylotic amyotrophy (CSA). However, the motor evoked potential (MEP) study revealed the pyramidal tract dysfunction above the levels of the pyramidal decussation. Furthermore, brain MRI revealed abnormal foci in both internal capsules which were characterized by hyperintense relative to cortical gray matter on T2-weighted images and still hyperintense to white matter on proton-density-weighted images. In addition, T2-weighted images demonstrated a low signal within the motor cortex and hyperintense lesions in the white matter of the precentral gyri. These MRI findings indicated the degeneration of the pyramidal tract and corresponded to those found in the patients with amyotrophic lateral sclerosis (ALS) which have been recently reported. It has been difficult to distinguish ALS from CSA. However, MEP and brain MRI studies were useful for distinguishing these two diseases in this patient. In addition, this patient showed typical MRI findings suggesting the degeneration of the pyramidal tract, although this patient had a relatively short course of illness and did not show obvious physical findings suggesting pyramidal tract dysfunction.

Amyotrophic Lateral Sclerosis↗

EMG activity of the muscles of the neck and forelimbs during different forms of locomotion.

We recorded the electromyographic (EMG) activity of 7 skeletal muscles in the forequarters and 1 in the hindquarters of 6 Thoroughbred horses during overground walking, swimming in a circular pool, and walking and trotting in a water treadmill. Bipolar fine wire electrodes were inserted into the muscles and the EMG signals were recorded using a telemetric system. The splenius exhibited tonic EMG activity during swimming. The brachiocephalicus showed its highest intensity during swimming followed by the walk and trot in the water treadmill and then walking overground. The triceps brachii caput longum had a similar activity pattern to the brachiocephalicus. The brachialis showed only weak EMG activity in all 3 types of locomotion. The extensor digitorum communis had higher intensity of EMG activity in the walk in the water treadmill than in other kinds of locomotion. The flexor digitorum profundus exhibited the most intense EMG activity during swimming. These results indicated that swimming evoked strong EMG activity in the antigravity muscles in spite of reduced gravitational force. Walking in the water treadmill may require more intensified EMG activity of the forelimb than the trot in the same treadmill.

Animals↗

Flight-training effect on the cervical muscle isometric strength of trainee pilots.

External stimulus/loading initiates adaptations within skeletal muscle. It has been previously found that the cervical area has the highest loading while performing flying maneuvers under +Gz. The first purpose of this study was to examine the neck muscle response to the physical environment associated with flight training, incorporating limited exposure to +Gz force, in a Pilatus PC-9 aircraft. The second purpose was to examine the short-term range of movement (ROM) response to flight training. Isometric cervical muscle strength and ROM was monitored in 9 RAAF pilots completing an 8-mo flight-training course at Pearce Airbase in Western Australia, and in 10 controls matched for gender, age, height, and weight. Isometric cervical muscle strength and ROM were measured at baseline and at 8 mo using the multi-cervical rehabilitation unit (Hanoun Medical, Downsview, Ontario, Canada). Results indicated that an increase in pilot neck strength was limited to flexion while in a neutral position. No strength changes were recorded in any other site in the pilots or for the controls. These findings suggest that short-term exposure to the physical environment associated with flight training had a limited significant effect on increasing isometric cervical muscle strength. No significant changes were observed in pilot ROM, indicating that short-term exposure to flight does not effect ROM.

Adult↗

Sitting posture and neck and shoulder muscle activities at different screen height settings of the visual display terminal.

The effects of the VDT screen height on working posture and electromyographic (EMG) activities of the neck and shoulder muscles were determined in 10 healthy subjects. The subjects performed a mouse-driven interactive task at the screen height settings of 80, 100 and 120 cm. Changes in body positions were measured by video image and frame analysis. Surface EMG recordings were done on the neck extensor muscle and the descending part of the trapezius muscle, both on the right side. The results of the postural analysis showed that at higher screen height settings, the neck became significantly more erect. The subjects also assumed a more backward-leaning trunk position at higher screen heights. The EMG activities of the neck and shoulder muscles were related to the neck angle and trunk inclination, respectively. A more flexed neck produced significantly higher neck extensor muscle activities. A backward-leaning trunk was also noted to decrease trapezius muscle activity in some subjects.

Adult↗

Eye and neck motor signals in periabducens reticular neurons of the alert cat.

The purpose of this work was to search for neurons in the ponto-medullary reticular formation which can carry horizontal eye position signals to dorsal neck muscles of the cat. The recordings were localized in an area of the ponto-medullary reticular formation which contains reticulo-spinal neurons projecting to the neck (Peterson et al. 1980). Experiments were performed on alert, head-fixed cats. Eye movements were measured by the search-coil technique. Neuronal activity was recorded extracellularly with glass microelectrodes. Neurons were localized both by stereotaxic coordinates and by their position with respect to the antidromic field potential profile of the abducens nucleus. EMG of longissimus capitis, obliquus capitis and splenius muscles were recorded. Vestibular stimulation was produced by a turn-table. Reticular cells were found below the abducens nucleus (1 to 3.5 mm below the center of the nucleus, A.P.:5.3 to 7.2 mm;L: 1 to 1.5 mm) showing a firing rate closely related to EMG during spontaneous saccades and, to a variable degree, to the ipsilateral horizontal component of eye position. 'Tonic' and 'burst-tonic' cells were found. During vestibular stimulation, the firing rate kept the same relationship with eye position and neck EMG. It is concluded that the analyzed reticular cells are good candidates to be reticulo-spinal neurons which mediate a signal allowing a synergistic movement of head and eye during orientation in the horizontal plane.

Abducens Nerve↗

Effects of applied vibration on triphasic electromyographic patterns in neurologically ballistic head movements.

Vibration of agonist or antagonist muscle tendon produced changes in the triphasic electromyographic pattern of neck muscles; EMG signals were rectified, averaged, and also integrated by planimetry. The triphasic EMG envelopes obtained during fast horizontal head rotation showed unmodified early agonist pulse, the action pulse (PA), under all conditions; increased antagonist pulse, the braking pulse (PB), only with antagonist muscle vibration; and increase of late agonist pulse, the clamping pulse (PC), only with agonist muscle vibration. Vibration experiments can be considered as a model for studying interactions between central and peripheral effects on control of normal movements.

Electromyography↗

The effect of muscle activation on neck response.

Prevention of neck injuries due to complex loading, such as occurs in traffic accidents, requires knowledge of neck injury mechanisms and tolerances. The influence of muscle activation on outcome of the injuries is not clearly understood. Numerical simulations of neck injury accidents can contribute to increase the understanding of injury tolerances. The finite element (FE) method is suitable because it gives data on stress and strain of individual tissues that can be used to predict injuries based on tissue level criteria. The aim of this study was to improve and validate an anatomically detailed FE model of the human cervical spine by implement neck musculature with passive and active material properties. Further, the effect of activation time and force on the stresses and strains in the cervical tissues were studied for dynamic loading due to frontal and lateral impacts. The FE model used includes the seven cervical vertebrae, the spinal ligaments, the facet joints with cartilage, the intervertebral disc, the skull base connected to a rigid head, and a spring element representation of the neck musculature. The passive muscle properties were defined with bilinear force-deformation curves and the active properties were defined using a material model based on the Hill equation. The FE model's responses were compared to volunteer experiments for frontal and lateral impacts of 15 and 7 g. Then, the active muscle properties where varied to study their effect on the motion of the skull, the stress level of the cortical and trabecular bone, and the strain of the ligaments. The FE model had a good correlation to the experimental motion corridors when the muscles activation was implemented. For the frontal impact a suitable peak muscle force was 40 N/cm2 whereas 20 N/cm2 was appropriate for the side impact. The stress levels in the cortical and trabecular bone were influenced by the point forces introduced by the muscle spring elements; therefore a more detailed model of muscle insertion would be preferable. The deformation of each spinal ligament was normalized with an appropriate failure deformation to predict soft tissue injury. For the frontal impact, the muscle activation turned out to mainly protect the upper cervical spine ligaments, while the musculature shielded all the ligaments disregarding spinal level for lateral impacts. It is concluded that the neck musculature does not have the same protective properties during different impacts loadings.

Accidents, Traffic↗

Changes in saccadic reaction time while maintaining neck flexion in men and women.

We investigated changes in saccadic reaction time in relation to the degree of increase in activity of neck extensor muscles when neck flexion occurred, and assessed the reliability of the measurements. Saccadic reaction time was measured firstly, during neck flexion angles set at 5 degrees increments from 0 degrees (resting position) to 25 degrees, with the chin either resting on a stand or not, and secondly, during shoulder girdle elevator muscles providing a relative muscle force of 30%, with the neck flexion angle maintained at 0 degrees by having the subjects rest their chins on a stand. Saccadic reaction time was evaluated by the latency to the beginning of eye movement toward the lateral target, which was moved at random intervals in 20 degrees amplitude jumps. Muscle activity in the trapezius muscle was evaluated using the mean amplitude of electromyogram recordings. Very high coefficients of reliability in muscle activity and saccadic reaction time were observed between the two sets of tests at 1-h intervals and also among the three trials with a 1-min rest. When their necks were flexed and the subjects rested their chins on a stand, muscle activity increased slightly in accordance with the enlargement of this angle, with no significant change in saccadic reaction time. With the chin not resting on the stand, muscle activity increased gradually, while the saccadic reaction time decreased to that obtained at an average neck flexion angle of 20 degrees. However, the angle where the shortest reaction time was obtained showed considerable individual variation (5-25 degrees ). Activity in the trapezius muscle at a 20 degrees neck flexion angle, with the chin not resting on the stand, was far less than that for 30% maximal voluntary contraction in shoulder girdle elevator muscles. Nevertheless, the saccadic reaction times were roughly equivalent under the two different sets of conditions. No sex differences were observed in terms of saccadic reaction time under any set of conditions.

Adolescent↗

[Assessment of stretching techniques for neck and arms muscles in physiotherapy following total laryngectomy].

After total laryngectomy and after complete treatment (radiotherapy) there are many functional disorders in the range of head and neck. These decrease muscle strength and flexibility of muscles of cervical spine. This causes disorders of physiological range of motions of this area. The introduction of the stretching techniques (postisometric relaxation) to the physiotherapy program allows to decrease negative effects of treatment, mentioned above. The therapy of main cervical muscles was carried out during a two week rehabilitation camp. Biomechanical-kinesiological tests were used to assess the effect of the physiotherapy program. The application of postisometrical relaxation and the massage of the cervical and arm muscles increased the average motor ranges and muscle strength. Cervical muscles disbalance was decreased at the same time.

Adult↗

Effects of variation in physical effort on frontalis EMG activity.

The physiological mechanisms underlying forehead muscle biofeedback for stress reduction are not well understood. A common hypothesis considers the frontales to be "key muscles" uniquely indexing the degree of tension present in the general musculature. We tested this hypothesis under conditions ranging from relaxation to maximum physical effort. The hypothesis was not supported. Changes in forehead muscle activity were limited primarily to reflecting changes in head and neck muscle tension.

Adult↗

Experimental infection of chickens with Ancylostoma caninum: migration and distribution of larvae in tissues.

Two hundred one-day-old male white leghorn chickens (Gallus domesticus) divided into 4 groups were inoculated with a single dose of 500, 1000, 2000 or 4000 filariform larvae of Ancylostoma caninum. Larval recoveries were made from different tissues at intervals from 4 hours to 72 hours after infection. Larval migration in the heart took place within 4 hours in all the groups except group A with 500 larvae and in thoracic muscle at 8 hours. Larvae also migrated into liver, lungs and neck muscle within 12 hours and into leg muscle at 18 hours. No larvae were recovered from the muscles after 72 hours of infection. Larvae did not migrate into kidney, spleen and central nervous system throughout the experimental period.

Ancylostoma↗

[Familial amyloidosis of the Finnish type (FAP) with extraocular muscle involvement].

Among 17 patients with amyloid polyneuropathy type IV in a Japanese family, we found a 72-year-old woman, who showed extraocular symptoms approximately 10 years after the onset of the disease. she developed weakness of the right facial muscles and dysarthria at age 57. She had atrophy and disturbance of movement of the tongue, along with difficulty in swallowing at age 62. At the age of 66, she felt diplopia when she looked toward the left, followed by difficulty of ocular movement. These manifestations progressed and at age 72, she was found to have mild ptopsis, mild to moderate disturbance of almost all extraocular muscles, moderate to severe disturbance of facial, masseter, pharyngeal, tongue and neck muscles. She also had slight weakness and atrophy of the limb and truncal muscles together with slight loss of pain and vibratory sensations in the distal parts of the limbs. FAP IV has sometimes been called cranial amyloidosis, but motor disturbance is limited to the middle and lower cranial nerve territories in the majority of the reported cases, and manifestations of the extraocular muscles are quite rare. According to the present investigation of the world literature, this is the second case of FAP IV with extraocular muscle involvement.

Aged↗

Electromyographic activity of the cervical musculature during dynamic lateral bending.

STUDY DESIGN: Electromyographic profiles from the neck muscles of five young, healthy men were obtained to study the muscular, activation patterns during dynamic external loading. OBJECTIVES: To examine the myoelectric activity of selected cervical muscles during dynamic loading, and to explore the reaction of the cervical muscles to different loading conditions. SUMMARY OF BACKGROUND DATA: The response of cervical muscles throughout dynamic external loading is not well understood. Electromyography provides information, within certain limitations, about the neural drive to various components of the musculature. Such information on activation, combined with geometric parameters of the musculoskeletal tissues, constitutes a tool for helping to understand the mechanism of head and neck impact-related injury. METHODS: Subjects were tested with the head and neck in the neutral and prebent positions. Dynamic loads were applied laterally to each subject by free-falling masses to create peak dynamic loads ranging from about 40 to 100 N. Force and electromyographic data were recorded, and cross-correlations from linear envelope electromyography were calculated. RESULTS: The peak electromyogram showed large differences between the two applied loads and between subjects. There were time delays (75-165 msec) between the peak applied force and the peak electromyogram. The muscles on the contralateral side demonstrated different electromyographic profiles between subjects. Within subjects, the electromyographic profiles from each muscle were consistent for the same trial conditions. CONCLUSIONS: Electromyographic profiles and the cross-correlator coefficients for cervical muscles have shown reproducible intrasubject muscle synergies, which were not sensitive to the magnitude of applied load and the posture of the head. Intersubject muscle activity patterns varied.

Adult↗

Effects of muscle type, castration, age, and compensatory growth rate on androgen receptor mRNA expression in bovine skeletal muscle.

The effect of testosterone on sexual dimorphism is evident by differential growth of forelimb and neck muscles in bulls and steers. Divergent hormone sensitivites may account for the differential growth rates of individual muscles. Therefore, the objective of this study was to compare androgen receptor (AR) expression in three different muscles of bulls and steers at various ages and growth rates. Thirty Montbéliard bulls and 30 steers were assigned to four slaughter age groups. Four or five animals of each sex were slaughtered at 4 and 8 mo of age. Animals in the remaining two slaughter groups (12 and 16 mo) were divided into groups of either restricted (R) or ad libitum (AL) access to feed. Five animals of each sex and diet were slaughtered at the end of the restricted intake period at 12 mo of age. To simulate compensatory growth, the remaining animals (R and AL) were allowed ad libitum access to feed until slaughter at 16 mo of age. Total RNA was extracted from samples of semitendinosus (ST), triceps brachii (TB), and splenius (SP) muscles. Androgen receptor mRNA was quantified in 200-ng total RNA preparations using an internally standardized reverse transcription (RT) PCR assay. Data were analyzed using 18S ribosomal RNA concentrations as a covariable. Steers had higher AR mRNA levels per RNA unit than bulls (P < .01). Androgen receptor mRNA levels differed between muscles (P < .05), with lowest expression in the SP. The pattern of AR expression differed (P < .05) for each muscle with increasing age. Between 4 and 12 mo of age, AR mRNA levels increased (P < .05) in SP but remained unchanged in the ST and TB. Feeding regimen had no effect on muscle AR expression, but steers exhibiting compensatory growth had higher AR mRNA levels than AL steers (P < .01) or bulls (P < .01). Our results show that AR expression is muscle-specific and may be modulated by circulating testicular hormones. These data suggest that the regulation of AR expression may be linked to allometric muscle growth patterns in cattle and compensatory gain in steers.

Aging↗

Head, neck, and mandible dynamics generated by 'whiplash'.

That injuries to the temporomandibular joint (TMJ) can be imposed by short-acting forces generated during rear-end collisions of motor vehicles was first proposed more than 50 years ago. Since that time, numerous anecdotal and clinical reports relating the onset of TMJ symptoms to low-velocity rear-end collisions have appeared in the literature. Various mechanisms of injury to the TMJ occurring during extension and flexion phases of 'whiplash' have been proposed. However, transient forces developed at the TMJ in impact velocity changes on the order of 8.0 kilometers per hour (km/h) have been shown to be well within typical physiologic ranges. This study applies current head/neck extension-flexion dynamic data to develop linear and angular force-time histories experienced at the TMJ. Fourteen test collisions of motor vehicles utilizing seven live test subjects were conducted in July 1993. Linear and angular accelerometers and high-speed photographic cameras recorded the vehicle and human-subject responses. Head accelerations and forces generated at the TMJ bore a generally linear relationship to the impact velocity changes in the range tested (3.9-10.9 km/h). Mandibular opening responses were measured on three test subjects. Neither neck hyperflexion nor hyperextension occurred for any subject on any trial. At some point in the series, all test subjects experienced neck muscle strain symptoms lasting 1-3 days. No TMJ symptoms were experienced. The head, neck, and mandible motions occurring in the 'whiplash' maneuver are more complex than previously described. The cervical muscle injury threshold appears to be reached in the 8.0 km/h range. Linear and rotational forces generated at the TMJ in rear-end impacts below the 11.0 km/h velocity-change level do not appear to be injurious.

Acceleration↗

Stability of the head in pitch (neck flexion-extension): studies in normal subjects and patients with axial rigidity.

The dynamic stability of the head in pitch during normal upright posture has been studied in normal subjects and patients with neurological disease affecting neck muscle tone by examining angular head acceleration responses to unpredictable linear motion of the trunk in the direction of surge. Within the frequency range of natural head movements the transfer function between head and trunk for both normal subjects and patients approximated a second-order linear differential equation involving inertia and coefficients of viscosity and elasticity. The degree of neck rigidity was determined by the damping ratio (viscosity:elasticity), which averaged .35 for normal subjects and ranged from 0.6 to 0.96 for patients with rigid syndromes. A patient with absent labyrinthine function and a "floppy" head had a damping ratio 0.18. The technique gives a numerical measurement of neck rigidity, which could be of value in characterising severity of disorder and response to therapy.

Adult↗

Innervation of the complexus ("hatching") muscle of the chick.

The complexus muscle of avians, also known as the "hatching" muscle, is notable for the dramatic, transient pseudohypertrophy which it undergoes around the time of hatching. The muscle is believed to be involved in specific dorsal and lateral head movements used for hatching. Innervation of the complexus muscle was studied in the hatchling chick by using horseradish peroxidase (HRP). HRP injections of the muscle showed that motor innervation arose, as expected, from the cervical motor column (C1-C6). However, additional innervation was also discovered; the spinal accessory nucleus (column of von Lenhossek), the nucleus supraspinalis, and the dorsal and ventral facial motor nuclei all contributed efferents to the hatching muscle. This observation constitutes the first description of dual innervation of a neck muscle by nuclei in both the brain and spinal cord. In addition, transganglionic transport of HRP revealed labelled primary afferent fibers from the hatching muscle ascending in the dorsal columns and terminating extensively within the vestibular complex, especially on the principal cells of the tangential nucleus. The tangential nucleus itself undergoes synaptic changes at the time of hatching. Possible functional relations between the tangential nucleus and the hatching muscle are discussed.

Accessory Nerve↗

CTG-repeat length in distal and proximal leg muscles of symptomatic and non-symptomatic patients with myotonic dystrophy: relation to muscle strength and degree of histopathological abnormalities.

Myotonic dystrophy (DM) is an autosomal dominant, multisystemic disorder with a variable phenotypic expression including muscle weakness and myotonia. The muscle wasting is most marked in distal limbs and in facial and neck muscles, although proximal limb muscles become affected as the disease progresses. The CTG-trinucleotide-repeat expansion associated with myotonic dystrophy is usually larger in muscle tissue than in leukocytes. It is unclear whether the repeat length itself bears any relation to the differences in the degree of weakness and atrophy between different muscles. We therefore analysed CTG-repeat lengths in blood and in proximal (m. vastus lateralis) and distal (m. tibialis anterior) muscles of patients with DM (n = 4) and non-symptomatic carriers of the mutant DM allele (n = 2) using conventional Southern blot hybridization. Muscle strength and histopathological abnormalities were evaluated for each muscle. In patients with clinical symptoms, the degree of paresis and morphological abnormalities was markedly more pronounced in m. tibialis anterior than in m. vastus lateralis. In these individuals, the CTG-repeat length was larger in muscles than in leukocytes, whereas in the two non-symptomatic carriers no difference could be detected. Furthermore, there was no clear difference in the repeat length between the two muscles in any of the patients. In conclusion, the selective muscular weakness and atrophy in DM do not seem to be related to differences in CTG-repeat length between different muscles.

Adult↗