Representation of facial muscles in human motor cortex. (65/322)

Whether there is a projection from the primary motor cortex (M1) to upper facial muscles and how the facial M1 area is modulated by intracortical inhibitory and facilitatory circuits remains controversial. To assess these issues, we applied transcranial magnetic stimulation (TMS) to the M1 and recorded from resting and active contralateral (C-OOc) and ipsilateral orbicularis oculi (I-OOc), and contralateral (C-Tr) and ipsilateral triangularis (I-Tr) muscles in 12 volunteers. In five subjects, the effects of stimulating at different scalp positions were assessed. Paired TMS at interstimulus intervals (ISIs) of 2 ms were used to elicit short interval intracortical inhibition (SICI) and ISI of 10 ms for intracortical facilitation (ICF). Long interval intracortical inhibition (LICI) was evaluated at ISIs between 50 and 200 ms, both at rest and during muscle activation. The silent period (SP) was also determined. C-OOc and I-OOc responses were recorded in all subjects. The optimal position for eliciting C-OOc responses was lateral to the hand representation in all subjects and MEP amplitude markedly diminished when the coil was placed 2 cm away from the optimal position. For the I-OOc, responses were present in more scalp sites and the latency decreased with more anterior placement of the coil. C-Tr response was recorded in 10 out of 12 subjects and the I-Tr muscle showed either no response or low amplitude response, probably due to volume conduction. SICI and ICF were present in the C-OOc and C-Tr, but not in the I-OOc muscle. Muscle activation attenuated SICI and ICF. LICI at rest showed facilitation at 50 ms ISI in all muscles, but there was no significant inhibition at other ISIs. There was no significant inhibition or facilitation with the LICI protocol during muscle contraction. The SP was present in the C-OOc, C-Tr and I-OOc muscles and the mean durations ranged from 92 to 104 ms. These findings suggest that the I-OOc muscle response is probably related to the first component (R1) of the blink reflex. There is M1 projection to the contralateral upper and lower facial muscles in humans and the facial M1 area is susceptible to cortical inhibition and facilitation, similar to limb muscles.  (+info)

Human extraocular muscles in mitochondrial diseases: comparing chronic progressive external ophthalmoplegia with Leber's hereditary optic neuropathy. (66/322)

AIMS: To compare the ultrastructural aspects of human extraocular muscles in two types of mitochondrial disease: chronic progressive external ophthalmoplegia (CPEO) and Leber's hereditary optic neuropathy (LHON). METHODS: Muscle samples of the medial rectus obtained from surgery in a sporadic case of CPEO associated with deleted mitochondrial DNA, and post mortem in a case of 3460/ND1 LHON were processed for electron microscopy (EM). The medial rectus from an autoptic time to fixation matched control was used to exclude postmortem artefacts. RESULTS: The CPEO specimen revealed focal areas of disruption and abnormalities of mitochondria in some muscle fibres, creating a "mosaic-like" pattern. In the LHON specimen a diffuse increase in both number and size of mitochondria (mean diameter 0.85 mum v 0.65 mum of control, p<0.0001) with swollen appearance and disorganised cristae filled all spaces of sarcoplasmic reticulum. In some areas the excessive number of mitochondria slightly distorted myofibrils. CONCLUSION: EM investigation of extraocular muscles in CPEO and LHON reveals marked differences. A "mosaic-like" pattern caused by a selective damage of muscle fibres was evident in CPEO, whereas a diffuse increase in mitochondria with preservation of myofibrils characterised the LHON case. These ultrastructural changes may relate to the different expression of the two diseases, resulting in ophthalmoplegia in CPEO and normal eye movements in LHON.  (+info)

The staircase phenomenon at the corrugator supercilii muscle in comparison with the hand muscles. (67/322)

BACKGROUND: Phonomyography (PMG) is a novel method to monitor neuromuscular block. It is non-invasive and can be applied to any muscle. It can be used interchangeably with mechanomyography (MMG). The staircase phenomenon has not been investigated for this method or at the corrugator supercilii muscle. The purpose of this work was to determine the staircase effect at three different muscles using two different methods. METHODS: In 10 patients undergoing general anaesthesia with sevoflurane, using a laryngeal mask airway without the aid of neuromuscular block, one piezo-electric microphone each was applied to the corrugator supercilii muscle and the first dorsal interosseus muscle. In addition, a force transducer was attached to the tip of the thumb to determine the force of the adductor pollicis muscle. Supramaximal stimulation at 1 Hz was used at the ulnar and the facial nerve. All signals were simultaneously recorded for 30 min. Data are presented as means (SD). RESULTS: The staircase effect was significantly positive for the first dorsal interosseus muscle and the adductor pollicis muscle. The signal potentiation was not significantly different between the first dorsal interosseus muscle with a maximum increase at 148 (19)% using PMG, and the adductor pollicis muscle at 154 (22)% using MMG. The evoked signals reached a plateau after 15-18 min at both muscles. There was only a small initial increase in signal height at the corrugator supercilii to a maximum of 117 (20)% at 7 min, after which the signals decreased to reach a plateau at 25 min. In comparison with the signal height of 105 (25)% at 30 min, there was no significant difference of signal heights throughout the observation period. CONCLUSIONS: A positive staircase phenomenon is found equally at the first dorsal interosseus muscle and the adductor pollicis muscle. There is no significant staircase effect at the corrugator supercilii muscle.  (+info)

Development of the stapes and associated structures in human embryos. (68/322)

The objective of this study was to clarify the development of the stapes in humans and its relationship with the cartilage of the second branchial arch. The study was carried out in 25 human embryos between 6 and 28 mm crown-rump length. The stapes develops at the cranial end of the second branchial arch through an independent anlage of the cartilage of this arch. Between the stapedial anlage and the cranial end of the Reichert's cartilage there is a formation called the interhyale, the internal segment of which gives rise to the tendon of the stapedial muscle. The stapedial anlage is a unique formation with two distinct parts: the superior part that will comprise the base and the inferior part that will be crossed by the stapedial artery during embryonic development and will constitute the limbs and the head of the stapes. According to the results, the otic capsule is not involved in formation of the base of the stapes.  (+info)

Comparative data on facial morphology and muscle thickness using ultrasonography. (69/322)

The purpose of this investigation was to measure the thickness of the masseter, levator labii superioris, and zygomaticus major muscles; to examine the reproducibility of ultrasonographically measured muscle thickness; to evaluate the association between facial and masticatory muscle thickness and vertical facial pattern; and to test whether the variation in muscle thickness is related to the variation in the body mass index (BMI) of different individuals. The thickness of the masseter, levator labii superioris and zygomaticus major muscles was measured bilaterally by ultrasonography in 47 (23 females, 24 males) healthy, fully-dentate young adults who volunteered for the study. The measurements for each individual were performed twice for the masseter muscle: during relaxation and during maximal clenching. Standardized lateral cephalograms of the subjects were traced to determine their facial morphology. The data obtained from the lateral cephalograms were used to divide the subjects into three groups according to their vertical facial pattern: low angle (n = 14), high angle (n = 17) and normal (n = 16). In the low angle group, the mean masseter muscle thickness was 15.20 (+/- 1.90) mm under relaxed conditions and 16.31 (+/- 2.18) mm during maximal clenching. In the high angle group, the respective measurements were 13.29 (+/- 2.52) mm and 14.72 (+/- 2.63) mm. In the vertically normal group, they were 13.56 (+/- 1.95) mm and 14.57 (+/- 1.83) mm. There was no relationship between vertical growth of the face and the thickness of the investigated muscles of facial expression. Masseter muscle thickness was found to be significantly correlated to vertical facial pattern and BMI, showing that individuals with a thick masseter had a vertically shorter facial pattern, whereas the muscles of facial expression showed no relationship with vertical facial pattern.  (+info)

Comparative anatomy of the facial motor nucleus in mammals, with an analysis of neuron numbers in primates. (70/322)

The facial motor nucleus (VII) contains motoneurons that innervate the facial muscles of expression. In this review, the comparative anatomy of this brainstem nucleus is examined. Several aspects of the anatomical organization of the VII appear to be common across mammals, such as the distribution of neuron types, general topography of muscle representation, and afferent connections from the midbrain and brainstem. Phylogenetic specializations are apparent in the proportion of neurons allocated to the representation of subsets of muscles and the degree of differentiation among subnuclei. These interspecific differences may be related to the elaboration of certain facial muscles in the context of socioecological adaptations such as whisking behavior, sound localization, vocalization, and facial expression. Furthermore, current evidence indicates that direct descending corticomotoneuron projections in the VII are present only in catarrhine primates, suggesting that this connectivity is an important substrate for the evolution of enhanced mobility and flexibility in facial expression. Data are also presented from a stereologic analysis of VII neuron numbers in 18 primate species and a scandentian. Using phylogenetic comparative statistics, it is shown that there is not a correlation between group size and VII neuron number (adjusted for medulla volume) among primates. Great apes and humans, however, display moderately more VII neurons that expected for their medulla size.  (+info)

Developmental changes in craniofacial morphology in subjects with Duchenne muscular dystrophy. (71/322)

Lateral cephalometric radiographs of 35 Japanese male patients suffering from Duchenne muscular dystrophy (DMD) were taken longitudinally from 10 to 20 years of age. Eighteen landmarks were placed and 15 angles and four linear distances calculated. Profile diagrams (profilograms) were produced to analyse changes in craniofacial morphological growth in the DMD subjects. The measurements were then compared with Japanese standards. In young patients with DMD, compared with the controls, the following were observed: a large gonial angle; clockwise rotation of the mandible; short sagittal length of the cranial base and protrusion of the upper incisors. In adult patients, the maxillary alveolus and the upper incisors were protruded, compared with the controls. Overbite in DMD subjects also showed a tendency to decrease. In the controls, mandibular growth direction tended to be straight down and forward, while in patients with DMD, the growth direction was down until approximately 16 years of age and, after that, a forward vector of growth was apparent. As a result, the tendency towards a clockwise rotation of the mandible in the adults was less than in the young patients. These findings showed that DMD significantly affects craniofacial morphology.  (+info)

Silent event-related fMRI reveals deficient motor and enhanced somatosensory activation in orofacial dystonia. (72/322)

Previous studies showed cortical dysfunction and impaired sensorimotor integration in primary generalized and focal hand dystonia. We used a whistling task and silent event-related fMRI to investigate functional changes in patients with blepharospasm and patients with a combination of blepharospasm and oromandibular dystonia (Meige's syndrome). Whistling served as a model for a skilful orofacial movement with a high demand on sensorimotor integration. It allowed us to study the oromandibular motor system that is clinically affected in Meige's syndrome but not in isolated blepharospasm. In Meige's syndrome, functional MRI revealed deficient activation of the primary motor and ventral premotor cortex within the mouth representation area during whistling. Compared with healthy controls, both forms of orofacial dystonia had increased activation of bilateral somatosensory areas and the caudal supplementary motor area (SMA) in common. While overactivity of somatosensory areas and caudal SMA in Meige patients was partly reversed by botulinum toxin treatment, impaired motor activation was not. We conclude that impaired motor activation appears to be specific for the clinically affected oromandibular motor system in Meige's syndrome while enhanced somatosensory activation is a common abnormality in both forms of orofacial dystonia independent of the affected motor system. Somatosensory overactivity indicates an altered somatosensory representation in orofacial dystonia while impaired motor activation may be a functional correlate of reduced cortical inhibition during oromandibular motor execution in Meige's syndrome.  (+info)