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PubMed1982

Organization of the facial nucleus in the rat.

The topographic organization of the facial nucleus motoneurons in the rat was investigated with the horseradish peroxidase retrograde tracing method using tetramethyl benzidine as a chromogen. Facial muscles were 'represented' on the facial nucleus in an orderly fashion. For example, the motoneurons supplying the rostral (nasolabial) muscles are laterally placed, whereas those supplying the caudal (auricular) muscles are medially placed. Notable findings in this study are: (i) the relatively unremarkable size of the nasolabial (vibrissal) muscle representation; there does not seem to be an increase in size of this part of the nucleus, commensurate with the specialized sensory functions of the vibrissal area of the face and (ii) the neurons supplying the posterior belly of the digastric are found in the suprafacial nucleus which lies about 1 mm dorsal to the main facial nucleus.

Animals↗

PubMed1989

Congenital nemaline myopathy. A clinical follow-up of twelve patients.

A clinical follow-up of 12 patients with congenital nemaline myopathy (CNM) is reported. The aims of the study were to characterise the disease further, to investigate the prognosis of CNM and the factors influencing it, to find guidelines for treatment and, through neuropsychological tests, electroencephalography and computed tomography of the head, to determine whether the central nervous system is affected. The following distribution of muscular weakness was constantly found: the weakest muscles were the facial muscles, the flexors of the neck and trunk, the dorsiflexors of the feet and the extensors of the toes. The distal limb muscles and the limb-girdle muscles were clearly weaker than the proximal limb muscles. No signs of involvement of the central nervous system were detected, and IQs showed a skew towards higher levels. The clinical state of health had deteriorated in 10 of the 12 patients and improved in 2. In addition to the grade of disease activity, prognosis seemed to be influenced mainly by the development of scoliosis and by the restricted respiratory capacity. Since no specific therapy is available for this disease, recommendations for the management of CNM include active rehabilitation and vigorous treatment of respiratory infections. Physiotherapy should focus especially on the maintenance of cardiorespiratory capacity and the prevention and treatment of scoliosis. Long periods of immobilisation should be avoided.

Adolescent↗

PubMed2004

Different levels of neuroprotection by two insulin-like growth factor-I splice variants.

We compared the neuroprotective effects of a liver-type isoform of insulin-like growth factor-I (IGF-IEa) and its splice variant, mechano-growth factor (MGF), isolated from active skeletal muscle. cDNAs of these peptides were injected into the facial muscle of adult rats prior to facial nerve avulsion. This resulted in significant neuroprotection of 88% and 37%, respectively, of motoneurons compared to control plasmid and avulsion-only groups. MGF is markedly more effective than the liver-type, systemic IGF-I for motoneuron survival, suggesting a major role for the peripheral target in adult neuronal maintenance and survival.

Alternative Splicing↗

PubMed1987

Multiple facial suspensions in protracted facial palsy.

Primary objective of multidirectional tractions of the facial muscles for protracted facial paralysis is to restore the facial symmetry. The facial suspension by multiple fascial strips in conjunction with other complemental minor surgical procedures realizes these complex tractions. Deformities requiring surgical procedures, regionally classified, are indicated for surgery as follows: 1) drooping of the eyebrow and upper eyelid, and loss of frontal creases, 2) lagophthalmos and ptosis of the lower eyelid, and 3) drooping of the cheeks and lips, and loss of the nasolabial fold. The multifascial suspension is a valuable adjunct to facial nerve surgery. It is capable of alleviating facial asymmetry due to paralysis and suppresses abnormal associated movements subsequent to surgical repair of facial nerves.

Adult↗

PubMed2002

Congenital muscular dystrophy with adducted thumbs, ptosis, external ophthalmoplegia, mental retardation and cerebellar hypoplasia: a novel form of CMD.

At least six different forms of congenital muscular dystrophy are associated with structural changes of the central nervous system, and three of these have been mapped: merosin-deficient congenital muscular dystrophy on chromosome 6q2, Fukuyama congenital muscular dystrophy on chromosome 9q31, and muscle eye brain disease on chromosome 1p32. Walker-Warburg syndrome, congenital muscular dystrophy with calf hypertrophy, pontocerebellar hypoplasia, and normal eyes, and congenital muscular dystrophy with severe mental retardation and cerebellar cysts are nosologically distinct and have been excluded from the known congenital muscular dystrophy loci with structural changes of the central nervous system. Here, we describe a novel congenital muscular dystrophy syndrome which is phenotypically distinct from the recognized forms of congenital muscular dystrophy with brain involvement. Two siblings, a boy and a girl, were born to consanguineous parents from Sicily. Both children were born with adducted thumbs and toe contractures. They were floppy from birth, walked late, showed profound generalized muscle weakness including facial muscles, elevated creatine kinase levels of 200-700U/l, and histological changes compatible with muscular dystrophy. In addition, both showed ptosis, external ophthalmoplegia, mild mental retardation, and mild cerebellar hypoplasia on MRI. Immunocytochemistry showed normal expression of muscle membrane proteins including laminin alpha 2, laminin beta 2, and alpha-dystroglycan. Linkage analysis excluded the candidate loci on chromosomes 6q2, 9q31, and 1q32. The gene locus for congenital muscular dystrophy 1B, MDC 1B, on chromosome 1q42 was also excluded. Adducted thumbs are a distinct clinical sign that has not been reported in congenital muscular dystrophy before and should facilitate recognition of further patients with this disorder.

Blepharoptosis↗

PubMed1999

Contractile activation characteristics of single permeabilized fibres from levator palpebrae superioris, orbicularis oculi and vastus lateralis muscles from humans.

1. We investigated the contractile activation characteristics of single membrane-permeabilized fibres from the following muscles from humans: the levator palpebrae superioris (LPS), an extraocular muscle; the orbicularis oculi (OO), a facial muscle; and the vastus lateralis (VL), a major muscle of the thigh. 2. Single permeabilized muscle fibres were isolated from each of the different muscles, attached to a sensitive force transducer and activated by rapid immersion in buffered solutions of varying [Ca2+] and [Sr2+]. Fibres were allocated into discrete populations based on their contractile characteristics, including their differential force responses during Ca2+ and Sr2+ activation. 3. With the exception of one fibre from the LPS, all 152 fibres sampled from the three different human muscles could be classified into either population I (slow, type I) or population II (fast, type II) based on their force-pCa(pSr) relations. The LPS muscle fibre which was unable to be classified into the two major fibre populations displayed a combination of the typical force-pCa(pSr) relations for mammalian fast and slow muscle fibres. 4. Although fibres from the LPS, OO and VL muscles had similar differential sensitivities to Ca2+and Sr2+, the steepness of the force-pCa(pSr) curves for fibres from the LPS and OO muscles were highly variable compared with those for fibres from the VL muscle. Specific forces (N cm-2) of the smaller diameter fibres from the LPS and OO muscles were significantly lower than those of fibres from the VL muscle. 5. The differences in the contractile activation characteristics between fibres from the VL muscle and those of fibres from facial (OO) muscles and extraocular (LPS) muscles, reflect the differences in their fibre composition that are responsible for their functional specificity.

Algorithms↗

PubMed2004

July 2003: 62-year-old female with progressive muscular weakness.

The July 2003 Case of the Month (COM). A 62-year-old female patient experienced progressive muscular weakness over the last ten years, involving shoulder and pelvic girdle muscles, paraspinal and facial muscles. A biopsy was taken from the left deltoid muscle where hepatitis vaccination had taken place 4 weeks previously. The specimen revealed macrophagic myofasciitis due to the injection of aluminium-bound vaccines. The finding can be reproduced experimentally by injecting vaccines in rats. The pathomechanism is supposed to involve immune stimulation due to long term persistence of the adjuvant. Macrophagic myofasciitis has been suggested to occasionally cause myopathy but is supposed to be unrelated to the underlying myopathy in our patient.

Aluminum Hydroxide↗

PubMed1998

Myasthenic syndrome of snake envenomation: a clinical and neurophysiological study.

In this prospective study, 65 consecutive patients with neurological manifestations after snake envenomation, were examined in order to describe the natural history of the reversible nature of muscle weakness. Snake envenoming led to a completely reversible muscle paralysis involving the external ocular muscles with sparing of the pupils, muscles of mastication, facial muscles, palatal muscles, neck and proximal limb muscles. The deep tendon reflexes were preserved with no sensory abnormalities. The muscular weakness usually set in within an hour of envenomation and lasted up to 10 days, with fatigability lasting for 12 days. Respiratory muscle paralysis led to ventilatory failure needing ventilation in severely envenomed patients. Motor and sensory nerve conduction were normal with normal resting compound motor action potentials on electromyography. Repetitive nerve stimulation gave rise to a decremental response during high frequency stimulation. The edrophonium test gave negative results. These manifestations are due to abnormalities of neuromuscular transmission and are not typical of myasthenia gravis. As the exact pathophysiology of venom-related neurotoxicity is not known, it is suggested that the neurological manifestations of snake envenoming be designated a myasthenic syndrome. Further studies to isolate the neurotoxin and its mechanism and exact site of blocking at the neuromuscular junction would pave the way for the development of a novel long-acting neuromuscular blocking agent.

Action Potentials↗

PubMed1994

Blast injuries to the facial nerve.

The recommended treatment of penetrating traumatic facial nerve injuries associated with immediate, total paralysis of the ipsilateral facial muscles generally includes facial nerve exploration and repair. We reviewed our experience with bullet injuries to the extratemporal facial nerve to determine the efficacy of this approach. Five patients with immediate, total facial nerve paralysis caused by bullet wounds near the extratemporal facial nerve were seen between July 1990 and December 1992. Of four patients who underwent surgical exploration, only one demonstrated complete transection of the facial nerve. Two of these four were followed up with serial electroneuronography, which demonstrated complete degeneration within the first week after injury. The fifth patient was followed up with serial electroneuronography without complete degeneration, and partial recovery was observed. We conclude that penetrating bullet injuries with immediate, total facial paralysis may not necessarily be associated with transection of the facial nerve. We propose a method of treating patients with these injuries using electroneuronography.

Adult↗

PubMedDate not supplied

Three silent periods in the orbiculari oculi muscles of man: normal findings and some clinical vignettes.

PURPOSE: To investigate how many true silent periods could be found in the orbiculari oculi muscles of man. MATERIAL AND METHODS: 10 subjects, clinically healthy (5 male, 5 female), with a mean age of 34 years-old (range: 23 to 48) were evaluated by mean of the blink reflex at resting and during contraction of the orbiculari oculi reflex according to protocols validated internationally. RESULTS: Three responses called R1, R2 and R3 were obtained in the orbicular oculi muscle at resting state which had latencies and amplitudes within normal limits. What was new was to obtain three silent periods when the subjects were evaluated during muscle contraction. The duration of the first silent period was statistically longer than the second one (p < 0.004) and shorter than the third silent period (p < 0.0001). In addition, this test was found useful in detecting more specific findings in patients with hemifacial spasm and Meigge syndrome. CONCLUSION: This is by the first time that three silent periods in the orbicular oculi muscles are consistently demonstrated. The refractoriness of the alpha motoneurons and the action of gamma-collateral activity seem to be the main conditions leasing to display the first two periods of muscle suppression. The modification of gamma motoneurons firing as well as a pause of muscle spindles in facial muscles due to the action of nociceptive stimuli traveling unmyelinated C fibers of the supraorbital nerve might be the most important mechanisms involved in the production of the third silent period. These results enables further clinical application of this test.

Adult↗

PubMed1977

[Biological and psychological aspects of geriatric dentistry].

A normal adult becomes a normal old man when going through a whole process of physiologic involution called natural ageing, which does not spare the stomato-gnathic system. The authors first review the biological aspects of ageing as seen on the skin and facial contour, on dentition and teeth, on periodontium, oral mucosa, tongue, salivary glands, facial and masticatory muscles, facial skeleton and temporomandibular joints. They then follow with the psychological aspects of ageing, going necessarily beyond the limits of dental medicine. The authors finally discuss means to prevent that natural ageing becomes pathologic.

Aged↗

PubMed1994

Brainstem reflexes in patients with olivopontocerebellar atrophy.

In 4 patients with familial olivopontocerebellar atrophy (OPCA) we have recently described an abnormal movement of facial muscles characterized by rhythmic muscle twitching during voluntary activation (facial action myoclonus). In the present article, we present the results of a neurophysiological study of brainstem reflexes in those 4 patients, in 4 other patients with OPCA but without facial action myoclonus, in 3 patients with pure cerebellar cortical atrophy, and in 6 normal volunteers used as control subjects. All patients had similar clinical features, but only the patients with facial action myoclonus and only one of the other patients with OPCA had brainstem atrophy detected on magnetic resonance imaging. Electrophysiological abnormalities were found in all patients with facial action myoclonus and consisted of myokymia in perioral muscles at rest, spread of spontaneous and reflex blinking to the orbicularis oris, and enhanced long-latency facial reflex responses to stimuli applied to the facial or trigeminal nerve. Other relevant electrophysiological abnormalities were the absence of jaw jerk in 2 patients, the absence of an R1 response of the blink reflex in 1 patient, and a markedly reduced compound muscle action potential of the facial nerve in another patient. Comparable electrophysiological abnormalities were found in only 1 of the patients with OPCA but without facial action myoclonus, and in none of the patients with pure cerebellar cortical atrophy. Facial action myoclonus is a clinical manifestation of a global brainstem functional derangement that may characterize a subgroup of patients with OPCA or constitute a distinctive step in the natural evolution of some forms of the disease.

Adult↗

PubMed2005

Facioscapulohumeral dystrophy presenting as infantile facial diplegia and late-onset limb-girdle myopathy in members of the same family.

We report a family with markedly variable myopathic weakness due to facioscapulohumeral muscular dystrophy (FSHD). The proband developed mild late-onset proximal limb weakness. Her two daughters had severe infantile facial diplegia, initially diagnosed as Möbius syndrome, and mild childhood-onset limb weakness and scapular winging. Results of facial muscle electromyography and muscle histopathology supported a myopathic disorder. This case study further highlights the broad clinical spectrum and intrafamily variability in FSHD, and the occasional absence of a positive correlation between fragment size and disease onset. Moreover, this study underscores the importance of considering FSHD in cases of infantile facial diplegia, especially in patients not demonstrating the full clinical features of Möbius syndrome. In difficult cases, facial muscle electromyography may help to differentiate myopathic from neuropathic weakness, and help guide further diagnostic studies.

Adolescent↗

PubMed2000

Long latency response of the mentalis muscle following transcranial magnetic stimulation with a circular coil in normal subjects.

Short latency response (SLR), middle latency response and long latency response (LLR) are elicited in facial muscles by transcranial magnetic stimulation. Although it has been said that the LLRs are elicited by the trigeminal nerve stimulation, a trigeminofacial reflex is recorded easily in normal subjects by the electrical stimulation in orbicularis oculi muscles as a blind reflex, but a trigeminal-facial reflex recorded in orbicularis oris, namely a snout reflex, is more difficult to record in normal subjects. The aim of this study is to demonstrate the LLR of lower facial muscles (mentalis muscle) by the transcranial magnetic stimulation, using a circular coil. The transcranial magnetic stimulations were performed over parieto-occipital scalp with frequencies of random and 0.3 Hz in 11 normal subjects and the responses in the mentalis muscle were recorded. The LLR of the mentalis muscle was recorded in all 11 subjects following SLRs. The latency, duration and LLR/SLR ratio were 37.4 msec, 20.3 msec and 9.1%, respectively. The waveform of the LLR varied trial to trial showing habituation with a stimulation of 0.3 Hz. At this time the LLR of the masseter muscle was not recorded following this transmagnetic stimulation. It was suggested that the LLR of the mentalis muscle is recorded by the transcranial magnetic stimulation of the trigeminal nerve with a circular coil. The ease and reliability of their recording make it possible to apply this LLR clinically as well as a blink reflex.

Adult↗

PubMed1978

Mechanisms of inactivation of noradrenaline in the iris sphincter, tracheal muscle and facial artery of cattle: implications for beta-adrenoceptor-mediated responses.

1 The role of neuronal and extraneuronal pathways of amine inactivation in regulating the inhibitory actions of noradrenaline was investigated in three bovine smooth muscle preparations in which the primary adrenoceptor is of the beta-type.2 The extraneuronal uptake inhibitor, 17beta-oestradiol, sensitized the inhibitory responses to noradrenaline in the facial artery, the iris sphincter and in tracheal muscle preparations, indicating a major role for non-neuronal processes in agonist-inactivation in all three preparations. Cocaine also increased responses to noradrenaline, pointing to a role for neuronal uptake either as a terminating mechanism or as a process limiting access of exogenous agonist molecules to their site of action.3 Cocaine did not enhance significantly responses to isoprenaline, a potent beta-adrenoceptor agonist which is not taken up neuronally. Further, relaxations to metaraminol, a sympathomimetic amine which is taken up extraneuronally, but much less so than noradrenaline, were also less enhanced by 17beta-oestradiol in the three preparations tested. These findings support the specificity of action of cocaine and 17beta-oestradiol as neuronal and extraneuronal uptake inhibitors in the present experiments.4 Studies of the uptake of [(3)H]-noradrenaline revealed that 17beta-oestradiol reduced the uptake of amine in the presence of cocaine, confirming a cocaine-resistant site of action for the steroid in all three preparations.5 It is concluded that extraneuronal uptake sites are located sufficiently close to the beta-adrenoceptors to modulate the concentration and duration of action of noradrenaline at these sites of action. It is proposed that in smooth muscles which contain a preponderance of beta-receptors, extraneuronal metabolism is a key event in terminating the inhibitory effects produced.

Animals↗

PubMed2002

Influence of early high-dose steroid treatment on Bell's palsy evolution.

The objective of this double-blind, randomized, placebo-controlled study was to test the efficacy of high-dose prednisone, administered as early as possible, in modifying the natural progression of Bell's palsy. Sixty-two consecutive patients, enrolled within 72 hours of facial palsy onset, were assigned to high dose intravenous prednisone in combination with intramuscular polyvitaminic therapy (group A) or polyvitaminic therapy alone (group B). Clinical grading of facial muscle strength and length of absence from work were evaluated. An early worsening of facial muscle strength was observed in controls, leading to the divergence in the trends of the grading scores in the two groups; this result was not confirmed in the long-term follow-up. Treated patients returned to work earlier than controls. In conclusion, early treatment based on high-dose corticosteroids slightly accelerates spontaneous improvement in Bell's palsy.

Adolescent↗

PubMed1989

[Gaze-evoked eyelid closure. Report of two cases].

Various types of associated movements of eyeballs with other cranial muscles have been described in the literature. Only a few observations, however, have been reported on the relation of ocular movements and facial muscles innervated by the facial nerve, especially the orbicularis oculi muscles. We report two cases presenting gaze-evoked involuntary contraction of the orbicularis oculi muscle and unilateral eyelid closure. Case 1 was a 38-year-old housewife who was admitted to our hospital because of gait disturbance and sensory deficits below the neck. She had a 5-year history of left facial palsy, disturbed horizontal eye movements, retrobulbar optic neuritis, spastic paraparesis and bladder and rectal disturbances, some of which had relapsed a few times. Neurological abnormalities were summarized as follows: bilateral pale discs, right Horner's sign, horizontal nystagmus, left facial nerve palsy of peripheral type, spastic paraparesis with left-sided predominance, sensory disturbances below the third cervical segment, truncal and limb ataxia, and bladder and rectal disturbances. Gaze-linked synchronized contraction of the left facial muscles was observed and on the left lateral gaze marked spasm of the left orbicularis oculi muscle with occasional lid closure was evoked. Case 2 was a 72-year-old female with cerebellar hemorrhage. Neurological abnormal findings included mild disorientation, meningeal irritation signs, horizontal nystagmus on lateral gaze, reduced response of reflex eye movement to the left, minimal weakness of the left facial muscles, ataxic dysarthria, mild left hemiparesis and hemisensory deficits with face, truncal and limb ataxia. She could move eyeballs to any direction but with effort to the left.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1989

Psychophysiological responses to stress in patients with myofascial pain-dysfunction syndrome.

The hypothesis was tested that patients diagnosed with myofascial pain-dysfunction (MPD) syndrome display a stereotypic response to stress via increased activity in the facial muscles. Twenty MPD patients and 20 matched control subjects were seated and exposed to affectively neutral, then stressful, then affectively neutral film clips. During film viewing, heart rates and skin-conductance levels were recorded along with bilateral EMG activity from the frontalis, temporalis, and masseter muscles and unilateral EMG activity from the forearm extensor. While both groups showed facial EMG responses to the stressor, MPD patients showed lower heart-rate and skin-conductance responses to experimental stress than did normals and a differentially higher frontalis EMG response. MPD patients also showed higher resting EMG levels than did normals at four of six facial muscle sites.

Arousal↗