Cardiofacial syndrome. Congenital heart disease and facial weakness, a hitherto unrecognized association.
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Child, Preschool↗
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Results for “FACIAL MUSCLES”
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Asymmetric crying facies and congenital anomalies.
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Crying↗
Fluctuating and intermittent facial weakness following a local anaesthetic.
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Adult↗
Oculopharyngeal myopathy with distal and cardiomyopathy.
Two patients are described with distinctive clinical features including an insidious onset, slow progression, bilateral ptosis, weakness of facial muscles, dysphagia, muscle atrophy, and weakness with a distal distribution in the extremities, and cardiomopathy with conduction system disorders. Electromyographic studies and muscle biopsy showed features highly suggestive of a myopathic disorder. One case is considered to be sporadic. The other seems to be a familial disorder, because of the presence of a mild atrioventricular block and right incomplete bundle branch block in the patient's son and the presence of eyelid ptosis in his sister. This may be a variant of oculopharyngeal myopathy with distal and cardiomyopathy. It will be necessary to perform long-term follow-up studies in these families.
Arm↗
Hemifacial spasm: treatment by posterior fossa surgery.
Nine cases of hemifacial spasm have been treated by posterior fossa exploration without mortality or significant morbidity. In only three was definite pathology found, but the hemifacial spasm was abolished in eight patients and markedly diminished in the remaining patient. The condition has reccurred in one patient. Microsurgical techniques make the operation safe and accurate. We suggest that this procedure is the best approach for hemifacial spasm requiring treatment. Where no definite pathology is found, the effectiveness of the procedure is probably due to fibrosis and hence mild trauma to the facial nerve induced by the sponge wrapped around the nerve.
Adult↗
Hemifacial spasm: a long term follow-up of patients treated by posterior fossa surgery and facial nerve wrapping.
Sixteen patients with hemifacial spasm were treated by posterior fossa surgery and wrapping of sponge around their facial nerve. A good or excellent result has been obtained in fourteen of the sixteen cases, and in seven cases followed by four years or longer. Two patients had a mild recurrence of their hemifacial spasm after a cessation of their spasm for eighteen months and two years following surgery. Contrary to the experience of other authors a definite vascular abnormality was found in only four cases. In the other twelve cases circumferential fibrosis about the nerve is again proposed as a mechanism for the effectiveness of the procedure.
Adult↗
Hemifacial spasm: treatment by posterior fossa surgery.
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Cranial Fossa, Posterior↗
Primary hemifacial spasm: a neurophysiological study.
A series of 53 cases of primary hemifacial spasm have been evaluated by means of blink reflexes and their results compared with a normal control group. Reflex responses were obtained by percutaneous electrical stimulus of both the supraorbital nerve (trigemino-facial reflex), and the facial nerve at the stylo-mastoid region (facio-facial reflex). The R2 response was considered abnormal when its latency was shortened (hyperactivity) or delayed (hypoactivity). Thirty-six out of 53 cases with primary hemifacial spasm showed abnormal responses, with a combination of facial nerve impairment (delayed R2 in the facio-facial reflex) and trigeminal-facial hyperactivity (shortened R2 in the trigemino-facial reflex). Five cases showed hyperactivity in both the trigemino-facial reflex and the facio-facial reflex reflexes. These results suggest a state of hyperexcitability, probably at the level of the facial nucleus, combined with a peripheral facial nerve involvement in a high proportion of patients with primary hemifacial spasm.
Adult↗
Hypoglossal-facial nerve anastomosis: a clinical and electrophysiological follow-up.
Eight patients with acoustic neuroma and five patients with hemifacial spasm, who had undergone hypoglossal-facial nerve anastomosis 1-14 years previously, were reviewed clinically and electrophysiologically with an electrically elicited blink reflex. Functional recovery from the anastomosis, as rigorously judged on a scale of good, fair and poor, was fair to poor. Electrically it was found that the blink reflex was present in eight patients, suggesting facial nerve re-innervation of the facial musculature. This was confirmed by the mild recurrence of hemifacial spasm in four patients. Implications for the future role of this operative procedure are discussed.
Facial Muscles↗
The association of hemifacial spasm and facial pain.
The recognition of an association between trigeminal neuralgia and ipsilateral hemifacial spasm has been delayed by confusion over the nomenclature of the two conditions. Three patients are presented who had facial pain associated with hemifacial spasm. The findings on investigation of these patients, and an analysis of the literature, suggests that the combination is almost inevitably associated with pathological processes in the posterior fossa, particularly anomalous, ectatic or aneurysmal blood vessels.
Facial Muscles↗
Botulinum toxin treatment of cranial-cervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial spasm.
In the past five years, 477 patients with various focal dystonias and hemifacial spasm received 3,806 injections of botulinum A toxin for relief of involuntary spasms. A definite improvement with a global rating greater than or equal to 2 on a 0-4 scale, was obtained in all 13 patients with spasmodic dysphonia, 94% of 70 patients with blepharospasm, 92% of 13 patients with hemifacial spasm, 90% of 195 patients with cervical dystonia, 77% of 22 patients with hand dystonia, 73% of 45 patients with oromandibular dystonia, and in 90% of 21 patients with other focal dystonia who had adequate follow up. While the average duration of maximum improvement lasted about 11 weeks after an injection (range seven weeks in patients with hand dystonia to 15 weeks in patients with hemifacial spasm), some patients benefited for over a year. Only 16% of the 941 treatment visits with follow up were not successful. Except for transient focal weakness, there were very few complications or systemic effects attributed to the injections. This study supports the conclusion that botulinum toxin injections are a safe and effective therapy for patients with focal dystonia and hemifacial spasm.
Adolescent↗
Occidental type cerebromuscular dystrophy: a report of eleven cases.
Occidental type cerebromuscular dystrophy (OCMD) forms a substantial distinct group within congenital muscular dystrophy (CMD). These patients invariably present with amyotrophy, multiple joint contractures, facial muscle involvement, normal or nearly normal intelligence, leukodystrophic appearance on CT scan, and dystrophic changes in muscle.
Biopsy↗
Hemifacial spasm in tuberculous meningitis.
Hemifacial spasm developed in a 47-year-old man with tuberculous meningitis. The spasms ceased completely following vigorous antituberculous treatment. A selective compression of the facial nerve root along its exit at the brainstem by a localised inflammatory process is the most tenable explanation for the development of the hemifacial spasm.
Facial Muscles↗
Major motor atrophic patterns in the face and neck: CT evaluation.
Cranial nerve deficits from various pathologic processes of the head and neck may result in characteristic patterns of denervation muscular atrophy. Such atrophic patterns may be clues to the location and extent of the lesion, particularly when cranial nerves are involved early in the course of the disease process. Thirty-six patients with computed tomographic (CT) evidence of muscular atrophy secondary to pathologic conditions involving the motor division of cranial nerves were examined. Five characteristic denervation muscular atrophy patterns seen on CT scans were identified. In several patients, identification of the muscular atrophy pattern was the only clue to the presence of a pathologic condition. Recognition of these atrophic patterns can prevent misinterpretation of their CT appearance and direct the CT examination to the course of the compromised cranial nerve from the brainstem to its peripheral innervation.
Accessory Nerve↗
Brain stem tonic inhibition of thermoregulation in the rat.
It was shown, by selective levels of decerebration, that a drop in body temperature occurred in a midpontine rat due to impairment in thermoregulatory responses. Lowering the level of decerebration to the lower pons restored a coordinated thermoregulatory response. The response was inadequate to raise the core temperature to normal level, but it was nevertheless an integrated response of vasoconstriction, piloerection, and shivering and was appropriately turned off by warming the skin or core. In intact unanesthetized rats, microinjection of a local anesthetic in the pontine tegmentum resulted in thermoregulatory responses, such as curling of the body, piloerection, tremor in facial muscles, and shivering. Similar injections in the pontine tegmentum of the midpontine decerebrate rat also restored some heat gain responses, including shivering. These experiments suggest that in rats the heat gain responses are powerfully regulated by a tonic inhibitory mechanism located in the midbrain and upper pons that exerts a complete inhibitory effect on the thermoregulatory areas caudal to the midbrain in decerebrate rats and a partial inhibitory effect in intact animals. These experiments also confirm the previous observation in the cat of the presence of a facilitatory area(s) in lower levels of the central nervous system which is responsible for coordinated heat gain responses observed in low-level decerebrate animals. In addition, these experiments suggest that the brain stem thermoregulatory mechanisms are in fact functioning in intact unanesthetized animals.
Animals↗
Upper airway walls impedance measured with head plethysmograph.
Respiratory input impedance (Zrs) measured by forced oscillations needs to be corrected for the motion of extrathoracic airway walls. Two methods of obtaining the impedance of this shunt pathway [upper airway impedance (Zuaw)] were compared in six normal subjects. In the first, flow was measured at the airway opening during Valsalva maneuvers, as described by Michaelson et al. (10). In the second, motions of upper airway walls were directly assessed during respiratory impedance measurements by use of a head plethysmograph. Larger upper airway impedance values were found during Valsalva maneuvers, corresponding to a larger upper airway resistance (Ruaw) (at 20 Hz, Ruaw = 9.1 +/- 4.7 compared with 7.0 +/- 2.1 cmH2O X 1-1 X s with the second method) and inertance (Iuaw) (Iuaw = 0.053 +/- 0.036 vs. 0.025 +/- 0.008 cmH2O X 1-1 X s2, P less than 0.05) and a lower upper airway compliance (Cuaw) (Cuaw = 0.78 +/- 0.33 vs. 1.15 +/- 0.15 ml X cmH2O-1, P less than 0.05). Active contraction of facial muscles during Valsalva maneuvers may be responsible for this finding. As a consequence, respiratory impedance values are undercorrected when using the Valsalva method, leading in normal subjects to an overestimation of respiratory compliance by 30% and an underestimation of inertance by 16% (P less than 0.05) and promoting positive frequency dependence of respiratory resistance. Substantial errors may be avoided by using a head plethysmograph, which permits measuring Zrs and Zuaw simultaneously.
Airway Resistance↗
Morphology of the external nose in Hipposideros diadema and Lavia frons with comments on its diversity and evolution among leaf-nosed Microchiroptera.
In some bats, a noseleaf is thought to help focus echolocation calls emitted through the nostrils. I studied the ontogenetic mode of the rhinarial cartilages and the associated facial muscles to assess how these rhinarial infrastructures interact with the noseleaf, and the inferred function of such a rhinarial complex. This study focuses on developmental stages of Hipposideros diadema and Lavia frons. Based on new data on these two rhinolophoids and a review of former studies concerning rhinopomatids, rhinolophoids and phyllostomids, the functional and phylogenetic implications of the rhinarial complex among leaf-nosed Microchiroptera are evaluated by the current study. Nasal emitting forms evolved several times independently in the Microchiroptera and share various features, irrespective of their phylogenetic position: the nostrils lie dorsally; the noseleaf has a well-developed horseshoe-shaped plate; the cartilago cupularis bears a large processus cupularis; M(iv) is extended on the lateral plate of the processus alaris superior. The unique similarities of the ontogenetic process of the nasal 'resonators' support the assumption that the rhinolophids + megadermatids and rhinopomatids may represent a natural group. In some features, the rhinolophids + megadermatids differ significantly from the phyllostomids. The noseleaf has a median flap and anterior-facing pockets. The processus alaris superior is half-tube-shaped or included in the lateral wall of the cupula nasi anterior. The cartilago accessoria and the attendant musculature perform an important function for supporting and moving the noseleaf. Similar emission of the echolocation pulses but alternative constructional designs of the external nose structures suggest that a separate history of rhinolophoids and phyllostomids might account for these differences.
Animals↗