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PubMed1979

Pathophysiological effects of Möbius syndrome on speech and hearing.

The effects of Möbius syndrome (MoS) on the functioning of the peripheral speech and hearing mechanisms has not hithertofore been described, to my knowledge. Speech and hearing function of an 8-year-old MoS speaker was studied. Data were obtained from several sources. These included: electromyography of the facial muscles, physical examination of the peripheral speech mechanism, speech and language test batteries, audiological evaluation, and acoustic analyses of the speech signal. A pathologic condition was found to exist in a number of cranial nerves and in structures in the oral cavity. This resulted in consonant and vowel misarticulations, which notably affected speech intelligibility. Middle ear dysfunction was also present. Neurological mechanisms are discussed to account for these pathologies in human communication. Strategies for speech therapy and future research in MoS are presented.

Abducens Nerve↗
PubMed2006

End-to-side intrapetrous hypoglossal-facialanastomosis for reanimation of the face. Technical note.

The aim of this paper was to report on further experience with a new technique for reanimation of the facial nerve. This procedure allows a straight end-to-side hypoglossal-facial anastomosis without interruption of the 12th cranial nerve or the need for graft interposition. It is technically demanding and time consuming but offers an effective, reliable, and extraordinarily quick means of reinnervating the facial muscles, including the orbicularis oculi muscle, thus avoiding the need for a gold weight in the eyelid or a fascial sling.

Adult↗
PubMed1983

A method of direct eyebrow lift.

A very simple procedure, requiring about twenty minutes, accomplishes moderate elevation and support of the eyebrows. It may be utilized for eyebrow ptosis alone, whether unilateral or bilateral; in conjunction with meloplasty and blepharoplasty; for equalizing asymmetrical eyebrows, and for further support of markedly ptotic upper lids. It has been used by the author in instances of partial and complete facial paralysis in conjunction with other procedures about the face to accomplish better symmetry. The duration of results with this procedure varies with tissue quality and healing, surgical technique, care given the area during healing by the patient, amount of frowning and vigorous facial muscle use by the patient, and aging. It is a useful adjunct, especially when used for moderate or subtle brow lifts.

Aging↗
PubMed1993

AAEM case report #26: seventh cranial neuropathy.

A 25-year-old man with acute, bilateral facial palsies is presented. He had a lymphocytic meningitis, history of tick bites, and lived in an area endemic for Lyme disease, which was ultimately confirmed by serology. Electrodiagnostic investigation included facial motor nerve study, blink reflex and electromyography of facial muscles, which were indicative of a neurapraxic lesion on the right and an axonopathic lesion on the left. The clinical course was consistent with these findings as the right side fully recovered and the left remained plegic. The clinical features of Lyme associated facial neuritis are reviewed, as is the electrodiagnostic evaluation of facial palsy.

Adult↗
PubMed1976

Aarskog syndrome: new oral-facial findings.

The Aarskog syndrome is characterized by short stature with typical facial, digital and genital anomalies. A further case is reported which presented with the uncommon finding of ophthalmoplegia and three previously unreported oral-facial findings: enamel dysplasia, a "col" deformity of the anterior mandible and a paresis of the facial muscles innervated by the VII cranial nerve. The implications of genetic heterogeneity in this nosologic classification are discussed.

Abnormalities, Multiple↗
PubMedDate not supplied

[Facial hemispasm and anomalies of the terminal portion of the vertebral artery (author's transl)].

A revision is suggested of the concept of "essential hemifacial spasm": 23 cases have been studied in whom the facial spasm was the only symptom present, while in 8 other cases in addition to the spasm of facial muscles other symptoms were noticed suggesting a pathology of the ponto-cerebellar angle. In many cases the symptom "spasm" and other concomitant symptoms are a direct consequence of an irritation due to compression on the nerve root (and/or on other nervous structures) owing to stretch of the distal tract of the vertebral artery or of the initial segment of the basilar artery. In five cases the AA. studied the vertebro-basilar circulation and in four patients they have actually found pathological elongations of the great vessels. In 18 other cases it was only possible to assume the presence of analogous alterations. The AA. suggest a study of the vertebro-basilar circulation in all the patients with "essential hemifacial spasm".

Adult↗
PubMed2001

Classification, assessment, and management of childhood ptosis.

Ptosis in childhood may both impair normal visual development and be cosmetically disfiguring. Ptosis may sometimes be a component part of a more extensive disorder involving the extraocular muscles, facial structures, or nervous system. Each patient must receive a thorough ocular examination, as well as careful assessment of the ptosis itself. Optimum outcome following surgical repair depends on the correct choice of operation for the specific type of ptosis and degree of levator muscle function.

Blepharoptosis↗
PubMed1985

Monitoring of facial nerve function during removal of acoustic tumor.

A method for locating the facial nerve during removal of medium to large acoustic tumors is described. The method consists of stimulating the tumor mass with short rectangular pulses of monopolar, constant voltage and then presenting the electrical responses from the facial muscles through a loudspeaker. In this way, regions of the tumor where there is no nerve present can be identified and large parts of the tumor removed safely and quickly. Monitoring spontaneous electromyogram (EMG) activity was used to reduce the risk of damaging the facial nerve when parts of medium to large acoustic tumors adhering to the nerve were removed from 20 patients operated upon consecutively by the same surgeon. Only 1 patient lost facial function with the nerve intact anatomically. Nine patients had normal function immediately after the operation and had no detectable weakness upon discharge 6-8 days later, 4 patients had weakness which was resolved, and 6 patients had partial loss of facial function at the time of discharge. The same method may be used to identify the facial nerve during other operations in which this structure is at risk.

Cranial Nerve Neoplasms↗
PubMed2006

Language-induced epilepsy, acquired stuttering, and idiopathic generalized epilepsy: phenotypic study of one family.

PURPOSE: Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language-induced epilepsy. METHODS: Nine members on three generations were studied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non-language-related tasks. RESULTS: Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features. CONCLUSIONS: This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE.

Adolescent↗
PubMed2001

[A case with trigeminal herpes zoster manifesting a long lesion of the spinal trigeminal nucleus and tract on MR T2-weighted image].

We reported a 53-year-old man with the right trigeminal herpes zoster with preceding neuralgia (preherpetic neuralgia) in the right upper cervical nerve area. He developed dysesthesia and scapular pain in the right second cervical nerve area. 5 days later, herpes zoster emerged in the area of the right maxillary division of trigeminal nerve. Furthermore, he developed paralysis on the right facial muscle on the 12th day after the onset of scapular pain. Neurological examination revealed decrease in superficial sensation accompanied by pain and dysesthesia in the areas innervated by the right maxillary division of trigeminal nerve and the right second cervical nerve, and the right peripheral facial nerve palsy. Any rash was not observed in the right second cervical nerve area throughout the course. The cerebrospinal fluid showed a mild mononuclear pleocytosis. The antibody titer for varicella zoster virus (VZV) was elevated in both cerebrospinal fluid and blood serum. T2-weighted magnetic resonance (MR) image revealed a continuously long high-signal lesion corresponding to the right spinal trigeminal nucleus and tract, extending from the lower pons to the second cervical segment of the spinal cord. This lesion could have resulted from a centripetal migration of VZV from the Gasser ganglion to the spinal trigeminal nucleus and tract, which was probably related to the preherpetic neuralgia in the upper cervical nerve area without rash.

Antibodies, Viral↗
PubMed1982

Technical and instrumental improvements in the surgical treatment of acoustic neurinomas.

Microsurgical removal of large acoustic neurinomas, more than 3 cm in diameter, has been performed by the lateral suboccipital transmeatal approach with the patient in the lateral position in 68 cases in the past 5 years. sixty-two patients (92%) returned to their former occupations and two died: one in the immediate postoperative period and the other 16 months later. The postoperative follow-up examination showed satisfactory facial nerve function in 70% of the patients. Hearing was preserved in six of 14 patients who had preoperatively retained their auditory function. The authors emphasize the usefulness of bipolar forceps for intraoperative stimulation of the facial nerve and for facial muscle monitoring. The value of the four-pronged hook and the multipurpose head-frame in this procedure is also discussed.

Adolescent↗
PubMed1994

Emotional imagery and physical anhedonia.

A previous study by Fitzgibbons and Simons (1992) confirmed that subjects identified as anhedonic consistently report a reduced experience of pleasure when confronted with hedonic stimuli (i.e., color slides). Evidence that this emotion-processing deficit extended from the verbal report to the physiological and behavioral domains was mixed but suggested that poor imagery might be associated with the reduced capacity to experience pleasure. The present experiment was designed to test this hypothesis more directly. Anhedonic and normal control subjects were presented scripts, which they were to imagine as vividly as possible. During both script presentation and imagery, heart rate, skin conductance, and facial muscle activity were recorded. Verbal reports of emotional experience were obtained subsequent to each image. The physiological data, as well as an imagery questionnaire, were consistent with the poor imagery hypothesis and also with data from other laboratories (Lang, 1984), which suggests that imagery may be a fundamental aspect of emotion processing in humans.

Adult↗