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PubMed1999

[A family with probable autosomal dominant bulbospinal muscular atrophy with gynecomastia].

We reported a 52-year-old man and his family with bulbospinal muscle atrophy (BSMA) and gynecomastia. The propositus presented with the clinical picture of late onset progressive bulbospinal muscular atrophy including postural tremor, general hyporeflexia, mild maturity onset diabetes, gynecomastia and sexual impotence. One of his brother and his two sons had gynecomastia. His elder son had ocular movement abnormality, associated movement of facial muscle and finger tremor. One of his brothers showed tongue fasciculation without gynecomastia. None of members examined had abnormal expansion of CAG repeats in the androgen receptor gene. We speculate that this family has a new clinical entity characterized by bulbospinal muscular atrophy with an autosomal dominant inheritance.

Aged↗
PubMed2006

Ipsilateral facial sensory and motor responses to basal fronto-temporal cortical stimulation: evidence suggesting direct activation of cranial nerves.

To clarify the generator mechanism of sensory and motor facial responses ipsilateral to electrical stimulation of the inferior fronto-temporal cortex in epilepsy patients. Out of 30 patients who have been evaluated with chronically implanted subdural electrodes for medically intractable partial seizure or brain tumor involving the basal frontal or temporal cortex, 4 patients (age ranging 24-57 years) showed sensory and motor responses in the ipsilateral face to high frequency electrical cortical stimulation of the inferior fronto-temporal cortex. We investigated motor evoked potentials (MEPs) in the facial muscle by single pulse stimulation in 2 out of 4 patients. Three patients showed both sensory symptoms and muscle contraction in the ipsilateral lower face when the orbitofrontal or basal temporal cortex was stimulated with 50 Hz electric current. One patient had only sensory symptoms in the lower face when ipsilateral basal temporal area was stimulated. MEPs at the left orbicularis oris muscle were constantly elicited with the onset latency of 7 ms throughout the stimulus rate of 2-30 Hz in 1 patient out of 2 patients was tested. In another patient, MEP onset latency was 3.0 ms with 11 Hz stimulation. With electrical stimulation of the basal fronto-temporal cortex, the ipsilateral facial twitch might occur through either the direct activation of the facial nerve by the current spread in the middle cranial fossa or through the mechanism similar to blink reflex.

Adult↗
PubMed1996

The relationship of vibrissal motor cortex unit activity to whisking in the awake rat.

Rats actively sweep their whiskers back and forth to locate and palpate objects within their immediate environment. Microstimulation studies in anesthetized rats have demonstrated the presence of a large vibrissal motor representation in agranular cortex. However, the manner in which motor cortex neurons contribute to whisking behavior in the awake animal is unknown. This study represents an initial investigation of the relationship between the activity of task-related neurons in vibrissal motor cortex and the electromyographic (EMG) activity of the deep vibrissal pad muscles in the awake, freely whisking rat. Each animal was gently held in an experimenter's hands while the animal whisked the air. A spring-loaded, metal microelectrode mounted in a removable, miniature microdrive provided stabile recordings of motor cortex unit activity. Fine-wire electrodes implanted in the mystacial pad simultaneously recorded facial muscle activity. Results showed that the discharge of task-related neurons was correlated with changing levels of muscle output. Unit discharge was related in a tonic or phasic-tonic fashion to EMG activity. No units were found to discharge rhythmically in a 1:1 fashion with the periodicity of the whisking pattern. These findings support a role for vibrissal motor cortex in the initiation and modulation of the overall level of mystacial pad muscular output, but not in the generation of bursts of EMG activity responsible for individual whisking sweeps.

Animals↗
PubMed1988

Monitoring Seizure Duration During Electroconvulsive Therapy.

Three alternative monitoring methods for assessing the duration of seizures during electroconvulsive therapy were studied. The mean integrated amplitude of the electroencephalogram, facial muscle electromyogram, and "cuff method" were compared with the reference single-channel unprocessed electroencephalogram in 78 sessions with 17 patients. The measures of seizure duration differed significantly (p < 0.001). The mean integrated electroence phalographic amplitude differences were small and an artifact of the sampling procedure. Larger, but clinically unimportant, discrepancies were obtained with the facial electromyogram amplitude. In contrast, there were marked differences between the electroencephalogram and the "cuff method," which suggest that the latter technique may be of limited usefulness.

Journal Article↗
PubMedDate not supplied

Cross-face nerve grafting in facial paralysis.

Facial paralysis causes a most disturbing deformity with functional, emotional and social consequences for the people afflicted. Since a few years most promising advances in the treatment of facial palsy have been developed and new ways of surgical management have been introduced. Cross-face nerve grafting connects the non-paralysed facial nerve branches to the paralyzed ones and by these means realizes a reanimation of the paralyzed side of the face. Fourteen cases have been operated according to this technique. The results of 10 cases with a sufficient follow-up are analysed. Good and satisfactory results were obtained in 8 cases. Two cases were considered bad with poor recovery of facial muscle activity. The overall results of cross-face nerve grafting are very gratifying. The technique should always be attempted in first instance. Other substitutional methods for further improvement should be reserved for later.

Adult↗
PubMed1994

Neuropathology of infant with Pena-Shokeir I syndrome.

In an infant with clinical features of Pena-Shokeir I syndrome, who survived for 182 days, neuropathologic examination revealed little myelination in peripheral nerves with group atrophy of muscle fibers, dysplasia of inferior olivary and dentate nuclei, and leptomeningeal heterotopia. Congenital peripheral neuropathy associated with minor brain anomalies is characteristic in this patient, and may cause absence of fetal movements leading to ankylosis of multiple joints, absence of breathing in association with pulmonary hypoplasia, absence of swallowing causing polyhydramnios, and absence of movements of facial muscles causing craniofacial anomalies.

Abnormalities, Multiple↗
PubMed1992

The directions of static skin tensions in the face: their roles in facial incisions.

The circular excisions of facial skin lesions were performed in 202 sites on 126 patients. The shapes of the resultant defects were observed to identify the directions of skin tensions in a static state. The round shapes remained in the forehead, temple, and nose. In the other areas of the face, the wounds were stretched in certain directions and became elliptical. The orientations of the facial static tensions were identical to the wrinkle lines in many facial areas. It is suggested that the wrinkle lines show the optimal directions of selective incisions in most facial areas except for the medial canthus and upper and lower lips.

Adolescent↗
PubMed1975

[Abnormalities of recovery of the facial nerve].

This paper presents 235 cases of facial palsy. These cases are part of a group of 455 cases and they were chosen because their clinical study and evolution were well documented. Abnormalities of facial nerve recovery are described and the importance of each one with reference to the group is determined. Two cases of particular interest are described, and comments are offered on the incidence of abnormality of recovery according to the type of lesion and to the importance of the electrical dysfunction.

Age Factors↗
PubMedDate not supplied

[Results of 13 hypoglosso-facial anastomoses].

BACKGROUND AND PURPOSE: Facial nerve paralysis significantly decreases the quality of life in patients after skull base tumors surgery. There are different options for surgical repair of the nerve. One of the most effective methods is the anastomosis of the distal trunk of the facial nerve with the proximal trunk of the hypoglossal nerve or with hypoglossal loop. MATERIAL AND METHODS: Thirteen hypoglosso-facial anastomoses had been performed in our department between 1999 and 2003. In all patients the facial nerve paralysis was due to the surgery in its proximity. In one case the patient suffered from petroclival chordoma, in one case ameloblastoma, one was with petroclival meningioma and the rest of cases were after vestibular schwannoma. The follow-up period lasted from 6 months to three years. The final result was expressed in House-Brackmann scale (HB) describing facial muscle strength. RESULTS: In two cases there was no improvement (HB 6), in two cases barely perceptible motion (HB 5), in five obvious weekness with disfiguring assymetry (HB 4) and in four obvious but not disfiguring dysfunction (HB 3). There was only one postoperative complication haematoma at the place of anastomosis requiring evacuation. CONCLUSION: Hypoglosso-facial anastomosis remains one of the most important techniques of the reanimation of the paralysed face. Its efficacy is limited, but it is a relatively safe procedure.

Anastomosis, Surgical↗
PubMed1981

Hemifacial spasm caused by cholesteatoma.

Hemifacial spasm is a rare disease. Usually a specific etiologic factor is not found and nonspecific treatments are used. A case of hemifacial spasm secondary to a middle ear cholesteatoma is presented. This origin should be considered in any patient who has hemifacial spasm.

Aged↗
PubMedDate not supplied

Reconstruction of total lower lip and chin defects using the composite radial forearm--palmaris longus tendon free flap.

BACKGROUND: Functional and aesthetic restoration of total lip and chin defects can be achieved using the composite radial forearm-palmaris longus tendon free flap. OBJECTIVE: To present the technique we use and our experience with this form of reconstruction in 10 consecutive patients with total lip and chin defects who were surgically treated between 1992 and 1998. METHODS: The palmaris longus tendon acting as a sling over which the flap is draped is responsible for long-term maintenance of vertical lip height and lip support. The factors responsible for this are the long-term maintenance of vertical lip height and lip support and the transfer of facial muscle activity to the neolip. RESULTS: All patients were satisfied with their final reconstructive result. Oral competence for deglutition and speech was achieved in all patients in our case series, with no incidence of drooling. CONCLUSION: We recommend the use of the composite radial forearm-palmaris longus tendon free flap for this type of reconstructive surgery. Arch Facial Plast Surg. 2000;2:53-56

Aged↗