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Organization of the facial nerve nucleus in the cat.

The location of efferent neurons in the brain stem of newborn kittens supplying the facial muscles was studied by means of horseradish peroxidase (HRP) used as a tracer of retrograde protein transport. Three to 15 microliters containing 0.3 to 1.5 mg of HRP was injected into the orbicularis oris and orbicularis oculi muscles of six kittens and in the seventh kitten, the ventral ramus of the facial nerve was transected and HRP was instilled on the cut end of the nerve. After a survival time of 24 hours, the animals were anesthetized, perfused by intracardic perfusion technique, and then they were fixed and the brain stems sectioned. Stains for peroxidase demonstrated the labelled neurons in the lateral division of facial nuclei in the ipsilateral side. The orbicularis oculi muscles were represented in the rostral pole of the lateral division of the facial nucleus dorsally while the orbicularis oris muscles were represented in the caudal pole of the lateral division of the facial nucleus ventrally. No labelled neurons were found in the nuclei of the Vth or any other brain stem nuclei. According to my knowledge, this method of localizing the neurons supplying the facial muscles has not been reported previously.

Animals↗

Hemispheric control of speech expression in aphasia. A mouth asymmetry study.

Clinical observation of aphasic patients often shows a dissociation between impaired propositional speech and preserved automatic speech. The question of differing hemispheric control between these speech tasks was examined by measuring asymmetry in facial muscle activity. In spite of right-sided facial palsy, greater opening of the right side of the mouth was seen for spontaneous speech, repetition, and word list generation, suggesting that the damaged left hemisphere still controlled motor output in these propositional tasks. However, greater opening of the left side of the mouth for serial speech and singing indicated at least a relatively greater role of the right hemisphere in these "automatic" tasks.

Adult↗

Nerve fiber analysis for the lingual nerve of the human adult subjects.

The neuro-motor control of the human tongue musculature had not been investigated in detail. This study identified first that the lingual nerve should play the neuro-motor control of some lingual muscles. Six en bloc samples (12 sides), including the tissues from the skull base to the hyoid bone, and three whole tongues were obtained from adult human cadavers. The former samples were used for the study of nerve fiber analysis of the lingual nerve with the aid of binocular stereomicroscope, and the latter samples were used for histological study by serial section method. On nerve fiber analysis of the lingual nerve from the trigeminal ganglion to the tongue musculature, we found that the motor- root of the trigeminal nerve gave off its supply to the lingual nerve and traveled into the lingual nerve, and branched to the superior and the inferior longitudinal muscles. On histological study, it was revealed that in the anterior part of the tongue the superior and the inferior longitudinal muscles surrounded the other lingual musculature and combined with the sub-mucosal connective tissues closely like the cutaneous muscle, for example, the facial muscles. The lingual nerve entered the inner side of the space between the genioglossus and the inferior longitudinal muscles with the lingual artery. These findings suggested that the superior and the inferior longitudinal muscles should be innervated by the motor fibers traveled into the lingual nerve from the motor root of the trigeminal nerve, and do not originate from the myotome originating in occipital somites but branchial muscles.

Adult↗

[Phylogenetic aspects of lip function].

Lips can be considered a phylogenic summary of nasolabial and facial muscle evolution. They represent the central point of facial morphogenesis. The progressive development of oral and facial functions is an elementary human necessity for feeding, suction, oral competence, swallowing, language, and mimics. Each discontinuity causes functional and anatomic disorders. Any damage to the lips creates facial unbalance. Precise knowledge of lip function is indispensable for optimal reconstruction.

Adolescent↗

Preservation of facial function during removal of acoustic neuromas. Use of monopolar constant-voltage stimulation and EMG.

The authors describe a modification in the way the facial nerve is stimulated electrically during operations to remove medium and large-sized (greater than 2 cm) acoustic tumors. This consists of monopolar stimulation with low internal impedance. Proper use of this modified stimulation technique together with acoustic monitoring of the electromyographic responses of facial muscles helps to preserve facial nerve function in patients undergoing these operations, and also decreases the duration of the operation.

Electric Stimulation↗

[An old man presenting with fluctuating bilateral multiple cranial nerve palsies and positive test for perinuclear antineutrophil cytoplasmic antibody].

A 70-year-old man admitted to a local hospital because of facial muscle weakness, tinnitus and facial pain in left side, was then given corticosteroid with a tentative diagnosis of Bell's palsy and his symptoms gradually improved. Since these symptoms recurred six months later, he was referred to our neurological service. As his brain CT revealed diffuse thickening and enhancement of the dura mater, he was thought to have hypertrophic pachymeningitis (HP). Intravenous antibiotics were started for aspiration pneumonia and his neurological symptoms gradually improved. HP caused by bacterial infection was thus likely and antibiotics, rifampicin and metronidazole, were administered orally as an outpatient. However, one month later, these symptoms were worsened with headache and double vision. He was then rehospitalized. MR imagings of the head with gadolinium disclosed diffuse meningeal thickening and enhancement, especially of the left-sided cerebellar tentorium. Erythrocyte sedimentation rate and C-reactive protein were moderately elevated. Serum angiotensin converting enzyme was within normal range. The test for cytoplasmic antineutrohil cytoplasmic antibody (ANCA) in the serum was negative, however, that for perinuclear ANCA was positive at a titer of 99 EU. Lumbar puncture showed normal findings and negative culture results for bacteria, fungi or mycobacteria. Dural biopsy specimens showed non-specific granulomatous inflammation of the dura with epithelioid histiocytes and Langerhans type multinuclear giant cells with caseous necrosis, however, with no presence of fungi or tubercle bacilli. After the oral administration of cyclophosphamide (100 mg, daily) and prednisolone (40 mg, daily), his neurological symptoms and laboratory findings have been gradually improved and he is well one year after discharge. This case together with previous reports suggests that ANCA positive HP without evidence of other organ involvements may belong to the limited form Wegener's granulomatosis. In the literatures of idiopathic HP, the treatment effect with corticosteroid alone is initially favorable, but transient. On the other hand, using the combined therapy of cyclophosphamide and prednisolone, the remission has been achieved in more than 90% of patients with WG. These data suggest that P-ANCA positive HP should be treated with a combination of corticosteroid and cyclophosphamide.

Aged↗

[Adult form of acid maltase deficiency presenting with pattern of muscle weakness resembling facioscapulohumeral dystrophy].

We report a 61-year-old female patient with adult form of acid maltase deficiency showing many clinical similarities to facioscapulohumeral muscular dystrophy (FSHD). She developed difficulty in raising her right arm in her thirties followed by leg weakness. She had the typical features of FSHD, including bilateral scapular winging sparing the levator scapulae and deltoid muscles, and Beevor's sign. Muscle involvement was asymmetrical. Facial muscles were not affected, while the neck flexor was weak. No muscle shortening or joint contracture was observed. On muscle CT, the lumbar paravertebral, gluteal and thigh muscles were replaced by adipose tissue, while the rectus femoris, gracilis, and sartorius muscles were spared. Serum creatine kinase level was not elevated. Muscle biopsy showed some vacuoles and many granular inclusions with high acid phosphatase activity. Acid maltase activity was very low in both muscle and cultured skin fibroblasts. Absence of shortening of affected muscles appears to be the characteristic finding suggesting metabolic myopathies with minimal fibrosis, rather than FSHD.

Diagnosis, Differential↗