1/18. Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/18. One and one-half syndrome with supranuclear facial weakness: magnetic resonance imaging localization.OBJECTIVE: To provide clinicoanatomical correlation for a small pontine tegmental ischemic stroke producing the one and one-half syndrome associated with supranuclear facial weakness. DESIGN: Case report. SETTING: Tertiary care center. PATIENT: A 70-year-old man developed left-sided facial weakness sparing the forehead, a left internuclear ophthalmoplegia, and a complete left horizontal gaze palsy immediately after percutaneous transluminal coronary angioplasty. magnetic resonance imaging demonstrated a small lesion in the left paramedian aspect of the dorsal pontine tegmentum. MAIN OUTCOME AND RESULTS: Electromyographic findings were consistent with supranuclear facial involvement. The patient had nearly complete recovery after 1 year. CONCLUSIONS: To our knowledge, this is the first report of supranuclear facial weakness in association with the one and one-half syndrome. The location of the lesion provides evidence of the existence of corticofugal fibers that extend to the facial nucleus in the dorsal paramedian pontine tegmentum.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
3/18. multiple sclerosis with caudate lesions on MRI.A 31-year-old woman displayed sleepiness and impairment of recent memory. T2-weighted MRI revealed high signal intensity lesions in the bilateral basal ganglia, thalamus, and brainstem. Although remission was achieved with corticosteroid therapy, she again displayed memory dysfunction and emotional disturbance one year later, at which time MRI disclosed new lesions in the right caudate nucleus and left frontal white matter. Corticosteroid therapy lead to improvement, and she suffered no recurrence on maintenance steroid therapy. These findings suggest that caudate lesions do occur in multiple sclerosis, the manifestations of which can be abulia and memory dysfunction, as in the present case.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
4/18. blepharospasm hemifacial spasm and tremors possibly due to isolated caudate nucleus lesions.Isolated caudate nucleus lesions have only rarely been documented to cause focal extrapyramidal dysfunction. Two cases with possible infarcts in the head of left caudate nucleus presenting with contralateral tremors and blepharospasm with hemifacial spasm are reported. The possible mechanisms for such a presentation are discussed.- - - - - - - - - - ranking = 6keywords = nucleus (Clic here for more details about this article) |
5/18. Automatic-voluntary dissociation: an unusual facial paresis in a patient with probable multiple sclerosis.A patient with multiple sclerosis is described who presented with a unilateral loss of voluntary function of his lower face muscles. However, in an emotional situation, there was strong involuntary innervation of these muscles: automatic-voluntary dissociation. The subcortical afferents to the facial motor nucleus are discussed. It is hypothesized that cortical disinhibition of midbrain nuclei underlies the accentuated involuntary innervation.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
6/18. Acute peripheral facial palsy simulating Bell's palsy in a case of probable multiple sclerosis with a clinically correlated transient pontine lesion on magnetic resonance imaging.A transient pontine lesion was demonstrated in a young adult male who had a complete acute peripheral facial nerve palsy due probably to multiple sclerosis. In the acute stage of the palsy. T2-weighted magnetic resonance imaging (MRI) revealed a high signal intensity in the ipsilateral pons in the region of the nucleus and pontine part of the facial nerve. The patient recovered completely 5 weeks after the onset of the palsy, and at this stage, the lesion in the brainstem was no longer demonstrable on MRI. The onset and course of the disease resembled the idiopathic form of facial palsy (Bell's palsy): the present findings along with recent MRI and topodiagnostic studies may indicate that in some cases of Bell's palsy the primary lesion is located centrally. By improved MRI techniques and intravenous contrast agents it may be possible to visualize and follow the disease process and ascertain more of the pathogenesis of Bell's palsy.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
7/18. hemifacial spasm. Occurrence in multiple sclerosis.We present six patients with hemifacial spasm and multiple sclerosis. To our knowledge, this association has not been described previously in the North American literature. magnetic resonance imaging was obtained in all the patients and plaques consistent with multiple sclerosis were identified. In two patients the plaques were seen in the area of the facial nucleus on the involved side. We suggest that hemifacial spasm can be a manifestation of multiple sclerosis. These cases illustrate the utility of magnetic resonance imaging in the investigation of hemifacial spasm. Our findings also support a central (nuclear) origin in multiple sclerosis associated with hemifacial spasm.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
8/18. Middle ear mucosa in Ramsay Hunt syndrome.We report two patients with Ramsay Hunt syndrome, together with histopathologic findings of the middle ear mucosa near the facial canal. An attempt was made to find specific antigens of varicella zoster virus (VZV) and herpes simplex virus (HSV) by an immunofluorescence method. Histopathologic examination revealed inflammation of the middle ear mucosa. Specific VZV antigens were demonstrated in the cytoplasm and nucleus of elliptically shaped cells and round cells, but no specific antigens of HSV were found. The findings suggest a pathogenetic relationship between VZV infection and inflammatory changes in the middle ear mucosa.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
9/18. Cranial nerve palsy as a delayed complication of attempted infanticide by insertion of a stylet through the fontanel. Case report.A child suffered a sixth and seventh cranial nerve palsy due to intracerebral insertion of a stylet. The stylet was introduced through the anterior fontanel, most probably in an attempt at infanticide. The migration of the stylet through the brain was monitored because the child was first examined 6 years earlier. At operation the cranial part of the stylet lay in the fourth ventricle, compressing the facial nerve as well as the nucleus of the abducens nerve. The lower part of the stylet had reached the C-5 level.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
10/18. Pontine supranuclear facial palsy.Two patients presented with a unilateral supranuclear facial palsy. Additional dysarthria was attributed to the pontine origin documented by magnetic resonance imaging on the contralateral side. The pontine disorder also was indicated by an isolated delay of the blink reflex R1 component or of the masseter reflex. We attribute the facial palsy to a lesion of a supranuclear fiber bundle supplying the facial nucleus. The location of the lesions favors these fibers taking a separate course from the main pyramidal tract at the mid- to upper pontine level.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
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