1/57. Wall-eyed bilateral internuclear ophthalmoplegia in central nervous system cryptococcosis.Only one case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) has been described in central nervous system cryptococcosis. The disorder was initially unilateral, then became bilateral with skew deviation and vertical upgaze deficit. We report a case of WEBINO in central nervous system cryptococcosis in a patient with acquired immune deficiency syndrome. magnetic resonance imaging revealed high signal on T2 images in the right midbrain, left frontal vertex, left splenium, and cerebellum. With treatment, the internuclear ophthalmoplegia improved; however, the convergence insufficiency remained. Disruption of input from cortical supranuclear locations or the region of the rostral interstitial nucleus of the medial longitudinal fasciculus has been proposed as a mechanism in the absence of convergence. This correlates in our patient with the lesions seen on magnetic resonance images.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
2/57. Isolated superior oblique palsies with brainstem lesions.Isolated unilateral superior oblique palsies resulting from brainstem lesions occurred in three patients. MRI documented contralateral tegmental lesions of the trochlear nucleus and adjacent intraaxial trochlear nerve. Lacunar infarct was the cause in two patients and a small hemorrhage in a third.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
3/57. 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) |
4/57. Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration.A 58-year-old Chamorro female patient, who died in 1993, was examined clinicopathologically. At the age of 51, she suffered from hemiparkinsonism, then bradykinesia, rigidity without tremor, and dementia. Extrapyramidal symptoms developed, and at the age of 57, vertical gaze palsy was noted. The clinical diagnosis was parkinsonism-dementia complex (PDC) with vertical gaze palsy. The brain showed atrophy in the frontal and temporal lobes, and the atrophy was accentuated in the dentate gyrus, Ammon's horn and parahippocampal gyrus. The basal ganglia, thalamus and midbrain were moderately atrophic. The substantia nigra and locus ceruleus were completely depigmented. Numerous neurofibrillary tangles (NFTs) were seen in the subiculum and amygdaloid nucleus. Many NFTs were evident in the parahippocampal gyrus, lateral occipitotemporal gyrus, insula, Sommer sector, basal nucleus of meynert, lateral nucleus of the thalamus, subthalamic nucleus and brain stem, and several were observed in the globus pallidus and hypothalamus. The Sommer sector, substantia nigra, locus ceruleus and basal nucleus of meynert showed severe loss of neurons, and a moderate loss of neurons was exhibited by the globus pallidus. These findings were apparently consistent with those associated with PDC. However, in this patient, severe neuronal loss was seen in the subthalamic nucleus and lateral nucleus of the thalamus, and grumose degeneration, which has not previously been reported in PDC, was seen in the dentate nucleus. In addition, many tufted astrocytes, which have been reported to occur in progressive supranuclear palsy (PSP) and postencephalitic parkinsonism, but scarcely observed in PDC, were present. Furthermore, astrocytic plaques, which have been considered as a specific finding of corticobasal degeneration (CBD), were observed in the cerebral cortex. On the other hand, granular hazy astrocytic inclusions, previously reported to occur in PDC, were not seen. Chromatolytic neurons were not observed. The question thus arises as to whether it is appropriate to consider this patient as having suffered from a combination of PDC, PSP and CBD. From the view points of absence of granular hazy astrocytic inclusions and chromatolytic neurons, and of tufted astrocytes in the neostriatum, it is conceivable that this patient is a case of a new disease entity.- - - - - - - - - - ranking = 8keywords = nucleus (Clic here for more details about this article) |
5/57. Isolated inferior rectus palsy as a result of paramedian thalamopeduncular infarction.The authors present the cases of two patients with isolated inferior rectus muscle paresis presumed to be caused by paramedian thalamopeduncular infarction that involved supranuclear descending pathways, just before the inferior rectus subnucleus in one patient, and just before subnucleus or fascicular fibers in the other patient. Both patients had no other associated neurologic dysfunction. The lesions that cause isolated inferior rectus palsy in these patients are documented by magnetic resonance findings. Although vascular ischemic lesions as the cause of isolated inferior rectus palsy were reported previously, to the authors' knowledge, it has not been demonstrated radiologically.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
6/57. Congenital fibrosis of the extraocular muscles associated with cortical dysplasia and maldevelopment of the basal ganglia.BACKGROUND: Congenital fibrosis of the extraocular muscles (CFEOM) is a rare condition that has been traditionally regarded as a primary eye muscle disease. Recent studies, however, suggest that CFEOM may be the result of a primary neuropathy with secondary myopathic changes. PURPOSE: To describe a previously unrecognized association between congenital fibrosis of the extraocular muscles and structural abnormalities of the brain. DESIGN: Small case series. methods: Detailed clinical examinations and neuroradiologic studies were performed on the three affected family members. In addition, genetic analysis of the family was performed. RESULTS: The three affected family members, mother and two children, have the ocular features of 'classic' congenital fibrosis of the extraocular muscles. All showed dilation of the left lateral ventricle secondary to hypoplasia of the body and tail of the ipsilateral caudate nucleus. There was fusion of an enlarged caudate nucleus head with the underlying putamen. Both children showed widespread bilateral cortical dysplasia. Genetic analysis of the family was inconclusive but consistent with linkage to the CFEOM1 locus on chromosome 12. Chromosomal analysis of the affected individuals did not show evidence of a deletion of chromosome 12 and haplotype analysis was not suggestive of a microdeletion. CONCLUSIONS: Cerebral cortical and basal ganglia maldevelopment can be found in individuals with CFEOM. This suggests that neuroimaging should be considered in the initial diagnostic evaluation of these patients, particularly if there is developmental delay.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
7/57. Isolated acquired unilateral horizontal gaze paresis from a putative lesion of the abducens nucleus.In three patients, acute horizontal gaze pareses developed that could not be overcome with the oculocephalic maneuver, indicating a putative lesion of the ipsilateral abducens nerve nucleus. None of the patients had a facial nerve paresis or evidence of a trigeminal sensory neuropathy. Although most lesions that affect the abducens nerve nucleus also damage the ipsilateral fasciculus of the facial nerve, small lesions in this region can produce an isolated horizontal gaze paresis.- - - - - - - - - - ranking = 6keywords = nucleus (Clic here for more details about this article) |
8/57. Supranuclear paralysis of monocular elevation.A man with bronchogenic carcinoma lost the ability to elevate his left eye voluntarily. His eyes were level in the primary position and the Bell phenomenon was normal, indicating that the ophthalmoplegia was caused by a supranuclear lesion. Other clinical and radiologic evedence indicated that there was a lesion in the rostral midbrain. A metastatic tumor, found in the right pretectum at autopsy, probably produced the ophthalmoplegia by interrupting axons destined for the superior rectus portion of the homolateral oculomotor nucleus and the interior oblique portion of the contralateral oculomotor nucleus.- - - - - - - - - - ranking = 2keywords = nucleus (Clic here for more details about this article) |
9/57. Vertical ophthalmoplegia in a demented patient with striatopallidodentate calcification.A case is presented here of a 73-year-old man who showed signs of dementia, supranuclear vertical ophthalmoplegia, pseudobulbar palsy, axial dystonia, mild rigidity, and parkinsonian gait. Computed tomography of the head revealed symmetrical calcification in the striatum, globus pallidus and dentate nucleus to an extraordinary degree. No metabolic conditions were observed that could explain the intracranial calcification. Oral administration of levodopa improved the patient's motor symptoms to some extent. ophthalmoplegia, parkinsonism and dementia combined are typically seen in patients with progressive supranuclear palsy. However, the present case and a few others that have been reported would seem to indicate that these unique symptoms might also be found in patients with intracranial calcification.- - - - - - - - - - ranking = 1keywords = nucleus (Clic here for more details about this article) |
10/57. A case of bilateral oculomotor nuclear palsy.To the best of our knowledge, isolated bilateral oculomotor nuclear palsy has not yet been reported in the literature, while bilateral oculomotor nuclear palsy with more widespread rostral brainstem infarction has often been reported. We present a patient having 'top of the basilar syndrome' with midbrain infarction selectively involving the bilateral oculomotor nucleus. A 61-year-old woman with two episodes of vertebrobasilar infarction presented with sudden onset of bilateral ptosis. Examination revealed pronounced bilateral ptosis. In the primary position, fixation of either eye produced an approximately 50 prism diopter exotropia. Adduction of the right eye was restricted to the midline. There was moderately decreased adduction of the left eye, severe limitation of depression, and moderately decreased elevation of both eyes. Abduction of both eyes was normal. The pupils were equal, round, and reactive to light. Bilateral ptosis is suggestive of oculomotor nuclear palsy. On the basis of clinical findings alone, we could not establish whether the precise location of the lesion was all the subdivisions of the oculomotor nucleus except the Edinger-Westphal nucleus or the central caudal nucleus and bilateral fascicles. However, because axial MRI showed a small midbrain infarct in the oculomotor nucleus region, we concluded that she had an isolated, pupil-sparing, bilateral oculomotor nuclear palsy caused by midbrain infarct.- - - - - - - - - - ranking = 5keywords = nucleus (Clic here for more details about this article) |
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