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Cases reported "Ocular Motility Disorders"
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1/155. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa.A tonic pupil may occur in isolation or as part of a systemic disorder. We report a patient who developed tonic pupils and an abduction deficit in the setting of polyarteritis nodosa. The combination of a tonic pupil and an abduction deficit should suggest the possibility of a vasculopathic disorder, because the ciliary ganglion and lateral rectus muscle are both supplied by the lateral muscular artery. Widespread small artery and arteriolar narrowing and occlusion are the hallmarks of polyarteritis nodosa. Unusual ischemic syndromes may occur, such as this rare combination of neuro-ophthalmic signs, by involvement of both the nutrient artery and its collaterals. We are unaware of other reports of neuropathic tonic pupils in association with polyarteritis nodosa.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/155. magnetic resonance imaging of acquired Brown syndrome in a patient with psoriasis.PURPOSE: To report the occurrence of acquired Brown syndrome and associated magnetic resonance imaging findings in a patient with psoriasis. methods: A 42-year-old woman with a history of psoriasis developed pain, double vision, and limited elevation of her left eye in adduction. An orbital magnetic resonance image with gadolinium enhancement was obtained. RESULTS: Orbital magnetic resonance image disclosed abnormal enhancement of the left trochlea/tendon complex. The patient's symptoms resolved with corticosteroid therapy. CONCLUSIONS: Acquired Brown syndrome may be associated with psoriasis. The inflammation of the trochlea/tendon complex that can cause acquired Brown syndrome can be demonstrated on magnetic resonance image.- - - - - - - - - - ranking = 7keywords = syndrome (Clic here for more details about this article) |
3/155. Posterior leukoencephalopathy syndrome may not be reversible.The association of an acute reversible encephalopathy with transient occipital lobe abnormalities on imaging studies is well known. This condition has been called reversible posterior leukoencephalopathy syndrome. The clinical presentation usually includes seizures, headache, altered mental status, and blindness, often associated with hypertension and immunosuppressants. The authors discuss a two-year-old male with down syndrome who presented 2 months after allogeneic bone marrow transplantation with severe oculogyric crisis, without other complaints. The patient was being treated for hypertension and was receiving cyclosporine for prophylaxis of graft-vs-host disease. A computed tomography scan of the head revealed marked bilateral lucencies mainly involving the white matter of the occipital lobes, with a few foci of punctate hemorrhage. The condition improved when cyclosporine was discontinued, but an area of leukomalacia was identified on follow-up magnetic resonance imaging. To the authors' knowledge, oculogyric crisis as a presentation of reversible posterior leukoencephalopathy has not been previously described. Recognizing this association is important, because patients receiving cyclosporine are often receiving other medications that can potentially cause dystonic eye movements, possibly leading to a delay in diagnosis and treatment, which can result in an irreversible neurologic deficit.- - - - - - - - - - ranking = 6keywords = syndrome (Clic here for more details about this article) |
4/155. Paraneoplastic opsoclonus-myoclonus secondary to malignant melanoma.Opsoclonus-myoclonus is a well-described paraneoplastic syndrome that most often occurs in association with small cell carcinoma of the lung and breast carcinoma. To the best of our knowledge, we describe the first reported case of paraneoplastic opsoclonus-myoclonus occurring is association with malignant melanoma. Antineuronal antibodies were not identified despite repeated testing. No therapeutic benefit was observed following corticosteroid administration.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
5/155. Acquired Pseudo-Brown's syndrome immediately following Ahmed valve glaucoma implant.A 76-year-old woman noted vertical and horizontal diplopia one day following placement of an Ahmed valve in the superonasal quadrant of her left eye. She was unable to elevate her left eye, especially in adduction. She refused implant removal and strabismus surgery alone failed to satisfactorily resolve her problem. On forced duction testing, the implant became wedged between the globe and orbit superonasally. Subsequent repositioning of the valve resulted in resolution of her motility problem. Implant-orbital disproportion can produce a pseudo-Brown's syndrome. Surgeons are encouraged to perform forced duction testing at the time of glaucoma implant placement to detect and prevent this complication.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
6/155. Palatal myoclonus in postinfectious opsoclonus myoclonus syndrome : a case report.An adult male presenting with acute onset opsoclonus, myoclonus and cerebellar ataxia is being reported. Patient had myoclonus involving limbs and palate. There are only a few reported cases associated with palatal myoclonus. Patient showed gradual spontaneous recovery. Possibility of underlying malignancy was excluded by detailed investigations.- - - - - - - - - - ranking = 4keywords = syndrome (Clic here for more details about this article) |
7/155. A case of paroxysmal tonic upgaze of childhood with ataxia.Paroxysmal tonic upgaze of childhood is a rare, distinctive, childhood syndrome that may be associated with ataxia and sometimes strabismus or amblyopia. Neurological examination as well as metabolic studies, electroencephalogram and neuroradiological investigations are normal in these patients. Although it has been considered as an age-related, dopa-sensitive dystonia, the exact pathogenetic mechanism is still unknown. Aggravation of attacks by fatigue, intercurrent infection or vaccination, and possible corticomesencephalic dysmaturation may underlie this abnormality. We report on a sporadic case of paroxysmal tonic upgaze with ataxia in which there was prompt aggravation of symptoms with sleep without response to levodopa treatment. This case suggests a different underlying pathogenetic mechanism from dopaminergic pathways for this syndrome.- - - - - - - - - - ranking = 2keywords = syndrome (Clic here for more details about this article) |
8/155. history of Joubert syndrome and a 30-year follow-up of the original proband.The 1960s were a period of great flowering in the recognition of neurologic disorders in children. The so-called ataxic cerebral palsies were an especially fertile field waiting for clarification. Congenital ataxia coupled with hyperpnea-apnea, abnormal eye movements, and retardation was identified as an autosomal-recessive syndrome eponimically associated with the senior author, Marie Joubert. The disorder, though rare, is increasingly recognized and a lay society dedicated to family support and research has been formed. In preparation for a recent symposium the original proband was re-examined 30 years later and the manifestations in adults clarified. Severe dysarthria was the most striking feature in this man, the hyperpnea-apnea had diminished, and the abnormal eye movements were less striking. Ataxia was still present but not severe. Poor judgment and borderline intelligence rounded out the clinical picture. Modern imaging has clarified, in part, the anatomic basis of this syndrome.- - - - - - - - - - ranking = 6keywords = syndrome (Clic here for more details about this article) |
9/155. Neuropathology of Joubert syndrome.Very little documentation of the neuropathologic changes in Joubert syndrome exists. This paper presents a detailed postmortem neuropathologic study of a clinically and radiographically well-documented case of Joubert syndrome. In addition to aplasia of the cerebellar vermis and fragmentation of the dentate nuclei, there was marked dysplasia of structures at the pontomesencephalic junction and caudal medulla. There was abnormal decussation of the superior cerebellar peduncles and an enlarged iter (rostral 4th ventricle) with elongated tegmental nuclei (including the locus coeruleus). neurons of the basis pontis and reticular formation appeared reduced. Extensive malformations of the medulla included hypoplasia of the inferior olivary nuclei, solitary nuclei and tracts, and the nucleus and spinal tracts of trigeminal nerve (cranial nerve V). Even more striking was dysplasia of the caudal medulla at the cervicomedullary junction, which was characterized by the absence of a posterior median sulcus, neuronal swelling and axonal spheroids in the region of malformed nuclei gracilis and cuneatus, and absence of pyramidal decussation. This study suggests that, in addition to vermal agenesis, Joubert syndrome is characterized by malformation of multiple brainstem structures. The latter could explain certain clinical features of the syndrome, including episodic hyperpnea and oculomotor apraxia.- - - - - - - - - - ranking = 8keywords = syndrome (Clic here for more details about this article) |
10/155. Restriction of elevation in abduction after inferior oblique anteriorization.PURPOSE: Inferior oblique anteriorization is gaining popularity for the treatment of dissociated vertical divergence associated with inferior oblique overaction. This procedure is based on the theory that moving the insertion of the inferior oblique muscle anterior to the equator changes its vector of force from one of elevation to one that opposes elevation. The purpose of this investigation is to describe, investigate the cause, and outline treatment for a complication I observed after inferior oblique anteriorization. This postoperative syndrome consists of a motility pattern that resembles marked residual inferior oblique overaction associated with a Y or V pattern. It is probably caused by a restriction of elevation of the abducting eye causing fixation duress, with a resultant upshoot of the contralateral adducting eye. methods: A retrospective chart review was conducted for all patients on whom I performed bilateral inferior oblique anteriorization for inferior oblique overaction associated with dissociated vertical divergence. patients in whom this postoperative syndrome developed were compared with those in whom it did not with respect to type and extent of surgery. In addition, cases of patients I treated or examined for this complication but whose inferior oblique anteriorization had been performed by other ophthalmologists were also analyzed. RESULTS: I performed bilateral inferior oblique anteriorization in 77 patients. In 29 patients the inferior oblique muscles were placed level with the insertions of the inferior rectus muscles, in 31 patients they were placed 1 mm anterior to the insertions of the inferior rectus muscles, and in 17 patients they were placed 2 mm anterior. The postoperative syndrome described here developed in two of the 77 patients; both had the inferior oblique muscles placed 2 mm anterior to the insertions of the inferior rectus muscle. These were also the only two patients in this series in whom the new insertion of the inferior oblique muscle was spread out laterally at the time of anteriorization. I have seen an additional six patients in whom this syndrome developed after undergoing operations by other ophthalmologists. In four, the inferior oblique muscles were placed 2 mm anterior to the insertions of the inferior rectus muscles, and in two they were placed 3 mm anterior. Of the eight patients I have observed with this complication, I reoperated on six. The surgical procedure consisted of denervation or extirpation of both inferior oblique muscles in four patients and conversion to standard recessions of the inferior oblique muscles in two patients. In all six patients,the versions were markedly improved and the Y orV pattern was eliminated after reoperation. CONCLUSIONS: Anteriorization of the inferior oblique muscles more than 1 mm anterior to the insertions of the inferior rectus muscle may cause a limitation of elevation in abduction, resulting in a Y or V pattern that mimics inferior oblique overaction. This may be more likely to occur if the new insertions of the inferior oblique muscles are spread out laterally at the time of anteriorization.- - - - - - - - - - ranking = 4keywords = syndrome (Clic here for more details about this article) |
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