Cases reported "ocular motility disorders"

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11/416. 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. ( info)

12/416. 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. ( info)

13/416. Post malaria cerebellar ataxia and ocular flutter: report of two cases.

    We report two cases of post malarial cerebellar ataxia presenting with severe ocular flutter, in a female of 72 years and a male of 20 years. Both patients had falciparum malaria infection. CT scans of both patients were within normal limits for the age. Cerebellar signs as well as ocular flutter responded very well to moderate doses of prednisolone therapy. ( info)

14/416. 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. ( info)

15/416. Normal reading despite limited eye movements.

    Many investigators have demonstrated that poor readers exhibit abnormal eye movements during reading. An association between defective vergence, accommodation, and poor reading skills has also been noted by some investigators. Children who are poor readers have been subjected to therapeutic interventions on the basis of the assumption that improving their eye movements, as part of a multifaceted program of "vision therapy," will yield commensurate improvement in reading performance. This approach has been controversial, and other authors have expressed opposing views. We report the ophthalmologic and reading assessments of two children with Mobius' syndrome who were average to above-average readers despite essentially absent horizontal eye movements. ( info)

16/416. Fresnel membrane prisms: clinical experience.

    BACKGROUND: There are few published reports on the clinical application of Fresnel membrane prisms in the treatment of diplopia in adults. The authors describe the use of these prisms in patients with fourth and sixth cranial nerve palsies, restrictive motility caused by thyroid-related orbital disease, and convergence insufficiency. methods: Of 209 patients who had been treated with Fresnel prisms, 141 were selected. The database included patients from a private practice in Montreal, seen from 1988 to 1996, and patients seen by orthoptists in the ophthalmology department of a children's hospital in Montreal between 1992 and 1996. All the patients had diplopia associated with fourth (48 patients) or sixth (43 patients) cranial nerve palsy, thyroid-related orbitopathy (18 patients) or convergence insufficiency (32 patients). After qualitative and quantitative assessment of the ocular misalignment, a Fresnel prism was selected for power and axis and for appropriate location on the spectacle lens. Ocular dominance and side of paresis or restriction were also considered in the placement of the prism. The patient's response to treatment was documented. RESULTS: The Fresnel prisms were oriented horizontally in 72 patients (51%), vertically in 55 (39%) and obliquely in 14 (10%). They were placed on the spectacle lens before the nondominant eye in 127 cases (90%), either covering the entire lens, or on the upper or lower segment or both. The patients were followed for an average of 15 (range 2 to 96) months. Of the 141 patients 113 (80%) had a successful outcome, with relief of their diplopia. Twenty-seven patients (19%) eventually had the prismatic correction ground into the lens, 70 (50%) chose to wear the Fresnel prism on a permanent basis because incorporation into the lens was not possible or because of cost, and 17 (12%) used the Fresnel prism as a temporary device before or after surgery. Most patients who converted to incorporated prisms did so when the prismatic power became stable, usually after 6 to 8 months. Eight patients (6%) stopped using the prism because of associated side effects, such as blurred vision, persistent diplopia, torsion or optical aberrations. INTERPRETATION: The Fresnel prism is an excellent device in treating diplopia in adult patients. It is a reasonable permanent option when incorporating the prism into the spectacle lens is not possible. A fused blurred image caused by a Fresnel prism placed in front of the nondominant eye is preferable to double but clear images. ( info)

17/416. Orbital blowout fracture with persistent mobility deficit due to fibrosis of the inferior rectus muscle and perimuscular tissue.

    A case of orbital blowout fracture accompanied by fibrosis of the inferior rectus muscle resulting in an irreversible orbital mobility deficit is reported. An 8-year-old girl with an orbital blowout fracture was treated with steroids for 10 days, as with other cases in our department. She exhibited a disturbance of vertical eye movement and a positive forced duction test result. Although surgery was performed on day 13, and on day 27 due to poor recovery after the first operation, almost no improvement of the ocular movement was noted. The results of a traction test, performed during the second operation, suggested that the inferior rectus muscle had adhered to the periosteum. magnetic resonance imaging performed 3 days after the second operation revealed fibrosis of the inferior rectus muscle and perimuscular tissue, resulting in an irreversible disturbance of the vertical ocular movement. The present findings suggest that the need for and timing of surgery in patients with blowout fractures should be determined on an individual basis. ( info)

18/416. Opsoclonus in a patient with cerebellar dysfunction.

    After two days of malaise, headache, nausea, and vomiting, a 26-year-old man suddenly developed opsoclonus and stance and gait ataxia, without myoclonus. Having excluded a paraneoplastic etiology, we assumed that the disorder was probably related to a viral infection. Spontaneous resolution occurred in about two months. Opsoclonus became flutter dysmetria and then resolved. Saccadic eye movement recording disclosed the occurrence of hypermetria, increased velocity, and delayed latency, which also resolved. In this patient, the correspondence between clinical and ocular motor abnormality courses suggests a transient cerebellar dysfunction as the possible pathophysiologic mechanism for opsoclonus. ( info)

19/416. Paroxysmal tonic upgaze: physiopathological considerations in three additional cases.

    Paroxysmal tonic upgaze of childhood has been described as a benign distinctive syndrome of abnormal ocular movement, with or without concomitant ataxia. After the first observation of four children, a further 29 patients have been reported with a wide spectrum of neurologic abnormalities such as ataxia, unsteady of gait, learning disabilities and mental retardation at follow-up. Electroencephalograms were normal in all the subjects and magnetic resonance imaging showed deficient myelination in only one patient. Recently it has been suggested that paroxysmal tonic upgaze could be a heterogeneous syndrome, ranging from a simply age-dependent manifestation to a clinical appearance of a variety of disorders affecting the corticomesencephalic loop of vertical eye movement. Moreover, it also could be an early sign of more widespread neurologic dysfunction. We describe three patients who presented paroxysmal tonic upgaze; in one, ataxia was present; in the second child, ataxia and language disorder also were observed; and in the third patient paroxysmal tonic upgaze was associated with loss of muscle tone (drop-attack-like events). On magnetic resonance imaging, a pinealoma compressing the dorsal mesencephalic region was detected. On the basis of our observations, we suggest that any insult with periaqueductal mesencephalic gray-matter involvement could be considered the basic condition for this peculiar clinical manifestation. ( info)

20/416. Lagophthalmos: an unusual manifestation of oculomotor nerve aberrant regeneration.

    PURPOSE: To describe a patient with unusual findings after regeneration of the oculomotor nerve. methods: Case report. RESULTS: A 35-year-old woman developed complete right third nerve paralysis after neurosurgical ligation of internal carotid-posterior communicating and internal carotid-ophthalmic artery aneurysms. Permanent ipsilateral lagophthalmos appeared as third nerve function spontaneously recovered. CONCLUSION: Lagophthalmos may rarely develop after aberrant regeneration of the oculomotor nerve, presumably caused by co-contraction of the levator and superior rectus muscles during the Bell's phenomenon. ( info)
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