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We treated one patient with bilateral internuclear ophthalmoplegia and another with skew deviation with extraocular muscle botulinum toxin injection. Both patients had pre-injection symptomatic diplopia in primary position, one for 1 month and the other for 12 months. Resolution of diplopia and complete and permanent binocular fusion in primary position was obtained within 3 to 4 days in both cases.
By measuring the angles stepwise through the whole field of gaze or through selected meridians the pattern of incomitance can be analyzed. It can be graphically demonstrated by "incomitance curves" or "-lines". A preliminary but very useful guess of the incomitance pattern can be obtained in performing the cover test turning the head of the patient passively in small steps. The Hess-Screen gives a quick and useful graphical overview. Synoptometer and Harms tangent screen allow a detailed study. The evaluation of the incomitance pattern allows conclusions on the etiology and explains the remaining binocular field and the head position. In neurogenic palsies, the angle increases in a linear fashion, whereas in passive motor disturbances the angle may increase sharply, change its direction in the opposite direction of gaze and show a zone of concomitance. Duanes retraction syndrome shows passive components. Examples of patients with specific incomitance patterns are shown.
Motility disturbances after retinal detachment surgery are rare complications. This retrospective study examined 2427 cases from the University Eye Hospital in Münster (1/84-6/89). All cases underwent scleral buckling procedures for retinal detachment. Twelve patients developed persistent diplopia. The scleral buckle needed to be removed in all cases; however, additional strabismus surgery had to be done in 5 cases to restore binocular single vision. Evaluation of the 12 cases suggested that the complications were caused by hypertrophic scarring rather than by the buckles, suturing material or muscular decompensation.
This retrospective study shows the clinical data of 81 patients with elevation in adduction. The relationships between the elevation in adduction and various ocular anomalies, such as horizontal deviations, refractive errors, nystagmus, torticollis, superior oblique muscle paralysis and dissociated vertical deviation, have been studied in order to specify the clinical characteristics of this phenomenon. The age of onset of the elevation in adduction and of the dissociated vertical deviation has also been considered, and a typical temporal progression of these symptoms has been found. In this sequence, we first noticed the onset of the horizontal deviation, then the appearance of the elevation in adduction, and finally the appearance of the dissociated vertical deviation. Although the elevation in adduction is frequently found in association with the complex syndromic pattern of congenital esotropia, this phenomenon may also accompany other types of anomalies of ocular motility.
UNLABELLED: HISTORY AND METHODS: Six weeks after onset of a monocular elevation paresis of the left eye a 78-year-old man presented with an anterior ischemic optic neuropathy of the right eye. This time a monocular elevation paresis was observed in the right eye. The diagnosis of arteritis temporalis was confirmed histologically. THERAPY AND CLINICAL COURSE: Systemic steroid therapy was administered. Five days later the patient developed signs of an acute vertebrobasilar insufficiency. After further steroid therapy the neurologic signs and the motility disorder resolved. The visual acuity of the right eye improved from 1/20 to 6/20, but the visual field remained poor. CONCLUSION: In retrospect the monocular elevation paresis was the initial sign of the temporal arteritis. The combination of the affected muscles and the change of the affected eye were hints at a prenuclear origin of the paresis. General signs of an acute vertebrobasilar ischemia supported this supposed localisation.
The congenital fibrosis syndromes (CFS), including congenital fibrosis of the extraocular muscles (CFEOM) and Duane syndrome (DS), are rare congenital strabismus syndromes that present with nonprogressive restrictive ophthalmoplegia with or without ptosis. Although historically believed to result from primary extraocular muscle (EOM) fibrosis, our laboratory's work is based on the hypothesis that these disorders result from distinct, but analogous, developmental defects of the oculomotor (nIII), trochlear (nIV), and abducens (nVI) nuclei. We have defined three inherited CFEOM phenotypes (CFEOM1-3) and have mapped each phenotype to a distinct genetic locus (FEOM1-3). Individuals with CFEOM1 are born with bilateral ptosis and both eyes fixed in a downward position with absent upgaze and aberrant horizontal gaze. This disorder maps to the FEOM1 locus on chromosome 12cen.(1,2) Neuropathology studies of CFEOM1 reveal the absence of the superior division of oculomotor nerve and its corresponding alpha motor neurons in the midbrain, with abnormalities of target EOMs.(3) These neuropathology findings parallel those previously identified in Duane syndrome, in which there is an absence of nVI and the abducens nerve.(4,5) Individuals with CFEOM2 are born with bilateral ptosis and exotropia. This atypical form of CFEOM maps to the FEOM2 locus on chromosome 11q13 and results from mutations in ARIX (PHOX2A).(6,7) ARIX encodes a homeodomain transcription factor protein previously shown to be required for nIII/nIV development in mouse and zebrafish.(8,9) Together, these findings support the hypothesis that the congenital fibrosis syndromes result from parallel defects in nIII, nIV, and nVI nuclear development. Functional studies of the CFEOM genes should provide additional insight into the unique features of the extraocular lower motor neuron axis in health and disease. (For full (refs. 1-9), see reference list of the main paper.)
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The superiority of magnetic resonance imaging (MRI) of the posterior fossa over x-ray computed tomography is demonstrated by four patients with neuro-ophthalmologic disorders. Advantages of this technique include lack of ionizing radiation, direct multiplanar imaging, increased sensitivity to pathology, increased tissue contrast, and no known adverse effects. It is concluded that MR, when available, should be the imaging modality of choice in patients with neuro-ophthalmologic disorders of suspected brainstem origin.
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Cerebral visual impairment is one of the main causes of childhood visual impairment in developed countries. These disorders are often linked with pre- or perinatal hypoxic brain injuries. The patterns of brain injuries depend on the severity and duration of hypoxia and the child's age. In premature children, periventricular leukomalacia affects the optic radiations and the subcortical visual brain. In full-term newborn babies, chronic hypoxia leads to the damage of the visual cortex and acute hypoxia damages the basal ganglia. They recover from cortical blindness in variable ways. Visual dysfunction is characterized by fixation troubles, subnormal acuity (crowding), difficulty with perceiving visual fields, movements, depth, cognitive defects (agnosia of images, objects or faces, visuospatial disorders), ocular motility disorders (tonic gaze deviation, strabismus, nystagmus). Accompanying these cerebral injuries, there are accommodation defects and optic disk abnormalities that vary according to the gestational age at the time of hypoxia.
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OBJECTIVE: To determine the types and frequency of symptomatic ocular motility disturbances following head trauma and their association with severity of trauma. DESIGN: Retrospective study of patients with (1) diplopia unless visual loss is present, (2) heterotropia for far or near targets, and (3) prior head injury. SETTING: Office and in-hospital consulting practice of a university neuro-ophthalmologist. SUBJECTS: Sixty patients with posttraumatic ophthalmoplegia. MAIN OUTCOME MEASURES: Paralytic and nonparalytic heterotropias were quantitated in prism diopters or percentage limitation of ductions. Convergence insufficiency was assessed by determining the near point of convergence. RESULTS: Fifty-one patients had nuclear or infranuclear findings, ie, trochlear palsies (n = 20), oculomotor palsies (n = 17), abducens palsies (n = 7), combined palsies (n = 5), and restrictive ophthalmopathy (n = 2). Nine patients had supranuclear dysfunction, including seven patients with convergence insufficiency. Bilateral ocular motor palsies and combined palsies were significantly (by means of chi 2 test) associated with head trauma of severity sufficient to cause corticospinal tract dysfunction. Individual or combined ocular motor palsies were not significantly (by means of chi 2 test) associated with intracranial hemorrhage and/or skull fracture or loss of consciousness. CONCLUSIONS: Trochlear palsy was the most common nuclear or infranuclear basis for traumatic diplopia, and convergence insufficiency was the most common supranuclear cause of double vision. Head trauma distinguished by upper motor-neuron signs was correlated with specific subsets of disordered ocular motility.
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PURPOSE: To report our experience with extraocular myocysticercosis, to highlight the role of ultrasound and orbital computerized tomography (CT) scan in these cases and to discuss the management. METHODS: This is retrospective study of 35 cases of orbital myocysticercosis confirmed on ultrasound and CT scan of the orbit. RESULTS: There were 18 males and 17 females. The average age of these patients was 19.6 years. The most common presenting feature was restricted ocular motility with diplopia and recurrent pain and redness. Ultrasound examination and CT scan of the orbit done for all these patients identified the cyst and the affected muscle. All extraocular muscles were noted to be involved in myocysticercosis. However the lateral rectus, medial rectus and the superior oblique were affected to a greater extent. All cases dramatically improved on treatment with albendazole and oral steroids. Surgical excision of cyst was done in 6 cases. CONCLUSION: A high index of suspicion should be entertained for extraocular muscle cysticercosis, especially in cases of acquired ocular motility disorder with recurrent ocular congestion. Ultrasound and CT scans of orbit play a vital role in diagnosis. Medical therapy with albendazole under cover of steroids is effective in most of the cases.
Null-cell carcinomas of the pituitary are extremely rare. We describe a 41-year-old woman with a large adenohypophyseal neoplasm presenting as a primary nonfunctioning tumor without pituitary insufficiency. Signs of mass effect with progressive unilateral ocular motility disorders and anterior pituitary failure developed rapidly. Histopathological examination of the trans-sphenoidally removed tumor showed a primary pituitary null cell tumor with high mitotic index. Pituitary carcinoma was suspected because of rapid relapse of ocular motility disorders and of intra-sellar tumor growth after surgery. Radiotherapy of the sellar and parasellar area with a total dose of 59.4 Gy was performed, achieving marked tumor reduction and a significant improvement of ocular motility disorders. However, 6.5 months after presentation the patient rapidly declined and died of carcinomatous meningitis. Less than 100 pituitary carcinomas have been published so far, most of them as single-case reports, and endocrine, immunohistochemical, and ultrastructural data have not been described in the majority of cases. At presentation, there are no specific symptoms that allow to distinguish benign from malignant tumor. Prognosis is poor, since no curative treatment has been established, but aggressive surgery and radiotherapy has been recommended. Our case highlights the poor prognosis of nonfunctioning pituitary carcinomas.
PURPOSE: To highlight the different presentations of extraocular muscle cysticercosis, which can result in acquired ocular motility disorders. METHODS: Ten cases of acquired motility disorders examined between January 1998 and January 1999 were diagnosed as extraocular muscle cysticercosis with computed tomography (CT). All patients were treated with corticosteroid therapy and albendazole. Repeat CT scans were performed in 5 patients. RESULTS: The inferior rectus muscle was most commonly affected with double elevator palsy the most common clinical presentation. Response to treatment was satisfactory with complete resolution of the ocular motility disorder in 8 patients. Initiating therapy with corticosteroids prior to the addition of cysticidal drugs prevented severe inflammatory reactions and residual strabismus. CONCLUSION: A high index of suspicion should be entertained for extraocular muscle cysticercosis in every case of recent acquired ocular motility disorder. Response to medical therapy is satisfactory.