[Space localization in cases of disorders of ocular motility in the horizontal plane].
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BACKGROUND: Ocular neuromyotonia is a rare ocular motility disorder characterized by involuntary contractions of one or several ocular motor muscles. In this report a typical case is presented. PATIENT AND METHODS: A 61-year-old female patient was referred with persistent diplopia despite previous surgical treatment for right 6 (th) nerve palsy. The palsy was caused by a meningeoma of the petrous bone, which had been incompletely resected. Subsequently, the patient had received radiation therapy. Strabismological and neuroophthalmological examinations were performed. The results coincide with data collected in our department from three other patients with ocular neuromyotonia (2 cranial nerve VI, 1 cranial nerve IV), over the past three years. RESULTS: The patient showed orthophoria at distance (5 m) and an exophoria of 6 degrees at near vision. The abduction and adduction of the right eye were restricted to 40 degrees and 35 degrees , respectively. After several seconds of eccentric gaze to the right, the right eye remained in an abducted position of approximately 25 degrees . The gaze to the left led to a retraction instead of an adduction of the right eye. The spasm of the lateral rectus muscle resolved after some seconds, returning to the aforementioned right eye motility. Under oral treatment with carbamazepine, initially 200 mg, later increasing to 400 mg per day, the symptoms improved significantly, did not resolve entirely, however. CONCLUSIONS: These typical findings permit the diagnosis of ocular neuromyotonia. The characteristic symptoms of ocular neuromyotonia and the typical history of a previous intracranial tumor, treated neurosurgically with adjuvant radiotherapy, lead to the hypothesis that ephaptic transmission in the cranial nerve is the underlying pathophysiological mechanism in the development of ocular neuromyotonia. Hereby, efferent impulses from non-twitch motoneurons could activate neighbouring axons, and spread both peripherally and centrally. Beside other mechanisms discussed, an involvement of proprioceptive elements and their reafference is also a possible cause for the prolonged muscle contraction.
PURPOSE: To analyze the contents of international publications in neuro-ophthalmology during the past decade. METHODS: Medline was searched with the Internet provider PubMed in December 2003, using "eye disease" as the medical subject heading and "optic nerve", "visual pathway", "visual cortex", "ocular motility", and "pupil" as subhead terms for retrieving English-language neuro-ophthalmic articles that were published between 1993 and 2002. RESULTS: A total of 9,585 English-written original articles available for analysis were concerned with a wide range of basic and clinical neuro-ophthalmic subjects, the order of frequency being ocular motility (22.8%), optic nerve (17.2%), visual functions (17.2%), visual cortex (10.1%), retina (8.6%), ocular adnexa (5.9%), optic chiasm (5.8%), brainstem (3.1%), and pupil (3.0%). The major optic nerve disorders included demyelinated optic neuritis, Leber hereditary optic neuropathy, and ischemic optic neuropathy. The major ocular motility disorders were supranuclear palsy, progressive external ophthalmoplegia, and ocular motor nerve disease. The articles were contributed from 73 countries. The top 10 countries ranked by share were USA (40.4%), United Kingdom (10.2%), Japan (8.7 %), Germany (6.0%), Italy (4.0%), Canada (3.5%), France (3.5%), Australia (2.2%), Israel (1.8%), and Turkey (1.8%). Researchers with diverse specialties contributed to the neuro-ophthalmic publications, including ophthalmology (40.5%), neurology/neuroscience (21.7%), and neurosurgery (5.9%). CONCLUSIONS: Neuro-ophthalmic papers were interdisciplinary, contributed by researchers with diverse specialties, and published in a wide range of biomedical as well as neurological journals.
Severe restrictive disorders of ocular motility are rarely described in high myopia. Their cause is still unknown. Several theories have been suggested: structural changes in the ocular muscles, sixth-nerve palsy or an elongation of the ocular globe. We report the case of a 44 years old man with high myopia of the left eye and disturbed ocular motility. Medical imaging techniques (echography, CT, MRI) allowed us to objectify the cause of ocular motility problem.
Opsoclonus and ocular flutter are rare but well-defined disorders of the saccadic system. Ocular flutter is a burst of back-to-back horizontal saccades without an intersaccadic interval. If these saccades occur in all directions, the involuntary eye movements are called opsoclonus. The most common aetiologies are paraneoplastic, postinfectious, toxic-metabolic and idiopathic. The underlying malignancy is usually neuronal crest tumors in children or lung, breast or gynaecologic cancer in adults. The appearance of these syndromes should prompt the search for an occult malignancy.
Neuro-ophthalmologic manifestations of whiplash injury may be evident or quite inapparent. Presentation is made of a young woman sustaining whiplash injury, who developed visual field impairment and reduced amplitude of accommodation with pronounced asthenopic discomforts. The potential visual system lesions secondary to whiplash injury, generally manifesting as impaired accommodation and convergence, ocular motility disorders, lesions of the posterior ocular segment and visual field impairments, are systematically described.
Following an analysis of echographic findings in 94 patients with posttraumatic ocular motility disorders, a flow-chart for detecting the basic pathomechanism was worked out. Normal muscle diameter is observed in entrapment of the perimuscular tissues or in a recent palsy, whereas increased muscle thickness suggests hematoma or incarceration, and thinned muscles an atrophy. A lack of change in muscle diameter when the patient looks in the direction in which motility is restricted is characteristic of atrophy or palsy, whereas an increase in muscle diameter is a sign of incarceration if a fracture has also been detected by echography; if not, muscle hematoma can be diagnosed.
PURPOSE: To improve existing MRI (Magnetic Resonance Imaging) methods of assessing ocular motility in horizontal gaze by using a multi-static fast imaging T2W MRI sequence, preventing patient head motion and convergence of the eyes during the study for the diagnosis and management of complex paralytic strabismus. SUBJECTS AND METHODS: A turbo-RARE image of the orbits was acquired in a set plane each time the patient fixed on a virtual image of one of 15 LEDs (Light Emitting Diodes). Positioning and securing the head was by a security cushion with an 8 degree tilt of the 25 cm quadrature head coil. After completion of the study (5 minutes) the MRI images were transferred to a work station and on to a magnetic-video tape to be replayed in cine mode on a PC using standard software. The qualitative analysis was completed by the study of quantitative parameters: visual axis, minor axis of the lens, the lines and angles of the horizontal rectus muscles with the optic nerve and the visual axis. We used digital image analysis to measure the cross-sectional areas of these muscles and also to determine muscle volumes. Twenty normal volunteers and 3 cases of oculomotor palsies were so studied. RESULTS: The experimental setup allowed us to obtain: 1) rapid (14 s) and high resolution images (256 x 196 x 3 mm); 2) which were free artifacts; 3) with no detectable eye convergence; 4) in which the lateral and medial rectus muscles and anatomical details of the orbit were clearly depicted; 5) from which additional functional information was obtained from the cine loop analysis; 6) wherein the visual axes did not converge during horizontal versions; 7) which permitted horizontal rectus muscle angles to be followed during contraction and relaxation; and 8) allowed the cross-sectional area of the four rectus muscles to be compared during contraction and relaxation (difference of 0.2 cm2). CONCLUSIONS: This method will provide useful functional quantitative information in the evaluation of ocular motility disorders. The procedure is readily clinically applicable
Two patients with diplopia presented with unilateral oculomotor palsy, defective elevation of the contralateral eye, and sparing of the levator palpebrae muscle. In each case, magnetic resonance imaging disclosed an infarction of the oculomotor nuclear complex in the mesencephalon. This clinical neuroradiological correlation is consistent with Warwick's scheme pertaining to the neuroanatomy of the oculomotor nuclear complex and demonstrates the utility of MRI in diagnosing ocular motility disorders of brainstem origin.
Ocular myasthenia is a localized form of myasthenia clinically involving only the extraocular, levator palpebrae superioris, and/or orbicularis oculi muscles. Ocular manifestations can masquerade as a variety of ocular motility disorders, including cranial nerve and gaze palsies. A history of variable and fatiguable muscle weakness suggests this diagnosis, which may be confirmed by the edrophonium (Tensilon) test and acetylcholine receptor antibody titer. Anticholinesterases, corticosteroids and other immunosuppressive agents, and other therapeutic modalities, including thymectomy and plasmapheresis, are used in treatment. As the pathophysiology of myasthenia has been elucidated in recent years, newer treatment strategies have evolved, resulting in a much more favorable prognosis than several decades ago. This review provides historical background, pathophysiology, immuno-genetics, diagnostic testing, and treatment options for ocular myasthenia, as well as a discussion of drug-induced myasthenic syndromes.
The authors report clinical features of ocular manifestations in patients with multiple sclerosis (MS), those that affect the visual sensory system and those that affect the ocular motor system. Disturbances of visual sensory function may precede, manifest coincidentally or follow the neurologic manifestations. Visual disturbances are common in MS and often a result of acute demyelinating optic neuropathy. Careful examination of MS patients, who have never suffered optic neuritis, may also reveal asymptomatic visual loss. Asymptomatic visual loss seems to be a universal feature of MS. Patients with multiple sclerosis may develop disorders of fixation, ocular motility and ocular alignment. Disorders of ocular motor system are frequently the initial sign of multiple sclerosis and occur as its presenting sign weeks, month, or years before other neurologic symptoms and signs develop.
We evaluated nine patients with external ophthalmoparesis and increased intracranial pressure. The eye movements normalized when the intracranial pressure was controlled. Investigations for an underlying cause of elevated cerebrospinal fluid pressure are warranted when ocular motility disorders are present.
This report is a case of a 4 year old male child who was admitted for meningitis. On clinical examination he was diagnosed as a case of oculo-suriculo-vertebral dysplasia with congenital heart disease, i.e., tetralogy of Fallots besides his presenting picture of meningitis. During his ophthalmic checkup for the conspicuous presence of epibulbar dermoid, he was discovered to have upper lid coloboma, double elevator palsy, and Grade 1 Duane retraction syndrome in his right eye while the pathognomic feature, a dermolipoma, was present in the left eye. The oculo-auriculo-vertebral dysplasia, as described by Goldenhar, is a disease complex of structures developed from the Ist and IInd branchial arch. The important feature of this case is the bilateral involvement of the disease complex over and above the presence of ocular motility disorders--Duane retraction syndrome and double elevator palsy.
The aim of ocular muscle surgery in Graves' disease is to determine how an optimal normalization of ocular motility disorders - diplopia, compensatory head posture, eyelid dispositions - can be achieved. The results of ocular muscle surgery of fibrotic ocular muscles allow the following conclusions to be drawn. Correcting the motility disorder can be precisely dosed by recessing only one fibrotic ocular muscle with a strabismic angle of up to 15 degrees. This leads to reproducible and dependable results, with a dose-effect coefficient independent of the initial strabismus angle. Indications as to the amount of surgery and which side should be varied according to horizontal an vertical deviations, and are also dependent on the compensatory head posture. Improvement of the binocular visual field is possible in nearly all cases. Over corrections occur more often when the muscle is not directly fixed at the sclera but adjusted on the following day. The time factor is important both before and after the operation. Before the operation, the motility status should have been stable for at least six months. Postoperatively, within the first few days, an insufficient correction should be expected, however this should not lead to premature revision of the amount of surgery. The surgery of the vertical rectus muscles influences the eyelid position. The upper lid retraction is improved with surgery on the vertical extraocular muscle and depends on prior upper lid motility. In contrast, an increase in lower lid retraction is not dependent on an inferior rectus recession. The results permit a precise series of steps to be drawn up in Graves' disease regarding surgical indications, proportionally correcting each side, and the dosage of ocular muscle operations.
Ocular myasthenia gravis is a not uncommon autoimmune disorder causing diplopia, ptosis, and weakness of lid closure. The predilection of myasthenia for the ocular muscles may be related to differences between limb and extraocular muscles in either physiological function or antigenicity. Clinically, ocular myasthenia can mimic any form of pupil-sparing ocular motility disorder. Dynamic abnormalities of myasthenic eye movements may reflect the primary hallmarks of the disease, which are fatigability and variability in strength, or secondary adaptive effects by the central nervous system. Tests to confirm the diagnosis include edrophonium challenge, repetitive nerve stimulation, single-fiber electromyography (EMG) of the frontalis, and assays for antibody directed against the acetylcholine receptor: all are less sensitive for ocular myasthenia than for generalized myasthenia. There is a higher incidence of other autoimmune conditions in myasthenia, notably thymoma and thyroid dysfunction. The differential diagnosis includes other diseases of the neuromuscular junction, such as Lambert-Eaton syndrome and botulism. Treatment consists of symptomatic use of acetylcholinesterase inhibitors and immunosuppression with steroids or azathioprine. Between 50 and 70% of patients with ocular myasthenia will eventually develop generalized disease: there is some retrospective data that steroids or azathioprine may reduce this by about 75%. The role of thymectomy in ocular myasthenia remains unclear.
Botulinum A toxin is used intramuscularly in the treatment of spastic neuromuscular disorders, strabismus, and laryngeal dystonia. The toxin has recently been reported as being useful for the cosmetic removal of glabellar furrows. The clinical effect of the toxin lasts four months or longer. Systemic side effects are rare and usually transient. We report the case of a psoriasiform eruption temporally related to the injection of botulinum A toxin into the medial rectus muscle to treat an ocular motility disorder. To our knowledge, this is the first case of a psoriasiform dermatitis caused by this agent.
Although extraocular muscle is perhaps the least understood component of the oculomotor system, these muscles represent the most common site of surgical intervention in the treatment of strabismus and other ocular motility disorders. This review synthesizes information derived from both basic and clinical studies in order to develop a better understanding of how these muscles may respond to surgical or pharmacological interventions and in disease states. In addition, a detailed knowledge of the structural and functional properties of extraocular muscle, that would allow some degree of prediction of the adaptive responses of these muscles, is vital as a basis to guide the development of new treatments for eye movement disorders.
OBJECTIVE: To review the clinical classification of strabismus, to describe the timing and method of strabismus screening examinations, and to discuss the principles of treatment. QUALITY OF EVIDENCE: Current literature (1983 to 1995) was searched via MEDLINE using the MeSH headings strabismus, ocular motility disorders, and amblyopia. Articles were selected based on their date of publication, clinical relevance, and availability. Preference was given to more recent articles, articles with large numbers of subjects, and well-designed cohort studies. Official recommendations from academic groups were analyzed. Descriptions of clinical tests and their illustrations are based on classic texts. MAIN FINDINGS: Primary care physicians should screen all low-risk children. High-risk children (low birth weight, family history of strabismus, congenital ocular abnormality, or systemic conditions with vision-threatening ocular manifestations) should be referred to an ophthalmologist for screening. Screening should be performed in the neonatal period, at 6 months, and at 3 years (Grade A recommendation), as well as at 5 to 6 years (Grade B recommendation). Screening examination includes inspection, examining visual acuity, determining pupillary reactions, checking ocular alignment, testing eye movements, and ophthalmoscopy. CONCLUSIONS: Primary care physicians are essential to early detection of strabismus and amblyopia. Early detection can help minimize visual dysfunction, allow for normal development of binocular vision and depth perception, and prevent psychosocial dysfunction.