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Ocular myasthenia gravis.

Myasthenia gravis may be limited solely to the eyes (ocular myasthenia) or may have systemic manifestations (generalized myasthenia). Most patients display blepharoptosis or ophthalmoparesis, resulting in diplopia, but the ocular motility disorder may take many forms. The diagnosis is often elucidated by variable muscle weakness with fatigability noted in the history and on examination. The ice test has supplanted the need for the edrophonium (Tensilon) test in many cases of ocular myasthenia. Therapy may be challenging, because many ocular myasthenics do not achieve significant clinical improvement with acetylcholinesterase inhibitors alone. Immunomodulatory treatment,such as systemic corticosteroids, is frequently effective, and emerging data suggest that this type of therapy may reduce the likelihood of ocular myasthenia becoming generalized.

Humans↗

Cine magnetic resonance imaging of eye movements.

Cine magnetic resonance imaging (MRI) is a technique in which multiple sequential static orbital MRI films are taken while the patient fixates a series of targets across the visual field. These are then sequenced to give a graphic animation to the eyes. The excellent soft tissue differentiation of MRI, combined with the dynamic imaging, allows rapid visualisation, and functional assessment of the extraocular muscles. Good assessment of contractility can be obtained, but the technique does not allow study of saccadic or pursuit eye movements. We have used this technique in 36 patients with a range of ocular motility disorders, including thyroid-related ophthalmopathy, blow-out fracture, post-operative lost or slipped muscle, and Duane's syndrome.

Eye Movements↗

Experimentally designed glaucoma implant surgery with mitomycin C in rabbit.

An attempt was made to observe the possibility of controlling intraocular pressure (IOP) without hypotony and ocular motility disorder by installing an experimentally designed glaucoma implant through a small conjunctival incision with the aid of a stylet and maintaining the aqueous reservoir using mitomycin C (MMC). The implant was made of silicone tube, of which one end was occluded by glue and on the same end 4 check-valve-like slits were made. Thirty-five healthy white rabbits were used and subdivided into 4 groups. In groups I to III, implants having 2.0, 2.5, 3.0 mm slit lengths, respectively, were installed with MMC application in one eye of each of the 10 rabbits. In group IV, a 2 mm slit-length implant was installed without MMC in one eye of each of the 5 rabbits. Pneumatonometry and ultrasonography were performed to check the IOP and the formation of aqueous reservoir in the implanted eyes for 8 weeks. In group I through III, there was a statistically significant 4-5 mmHg pressure-lowering effect in the implanted eyes compared to the contralateral control eyes for 8 weeks. The aqueous reservoirs were observed throughout the follow-up period. In group IV, we could observe neither a pressure-lowering effect nor aqueous reservoir formation in the implanted eyes after 2 weeks postoperatively. Hypotony did not occur in implanted eyes in any of the groups. This study shows the possibility of IOP control by installing a specially designed glaucoma implant with application of MMC.

Animals↗

Remission of superior oblique myokymia after microvascular decompression.

Superior oblique myokymia (SOM) is an ocular motility disorder characterized by oscillopsia, vertical or torsional diplopia, sometimes combined with pressure sensation. Although the pathophysiological basis is unclear, isolated case reports have documented its association with intracranial pathological processes. We present a case of SOM associated with a vascular compression of the fourth nerve at the root exit zone. Following microneurosurgical decompression, SOM completely resolved and paralysis of the fourth nerve occurred. This was less disturbing.

Decompression, Surgical↗

Synergistic divergence: saccadic velocity analysis and surgical results.

The clinical findings are described in four patients with synergistic divergence (SD), an ocular motility disorder which is considered to be a variant of Duane retraction syndrome (DRS). Saccadic velocity studies in three patients demonstrated preserved abducting saccades in the affected eye with equally reduced amplitudes on both attempted abduction and adduction. The preserved saccades on attempted abduction of the affected eye, coupled with electromyographic evidence that shows maintenance of normal reciprocal innervation with respect to gaze on attempted abduction as dictated by Sherrington's law, strongly suggest that the abducens nerve is not necessarily absent or severely hypoplastic in this condition. One patient showed typical DRS in the contralateral eye and had a son with bilateral DRS, attesting to a possible genetic and etiologic relationship between SD and DRS. Three patients underwent strabismus surgery. Review of our surgical results and an analysis of the literature indicate that a crippling procedure to the ipsilateral lateral rectus muscle, such as extirpation, may be necessary to eliminate simultaneous abduction. Overcorrection did not occur following lateral rectus extirpation.

Adolescent↗

Benign opsoclonus in preterm infants.

Opsoclonus is a rare childhood ocular motility disorder characterized by irregular, chaotic, involuntary bursts of high amplitude, back-and-forth oscillations of the eyes,without pause intervals. Although this disorder is associated with neuroblastoma and other neural crest tumors, as well as with other neurologic abnormalities, it was also reported in healthy infants. The purpose of this study was to prospectively investigate the prevalence of opsoclonus in preterm infants, and to find any conditions associated with this disorder. Between August 2000 and April 2003, 528 consecutive preterm infants with gestation of less than 33 weeks or birth weight of less than 1500 gm were examined in accordance with our medical centers' retinopathy of prematurity screening policy. Opsoclonus was identified in three patients (0.6%), who were all otherwise physically and neurologically intact. One patient developed threshold retinopathy of prematurity that resolved after laser treatment. On follow-up examinations, the opsoclonus gradually disappeared by the age of 6 months in all infants,with no other complications. This study suggests that opsoclonus can be a benign, self-limiting phenomenon in preterm infants as was previously reported in term infants. However, because of the risk of severe associated illnesses, it is prudent to perform several diagnostic procedures and to continue careful follow-up until this phenomenon spontaneously resolves.

Female↗

Locomotor and oculomotor impairment associated with cerebellar dysgenesis in Zic3-deficient (Bent tail) mutant mice.

We examined the adult neural phenotypes of the Bent tail mutant mouse. The Bent tail mutant mouse was recently shown to lack a submicroscopic part of the X chromosome containing the Zic3 gene, which encodes a zinc-finger protein controlling vertebrate neural development. While nearly one-fourth of hemizygous Bent tail (Bn/Y, Zic3-deficient) mice developed neural tube defects in their midbrain and hindbrain region, the other Bn/Y mice showed apparently normal behaviour in a C57BL/6 genetic background. A battery of behavioural and eye movement tests revealed impaired spontaneous locomotor activity, reduction of muscle tone and impairments of vestibuloocular and optokinetic eye movements in these mice. Morphological examination of the mutant brain showed a significant reduction in the cell numbers in the cerebellar anterior lobe and paraflocculus-flocculus complex. Our results indicate that the cerebellar dysgenesis characterized by subregional hypoplasia affects the locomotor activity, muscle tone and eye movement control of the mice. These findings may have some clinical implications in relation to disorders characterized by cerebellar dysgenesis, such as Joubert syndrome.

Animals↗

Ophthalmic complications of spina bifida and hydrocephalus.

The article reviews the ophthalmic literature on spina bifida and hydrocephalus and presents the findings of a six-year survey of 322 children. Ophthalmic complications were found to occur very frequently. Forty-two per cent had a manifest squint, 29% lateral rectus palsy or musculoparetic nystagmus, 14% papilloedema and 17% optic atrophy. Only 27% definitely had normal visual function. Seventy per cent of proven episodes of shunt dysfunctions had positive ophthalmological evidence of raised intracranial pressure. The sudden appearance of a squint, other ocular motility disorder or papilloedema usually denotes uncontrolled hydrocephalus. Shunt surgery is the first priority but may not restore normal ocular motility and visual function. Subsequent treatment should be tailored to the special needs of these children. Regular ophthalmic supervision of these children saves life and sight.

Adolescent↗

Giant axonal neuropathy: visual and oculomotor deficits.

Giant axonal neuropathy, a generalised disorder or neurofilaments, presents as a chronic, progressive peripheral neuropathy in childhood. Evidence for central nervous system involvement is demonstrated in this study of four male patients with giant axonal neuropathy who had defective visual function and abnormal ocular motility. The visual system was studied by electroretinography, which showed normal retinal function, and by visual evoked potentials, which showed disease of both optic nerves and retrochiasmal visual pathways. The ocular motility disorder, studied by electrooculography, comprised defective pursuit, inability to maintain eccentric gaze with gaze paretic and rebound nystagmus, abnormal optokinetic responses and failure of suppression of the vestibulo-ocular reflex by fixation. These findings suggested involvement by giant axonal neuropathy of the cerebellar and brain stem pathways important in the control of ocular motility.

Axons↗

Medical treatment of nystagmus and ocular motor disorders.

An increased compendium of drugs useful in ocular motor system dysfunction has expanded our capacity to treat selected ocular motility disorders. Adjunctive therapeutic modes (e.g., Fresnel prisms and orthoptic exercises) can also be beneficial. PAN and see-saw nystagmus can be treated with baclofen. Downbeat nystagmus may respond to clonazepam therapy, and prisms may help if the nystagmus can be modified with convergence. Congenital nystagmus may respond minimally to drugs (e.g., baclofen), but prisms or surgical procedures, or both, are still the primary treatment modalities. Innovar may be helpful in patients with severe, incapacitating vestibular disorders, and scopolamine alone or in combination with promethazine may be beneficial in patients with milder ambulatory acute peripheral vestibular disorders. Benign positional vertigo is best treated initially with positional exercises before drug therapy is instituted. Opsoclonus and ocular flutter have been treated successfully with corticosteroids, propranolol, and clonazepam, while microflutter, an extremely rare disorder, can resolve with baclofen. Although therapy with carbamazepine, 5-hydroxtryptophan, and scopolamine has been useful in selected patients with ocular palatal myoclonus, most do not respond to drug treatment. It is not usually necessary to treat voluntary nystagmus, but Fresnel prism lenses should be remembered in refractory patients. Potentially reversible and pseudointernuclear ophthalmoplegias also were discussed. Orthoptic exercises can be beneficial in posttraumatic internuclear ophthalmoplegia. Selected supranuclear palsies can be improved completely with the proper drug regimen. Lastly, superior oblique myokymia can be treated successfully with carbamazepine, with tight surveillance for possible adverse side effects. Descriptive phenomenology and pathophysiological localization must be correlated with brain stem neurochemistry and neuropharmacology to medically treat additional ocular motor system disorders.

Adolescent↗

Neuro-ophthalmological evaluation of patients with myelomeningocele and Chiari malformations.

Twenty-eight patients (14 females and 14 males aged between four and 34 years) with myelomeningocele and Chiari malformations were examined neuro-ophthalmologically. All patients had been under proper neurosurgical care and no reduction of visual function was found that could be related to optic atrophy or other changes in the visual pathways. However, ocular motility disorders, including strabismus, were common, although no case of down-beat nystagmus was observed. Manifest strabismus was found in 11 patients. Esotropia with or without A-pattern was seen in nine patients. Spontaneous or gaze-related nystagmus and abnormal optokinetic nystagmus represented the most common disturbances of ocular motility. Abnormalities of horizontal eye-movements were more common than those of vertical eye-movements. Strabismus of the manifest type was combined with other ocular motility disturbances in most cases.

Adolescent↗

[Dissociated ophthalmoplegia of abduction: so-called posterior internuclear ophthalmoplegia in Fisher's syndrome].

The anterior internuclear ophthalmoplegia, a dissociated ophthalmoplegia of adduction secondary to an ipsilateral supranuclear paresis of the internal rectus, represents a well-know clinical picture. The dissociated ophthalmoplegia of abduction, however, sometimes referred to as posterior internuclear ophthalmoplegia, is debated. Such an oculomotor disturbance emerging from an ophthalmoplegic polyneuropathy (Fisher syndrome) is presented and illustrated by oculographic tracings. A secondary finding was a limited range of accommodation. Oculographically, the dissociated ophthalmoplegia of abduction reflects a true mirror image of the anterior internuclear ophthalmoplegia. Because of anatomical considerations the dissociated ophthalmoplegia of abduction cannot be regarded as internuclear ophthalmoplegia, and the terminology of posterior internuclear ophthalmoplegia is not tenable. Discussing the pathophysiology of the Fisher Syndrome, it is reflected upon an infranuclear lesion causing an ocular motility disorder featuring supranuclear aspects, and avoiding limitations of peripheral nerve damage.

Accommodation, Ocular↗

[A clinical and epidemiological description of a series of patients diagnosed as suffering from progressive supranuclear palsy].

INTRODUCTION: Progressive supranuclear palsy is a disease that normally presents only sporadically in adults and courses in a progressive, chronic manner. It is characterised by the presence of supranuclear ophthalmoplegia, postural instability, a Parkinsonian syndrome, pseudobulbar affect, cervical dystonia and cognitive impairment. PATIENTS AND METHODS: We conducted a descriptive study of clinical and epidemiological features in a series of 18 patients who satisfied the mandatory NINDS-SPSP clinical eligibility criteria for the likely diagnosis of progressive supranuclear palsy, using the scale developed by Golbe et al. RESULTS AND CONCLUSIONS: The mean age of onset of the disease was 58.6 +/- 8.2 years, 55.5% of the patients were males, the average history of the disease at the time of diagnosis was 4.39 +/- 2.3 years, and there was a diagnostic subregister in the first 4 years of the disease. Gait disorders, falls and slowness were the most frequently observed presenting forms of the disease. During their first four years with the disease, 75% of the patients were totally independent when it came to carrying out activities of daily living, whereas after the fourth year there was a predominance of the need for aid and absolute dependence. Dysphagia was more frequent in the later stages of the disease. Ocular motility disorders and impaired cognitive functioning were obvious in the initial stages of the disease, and there was a strong correlation between the length of time the disease had been coursing and the severity of the ocular and cognitive disorders.

Cognition Disorders↗

New muscle growth in denervated extraocular muscle.

Current therapies for paralytic ocular motility disorders often provide less than satisfactory results. Promising new techniques of nerve grafting and muscle transplantation used to treat muscle paralysis elsewhere in the body may have application to the ocular motor system. In studying a procedure designed to reinnervate paretic extraocular muscle (e.o.m.), we produced not only the expected nerve growth but also abundant new muscle growth. A considerable amount of this muscle growth occurred within the axon depleted nerve fascicles of the paretic muscle, suggesting that this structure was a particularly good matrix for muscle fiber growth. These observations suggest that new, entire neuromuscular units may be induced to grow in the plane of a paretic muscle if appropriate surface characteristics can be established.

Animals↗

Intraoperative eye position after sub-Tenon's anesthesia for cataract surgery.

BACKGROUND: To evaluate the incidence and extent of ocular deviation associated with sub-Tenon's anesthesia. METHODS: Seventy-five consecutive patients undergoing routine phacoemulsification with sub-Tenon's anesthesia at the Eye Unit, Royal Gwent Hospital, were prospectively enrolled in this observational case series. The exclusion criteria were patients taking warfarin, or patients with preexisting ocular motility disorders, allergy to local anesthetics, or previous surgery for squint or retinal detachment. Eleven patients were subsequently excluded from the study group as they required additional anesthesia blocks before measurements were taken for ocular deviation. We injected 5 mL of a local anesthetic mixture of 2% lignocaine and 7.5 mg/mL levobupivacaine in the sub-Tenon's space in the inferonasal quadrant. Ocular deviation was assessed objectively by means of the Krimsky test. Forced duction tests were performed before and after anesthesia. RESULTS: Hyperdeviation was documented in 49 (77%) eyes, exodeviation in 62 (97%), and hypodeviation in 6 (9.4%). No esodeviations were documented. Only 2 patients were orthophoric after anesthesia. Mean vertical deviation was 4.4 prism dioptres (PD) (SD 4.7 PD, 95% CI 3.2-5.5 PD, range 0-14 PD). Mean horizontal deviation was 19.5 (SD 9.6, 95% CI 17-22, range 0-50) PD. Forced duction test revealed no mechanical restriction before or after anesthesia. INTERPRETATION: This study suggests that there is an appreciable ocular deviation with sub-Tenon's anesthesia. Intraoperative ocular deviation may make certain procedures more difficult; therefore, we recommend a low threshold for an additional corrective block administered before surgery to rectify the deviation.

Anesthesia, Local↗

[Thalamic dementia due to infarct of the left thalamus and genum of the right internal capsule].

INTRODUCTION AND OBJECTIVES: Thalamic dementia is the clinical consequence of a disorder of both thalami. It is generally secondary to bilateral paramedial thalamic infarcts due to disorders of small blood vessels or cardioembolism. We report a case of dementia of acute onset involving the left thalamus and the genum of the right internal capsule. CLINICAL CASE: A 33 year old man, HIV positive, category B2, admitted to hospital for tuberculous meningitis presented with the acute onset of somnolence, followed by marked bradypsychism, personality changes, marked disorder of executive explicit memory without associated praxic, gnosic or language disorders. Ocular motility remained normal. There was left central facial paralysis with inverse emotive voluntary dissociation. The other cranial nerves were normal. There was left hemiparesia with extensor plantar reflex. No other alterations. Cerebral MR imaging was compatible with paramedial infarcts of the left thalamus and genum of the right internal capsule. CONCLUSIONS: Thalamic dementia generally occurs in bilateral paramedian thalamic disorders. There are cases of disorders of executive memory secondary to infarcts of the genum of the internal capsule due to interruption of the thalamotemporal pathways and a contralateral paramedial thalamic lesion.

Adult↗

Vestibular perception of angular velocity in normal subjects and in patients with congenital nystagmus.

A technique is described for the assessment of vestibular sensation. The two main goals of the study were (i) to compare the perception of angular velocity with the eye velocity output of the vestibulo-ocular reflex and (ii) to study vestibular function in patients with congenital nystagmus; this was needed since most previous studies, based on eye movement recordings, have been inconclusive. Subjects indicated their perceived angular velocity by turning by hand a wheel connected to a tachometer. The vestibular stimuli used consisted of sudden deceleration from rotation at a constant horizontal velocity of 90 degrees /s ('stopping' responses). Eye movements were recorded simultaneously with electro-oculography. In normal subjects the perceived angular velocity decayed from the moment of deceleration in an exponential fashion. The mean time constant of sensation decay was approximately 16 s. Eye movement velocity decayed with a similar exponential trajectory (time constant 16 s). Congenital nystagmus patients showed markedly shortened vestibular sensation (mean time constant 7 s). The following conclusions can be drawn: (i) the similarity of the eye velocity and perceptual responses suggests that these two systems receive a vestibular signal which has been similarly processed; (ii) the time constant of the responses indicates that this vestibular signal probably originates in the same brainstem 'velocity storage' integrator; (iii) the technique described is useful for clinical assessment of vestibular function, particularly in patients with ocular motility disorders; (iv) patients with congenital nystagmus have short vestibular time constants, which is probably due to changes induced in velocity storage processing by the persistent retinal image motion present in these patients.

Adult↗