Brown syndrome: clinical features and results of superior oblique tenotomy / Síndrome de Brown: quadro clínico e resultados da tenectomia do oblíquo superior

ABSTRACT Purpose: This study was conducted to further define the specific clinical characteristics of patients with Brown syndrome and evaluate the outcomes of superior oblique tenotomy in its surgical management. Methods: A retrospective analysis of the medical charts of 45 patients with Brown syndrome was performed, which revealed that 11 patients underwent superior oblique tenotomy due to abnormal head posture and/or hypotropia and 1 patient underwent bilateral superior oblique tendon elongation with a silicone band due to abnormal head posture. In the last patient, silicone bands were removed at the postoperative 3rd month due to the lack of improvement in the abnormal head posture and the limitation of elevation in adduction. Simultaneous horizontal rectus muscle surgery was performed in four patients. Results: There was a predominance of female gender, right eye, congenital form, unilaterality, A-pattern, and an abnormal head posture type with a combination of chin up and head tilting. Bilateral form was observed only in female patients. Amblyopia was detected in two patients. Among patients aged >5 years, 40% had reduced stereopsis. Abnormal head posture was found in 60% of patients. More than half of them were diagnosed with a vertical and/or horizontal deviation. Tenotomy procedure eliminated the abnormal head posture in all patients and significantly improved the mean limitation of elevation in adduction and hypotropia (p=0.001, p=0.012). Two patients developed inferior oblique overaction in the operated eye. There was complete spontaneous resolution in two patients. Conclusions: The clinical features of patients with Brown syndrome in our study are considerably consistent with those of previous reports. The present study demonstrated the effectiveness of superior oblique tenotomy with less overcorrection in the surgical treatment of Brown syndrome.(AU)

RESUMO Objetivo: Definir mais detalhadamente as características clínicas específicas de pacientes com síndrome de Brown e avaliar os resultados da tenectomia do músculo oblíquo superior no manejo cirúrgico da síndrome de Brown. Métodos: Prontuários de 45 pacientes com síndrome de Brown foram analisados retrospectivamente. Onze pacientes submetidos à tenectomia do músculo oblíquo superior devido a postura anormal da cabeça ou a hipotropia e um paciente submetido ao alongamento bilateral do tendão do oblíquo superior com uma faixa de silicone devido a postura anormal da cabeça. Neste último paciente, a faixa de silicone foi removida no terceiro mês pós-operatório devido à ausência de melhora na postura anormal da cabeça e à limitação da elevação em adução. Quatro pacientes submeteram-se simultaneamente à cirurgia do músculo reto horizontal. Resultados: Houve predominância de sexo feminino, olho direito, forma congênita, acometimento unilateral, padrão em "A" e um tipo de postura anormal da cabeça combinando queixo elevado e inclinação da cabeça. A forma bilateral foi vista apenas em pacientes do sexo feminino. Foi constatada ambliopia em 2 pacientes. Dentre os pacientes acima de 5 anos de idade, 40% tinham estereopsia reduzida. Postura anormal da cabeça estava presente em 60% dos pacientes. Mais da metade dos pacientes foi diagnosticada com um desvio vertical, horizontal ou ambos. O procedimento de tenectomia eliminou a postura anormal da cabeça em todos os pacientes e melhorou significativamente a limitação média da elevação em adução e a hipotropia (p=0,001 e p=0,012). Dois pacientes desenvolveram hiperação do músculo oblíquo inferior no olho operado. Resolução completa ocorreu espontaneamente em 2 pacientes. Conclusões: O quadro clínico dos pacientes com síndrome de Brown no nosso estudo é bastante consistente com os relatos iniciais na literatura. Este estudo mostrou a eficácia da tenectomia do oblíquo superior, com menor hipercorreção no tratamento cirúrgico da síndrome de Brown.(AU)

Síndrome de flutter ocular e ataxia de tronco pós-infecciosa / The post-infectious ocular flutter syndrome and truncal ataxia

OBJETIVO: Descrever um quadro clínico subagudo pós-infeccioso caracterizado por ataxia de tronco e flutter ocular. RELATO DE CASO: Um homem de 37 anos previamente hígido, pouco após um quadro sistêmico inespecífico que se resolveu espontaneamente, deu início a movimentos involuntários hipercinéticos dos olhos, da cabeça e desequilíbrio importante. Os sintomas evoluíram em poucos dias. Ao exame, apresentava ataxia de tronco e presença de flutter ocular. O líquor mostrou pleocitose discreta. Ressonância magnética (RM) de crânio e exames laboratoriais normais. Houve melhora espontânea e total dos sintomas em cerca de três semanas. CONCLUSÃO: A síndrome de flutter ocular e ataxia de tronco é rara e é destacado a benignidade do quadro.

OBJECTIVE: To describe a post-infectious subacute clinical picture characterized by truncal ataxia and ocular flutter. CASE REPORT: A healthy 37-year-old man, a few days after spontaneously resolved nonspecific systemic disease, initiated involuntary hyperkinetic movements of the eyes, head, and major imbalance. The symptoms progressed within a few days. He presented ataxia of the trunk and presence of ocular flutter. CSF showed pleocytosis. Magnetic resonance of the skull and laboratory tests were normal. There was spontaneous and total improvement of symptoms in about three weeks. CONCLUSION: The ocular flutter syndrome and truncal ataxia is rare and the benignity of the condition is highlighted.

Ocular flutter following Zika virus infection.

Zika virus (ZIKV) is an emerging flavivirus which has been linked to a number of neurologic manifestations such as Guillain-Barré syndrome (GBS), transverse myelitis, and meningo-encephalitis. Ophthalmologic manifestations are increasingly being reported; however, ocular dyskinesias have not been described in this context to date. Herein, we report a case of a 22-year-old female who presented with ocular flutter and associated Guillain-Barré syndrome following acute ZIKV infection. We speculate that although such symptoms may have originated from a direct viral insult, a post-infectious autoimmune mechanism may not be excluded. Physicians should include ZIKV as well as other flaviviruses in their diagnostic workup for all patients with ocular flutter/opsoclonus, after excluding other non-infectious causes of central nervous system pathology. To the best of our knowledge, this is the first report on the association of ocular flutter, GBS, and ZIKV infection.

Response to correction of refractive errors and hypoaccommodation in children with congenital Zika syndrome.

PURPOSE: To describe the immediate response to correction of refractive errors and hypoaccommodation in children with congenital Zika syndrome (CZS). METHODS: Children born between May and December 2015 with a confirmed diagnosis of CZS and enrolled in a multidisciplinary early intervention program were included in this study. All children received a comprehensive ophthalmic examination, including dynamic retinoscopy and cycloplegic refraction. Children were prescribed their full correction if they met the criteria for refractive error, and additional plus 3.00 overcorrection for strabismus, accommodative dysfunction, and/or low vision. Monocular and binocular visual responses to Lea Grating Test at 30 cm, with and without eyeglasses, were measured on day 1 of glasses wear. RESULTS: A total of 60 children were evaluated (mean age at evaluation, 11.5 ± 1.1 months; range, 9.0-16.0 months). Lea Grating Test responses were abnormal in all children prior to spectacle correction. Hypoaccommodation was present in 17 of 21 children (81%). Overcorrection was prescribed for all children. Visual responses were subnormal even with glasses use; however, immediate improvement in binocular vision was found in 37 children (62%) and in 74 of 119 eyes (62.2%). For the monocular visual improvement, 27 of 115 eyes (23.5%) had structural abnormalities, and 44 of 115 eyes (38.3%) were structurally normal. There was a statistical difference between the cycloplegic refraction of the children in August and in November, including emmetropia (P = 0.001), hyperopia (P = 0.000), myopia (P = 0.007), and astigmatism (P = 0.004). CONCLUSIONS: Eyeglasses can improve visual acuity in children with CZS. Significant changes in their refractive status over time requires periodic updates.

Spinocerebellar ataxia type 2: Measures of saccade changes improve power for clinical trials

BACKGROUND:Saccadic eye movement abnormalities are common in patients with spinocerebellar ataxia type 2, but it is unclear how these alterations progress over time. The aim of this study was to assess the progression of saccade involvement in spinocerebellar ataxia type 2 patients, identify its main determinants, and evaluate its usefulness as outcome measures in clinical trials.METHODS:A prospective 5-year follow-up study was performed with 30 spinocerebellar ataxia type 2 patients and their matched healthy controls, who were evaluated a total of four times by clinical and electrooculographical assessments of horizontal saccades and by the scoring of ataxia.RESULTS:Patients showed significant decreases in saccade peak velocity and saccade accuracy as well as increases of saccadic latency during the follow-up period. Annual progression rates were significantly higher in patients compared to controls. Faster progression rates of saccade slowing were associated with higher trinucleotide cytosine-adenine-guanine repeat expansions. Sample-size estimates for two-arm trials would require 19 patients per group to detect a 50 percent reduction in disease progression using saccade peak velocity as outcome variable, but 44 and 124 patients using saccade latency and accuracy, respectively (power, 80 percent; alpha = 0.05).CONCLUSIONS:Electrooculographical measures of saccade changes are useful for the objective quantification of disease course in spinocerebellar ataxia type 2. The progression rate of saccade slowing is influenced by the expansion size, providing novel insight into the cumulative polyglutamine neurotoxicity, and supporting the usefulness of saccade peak velocity as a sensitive biomarker during the natural history of the disease, and as suitable outcome measure for therapeutic trials (AU)

Spinocerebellar ataxia type 2: Measures of saccade changes improve power for clinical trials.

BACKGROUND: Saccadic eye movement abnormalities are common in patients with spinocerebellar ataxia type 2, but it is unclear how these alterations progress over time. The aim of this study was to assess the progression of saccade involvement in spinocerebellar ataxia type 2 patients, identify its main determinants, and evaluate its usefulness as outcome measures in clinical trials. METHODS: A prospective 5-year follow-up study was performed with 30 spinocerebellar ataxia type 2 patients and their matched healthy controls, who were evaluated a total of four times by clinical and electrooculographical assessments of horizontal saccades and by the scoring of ataxia. RESULTS: Patients showed significant decreases in saccade peak velocity and saccade accuracy as well as increases of saccadic latency during the follow-up period. Annual progression rates were significantly higher in patients compared to controls. Faster progression rates of saccade slowing were associated with higher trinucleotide cytosine-adenine-guanine repeat expansions. Sample-size estimates for two-arm trials would require 19 patients per group to detect a 50% reduction in disease progression using saccade peak velocity as outcome variable, but 44 and 124 patients using saccade latency and accuracy, respectively (power, 80%; alpha = 0.05). CONCLUSIONS: Electrooculographical measures of saccade changes are useful for the objective quantification of disease course in spinocerebellar ataxia type 2. The progression rate of saccade slowing is influenced by the expansion size, providing novel insight into the cumulative polyglutamine neurotoxicity, and supporting the usefulness of saccade peak velocity as a sensitive biomarker during the natural history of the disease, and as suitable outcome measure for therapeutic trials.

Fundamentos para una plataforma nacional de interconsulta digital en trastornos de la motilidad ocular / Foundations for a national platform of digital consultation related to eye movement disorders

Se realizó estudio descriptivo de 187 pacientes recibidos en atención terciaria de Neuroftalmología, desde fuera de la capital, por trastornos de la motilidad ocular, entre enero/2010 y diciembre/2011, con el objetivo de caracterizar las interconsultas físicas por trastornos de la motilidad ocular solicitadas a servicios de Neuroftalmología de nivel terciario. Las variables estudiadas describieron particularidades epidemiológicas, origen y finalidad de las remisiones. Se evaluó además la posibilidad de interconsulta digital como opción alternativa en cada uno de los casos y sus beneficios potenciales. El 63,1 por ciento de las remisiones procedieron desde fuera de la región occidental, donde se encuentran enclavados los dos centros de atención terciaria de la especialidad. La distancia global recorrida por remisiones físicas fue de 70 286,9 Km. Del total de remisiones realizadas, 131 (70,1 por ciento) fueron clasificadas tributarias de interconsulta digital, lo que representaba una reducción de 54 823,2 Km en términos de distancia global recorrida. Las 56 remisiones restantes pudieron beneficiarse de interconsulta previa a la remisión física según criterio de experto, fundamentalmente para inicio de tratamiento e indicación de estudios complementarios. La Telemedicina aplicada a los trastornos de la motilidad ocular constituye una realidad necesaria y posible con los recursos humanos y estructurarles actuales con los que cuenta la especialidad en el país(AU)

This paper presents a descriptive study in 187 patients with eye movement disorders, received in Third-level Neurophthalmology Units, from far outside Havana, between January/2010 and December/2011, in order to characterize office consultation related to eye movement disorders in Third-level Neurophthalmology Units. Various subjects connected to epidemiological elements, genesis and purpose of the office evaluation were studied. The possibility and advantages of digital consultation were evaluated in each case. Interconsultation from outside the west region, where are both Third-level Neurophthalmology Units, were 63,1 percent. Total traveled-length owing to office evaluation was 70 286, 9 Km. 131 (70,1 percent) patients were classified as candidate for digital cases, reducing 54 823,2 Km the global traveled-length. For the remaining 56 cases, telemedicine consultation previous to the office one could be helpful according to the expert consensus, mainly to begin treatment or to do complementary tests. Telemedicine for eye movement disorders is in fact necessary. It is also possible oriented to all people and structural tools that have the specialty around the country(AU)

Saccade velocity is reduced in presymptomatic spinocerebellar ataxia type 2.

OBJECTIVE: A characteristic feature of spinocerebellar ataxia type 2 (SCA2) is saccadic slowing at early disease stages. We sought to determine whether this sign is detectable before clinical manifestation and quantifies the disease progression throughout life in linear fashion. METHODS: In a specialized ataxia clinic, 54 presymptomatic carriers of SCA2 polyglutamine expansions and 56 relatives without mutation were documented with regard to their maximal saccade velocity (MSV). RESULTS: Among the control individuals, a significant effect of aging on MSV was observed. After elimination of this age influence through a matched-pair approach, a presymptomatic decrease of MSV could be shown. The MSV reduction was stronger in carriers of large expansions. In the years before calculated disease manifestation, the MSV impairment advanced insidiously. CONCLUSION: Saccade velocity is a sensitive SCA2 endophenotype that reflects early pontine degeneration and may be a useful diagnostic parameter before the onset of ataxia. SIGNIFICANCE: Future neuroprotective therapies of polyglutamine neurodegeneration may be assessed by MSV from earliest to prefinal disease stages.

Outpatient evaluation of vision and ocular motricity in 123 children with cerebral palsy.

PURPOSE: Children with cerebral palsy (CP) may present varied ophthalmological problems. Paradoxically, however, investigation of visual function and possible related disorders is not routinely conducted in this population during medical consultations. METHOD: This paper proposes a simple, practical guide for assessing vision and ocular motricity in children with CP. One-hundred and twenty-three patients (mean age: 8.4 years +/- 2.3) from the Sarah Network of Rehabilitation Hospitals (Brasilia, Rio de Janeiro and São Luis units) were studied. Various parameters were investigated (smooth pursuit movements, strabismus, visual acuity, visual field, visuospatial neglect and nystagmus) using the following assessment tools: adapted smooth pursuit test for ocular motricity; Snellen test for visual acuity; confrontation method for visual field; cancellation test (star test) for visual neglect; and cover test for strabismus. Nystagmus was assessed based on patient observation. RESULTS: Eighty-one children completed all the evaluations. Among these, 38 (47%) had no visual problems (hemiplegia 60.5%; diplegia 41.4%; triplegia 12.5%; mixed tetraplegic 33.3%); 23 had difficulties in one of the tested items; and 20 had problems in two or more areas of vision. CONCLUSIONS: Visual and oculomotor tests are of significant importance in children with CP and provide relevant information for creating a rehabilitation programme aimed at the individual as a whole.

Can oculomotricity be altered in patients with tinnitus only? A preliminary study.

The study of oculomotricity is performed by evaluating three systems: saccadic ocular movements (SOMs), optokinetic nystagmus (OKN), and smooth pursuit eye movements (SPEMs). Our aim was to study oculomotricity in patients with a complaint of only tinnitus and to compare it with the value of our control group. We studied the SOMs, OKN, and SPEMs in 25 patients complaining only about tinnitus and in 35 normal adults and compared the results. The data analysis showed a significant difference in the value of the SOMs and SPEMs between the two groups. Sensorineural tinnitus can originate in the organ of Corti, in the cochlear nerve, or in the auditory pathways of the central nervous system. The auditory cortex connects with visual areas and with the superior colliculus. The latter structure is involved in the origin of SOMs and OKN. In our study, we found an increased delay in saccadic tests. In the SPEMs, we observed an increase in the degree of distortion, and a reduction in the gain. This outcome is in accordance with the literature. However, we detected a few alterations in the OKN, and this finding is in partial agreement with the studies analyzed. Alterations in oculomotricity can indicate involvement of the central nervous system in patients with a complaint of only tinnitus.

Torsiones oculares: seudohiperfunción de los oblicuos inferiores / Ocular torsions: inferior oblique overaction

Se plantea que la extorsión bilateral produce modificaciones globales de las acciones de los músculos rectos que pueden determinar el desarrollo de una desviación tipo -V- y simular una sobreacción de los oblicuos inferiores, con elevación en adducción. El análisis del siguiente caso clínico sugiere los planteamientos anteriores. Se trata de una paciente con endotropia alternante, hipermetropia alta y astigmatismo, con doble elevación en addución con lentes, la cual desaparece al retirarlos. Se obtiene ortoposición postoperatoria, con lentes persiste la elevación en addución. El cuadro se mantiene estable desde los 6 años (fecha de la operación) hasta los 32 (último control). El diagnóstico de extorsión se hizo mediante el test de Maddox modificado y fotografías del fondo de ojo, la cual puso en evidencia una extorsión de 15º en cada ojo en PPM y una disminución total de 4º durante la adducción. Esta pérdida de extorsión produce una reconversión del movimiento horizontal en elevación con modificación de los planos de acción de los rectos medios y upshoot en adducción, sin sobreacción de los oblicuos inferiores, los cuales no se contraen en las miradas horizontales.

Bilateral ocular extorsion may produce global modifications on recti eye muscles action with V pattern strabismus and bilateral upshoot, with apparent inferior oblique overaction. The report of the following case is supportive of this concept. We present a patient having alternant esotropia, high hyperopia and astigmatism, with bilateral upshoot with glasses, that disappears without them. The patient is orthophoric after surgical treatment. This patient remain in the same condition from the age of six (when she was operated on) until today (32 years old). The double red Maddox lenses and fundus photography were used to reveal a 15º bilateral extorsion diagnosis, and the further loss of abnormal torsion in extreme adduction. This degree of torsional change is converted in hyperdeviation, explaining the upshoot in adduction without any inferior oblique muscle overaction.

Síndrome de Fells-Waddel / Waddell Fells syndrome

Objetivo: Describir la fenomenología, génesis y alteraciones musculares del Síndrome de Fell-Waddel (FW) como parte de los desórdenes inervacionales congénitos. Se presentan dos casos. Método: Se realizó un análisis de la genética del rombencéfalo para comprender las posibles alteraciones del FW. Se analizaron dos casos, a los que se realizó la cirugía bajo anestesia tópica para el análisis de las funciones musculares. Resultados: En ambos casos se demostró la restricción en las diferentes rotaciones oculares, siendo mayor en el adulto. El debilitamiento de los rectos inferiores provocó una exotropia importante al frente, la divergencia en el intento de depresión y convergencia en el intento de elevación que tuvo sólo leve mejoría con el debilitamiento de los oblicuos superiores, en el segundo caso se analiza con cirugía bajo anestesia tópica su persistencia con los oblicuos superiores desinsertados. Conclusiones: Considerando la anomalía reportada de la división superior del III nervio en las disgnesias congénitas, podemos atribuir los cambios presentados en ambos casos a inervaciones aberrantes de la división inferior del III nervio craneal a los rectos laterales lo que correspondería con la divergencia e el intento de depresión, y a su vez la convergencia en el intento de elevación seria secundaria a la orden inhibitoria a los rectos laterales, quedando funcionando los restos mediales. El debilitamiento de los rectos inferiores, cirugía más frecuente en los síndromes de fibrosis de músculos extraoculares tipo I provocará una exotropia al frente que debe ser corregida.

Objective: To describe clinical characteristics, genetic and muscular anomalies in Fells-Waddel syndrome (FW), a congenital cranial dysinnervation disorder. Method: We analyzed the genetics of rhombencephalon to explain alterations in FW. We present two cases of FW which underwent surgery where orbital alterations were demonstrated. Topical anesthesia was used in the second case for analysis of muscular functions. Results: Restriction was demonstrated in both cases, but it was worse in the adult. Recession of the inferior rectus caused a big exotropia. Divergence in depression and convergence in elevation were improved only partially after recession of superior oblique. In case 2, the patient was alert under topical anesthesia, and while these muscles were free, the anomalies persisted. Conclusions: There is an anomaly of superior division of III nerve in these cases, so these movements can be secondary to aberrant innervations of inferior division of the III nerve to the lateral rectus, this can explain divergence during depression. Convergence in elevation is explained because of the inhibitory effect on the lateral rectus while de medial rectus are still functional. Recession of inferior rectus is the most common surgery in these cases, which will cause a big exotropia that must be corrected.

Síndrome de Brown bilateral / Bilateral Brown syndrome

Objetivo: El síndrome de Brown (SB) se caracteriza por restricción activa y pasiva de la elevación en adducción. Puede ser congénito o adquirido, intermitente o permanente, uni o bilateral; este último se presenta en el 10 por ciento de los casos. Nuestro objetivo es describir 9 casos de SB bilateral y revisar la literatura al respecto. Pacientes: se describen características clínicas de 9 casos y se revisa la literatura. Resultados: De los 9 casos, 2 eran de sexo masculino, y 7 de sexo femenino. Edad promedio: 5.7 años. 1 caso intermitente. Los casos publicados en diferentes series son: 13/126 (Brown), 3/8 (Clark y Noel), 7/36 (Waddell ) y 1/30 (Eustis). Entre los casos bilaterales hay alta incidencia de casos familiares. Conclusión: Se confirma que el cuadro es más común en mujeres. 7/9 tenía alineamiento en posición primaria. Todos tenían déficit bilateral de elevación y anisotropía en V. Frente a un caso de SB bilateral es conveniente examinar a los familiares en busca de casos asintomáticos.

Objective: The motility defect known as Brown´s síndrome is characterized by active and passive restriction of elevation in adduction. It may be either congenital or acquired, intermittent or permanent, uni or bilateral. It is bilateral in 10 percent of cases. Our purpose is to report 9 cases with bilateral Brown´s syndrome and review the cases previously published. Patients: 9 cases are described: 2 males and 7 females, average age 5,7 years who had bilateral Brown´s syndrome and review. Results: 9 cases are described: 2 males and 7 females, average age 5,7 years who had bilateral Brown´s syndrome, 1 of them was intermittent. Bilateral cases published are: 13/126 in Brown´s series, 3/28 in Clark and Noel´s, 7/36 in Waddell´s and 1/30 in that of Eustis. Among bilateral cases there is a striking incidence of familial cases. Conclusion: Brown´s statement that the disorder is more common in females than in males is confirmed in our series. It should be noted that 7 out of 9 of these patients had normal alignment in primary gaze. All cases had bilateral elevation deficit and V pattern. In bilateral cases examination of the relatives should be performed in order to detect asymptomatic familial cases.

Vertebro-basilar syndromes causing oculo-motor disorders.

PURPOSE OF REVIEW: All functional classes of eye movements require exquisite coordination between cortical, basal ganglia and brainstem centers involved in ocular motor control. Vertebrobasilar stroke may produce a wide spectum of isolated or combined eye-movement disorders. The intent of this article is to summarize the curent knowledge of eye-movement disorders occurring in infarcts involving the different arterial territories of the vertebrobasilar circulation. RECENT FINDINGS: In the last few years there has been an improvement in our understanding of pathophysiological mechanisms that are related mainly to abnormal vergence disorders due to thalamic-subthalamic infarcts, as well as of the peculiar symptoms resulting from otolith pathway involvement occurring in lateral medullary infarcts. Moreover, progress in neuroimaging technology has implicated neurovascular contact of the trochlear nerve in instances of superior oblique myokimia. SUMMARY: Eye-movement disorders commonly occur in vertebrobasilar stroke, although they are often unappreciated. They may make it possible to establish specific anatomical correlates, as well as the probable nature of the underlying pathology.

Palpebral fissure height and downgaze in patients with Graves upper eyelid retraction and congenital blepharoptosis.

PURPOSE: To compare the relation between vertical eyelid fissure height and downgaze in healthy subjects and in patients with Graves upper eyelid retraction and congenital blepharoptosis. METHODS: With a caliper rule, the authors measured the vertical eyelid fissure height of 25 patients with no previous history of eye disease, 34 patients with Graves disease, and 16 patients with congenital blepharoptosis. All measurements were done at the primary position and at four levels of downgaze (10 degrees, 20 degrees, 30 degrees, and 40 degrees). In 13 patients with Graves disease, vertical phoria was quantified with a Maddox rod and prism. RESULTS: The relation between vertical eyelid fissure height and downgaze is linear. The slope was identical for the control subjects and for the patients with Graves disease (b = -1.36) but it was almost zero for the patients with congenital blepharoptosis (b = -0.037). When vertical eyelid fissure height was corrected to arc values, the slope of the relation between vertical eyelid fissure height and downgaze was significantly greater for the patients with Graves disease. Vertical phoria was not related to the amount of Graves upper eyelid retraction. CONCLUSIONS: The data support the hypotheses that upper eyelid retraction secondary to Graves eye disease results from hyperactivity of the levator palpebrae superioris muscle.

Peripheral ocular motor disorders.

In the reviewed period, articles on peripheral eye movement disorders covered interesting aspects. Localizing value of associated signs, repetitive presentations of palsies, and classical quotations are stressed for the oculomotor nerve. The superior oblique is correlated to central nervous system disorders when overacting in pediatric patients or when ocular torsion is matched to the perceived vertical tilt. The family "pseudo" brought two of its members: "pseudo" myasthenia and "pseudo" myotonia. Mitochondrial citopathies with ocular manifestations can overlap among the different clinical types, eg, Kearns-Sayre, MELAS (mitochondrial encephalopathy-lactic acidosis and strokelike episodes), MERFF (myoclonic epilepsy and ragged red fibers). The diagnostic value of DNA mutations is emphasized in those syndromes. Imaging of the carotid arteries provides useful hints in cases where the lumen is narrowed due to internal processes or external compression; its interpretation is not only of diagnostic but of prognostic value. Certain otorhinolaryngology surgical procedures can damage the orbital muscles and produce serious inconvenience to the ocular motility. Analyzing the involved structures the therapeutic gesture can be determined. Diplopia after cataract surgery or retinal detachment repair is due to different factors, anesthetics, or implant location and is implied in every case.