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.

Ptose miogênica na distrofia muscular oculofaríngea / Miogenic ptosis in oculopharyngeal muscular dystrophy

RESUMO Relato de caso de distrofia muscular oculofaríngea, doença genética de herança autossômica dominante e uma das causas de ptose miogênica adquirida. A paciente apresentou quadro de ptose palpebral bilateral e disfagia, achados clínicos típicos da doença, foi submetida a tratamento cirúrgico da ptose, com bom resultado estético e funcional.

ABSTRACT The authors report a case of oculopharyngeal muscular dystrophy, an autosomal dominant genetic disease, which leads to miogenic ptosis. This patient presented bilateral palpebral ptosis and dysphagia and underwent ptosis surgical treatment, with a good functional and aesthetic result.

Skew deviation e ocular tilt reaction versus paralisia do nervo troclear / Skew deviation and ocular tilt reaction versus trochlear nerve palsy

Skew deviation é o desalinhamento vertical dos olhos causado por disfunção unilateral no tronco cerebral, cerebelo ou sistema vestibular periférico, comprometendo as vias por onde transitam impulsos dos órgãos otolíticos aos núcleos oculomotores. Está frequentemente associado a torção ocular e inclinação da cabeça, compondo, assim, a ocular tilt reaction. Neste artigo os autores ressaltam aspectos históricos, fisiológicos, etiopatogênicos e clínicos que contribuem para caracterizar ambas as condições, além da diferenciação clínica com a paralisia do nervo troclear.

Skew deviation is the vertical misalignment of the eyes caused by unilateral dysfunction in the brain stem, cerebellum or peripheral vestibular system, resulting imbalance in otolith input to the oculo-motor system. It's often associated with ocular torsion and head tilt, which together comprise the ocular tilt reaction. In this article the authors emphasize the historical, physiological, etiopathogenesis and clinical aspects that contribute to characterize both conditions, and help to differentiate them clinically from trochlear nerve palsy.

Opsoclonus-mioclonus: uma manifestação clínica aguda de neuroblastoma / Opsoclonus-myoclonus: an acute clinical neuroblastoma's manifestation

Objetivo: Descrever o caso de uma criança do sexo masculino que apresenta sintomas de forma súbita de Opsoclonus-mioclonus associada a tumor da crista neural. Descrição: Os autores relatam um caso de uma criança do sexo masculino de 1 ano e 10 meses que apresenta, subitamente, instabilidade de tronco e membros, tremores e ataxia. Três dias depois aparece opsoclonus, caracterizando a Síndrome de Opsoclonus-mioclonus. Uma massa tumoral retroperitoneal compatível com neuroblastoma foi encontrada em tomografia computadorizada de abdome. Discussão: Quando é feito o diagnóstico de Opsoclonus-mioclonus, a pesquisa de tumores deve periodicamente ser pesquisada, uma vez que estes podem se manifestar agudamente ou tardiamente...

Objective: To describe the case of a male child who presents sudden symptoms of Opsoclonus-myoclonus associated to a neural crest tumor. Description: The authors report a case of a 22-month-old male child that suddenly presents instability from trunk and limbs, trembling and ataxia. Three days later, the opsoclonus appeared and opsoclonus-myoclonus Syndrome was diagnosed. A retroperitoneal tumor, compatible with neuroblastoma was found through an abdominal computed tomography scan. Discussion: When the Opsoclonus-myoclonus is diagnosed, the search for tumors must persist periodically, since these tumors can present acute or late manifestation...

A Case of Acquired Brown Syndrome after Surgical Repair of a Medial Orbital Wall Fracture

A case of acquired Brown syndrome caused by surgical repair of medial orbital wall fracture is reported in the present paper. A 23-year-old man presented at the hospital with right periorbital trauma. Although the patient did not complain of any diplopia, the imaging study revealed a blow-out fracture of the medial orbital wall. Surgical repair with a calvarial bone autograft was performed at the department of plastic surgery. The patient was referred to the ophthalmologic department due to diplopia that newly developed after surgery. The prism cover test at distant fixation showed hypotropia of the right eye, which was 4 prism diopters (PD) in primary gaze, 20 PD in left gaze, while orthophoric in right gaze. Eye movement of the right eye was markedly limited on elevation in adduction with normal elevation in abduction with intorsion in the right eye present. Forced duction test of the right eye showed restricted elevation in adduction. Computerized tomography scan of the orbits showed the right superior oblique muscle was entrapped between the autografted bone fragment and posterior margin of the fracture. When repairing medial orbital wall fracture that causes Brown syndrome, surgeons should always be careful of entrapment of the superior oblique muscle if the implant is inserted without identifying the superior and posterior margin of the orbital fracture site.

Presumed Metastasis of Breast Cancer to the Abducens Nucleus Presenting as Gaze Palsy

A 51-year-old woman with breast cancer presented with progressive diplopia. Neuro-ophthalmologic examination revealed right gaze palsy and peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) was normal. However, two months later a repeat brain MRI revealed an enhancing round nodular mass at the right facial colliculus of the lower pons, at the location of the abducens nucleus. Localized metastasis to the abducens nucleus can cause gaze palsy in a patient with breast cancer.

Regeneração aberrante do nervo oculomotor secundária a aneurisma intracraniano: relato de caso / Aberrant regeneration of the oculomotor nerve followed by intracranial aneurysm: case report

Relatar um caso de regeneração aberrante secundária à paralisia aguda do nervo oculomotor causada por aneurisma intracraniano. Paciente atendida em fevereiro de 2006 queixando-se de dor de cabeça acompanhada de visão dupla e queda da pálpebra no olho direito. Na avaliação da motilidade ocular extrínseca, verificou-se incapacidade da adução, da supradução e da infradução associada à blefaroptose no olho direito. Com relação à motilidade intrínseca, midríase paralítica no olho direito. Formulou-se diagnóstico de paralisia aguda de nervo oculomotor no olho direito e solicitou-se avaliação neurológica. No Departamento de Neurocirurgia, após ser diagnosticada presença de aneurisma de artéria comunicante posterior, a paciente foi submetida a tratamento cirúrgico. Em dezembro de 2006, observou-se melhora relativa da adução, mantendo a incapacidade da supradução e da infradução com blefaroptose melhorada à adução do olho direito. Com relação à motilidade intrínseca, miose no olho afetado. O diagnóstico de regeneração aberrante do nervo oculomotor pós-paralisia aguda foi formulado baseando-se na anamnese e nos exames oftalmológicos seqüenciais.

To report a case of aberrant regeneration followed by acute palsy of the oculomotor nerve caused by intracranial aneurysm. A 59-year-old patient was attended in February 2006 complaining of headache with diplopia and blepharoptosis in the right eye. At the external ocular motility exam. Aduction, supraduction and infraduction defects with blepharoptosis in the right eye were observed. Regarding the internal ocular motility, mydriasis in the right eye. Acute palsy of the oculomotor nerve in the right eye was diagnosed and neurological examination was requested. At the Department of Neurosurgery, after having diagnosed aneurysm of the posterior communicating artery, the patient was submitted to an operation. In December 2006, it improvement of the aduction was observed, supraduction and infraduction defects remained and blepharoptosis improved during aduction of the right eye. In the internal ocular motility, miosis in the affected eye. The diagnosis of the aberrant regeneration of the oculomotor nerve after acute palsy was formulated based on anamnesis and ophthalmological follow-up tests.

Panayiotopoulos syndrome.

Panayiotopoulos syndrome is a benign childhood epileptic illness characterized by episodic autonomic symptoms. We present a 9 year old boy with this condition, who presented with episodes of severe vomiting, fever, deviation of eyes and altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroradiologic and metabolic investigations.

Double elevator palsy, subtypes and outcomes of surgery

To describe the clinical manifestations of subtypes of double elevator palsy and to report the outcomes of surgery in these patients. This retrospective study was conducted on hospital records of patients with double elevator palsy at Labbafinejad Medical Center over a ten-year period from 1994 to 2004. Patients were classified into three subgroups of primary elevator muscle palsy [9 subjects], primary supranuclear palsy with secondary inferior rectus restriction [4 subjects] and pure inferior rectus restriction [7 subjects] according to forced duction test [FDT], force generation test [FGT] and Bell's reflex. Patients in the first group underwent Knapp procedure, the second group received Knapp procedure and inferior rectus recession simultaneously and in the third group vertical recess-resect or mere inferior rectus recess operation was performed. Success was defined as final residual deviation of 5 PD or less and 25% improvement or more in restriction after all operations. Overall 20 subjects including 10 male and 10 female patients with mean age of 12.6 +/- 9.3 [range 1.5-32] years were operated during the mentioned period which included 9 cases of primary elevator muscle palsy, 4 patients with primary supranuclear palsy and secondary inferior rectus restriction, and 7 subjects with pure inferior rectus restriction. Mean follow-up was 22.0 +/- 20.0 [range 3-63.5] months. Mean pre and post-operative deviation was 32.0 +/- 8.0 PD and 3.8 +/- 8.0 PD [P < 0.001] respectively, and mean restriction before and after the operation[s] was -3.5 +/- 0.7 and -2.3 +/- 1.2 [P < 0.001], respectively. Success rate was 77% for correction of deviation and 80% for improvement in muscle restriction. Surgery for double elevator palsy must be individualized according to FDT, FGT and Bell's reflex. The outcomes are favorable with appropriate surgical planning

Evaluation of the "spasticity of conjugate gaze phenomenon" in unilateral cerebral lesions

The spasticity of conjugate gaze phenomenon (SCG) is a subtle motor ocular sign that usually indicates unilateral acute cerebral lesion. We analyzed the sensitivity of this sign in a prospective blinded study using 57 patients. All patients had monohemispheric brain lesions without resting deviation of the eyes and no motor complaints. Fourteen individuals without cerebral lesions were included as controls. Patients and controls were submitted to a brain magnetic resonance image. We observed SCG in just 1/57 (sensitivity of 1.7 percent), while all controls had a normal Bell's phenomenon. We speculate that SCG may disappear over time and is not useful to diagnose a long-lasting unilateral brain lesion.

O fenômeno do "desvio conjugado espástico ocular" é um sinal motor ocular sutil que habitualmente indica lesões cerebrais mono-hemisféricas agudas. Avaliamos a sensibilidade deste sinal num estudo prospectivo cego em 57 pacientes. Todos os pacientes apresentavam lesões cerebrais mono-hemisféricas, ausência de queixas motoras ou de desvio conjugado dos olhos em repouso. Quatorze indivíduos sem lesão cerebral foram incluídos como controle. Pacientes e indivíduos controle foram submetidos a exame de ressonância magnética do crânio. Observamos o sinal do desvio conjugado espástico ocular em somente 1/57 pacientes (sensibilidade 1,7 por cento), enquanto todos os controles apresentaram o fenômeno de Bell. Especulamos que este sinal desaparece com o tempo, e que o mesmo não é útil para o diagnóstico de pacientes com lesões cerebrais monohemisféricas de longa duração.

Síndrome de Brown bilateral associada com hipermobilidade articular benigna: relato de caso / Bilateral Brown's syndrome associated with benign joint hypermobility: a case report

A síndrome de Brown é caracterizada por grande limitação de elevação em adução, elevação ligeiramente diminuída ou normal na abdução, anisotropia em "Y" ou "V", intorção do olho em supraversão e ducção forçada positiva. Sua causa se deve à inelastibilidade do músculo oblíquo superior ou por sua contenção em sua própria bainha. A hipermobilidade articular benigna é doença hereditária do tecido conectivo caracterizada por aumento da mobilidade em diversas articulações. Sua prevalência é muito variável em relação à idade, sexo e etnia, variando de 2 a 35 por cento em homens e de 5 a 57 por cento e mulheres. Neste relato os autores descrevem um caso de síndrome de Brown associada com hipermobilidade articular benigna e atentam para a associação pouco referida na literatura. J.C.S, masculino, 6 anos de idade, pardo, estudante, foi encaminhado à Universidade de Santo Amaro com queixa de exotropia há dois anos que aumentava na supraversão. Paciente com o diagnóstico de síndrome de Brown bilateral teve o diagnóstico de hipermobilidade articular benigna pelo Reumatologista. O paciente com hipermobilidade articular benigna pode desenvolver sintomas articulares como artralgia devido a uma inflamação articular. Acreditamos na possibilidade de que síndrome de Brown possa ter ocorrido devido a processo inflamatório na tróclea que teve início devido a hipermobilidade articular benigna.

Brown's syndrome is characterized by a limitation of elevation in adduction, slight or normal limitation of elevation in abduction, divergence in straight upgaze (V-pattern), intorsion in upgaze and positive forced duction. It is caused by a tight or inelastic superior oblique tendon. Benign joint hypermobility is a hereditary disease of the connective tissue characterized by an increase of mobility in diverse joints. Its prevalence is very changeable regarding age range, sex and ethnicity, varying from 2 to 35 percent in men and 5 to 57 percent in women. In this case the authors describe a case of Brown's syndrome associated with Benign joint hipermobility and call attention to a little described association in the literature. J.C.S, male, 6 years old, mulatto, student, was referred to the University of Santo Amaro with complaint of exotropia for 2 years that it increased in supraversion. The patient with the diagnosis of bilateral Brown's syndrome, was diagnosed as having benign joint hipermobility by the reumatologist. The patient with benign joint hipermobility can develop symptoms such as arthralgia caused by a joint inflammation. We believe in the possibility that Brown syndrome has occurred, caused by an inflammatory process in the trochlea that started because of the benign joint hypermobility.

Antielevation Syndrome after Unilateral Anteriorization of the Inferior Oblique Muscle

PURPOSE: To report antielevation syndrome with restriction of elevation on abduction in the operated eye and overaction (OA) of the inferior oblique muscle (IO) of the contralateral eye after unilateral IO anteriorization (AT). METHODS: Medical records were reviewed retrospectively in 8 of 24 patients who underwent unilateral IOAT. Four patients were referred from other hospitals after the same surgery. RESULTS: Four patients had infantile esotropes. The rest showed accommodative esotropia, superior oblique palsy, exotropia, and consecutive exotropia. The mean amount of hyperdeviation was 16.3 PD (10~30). The mean restriction of elevation on abduction in the operated eye was -1.6 (-1~-4) and IOOA of the contralateral eye was +2.7 (+2~+3). IOAT of nonoperated eyes in 4 patients, IO weakening procedure of anteriorized eyes in 2 patients, and IO myectomy on an eye with IOAT in 1 patient were performed. Ocular motility was improved after surgery in all patients. CONCLUSIONS: Unilateral IOAT may result in antielevation syndrome. Therefore bilateral IOAT is recommended to balance antielevation in both eyes. A meticulous caution is needed when performing unilateral IOAT.

Síndrome de Joubert y signo del molar en el conjunto malformativo cerebelo-óculo-renal en dos pacientes / Joubert Syndrome and molar sign in the renal-ocular-cerebelar complex in two patients

El síndrome de Joubert (SJ) es una afección autosómica recesiva caracterizada por taquipnea neonatal episódica, anormalidades oculomotoras (apraxia oculomotora, nistagmus, estrabismo), hipotonía desde el nacimiento con posterior aparición de ataxia, retardo madurativo, deficiencia mental y algunos rasgos faciales distintivos. Es clínicamente heterogéneo presentando algunos pacientes amaurosis congénita de Leber, nefronoptisis y/o enfermedad renal medular quística. Existe igualmente heterogeneidad genética. Las imágenes de resonancia magnética revelan hipoplasia/aplasia de vermis, prominencia y elongación de los pedúnculos cerebelosos superiores y fosa interpeduncular ensanchada, evocando conjuntamente la silueta de una muela o “signo del molar”. También se evidencian alteraciones morfológicas del 4º ventrículo, que adquiere forma de “alas de murciélago”. El SJ es incluido actualmente en el espectro malformativo de síndromes cerebelo-óculo-renales (SCOR). Objetivo: Presentar los casos clínicos de dos pacientes con SJ diagnosticado por hallazgos clínicos y resonancia magnética, y revisar los aportes de las recientes investigaciones genéticas.

Atypical presentation of a unilateral septic thrombosis of the cavernous sinus.

A 7 year-old boy with unilateral septic cavernous sinus thrombosis following a pyoderma in the eyebrow is described. Problems in diagnosis and the role of magnetic resonance imaging (MRI) in the management are highlighted. Patient recovered with mild residual sequelae. The unique feature of this case was absence of chemosis and non-toxic presentation of the patient.

Ocular Motility Disturbances in Orbital Wall Fracture Patients

It is difficult to identify the exact cause of ocular motility disturbances in orbital wall fracture patients. By performing CT and ocular motility tests before and after surgery, this study analyzes the functions of the extraocular muscles and determines correlations between the results. Between February 2001 and January 2003, 45 eyes of 45 patients with orbital wall fractures, whose medical records could be traced back at least 6 months, underwent surgical repair in our hospital. All variables were analyzed using the independent t-test, paired t-test, and Chi-square test. There was no significant difference in the location and degree of fracture and the incarceration pattern of 6 patients who had moderate or severe diplopia, and of the remaining patients 6 months after surgery. However, in the case of diplopia, the sum of ocular motility limitation was 5.67 +/- 4.18, and the degree of extraocular motility disturbance was 3.67 +/- 2.42 before surgery. When there was no diplopia, the sum of ocular motility limitation was 1.13 +/- 1.38, and the degree of extraocular motility disturbance was 1.08 +/- 1.16 (p < 0.005, independent t-test). Ocular movement was successfully recovered by surgical reduction within 3 weeks from trauma. Postoperative ocular motility disturbance was more related to various ocular motility test results than CT findings. Ocular motility disturbances can remain after surgery if ocular motility limitation and extraocular motility disturbance are significant after trauma. Additional studies on the various tests to examine functions of extraocular muscles are required to identify and analyze the exact cause of ocular motility disturbance.

Acquired Simulated Brown Syndrome following Surgical Repair of Medial Orbital Wall Fracture

Simulated Brown syndrome is a term applied to a myriad of disorders that cause a Brown syndrome-like motility. We encountered a case of acquired simulated Brown syndrome in a 41-year-old man following surgical repair of fractures of both medial orbital walls. He suffered from diplopia in primary gaze, associated with hypotropia of the affected eye. We performed an ipsilateral recession of the left inferior rectus muscle as a single-stage intraoperative adjustment procedure under topical anesthesia, rather than the direct approach to the superior oblique tendon. Postoperatively, the patient was asymptomatic in all diagnostic gaze positions.