A Case of Acquired Ocular Motor Apraxia Accompanied with Esotropia due to Multiple Brain Infarcts

PURPOSE: We present a case of acquired ocular motor apraxia accompanied with esotropia due to multiple brain infarcts. CASE SUMMARY: A 59-year-old male was referred for diplopia that started 9 years before presentation and continued after multiple brain infracts including right cerebellum, right occipital lobe, medulla oblongata and inferior pons. At initial examination, his best corrected visual acuity was 20/22 in the right eye and 20/25 in the left eye and he had 12 prism diopter (PD) esotropia at distance and near in primary gaze with correction. His duction and version were normal; however, his horizontal saccade was notably decreased. Two years and 8 months after presentation, the patient had 15 PD esotropia at distance and near with correction. His duction and version were normal and vertical saccadic eye movements were observed. However, horizontal saccade disappeared. The patient also exhibited a distinguishing head thrust following the order for saccadic eye movement. He was diagnosed with an acquired ocular motor apraxia accompanied with esotropia. During the follow-up period the patient underwent bilateral recession of the medial rectus. The usual diplopia and his horizontal esodeviation improved to 3 PD of esotropia at distance. CONCLUSIONS: Multiple brain infarcts can result in an acquired ocular motor apraxia accompanied with esotropia. Varying types of ocular motor disorders should be considered in patients with a previous medical history of brain infarct.

Congenital Ocular Motor Apraxia: Clinico-Radiological Analyses

BACKGROUND: Congenital ocular motor apraxia (COMA) is characterized by impaired voluntary saccades and abnormal head thrusts. However, mechanism of this disorder remains to be elucidated. METHODS: This study analyzed the eye movements and imaging findings in 16 patients with COMA, who had been recruited from 2003 to 2009 at the Neuro-Ophthalmology Clinic of Seoul National University Bundang Hospital. RESULTS: All the children showed impaired saccades and smooth pursuit in the horizontal direction. One of them also exhibited impaired vertical saccades and smooth pursuit. Eight children showed excessive blinks in association with an attempt to generate saccades. The typical head thrust usually developed around the age of eight months and had resolved by the age of 6-7 years. History of spasmus nutans was confirmed in seven children. Fourteen children showed cerebellar vermian hypoplasia, mostly in the inferior portion, and five of them also had dysgenesis of the corpus callosum. The severity of cerebellar vermian hypoplasia was correlated with developmental delay, as determined by the age of independent walking. CONCLUSION: Cerebellar vermian hypoplasia is a frequent finding in COMA. Dysfunction of the oculomotor vermis may responsible for the impaired saccades and smooth pursuit in COMA. The occasional association of COMA with spasmus nutans indicates a common pathophysiology of these benign developmental disorders.

Apraxia oculomotora congénita / Ocular toxoplasmosis

La apraxia oculomotora congénita, también conocida como Síndrome de Cogan, es una enfermedad hereditaria rara que afecta al ojo, caracterizada por la incapacidad para ejecutar movimientos oculares horizontales voluntarios. Descrita por Cogan en 1952, de etiología desconocida, clásicamente se le considera un desorden esporádico, con herencia autosómica dominante en algunos casos y en otros sugieren alteraciones metabólicas parciales o defectos del desarrollo neurológico. Acude al servicio de oftalmologíí pediátrica un paciente masculino de ocho meses de edad y al examen de motilidad ocular se detecta que cuando el niño intenta mover los ojos hacia el objeto que le llama la atención, realiza movimientos bruscos laterales de la cabeza. Se realizan estudios de neuro imágenes y electroencefalograma, no se encontraron alteraciones. Este es un diagnóstico de exclusión donde deben descartarse otras causas de defecto de fijación y movimientos cefálicos anómalos. En este momento con dos años de edad se constata una mejoría dada por disminución de las sacudidas cefálicas. Sin embargo, el retraso del desarrollo psicomotor, especialmente del lenguaje, sí puede requerir educación especial. Esta enfermedad es poco frecuente por lo que su detección temprana contribuye a un mejor pronóstico visual

The congenital ocular motor apraxia, also known as Cogan´s Syndrome, is a rare heredity disease that affects the eye in that to the extent that it can not move horizontally at will. Of unknown etiology, it was described by Cogan in 1952 and, classically considered as a sporadic disease with dominant autosomal heredity in some cases, and as an indicator of partial metabolic alterations or neurological development defects in others. An 8 month-old male patient was seen at the pediatric ophthalmology department. On the ocular motility exam, it was found that the child attempted to move his eyes towards an object that got his attention, but instead of this, he moved his head laterally and abruptly. Some studies based on neuroimaging and electroencephalograms were performed; No alteration was found. This is an exclusion diagnosis because other causes of fixation defects and anomalous head movements should be ruled out. Presently, the patient is 2 years old and has improved due to decrease in sudden head movements. However, his retarded psychomotor development, particularly speaking, does require special education. This disease is not common, that is why early detection may contribute to a better visual prognosis

Two Cases of Congenital Ocular Motor Apraxia Associated With Cerebellar Vermian Dysplasia

Congenital ocular motor apraxia is a rare syndrome characterized by rotational head thrusts that attempt to compensate for the lack of voluntary eye movements. We describe the clinical, oculographic and magnetic resonance imaging features of two children with congenital ocular motor apraxia.

Ocular Motor Apraxia after Sequential Bilateral Striatal Infarctions

Ocular motor apraxia has been reported in bilateral frontoparietal lesions. We report a case of ocular motor apraxia after bilateral striatal infarctions. The patient had impaired voluntary saccades and smooth pursuits in the vertical and horizontal planes with an intact vestibulo-ocular reflex. Magnetic resonance imaging showed an old left putaminal infarction and an acute infarction in the right caudoputaminal area. This case demonstrates that ocular motor apraxia may occur in bilateral basal ganglia lesions.

Congenital Ocular Motor Apraxia with Cerebellar Vermian Hypoplasia

Congenital ocular motor apraxia is characterized by impaired voluntary saccades and abnormal head thrusts to induce a fixation. We report a case of a 7-year-old boy who shows typical finding of congenital ocular motor apraxia. He had a history of spasmus nutans. His developmental milestones were delayed. Brain MRI demonstrated cerebellar vermian hypoplasia, especially in the inferior portion. We report on a case of congenital ocular motor apraxia associated with cerebellar vermian hypoplasia.

Congenital Ocular Motor Apraxia with Cerebellar Vermian Hypoplasia

Congenital ocular motor apraxia is characterized by impaired voluntary saccades and abnormal head thrusts to induce a fixation. We report a case of a 7-year-old boy who shows typical finding of congenital ocular motor apraxia. He had a history of spasmus nutans. His developmental milestones were delayed. Brain MRI demonstrated cerebellar vermian hypoplasia, especially in the inferior portion. We report on a case of congenital ocular motor apraxia associated with cerebellar vermian hypoplasia.

Joubert Syndrome Presented with Ocular Motor Apraxia

Agenesis of the cerebellar vermis, episodic hyperpnea alternating with apnea, abnormal eye movement, ataxia, and psychomotor retardation are shown in Joubert syndrome. This rare syndrome is inherited as an autosomal recessive trait with variable expression. We report the first cases of Joubert syndrome in Korea, to our knowledge, which showed ocular motor apraxia and agenesis of the cerebellar vermis by Magnetic Resornance Image. From these two cases, we describe the correlation between the cerebellar vermian dysfunction and the ocular motor apraxia.

Unilateral congenital ocular motor apraxia: a case report

Congenital ocular motor apraxia (COA), first described by Cogan in 1953, is a rare disorder which shows characteristic defects of the horizontal voluntary saccades, and compensatory head thrust. Until now, most cases have showed a presumably congenital origin, bilaterality, and a tendency to various stages of recovery with aging. But the cause and mechanism of COA are not completely known. Occasionally, it combines with other neurologic abnormalities and metabolic diseases such as Gaucher's disease exhibit similar clinical characteristics to COA. We recently experienced a case of a 3-year-old girl who showed the clinical features of unilateral congenital ocular motor apraxia.

Congenital Ocular Motor Apraxia

Congenital ocular motor apraxia (COA), first described by Cogan in 1953, is a rare disorder which shows characteristic defect of horizontal voluntary saccades and head thrust. Until now, most cases have showed presumably congenital in origin, bilaterality, and tendency of various recovery with aging. But the causes and mechanisms of COA are not known. Occasionally, it combines with other neurologic abnormalities and the metabolic diseases such as Gaucher's disease which exhibits similar clinical characteristics of COA. We recently experienced a 3 years old girl who showed clinical features of unilateral congenital ocular motor apraxia.

Congenital Ocular Motor Apraxia

Congenital ocular motor apraxia (COA), first described by Cogan in 1953, is a rare disorder which shows characteristic defect of horizontal voluntary saccades and head thrust. Until now, most cases have showed presumably congenital in origin, bilaterality, and tendency of various recovery with aging. But the causes and mechanisms of COA are not known. Occasionally, it combines with other neurologic abnormalities and the metabolic diseases such as Gaucher's disease which exhibits similar clinical characteristics of COA. We recently experienced a 3 years old girl who showed clinical features of unilateral congenital ocular motor apraxia.