"Eleven mm recession from insertion of 4 horzontal muscles" was performed for 3 patients with sensory nystagmus and "Classic + 1mm or "Clasic + 40% augmented procedure" were performed for 5 patients with congenital nystagmus who have face turn. The degree of nystagmus was decreased from 20 degrees - 45 degrees to within +/- 5 degrees in 4 of 5 congenital nystagmus patients although degrees of nystagmus were not decreased. No significant diminution or abnomality of the ocular motility was noted after the extensive recession and resection.
Humans ; Nystagmus, Congenital

"Eleven mm recession from insertion of 4 horzontal muscles" was performed for 3 patients with sensory nystagmus and "Classic + 1mm or "Clasic + 40% augmented procedure" were performed for 5 patients with congenital nystagmus who have face turn. The degree of nystagmus was decreased from 20 degrees - 45 degrees to within +/- 5 degrees in 4 of 5 congenital nystagmus patients although degrees of nystagmus were not decreased. No significant diminution or abnomality of the ocular motility was noted after the extensive recession and resection.
Humans ; Nystagmus, Congenital

No abstract available.
Nystagmus, Congenital ; Turner Syndrome

Twenty patients were operated on under the diagnosis of congenital nystagmus(CN). Of these, nineteen were motor CN and one was sensory CN. Head turning was toward the leftside in 13 patients(65.0%) and the rightside in 7(35.0%). Preoperatively, the degree of head turn ranged from 20 degrees to 45 degrees with a mean of 34.5 degrees. Primarily, modified Kestenbaum procedures were performed and, if necessary, 2nd and/or 3rd operations(Anderson's procedure, Goto's procedure) were performed additionally. The degrees of head turn after the 1st operation ranged from -5 degrees to 30 degrees with a mean of 11.5 degrees. On an average, 23.5 degrees was corrected by 14.8mm of operation on muscles(resection plus recession) for each eye. By these procedures, seventeen patients(85.0%) showed improvement or cure(75% after 1st operation and 10% after 2nd and/or 3rd operation).
Diagnosis ; Head ; Humans ; Nystagmus, Congenital*

Normally, the fast phase of optokinetic nystagmus (OKN) beats on the opposite direction to the movement of an optokinetic stimulus. The fast component of OKN beating in the same direction are called "reversed OKN". Eventhough the mechanism of reversed OKN is still disputed, it is well known that reversed OKN occurs exclusively in patients with congenital nystagmus, or in some cases, with acquired neurologic disease. It is easy to diagnose the congenital nystagmus when the spontaneous nystagmus can be seen at birth. But when the congenital nystagmus can be seen only on eccentric gaze or when the patient has a wide neutral region around the primary position, the abnormal eye movement can not be detected until a medical examination is performed. It is thought that causes of reversed OKN may be the abnormal neural decussation of the visual system or spontaneous nystagmus. Recently, we experienced two cases of bidirectional reversed OKN as a congenital nystagmus. One patient had bilateral reversed optokinetic nystagmus and gaze evoked nystagmus, whereas the other patient had periodic alternative nystagmus and bilateral reversed OKN. Bilateral reversed OKN may be one of the pathognomic signs of congenital nystagmus.
Eye Movements ; Humans ; Nystagmus, Congenital ; Nystagmus, Optokinetic* ; Parturition

Congenital nystagmus is an involuntary, rhythmic oscillation of the eyes noted during the first months of life. The nystagmus may diminish in a particular pasition of gaze referred to as the null point or neutral zone. If the null point is located in the primary position, there is no need for a compensatory head posture, But, if a patient has an eccentric null point, torticollis occurs in which the head is rotated so that the eyes have a minimal nystagmus. This abnormal head posture can be very marked, cosmetically disturbing and disadvantageous both to the body posture and psyche. Attempts at surgical management were reported by Kestenbaum and Anderson in early 1950's. We obtained following results in 4 cases operated on utilizing similar procedures of Park's method and Dyer's principles of strabismus operations that recession of a medial rectus and lateral rectus muscle must not exceed 4.5mm - 5.5mm and 6.5mm - 7.5mm, respectively. One week after the operation, the head turn disappeared in 3 of 4 cases, and one revealed mild residual turn. Two months after the operation, 2 of 4 cases kept their head straight, and the other two showed a return of head turn in about one half amount of the preoperative condition.
Head ; Humans ; Nystagmus, Congenital* ; Posture ; Strabismus ; Torticollis

Congenital nystagmus is an involuntary, rhythmic oscillation of the eyes noted during the first months of life. The nystagmus may diminish in a particular position of gaze referred to as the null point or neutral zone. If the null point is located in the primary position, there is no need for a compensatory head posture. But, if a patient has an eccentric null point, torticollis occurs in which the head is rotated so that the eyes have a minimal nystagmus. This abnormal head posture can be very marked, cosmetically disturbing and disadvantageous both to the body posture and psyche. Attempts at surgical management were reported by Kestenbaum and Anderson in early 1950's. We obtained following results in 4 cases operated on utilizing similar procedures of Park's method and Dyer's principles of strabismus operations that recession of a medial rectus and lateral rectus muscle must not exceed 4.5mm-5.5mm and 6.5mm-7.5mm, respectively. One week after the operation, the head turn disappeared in 3 of 4 cases, and one revealed mild residual turn. Two months after the operation, 2 of 4 cases kept their head straight, and the other two showed a return of head turn in about one half amount of the preoperative condition.
Head ; Humans ; Nystagmus, Congenital* ; Posture ; Strabismus ; Torticollis

Congenital nystagmus is an involuntary, rhythmic oscillation of the eyes noted during the first months of life. The nystagmus may diminish in a particular position of gaze referred to as the null point or neutral zone. If the null point is located in the primary position, there is no need for a compensatory head posture. But, if a patient has an eccentric null point, torticollis occurs in which the head is rotated so that the eyes have a minimal nystagmus. This abnormal head posture can be very marked, cosmetically disturbing and disadvantageous both to the body posture and psyche. Attempts at surgical management were reported by Kestenbaum and Anderson in early 1950's. We obtained following results in 4 cases operated on utilizing similar procedures of Park's method and Dyer's principles of strabismus operations that recession of a medial rectus and lateral rectus muscle must not exceed 4.5mm-5.5mm and 6.5mm-7.5mm, respectively. One week after the operation, the head turn disappeared in 3 of 4 cases, and one revealed mild residual turn. Two months after the operation, 2 of 4 cases kept their head straight, and the other two showed a return of head turn in about one half amount of the preoperative condition.
Head ; Humans ; Nystagmus, Congenital* ; Posture ; Strabismus ; Torticollis

Genetic Diseases, X-Linked ; Humans ; Nystagmus, Congenital

BACKGROUND AND OBJECTIVES: Congenital nystagmus (CN) is an ocular oscillation that usually manifests during early infancy. To report a novel mutation in FERM domain containing 7 (FRMD7) gene in a Korean family with CN. MATERIALS AND METHODS:Genomic DNA was prepared from peripheral blood leukocytes and direct sequencing of the entire coding and adjacent intronic regions was performed to detect sequence variation of FRMD7 gene, where mutations were found recently in patients with familial CN. The family showed an X-linked pattern of inheritance without father-to-son transmission. RESULTS: Three family members with CN exhibited two sequence variations which were a novel mutation (c. 875T>C; Leu292Pro) and a polymorphism (c. 1403G>A; Arg468His, dbSNP rs#6637934). The proband was hemizygous for both variations and his mother and maternal grandmother were heterozygous carriers. CONCLUSION: This study provides an additional evidence for mutations in FRMD7 as a common cause of X-linked CN and expands its mutation spectrum.
Clinical Coding ; DNA ; Humans ; Introns ; Leukocytes ; Mothers ; Nystagmus, Congenital* ; Wills