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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twins girls presented with monocular nystagmus, head-nodding, and tilting in early infancy. This disorder resolved without therapy or residual defect. This case represents the first documented report of spasmus nutans presenting with only monocular nystagmus in twins. The etiology of spasmus nutans and monocular nystagmus is discussed in light of recent clinical and experimental observations.
J Pediatr Ophthalmol Strabismus
PMID:Acquired monocular nystagmus in monozygous twins. 57 15

Head nodding (to-and-fro turning about the vertical cervical axis) associated with abnormal eye movements may be seen in spasmus nutans and congenital nystagmus. In the absence of abnormal eye movements, it may be indicative of neurological disease (eg, cerebellar disease, basal ganglia dysfunction). We report a neurologically normal infant without nystagmus but with intermittent head nodding and intermittent esotropia, whose head movements manifested only when his eyes were straight. The head movements ceased with the occlusion of either eye or spontaneous onset of esotropia. When his head was forcibly stabilized, he immediately developed esotropia. The head movement presumably facilitated fusion, although the mechanism of action is unknown.
J Pediatr Ophthalmol Strabismus
PMID:Head nodding associated with intermittent esotropia. 224 38

Near-evoked nystagmus was evident in two children with spasmus nutans by clinical observation and electro-oculographic recording. In one child the nystagmus appeared to be evoked by fusional convergence and in the other by convergence-accommodation. These cases represent an atypical form of spasmus nutans in which the nystagmus is modulated by centers controlling visuomotor changes with near viewing.
J Pediatr Ophthalmol Strabismus
PMID:Near-evoked nystagmus in spasmus nutans. 372 97

Patients with congenital types of nystagmus, including congenital nystagmus (CN), latent/manifest latent nystagmus (LMLN) and combinations of the two, can be identified by waveform analysis and classified into three unambiguous groups. This categorization by waveform is supported by different clinical signs, including the relationship to strabismus. Strabismus is essential for LMLN but incidental to CN; most CN patients do not have strabismus. Seventy-seven percent of CN patients have a convergence null, 57% a gaze angle null and nearly half have both; only 14% have neither. Also supporting this patient grouping by waveform is the high incidence of patients in each of the two major groups (ie, they had either only CN waveforms or only the LMLN waveform). Comparing the incidence of each CN waveform, or combinations of waveforms, in families with that in the general CN population, reveals that heredity plays a role in determining waveform; heredity also affects other characteristics, such as gaze angle or convergence nulls. The nystagmus blockage syndrome has at least two mechanisms and the patients, therefore, can belong to either of two groups. Spasmus nutans is hypothesized to be an oscillation of the vergence system and therefore, unrelated to CN or LMLN.
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PMID:Congenital, latent and manifest latent nystagmus--similarities, differences and relation to strabismus. 383 87

Head turning in patients with spasmus nutans seems to transiently dampen the nystagmus. This was evident in two children by clinical observation and electrooculographic recording. The suppression of the nystagmus may have been mediated by the vestibular system.
J Pediatr Ophthalmol Strabismus
PMID:Habitual head turning in spasmus nutans: an oculographic study. 399 73

Subacute necrotizing encephalomyelopathy (SNE), or Leigh's disease, is an unusual neurologic disorder that is associated with nystagmus, strabismus, and optic atrophy in the majority of cases, and is most often described in infants. The defect currently thought to account for SNE is the presence of an inhibitor factor in the thiamine pathway. We saw a patient whose eye findings initially were believed to represent spasmus nutans, but whose clinical course, computed tomographic findings, and autopsy findings were typical of SNE.
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PMID:Leigh's subacute necrotizing encephalomyelopathy manifesting as spasmus nutans. 674 83

An eight-month-old child who presented with signs of spasmus nutans subsequently developed obstructive hydrocephalus, bilateral optic atrophy, and loss of vision due to a juvenile pilocytic astrocytoma involving the optic nerves, chiasm, and anterior third ventricle. It appears that gliomas arising from this region may produce signs that imitate spasmus nutans.
J Pediatr Ophthalmol Strabismus
PMID:Optic glioma masquerading as spasmus nutans. 706 62

Current models of the ocular motor system are usually presented in their most reduced form, are unilateral in architecture, and precise yoking is presumed. Although this simplifies the models, it does not accurately simulate the actual neuroanatomy and limits the models to simple, stereotyped responses. Studies of normal humans and monkeys have demonstrated striking disconjugacies in normal responses. Normal saccades may be disconjugate, or 1 eye may exhibit a dynamic overshoot. Asymmetric vergence can result in disconjugate saccades, unequal magnification spectacles cause differential saccadic gain adjustment, and saccades to unequal disparities also cause unequal saccades in the 2 eyes. In strabismus, deviated eyes typically do not mimic the movements of the fixating eye nor do their latent or congenital nystagmus waveforms duplicate those of the fixating eye. In spasmus nutans, each eye oscillates independently of the other. In achiasmatic dogs, uni-ocular saccades and uni-ocular nystagmus waveforms are seen; the same may be true in human achiasma. These data from both normals and those with abnormalities suggest that current models for ocular motor control are inadequate representations of the actual system. The inability of unilateral, yoked control (or even bilateral, yoked control) system models to duplicate the ocular motor responses of binocular mammals suggests that their ocular motor systems evolved from the bilateral, independent control systems seen in chameleons. One need only postulate a yoking overlay superimposed on two independent control systems to achieve conjugacy (bilateral, yoked, independent control) of the eyes. Abnormalities producing grossly disconjugate eye movements may then be simulated using the independent control of each eye released by a deficiency in the yoking overlay. Independent control of each eye coupled with the essential bilateral brain stem architecture implies that each individual muscle is driven by independent populations of neurons (burst cells, neural integrator cells, etc.). The agonist muscles of each eye are usually coordinated (yoked) but may function independently if the task dictates or if binocularity did not develop. Models based on the above architecture would be robust and could duplicate the many responses (both normal and abnormal) possible from the neurophysiological system.
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PMID:Evidence suggesting individual ocular motor control of each eye (muscle). 799 79

A small number of children who develop disconjugate nystagmus, torticollis, and head titubation (spasmus nutans) have been found to have optic chiasm or third ventricle gliomas. However, the prevalence of glioma or other developmental abnormalities in this disorder is unknown because no large series of spasmus nutans cases has previously been reported. A reviewer of the records of 67 consecutive children initially diagnosed with spasmus nutans and followed for an average of 3.3 years at the St Louis Children's Hospital revealed the following: 61% had a history of prematurity, developmental delay, or other systemic abnormality; strabismus, most commonly infantile esotropia, developed in 55%; 43% had neuroimaging studies; and 0% had evidence of a glioma or showed signs of tumor on follow-up examinations. From this consecutive patient series, we estimate the prevalence of tumor in spasmus nutans to be less than 1.4%. Without other evidence of an intracranial mass lesion, neuroimaging of infants initially diagnosed with spasmus nutans may not be immediately warranted.
J Pediatr Ophthalmol Strabismus
PMID:Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans) 896 30

In the recent pediatric ophthalmology and strabismus literature, there have been some important papers. Topics have included ocular outcomes in children born before 32 weeks of gestation, a practical correlate of Teller visual acuities and visual behavior in a real environment in a severely handicapped child, and a debate on the indications and efficacy of population screening for amblyopia. New information on the creation of refractive errors by earlier eyelid closure than used previously and a new torsionometer represent new sensory information. New information about motor adaptations in strabismus includes a new evaluation of saccade disconjugacy in deep amblyopia and in anisometropia. Spasmus nutans may be a long-term rather than a self-limiting disease, and the debate on neuroimaging in this condition has been evaluated. A subtle predictor of outcome in the surgical treatment of partially accommodative esotropia is presented, as well as an important case report on the treatment of a metabolic cause of strabismus in chronic progressive external ophthalmoplegia with coenzyme Q10. Finally, some information is presented on the treatment of hereditary retinal dystrophies with nonautologous retinal pigment epithelial cell transplants to the subretinal space of the dystrophic retina-the first experimental model of a successful biologic treatment of which I am aware and a possible indicator of future trends in human disease.
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PMID:Pediatric ophthalmology. 1016 5


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