Horizontal gaze palsy …

Yin Allison Liu MD PhD

Neuro-Ophthalmology & Neuro-Otology

Updated 04.05.2023
Released 07.19.2001
Expires For CME 04.05.2026

Horizontal gaze palsy

Author: Yin Allison Liu MD PhD

See Contributor Disclosures

Editor: Heather E Moss MD PhD

Horizontal gaze palsy

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Introduction

Overview

The author reviews the differentiating signs, causes, and management of horizontal gaze palsies. These are disorders in which the supranuclear control of lateral eye movements is impaired. Sometimes the palsy is selective for some types of eye movements but not others (eg, selective saccadic palsy). When acquired, horizontal gaze palsy usually points to damage of specific pontine structures. Congenital forms occur too, such as the Mobius syndrome and horizontal gaze palsy associated with pendular nystagmus and scoliosis. The genetic defect for the latter has been described, and it involves a protein that likely plays a role in axonal guidance during development.

Key points

	• Horizontal gaze palsy is usually due to lesions of supranuclear, nuclear, and infranuclear pathways of horizontal of eye movements in the pons.
	• Palsy of all types of horizontal movements implicates the abducens nucleus, whereas palsies of saccades alone are due to lesions of the parapontine reticular formation.
	• Congenital syndromes with horizontal gaze palsies include Mobius syndrome, Cogan congenital ocular motor apraxia, and horizontal gaze palsy with progressive scoliosis.

Historical note and terminology

Gaze palsy is an ambiguous term. It is best restricted for deficits in conjugate eye movements that affect both eyes. Thus, strictly unilateral problems such as palsies of cranial nerves III, IV, or VI are not gaze palsies, even though they do affect gaze. Likewise, impairments in vergence control, such as convergence or divergence insufficiency, are not gaze palsies, as they do not involve conjugate eye movements. In fact, vergence is often spared in horizontal gaze palsies, with some patients using a near reflex as a compensatory maneuver to get at least the adducting eye to move (75).

Gaze palsy also implies that the deficit is limited to some directions of conjugate movements only. Diffuse reduction of all eye movements is best termed ophthalmoparesis. These are most commonly myopathic in origin, occurring with mitochondrial disorders (chronic progressive external ophthalmoplegia, Kearns-Sayre syndrome, MELAS), myotonic dystrophy, oculopharyngeal dystrophy, thyroid eye disease, myasthenia gravis, and congenital fibrosis, among others.

The term “gaze palsy” requires further elaboration. There are many different types of conjugate eye movements, including saccades, pursuit, optokinetic, and vestibulo-ocular responses. The anatomic systems that control these diverge and converge at various levels, and it is possible for some lesions to impair some eye movement systems and spare others. Hence, a left saccadic palsy is a selective gaze palsy affecting only leftward saccades but not leftward pursuit or vestibulo-ocular responses. A palsy affecting all types of eye movements should be designated as nonselective gaze palsy. In contrast, the terms “partial” or “complete” when applied to gaze palsy indicate whether some motion across midline in the paretic direction is present.

An even more fundamental distinction is between vertical and horizontal gaze palsies. Most gaze palsies affect one direction in one plane of eye movement only, reflecting the separation of the prenuclear control systems for vertical and horizontal eye movement.

Clinical manifestations

Presentation and course

Horizontal nonselective gaze palsy. The cranial nerve VI nucleus contains neurons that project to the ipsilateral lateral rectus and interneurons that connect to the contralateral medial rectus and travel in the medial longitudinal fasciculus. Nuclear VI lesions cause ipsilateral gaze palsy, affecting all conjugate eye movements toward the side of the lesion. The eyes may be deviated contralaterally (11), and there may be ipsilateral gaze-evoked nystagmus (85). There often is an ipsilateral facial palsy (85; 105; 109), due to the proximity of the VII nerve fascicle to the VI nerve nucleus, though not always (88). Loss of all horizontal eye movements occurs with a lesion that affects both VI nuclei, as with unusual pontine strokes (42) and demyelination (45; 49). This can be associated with the locked-in syndrome in which facial and body motor control is also lost, but vertical eye movements are spared (59). In some cases, pontine demyelinating lesions that affect the outputs of the VI nucleus (ie, both the medial longitudinal fasciculus, carrying the projections to the medial rectus subnucleus of III for adduction, and the abducens fascicle) can cause a nonselective gaze palsy that mimics a VI nuclear lesion (86).

Horizontal saccadic palsy. The parapontine reticular formation, just ventral to the cranial nerve VI nuclei, contains neurons that send saccadic commands to the cranial nerve VI nuclei. Lesions of the parapontine reticular formation cause saccades towards the side of the lesion to be slow (73) and if severe, impossible across the midline (102). Pursuit and the slow phases of optokinetic and vestibular nystagmus are relatively spared, though they may be impaired in the ipsilateral abducting eye because the cranial nerve VI fascicles travel through the parapontine reticular formation (102; 77). This often causes esotropia also (30). As with cranial nerve VI nuclear lesions, there may be associated ipsilateral cranial nerve VII palsy and ataxia (73). Spontaneous vertical-torsional oscillations have been described as an associated sign (136).

Bilateral paramedian lesions of the parapontine reticular formation cause bilateral horizontal saccadic palsy with normal vertical saccades. Pursuit is often affected because its pathways are nearby, but horizontal vestibulo-ocular response is normal (09). There can be an associated bilateral internuclear ophthalmoplegia and cranial nerve VI palsy (59; 09). Patients may sometimes compensate by substituting vergence eye movements to generate lateral eye shifts (60), which can cause confusion with functional convergence spasm.

Horizontal midbrain gaze paresis. Midbrain lesions can disrupt descending ocular motor inputs for horizontal gaze. Slow, hypometric contralateral horizontal saccades occur with either ipsi- or contra-directional pursuit impairment (141; 16). Bilateral gaze palsies can also occur (25). The clue to midbrain origin is concurrent cranial nerve III damage and ipsilateral ataxia (141; 16; 46).

Horizontal “one-and-a-half” syndrome. The cranial nerve VI nucleus, medial longitudinal fasciculus, and parapontine reticular formation are neighbors in the pontine tegmentum. A medial pontine lesion may affect them simultaneously. Combined damage to the medial longitudinal fasciculus and parapontine reticular formation, or the medial longitudinal fasciculus and VI nucleus, causes the one-and-a-half syndrome. Adduction is impaired during contralateral gaze (“the half”), representing an internuclear ophthalmoplegia from medial longitudinal fasciculus damage. There is ipsilateral horizontal gaze palsy (“the one”), which affects all eye movements if the VI nucleus is involved (103; 14; 68), or saccades preferentially, with sparing of at least vestibular slow phases if the parapontine reticular formation is affected (14; 38). In primary position the eyes may be orthotropic (66; 140), exotropic (33; 116; 135; 38), or esotropic if the VI fascicle is damaged (135). Cerebellar dysarthria and ataxia of the trunk or limbs are frequent (116; 66; 93). Less often there is contralateral hemiparesis (66; 38; 140) or hemisensory loss (116; 66; 140). There may be an ipsilateral trigeminal deficit or facial palsy (116; 135; 68; 93).

Vertical and horizontal saccadic palsy. Bilateral lesions of the parapontine reticular formation can sometimes cause slowing of saccades and loss of quick phases of vestibular and optokinetic nystagmus in both vertical and horizontal planes (56), with normal pursuit and slow phases of optokinetic and vestibulo-ocular responses. There is disagreement over whether vertical saccadic impairment is more likely with caudal versus rostral parapontine reticular formation lesions (62; 56).

Cortical gaze palsies. Unilateral lesions of the frontal or parietal eye fields cause only subtle abnormalities of eye movements that require recordings to detect. Bilateral parietal or frontal lesions are more likely to impair gaze but usually in all directions. These can form part of a Balint syndrome. The saccadic problems include several aspects. One is severely inaccurate saccades, with the eyes wandering in search of a clearly visible target (64; 79; 106). Another is acquired ocular motor apraxia, in which subjects cannot initiate saccades to commands or (sometimes) visual objects but can do so spontaneously in reaction to sudden noises or actions (31). In some cases, but not others, pursuit is also limited in range; however, vestibulo-ocular response range is invariably normal to the Doll eye maneuver.

Occasionally, patients have bilateral lesions of both frontal and parietal regions (61). The gaze palsies in these cases are severe, with almost immobile eyes. Again, saccades to unexpected noises may remain (58). Gaze shifts are accompanied by head turns (104). There is no pursuit or convergence.

Mobius syndrome. Mobius syndrome is a mixed group of congenital anomalies that overlaps phenotypically with other congenital orders of cranial nerves. The core diagnostic criteria include facial palsies and abnormal horizontal eye movements, usually but not always bilateral. Bilateral horizontal gaze palsy is the most frequent ocular motor pattern (113). More unusual are isolated abduction deficits and vertical gaze impairment. The facial palsy is due to a nuclear defect in some cases but, in others, may reflect supranuclear damage (132). Other defects include limb and craniofacial deformities. Limb defects are missing digits or amputations of the distal limb (87). The Mobius-Poland anomaly includes absence of one pectoral muscle with or without ipsilateral hand deformity (48; 98). Craniofacial abnormalities include micrognathia, tongue malformations, oligodontia, facial or oral clefts, and palatal or pharyngeal abnormalities (87; 133). General motor disability and incoordination are common (133), and autism as well as a various degrees of learning disabilities have been reported in such patients as well (71).

Probably related to Mobius syndrome is the Athabascan brainstem dysgenesis syndrome, which occurs in Navajo and Apache children and consists of horizontal gaze palsy, sensorineural deafness, central hypoventilation, developmental delay, and sometimes swallowing dysfunction, vocal cord paralysis, facial paresis, seizures, and cardiac outflow tract anomalies (65).

Cogan congenital ocular motor apraxia. Some children from infancy have an intermittent failure to generate either horizontal saccades or quick phases of nystagmus (57), sometimes overcome by blinking, or compensated for by a head thrust. The head thrust may serve to trigger an eye movement or can be used to drag the eye over to the target, without a saccade. Head thrust is not pathognomonic of this syndrome because patients with other causes of saccadic failure use it. Congenital ocular motor apraxia may be unilateral (23).

A few patients have strabismus and nystagmus (57). Other signs are frequent, including infantile hypotonia and developmental motor and speech delay (108; 57), depending on the etiology.

Vertical saccades are normal in most. The presence of a vertical element of ocular motor apraxia indicates a need to investigate for a cause, such as Joubert syndrome (127), as this is atypical in the usual idiopathic form.

Horizontal gaze palsy with scoliosis. In this disorder all horizontal conjugate eye movements are absent (138; 01). Patients may use convergence to shift gaze, mimicking convergence spasm (69). Several patients have been described with horizontal nystagmus and pendular nystagmus; others have developmental delay, microcephaly, and scoliosis (122; 101; 40; 19; 67). Some patients also present with overlapping epilepsy and learning difficulties (84). MRI of the brain shows brainstem hypoplasia with absence of facial colliculi, a deep midline pontine cleft, and butterfly configuration of the medulla. Diffusion tensor imaging shows absence of decussating superior cerebellar peduncle and transverse pontocerebellar fibers (111; 128).

Pontine tegmental cap dysplasia. First described in 2007, this occurs as sporadic cases of children with horizontal gaze palsy, ataxia, and cranial nerve deficits (including deafness, facial palsy, trigeminal numbness, and dysphagia), in addition to radiologic signs of vermis hypoplasia, reduced volume of the ventral pons and middle cerebellar peduncles, absent inferior olivary eminence, and a vaulted pontine tegmentum (10).

Prognosis and complications

Recovery of gaze palsies varies with etiology. For the one-and-a-half syndrome, recovery of the eye movement abnormalities is the rule when it is due to demyelination or infarction, though this is typically incomplete and variable after infarction (116; 135).

Not surprisingly, abnormal ERG in Cogan oculomotor apraxia carries a worse visual prognosis (82). In addition, patients with oculomotor apraxia have a high likelihood of abnormal language, motor, and behavioral development, regardless of whether their imaging is abnormal (83).

Clinical vignette

A 32-year-old man presented with 6 months of new intermittent headache, drop attacks, and occasional horizontal diplopia. His examination showed limitation of right and left gaze in both eyes in saccades, pursuit, and oculocephalic maneuver. He also had gaze-evoked nystagmus in all directions and bilateral facial weakness affecting both the upper and lower face. CT scan showed a large, calcified lesion in the fourth ventricle, which proved on resection to be an ependymoma. On examination one month after surgery, his right gaze had improved, but he still had partial limitation of left gaze.

Horizontal gaze palsy eye movements

Patient has partial left gaze palsy. (Contributed by Dr. Jason Barton.)
Horizontal gaze palsy (CT)

There is a large IV ventricular tumor compressing the dorsal pons, which contains the VI nuclei and VII fascicles. (Contributed by Dr. Jason Barton.)

He had bilateral horizontal gaze paresis that also involved vestibular eye movements, indicating a lesion of the VI nuclei on the floor of the IV ventricle. The fascicles of the VII nerves were affected as they curled around the VI nuclei, in the facial colliculi. Gaze-evoked nystagmus presumably reflected floccular damage.

Biological basis

Etiology and pathogenesis

Any disorder that disrupts the anatomical pathways for horizontal gaze can manifest as a horizontal gaze palsy.

Pontine disorders. The final common pathway for all horizontal eye movements begins in the VI nucleus, which contains two populations of neurons. One projects to the ipsilateral lateral rectus, forming the VI fascicle and nerve. The other crosses the midline to ascend in the contralateral medial longitudinal fasciculus, terminating on neurons in the III nuclear complex that innervate the contralateral medial rectus. The causes of gaze palsies due to damage in this region reflect the typical range of pontine pathology. This has been best documented for the horizontal one-and-a-half syndrome, where the most frequent causes are brainstem infarction and multiple sclerosis (135). The next most frequent etiology is pontine hemorrhage (120; 95; 135). In a prospective study of ocular motility abnormalities following ischemic or hemorrhagic stroke, 8 of 16 cases of horizontal gaze palsy or paresis were due to brainstem lesions (112). Tumors include intrinsic lesions such as gliomas (116; 135) and metastases (34; 68) or compression by ependymomas or astrocytomas (135; 93). Horizontal gaze palsy has been described as the sole sign of a pontine tuberculoma (115).

Supranuclear disorders. Prior to the VI nucleus, the prenuclear inputs for vestibulocular response, pursuit, and horizontal saccades take different routes. Although typical anatomical localization of horizontal gaze palsy is usually at a cerebral cortex or brainstem level, horizontal gaze palsy has also been reported from strokes localized to the subcortical gaze controlling pathways (06) and anterior internal capsule hemorrhage (28).

Vestibulocular response. The VI nucleus receives input from the contralateral vestibular nuclear complex in the lateral medulla. This is the afferent arc of the horizontal vestibulo-ocular response (VOR). The efferent arc originates in the horizontal semicircular canals and projects through the vestibular component of the VIII nerve in the internal auditory canal and cerebellopontine angle. Lesions to this pathway can impair horizontal gaze that is mediated by the vestibulo-ocular response.

Pursuit. Pursuit of a moving target requires accurate perception of visual motion signals. This is processed in regions of extrastriate cortex, particularly middle temporal areas. Pursuit signals likely originate in medial superior temporal areas as well as a subregion of the frontal eye field. These signals are biased for targets moving towards that hemisphere (ipsidirectional). Axons from these sites project ipsilaterally through the posterior internal capsule to pontine nuclei, which project in turn to the contralateral flocculus (50). Axons from the flocculus project to the vestibular nucleus and nucleus prepositus hypoglossi, which then project to contralateral VI nucleus (72). This double decussation explains why both ipsi- and contra-directional pursuit defects occur with cerebellar or brainstem lesions (47; 124; 50).

Brainstem control of horizontal gaze

Only excitatory pathways are shown; inhibitory pathways exist as well. Final common pathways for all horizontal eye movements are shown as thick black arrows. Saccadic commands (blue) are generated in the ipsilateral parapontine r...

Impaired horizontal pursuit can occur due to lesions that impair perception of visual motion signals (eg, middle temporal areas of extrastriate cortex) and disrupt pursuit signals (eg, medial superior temporal areas as well as a subregion of the frontal eye field, flocculus, vestibular nucleus, and nucleus prepositus hypoglossi) (72).

Saccades. Horizontal saccadic commands originate in the contralateral parietal (78) and frontal eye fields (99). These projects parallel to brainstem ocular motor areas, including the superior colliculus. Pathways for horizontal saccades decussate between the midbrain and pons. Lesions below this level impair ipsilateral saccades. In the pons, the parapontine reticular formation is the immediate prenuclear generator of ipsilateral horizontal saccades. It contains excitatory burst neurons projecting to the ipsilateral VI nucleus, inhibitory neurons that suppress the contralateral VI nucleus, and omnipause neurons—tonically active cells that only cease firing during a saccade. The latter may gate the system and coordinate activity across different muscles. Long-lead burst neurons for vertical gaze are also found in the parapontine reticular formation, though the immediate prenuclear structure for vertical saccades is the rostral interstitial nucleus of the medial longitudinal fasciculus.

Impaired horizontal saccades can occur due to lesions at the site of signal origin (contralateral parietal and frontal eye fields) (99; 78). In the pons, the parapontine reticular formation is the immediate prenuclear generator of ipsilateral horizontal saccades. The most common causes of bilateral frontal or parietal lesions with gaze palsy are bilateral watershed zone infarction (104; 37) or metastases (61; 58).

Brainstem control of horizontal gaze

Acquired disorders without a discrete lesion. Some disorders without a discrete lesion affecting the horizontal eye movement pathways can cause horizontal gaze palsies. Rare cases may represent paraneoplastic phenomena (90). Wernicke encephalopathy can also cause gaze palsies (74), as can other disorders with a predilection for the periventricular spaces.

Developmental, genetic, or inherited disorders. Several developmental, genetic, or inherited metabolic disorders have horizontal gaze palsies. Horizontal gaze is impaired in 8% of infantile or juvenile neuronopathic forms of Gaucher disease, an autosomal recessive disease (32). Severity varies from mild horizontal saccadic slowing to absence of horizontal saccades (126) with head thrusts that simulate ocular motor apraxia (32; 53; 15). Horizontal pursuit and vestibulo-ocular response are spared. Diagnosis requires detecting excess glucosyl ceramides in liver biopsy and decreased beta-glucocerebrosidase levels in fibroblasts. Several of the autosomal dominant spinocerebellar ataxias have associated eye movement problems. Seventy percent of patients with SCA2 have slowed saccades (44), most often upward but sometimes horizontal. All patients have a pancerebellar syndrome, with onset ranging widely from early childhood to late adulthood. Spinocerebellar ataxias are now known genetically to be triplet-repeat polyglutamine disorders; SCA2 involves a gene on chromosome 12. Genetic testing is available. Some sporadic cerebellar degenerations have slow horizontal saccades that are hard to initiate (143).

Mobius syndrome. There are likely multiple etiologies that result in this syndrome. In some patients, imaging suggests a perinatal vascular insult. Mobius syndrome has been associated with prenatal exposure to misoprostol, which is a drug used as an abortifactant (130), and other toxic insults have been postulated. A genetic etiology is likely in some patients with Mobius syndrome who have family members with a similar syndrome or milder features, such as isolated facial palsy or digital contractures and harbor a genetic defect associated with one of the other cranial dysinnervation syndromes. Genetic mutations associated with other congenital cranial dysinnervation disorders are not common in Mobius syndrome (81). De novo mutations in two unrelated pathways have been reported to cause Mobius syndrome (125). Thus far, only mutation in PLXND1 and REV3L are confirmed to cause Mobius syndrome (92).

Cogan congenital ocular motor apraxia. Twenty-five percent of congenital ocular motor apraxia is idiopathic (57). There are numerous causes of symptomatic congenital ocular motor apraxia, including structural lesions (57; 83). Most frequent are agenesis of the corpus callosum (96; 43; 18) and cerebellar hypoplasia (118), as with Joubert syndrome, Dandy Walker malformation, and vermis hypoplasia (57). Brainstem tumors (142), posterior fossa lipoma (123), hydrocephalus, porencephalic cysts, microcephaly, and megalocephaly (57) are less common. Diverse other conditions can be associated, including inborn metabolic disorders such as Gaucher disease, infantile Refsum disease, GM1 gangliosidosis, and propionic acidemia, as well as dysmyelinative disorders such as Pelizaeus-Merzbacher disease and Krabbe leukodystrophy (57). There is evidence for a genetic basis in some patients based on co-occurrence in monozygotic twins (110; 18), siblings (51; 107; 54), or successive generations (129). One family had associated deletions of the NPHP1 gene for juvenile nephrophthisis, on chromosome 2q13 (13).

Horizontal gaze palsy with scoliosis. Electrophysiological studies have reported the intriguing finding of absent decussations of the dorsal columns and pyramidal tracts in this condition (80; 19), with similar findings of predominantly ipsilateral cortical neural activity with motor responses on fMRI (55). Absence of decussations in the pons and superior cerebellar peduncle has been demonstrated with diffusion tensor imaging studies (119; 97; 07), though the corpus callosum shows normal interhemispheric connectivity (55). The defect in familial horizontal gaze palsy with scoliosis involves a ROBO3 gene, which is similar to genes involved in the guidance of developing axons (70; 19; 24; 52). The mutations that have been described in different families are numerous and scattered throughout the ROBO3 genome (02). However, not all families have a mutation in the ROBO3 gene (03).

Pontine tegmental cap dysplasia. Impaired axon guidance is thought to underlie this rare disorder with MR tractography showing an abnormal bundle of horizontally oriented axons in the dorsal pons and no decussation in the cerebellar peduncles (21). Boney abnormalities are common with duplication, stenosis, or atresia of internal auditory canals and atresia or stenosis of the vestibulocochlear canals (94).

Ocular motor apraxia with ataxia. Ocular motor apraxia with ataxia occurs in ataxia-telangiectasia, a disease caused by mutations in a gene coding for a DNA-binding protein kinase involved in cell-cycle regulation (114). Rarely, ataxia-telangiectasia can present as a movement disorder and ocular motor problem without telangiectasia (29). Unlike Cogan ocular motor apraxia, ataxia-telangiectasia affects both vertical and horizontal eye movements (32).

A similar condition is ataxia with ocular motor apraxia (AOA), which consists of ataxia, choreoathetosis, and vertical and horizontal ocular motor apraxia (04). Multiple distinct phenotypes and genes have been identified (20).

Horizontal gaze is impaired in 8% of infantile or juvenile neuronopathic forms of Gaucher disease, an autosomal recessive disease (32). Severity varies from mild horizontal saccadic slowing to absence of horizontal saccades (126) with head thrusts that simulate ocular motor apraxia (32; 53; 15). Horizontal pursuit and vestibulo-ocular response are spared. Diagnosis requires detecting excess glucosyl ceramides in liver biopsy and decreased beta-glucocerebrosidase levels in fibroblasts.

Several of the autosomal dominant spinocerebellar ataxias have associated eye movement problems. Seventy percent of patients with SCA2 have slowed saccades (44), most often upward but sometimes horizontal. All patients have a pancerebellar syndrome, with onset ranging widely from early childhood to late adulthood. Spinocerebellar ataxias are now known genetically to be triplet-repeat polyglutamine disorders; SCA2 involves a gene on chromosome 12. Genetic testing is available. Some sporadic cerebellar degenerations have slow horizontal saccades that are hard to initiate (143).

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References

01: Abdrabou A, Hacking C, Sheikh Y, et al. Horizontal gaze palsy with progressive scoliosis. Available at: radiopaedia.org. Accessed 2023.
02: Abu-Amero KK, al Dhalaan H, al Zayed Z, Hellani A, Bosley TM. Five new consanguineous families with horizontal gaze palsy and progressive scoliosis and novel ROBO3 mutations. J Neurol Sci 2009;276(1-2):22-6. PMID 18829051
03: Abu-Amero KK, Faletra F, Gasparini P, et al. Horizontal gaze palsy and progressive scoliosis without ROBO3 mutations. Ophthalmic Genet 2011;32(4):212-6. PMID 21510772
04: Aicardi J, Barbosa C, Andermann E, et al. Ataxia-ocular motor apraxia: a syndrome mimicking ataxia-telangiectasia. Ann Neurol 1988;24:497-502. PMID 3239952
05: Akinci G, Oztura I, Hiz-Kurul S. Anti-GQ1b-negative Miller Fisher syndrome presented with one-sided horizontal gaze palsy. Turk J Pediatr 2010;52(3):317-20. PMID 20718193
06: Alshahrani AM. Horizontal gaze defect as a result of subcortical stroke: case report and review of the literature. Case Rep Neurol 2021;13(1):140-4. PMID 33790772
07: Avadhani A, Ilayaraja V, Shetty AP, Rajasekaran S. Diffusion tensor imaging in horizontal gaze palsy with progressive scoliosis. Magn Reson Imaging 2010;28(2):212-6. PMID 20071118
08: Bae YJ, Kim JH, Choi BS, Jung C, Kim E. Brainstem pathways for horizontal eye movement: pathologic correlation with MR imaging. Radiographics 2013;33(1):47-59. PMID 23322826
09: Baloh R, Furman J, Yee R. Eye movements in patients with absent voluntary horizontal gaze. Ann Neurol 1985;17:283-6. PMID 3873208
10: Barth PG, Majoie CB, Caan MW, et al. Pontine tegmental cap dysplasia: a novel brain malformation with a defect in axonal guidance. Brain 2007;130(Pt 9):2258-66. PMID 17690130
11: Bennett A, Savill T. A case of permanent conjugate deviation of the eyes and head, the result of a lesion limited to the sixth nucleus; with remarks on associated lateral movements of the eyeballs and rotation of the head and neck. Brain 1889;12:102-16.
12: Berlit P, Rakicky J. The Miller Fisher syndrome. Review of the literature. J Clin Neuroophthalmol 1992;12:57-63. PMID 1532603
13: Betz R, Rensing C, Otto E, et al. Children with ocular motor apraxia type Cogan carry deletions in the gene (NPHP1) for juvenile nephronophthisis. J Pediatr 2000;136(6):828-31. PMID 10839884
14: Bogousslavsky J, Miklossy J, Regli F, Deruaz J, Despland P. One-and-a-half syndrome in ischaemic locked-in state: a clinico-pathological study. J Neurol Neurosurg Psychiatry 1984;47:927-35. PMID 6481386
15: Bohlega S, Kambouris M, Shahid M, Al Homsi M, Al Sous W. Gaucher disease with oculomotor apraxia and cardiovascular calcification (Gaucher disease type IIIC). Neurology 2000;54(1):261-3. PMID 10636167
16: Bolling J, Lavin PJ. Combined gaze palsy of horizontal saccades and pursuit contralateral to a midbrain haemorrhage. J Neurol Neurosurg Psychiatry 1987;50(6):789-91. PMID 3612157
17: Bomfim RC, Távora DG, Nakayama M, Gama RL. Horizontal gaze palsy with progressive scoliosis: CT and MR findings. Pediatr Radiol 2009;39(2):184-7. PMID 19020872
18: Borchert M, Sadun A, Sommers J, Wright K. Congenital ocular motor apraxia in twins. Findings with magnetic resonance imaging. J Clin Neuroophthalmol 1987;7:104-7. PMID 2956282
19: Bosley TM, Salih AM, Jen JC, et al. Neurologic features of horizontal gaze palsy and progressive scoliosis with mutations in ROBO3. Neurology 2005;64:1196-203. PMID 15824346
20: Bras J, Alonso I, Barbot C, et al. Mutations in PNKP cause recessive ataxia with oculomotor apraxia type 4. Am J Hum Genet 2015;96(3):474-9. PMID 25728773
21: Briguglio M, Pinelli L, Giordano L, et al. Pontine tegmental cap dysplasia: developmental and cognitive outcome in three adolescent patients. Orphanet J Rare Dis 2011;6:36. PMID 21651769
22: Campos EC, Schiavi C, Bellusci C. Surgical management of anomalous head posture because of horizontal gaze palsy or acquired vertical nystagmus. Eye 2003;17:587-92. PMID 24694546
23: Catalano R, Calhoun J, Reinecke R, Cogan D. Asymmetry in congenital ocular motor apraxia. Can J Ophthalmol 1988;23:318-21. PMID 3228780
24: Chan WM, Traboulsi EI, Arthur B, Friedman N, Andrews C, Engle EC. Horizontal gaze palsy with progressive scoliosis can result from compound heterozygous mutations in robo3. J Med Genet 2006;43(3):e11. PMID 16525029
25: Chen CM, Wei JC, Huang TY. Mesencephalic bilateral horizontal gaze palsies. Neurology 2008;71(13):1039. PMID 18809843
26: Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barre syndrome. Neurology 1993;43:1911-7. PMID 8413947
27: Chiba A, Kusunoki S, Shimizu T, Kanazawa I. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol 1992;31:677-9. PMID 1514781
28: Chu N, Beecher G, Hussain MW. Supranuclear horizontal gaze palsy following anterior internal capsule hemorrhage. Can J Neurol Sci 2020;47(6):822-3. PMID 32507124
29: Churchyard A, Stell R, Mastaglia F. Ataxia telangiectasia presenting as an extrapyramidal movement disorder and ocular motor apraxia without overt telangiectasia. Clin Exp Neurol 1991;28:90-6. PMID 1726561
30: Coats DK, Avilla CW, Lee AG, Paysse EA. Etiology and surgical management of horizontal pontine gaze palsy with ipsilateral esotropia. J AAPOS 1998;2(5):293-7. PMID 10646751
31: Cogan D. Ophthalmic manifestations of bilateral non-occipital cerebral lesions. Brit J Ophthalmol 1965;49:281-97. PMID 14317977
32: Cogan DG, Chu FC, Reingold D, Barranger J. Ocular motor signs in some metabolic diseases. Arch Ophthalmol 1981;99(19):1802-8. PMID 7295130
33: Crevits L, de Rueck J, vander Eecken H. Paralytic pontine exotropia in subarachnoid hemorrhage. A clinicopathologic correlation. Clin Neurol Neurosurg 1975;78:269-76. PMID 1234038
34: Crisostomo E. One-and-a-half syndrome with facial paralysis. J Clin Neuroophthalmol 1985;2:129-32. PMID 2934432
35: Cronemberger MF, de Castro Moreira JB, Brunoni D, et al. Ocular and clinical manifestations of Mobius syndrome. J Pediatr Ophthalmol Strabismus 2001;38(3):156-62. PMID 11386648
36: Davis T, Lavin P. Pseudo one-and-a-half syndrome with ocular myasthenia. Neurology 1989;39:1553. PMID 2812341
37: Dehaene I, Lammans M. Paralysis of saccades and pursuit. Neurology 1991;41:414-5. PMID 2006011
38: Deleu D, Solheid C, Michotte A, Ebinger G. Dissociated ipsilateral horizontal gaze palsy in one-and-a-half syndrome: a clinicopathological study. Neurology 1988;38(8):1278-80. PMID 3399079
39: dos Santos AV, Matias S, Saraiva P, Goulao A. MR imaging features of brain stem hypoplasia in familial horizontal gaze palsy and scoliosis. AJNR Am J Neuroradiol 2006;27(6):1382-3. PMID 16775302
40: El Bahri-Ben Mrad F, Gouider-Khouja N, Gabsi S, et al. Lateral gaze palsy and progressive scoliosis in 4 Tunisian families. Rev Neurol (Paris) 2004;160:307-10. PMID 15037843
41: Evoli A, Tonali P, Bartoccioni F, Lo Monavo M. Ocular myasthenia: diagnosis and therapeutic problems. Acta Neurol Scand 1988;77:31-5. PMID 3354309
42: Felicio AC, Bichuetti DB, Marin LF, dos Santos WA, Godeiro-Junior C. Bilateral horizontal gaze palsy with unilateral peripheral facial paralysis caused by pontine tegmentum infarction. J Stroke Cerebrovasc Dis 2009;18(3):244-6. PMID 19426898
43: Fielder A, Gresty M, Dodd K, Mellor D, Levene M. Congenital ocular motor apraxia. Trans Ophthalmol Soc UK 1986;105:589-98. PMID 2432703
44: Filla A, de Michele G, Banfi S, et al. Has spinocerebellar ataxia type 2 a distinct phenotype. Neurology 1995;45:793-6. PMID 7723972
45: Fok A, Barton JJS. Teaching video NeuroImage: bilateral horizontal gaze palsies with vertical ocular dysmetria from a demyelinating lesion of the pontine tegmentum. Neurology 2021;97(18):e1868-9. PMID 34099527
46: Fong C. Claude's syndrome associated with supranuclear horizontal gaze palsy caused by dorsomedial midbrain infarction. Acta Neurol Taiwan 2005;14:147-50. PMID 16252618
47: Furman J, Hurtt M, Hirsch W. Asymmetrical ocular pursuit with posterior fossa tumours. Ann Neurol 1991;30:208-11. PMID 1897912
48: Gadoth N, Biedner B, Torok G. Mobius syndrome and Poland anomaly: case report and review of the literature. J Ped Ophthalmol Strabismus 1979;16:374-6. PMID 521879
49: Gamea MS, AlAkkad A, Ibraheem R. Complete bilateral horizontal gaze palsy as initial manifestation of multiple sclerosis: a case report. J Mult Scler (Foster City) 2021;8(10):273.
50: Gaymard B, Pierrot-Deseilligny C, Rivaud S, Velut S. Smooth pursuit eye movement deficits after pontine nuclei lesions in humans. J Neurol Neurosurg Psychiatry 1993;56(7):799-809. PMID 8331357
51: Godel V, Nemet P, Lazar M. Congenital ocular apraxia – familial occurrence. Ophthalmologica 1979;179:90-3. PMID 548846
52: Gowda VK, Suryanarayana SG, Shivappa SK, et al. Horizontal gaze palsy with progressive kyphoscoliosis. Indian J Pediatr 2021;88(7):728-9. PMID 32691295
53: Gross-Tsur V, Har-Even Y, Gutman I, Amir N. Oculomotor apraxia: the presenting sign of Gaucher disease. Pediatr Neurol 1989;5(2):128-9. PMID 2712947
54: Gurer Y, Kukner S, Kunak B, Yilmaz S. Congenital ocular motor apraxia in two siblings. Pediatr Neurol 1995;13:261-2. PMID 8554667
55: Haller S, Wetzel SG, Lutschg J. Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis. Neuroradiology 2008;50(5):453-9. PMID 18214457
56: Hanson M, Hamid M, Tomsak R, Chou S, Leigh R. Selective saccadic palsy caused by pontine lesions: clinical, physiological and pathological correlations. Ann Neurol 1986;20:209-17. PMID 3752965
57: Harris C, Shawkat F, Russell-Eggitt I, Wilson J, Taylor D. Intermittent horizontal saccade failure (‘ocular motor apraxia’) in children. Brit J Ophthalmol 1996;80:151-8. PMID 8814747
58: Hausser C, Robert F, Giard N. Balint’s syndrome. Can J Neurol Sci 1980;7:157-61. PMID 7407722
59: Hawkes C. “Locked-in syndrome”: report of seven cases. Brit Med J 1974;4:379-82. PMID 4425887
60: Hayashi R. Convergence substitution in pontine tegmental hemorrhage: an observation with frenzel goggles. Intern Med 2006;45(8):553-5. PMID 16702751
61: Hecaen H, de Ajuriaguerra J. Balint’s syndrome (psychic paralysis of visual fixation) and its minor forms. Brain 1954;77:373-400. PMID 13208876
62: Henn V, Lang W, Hepp K, Reisine H. Experimental gaze palsies in monkeys and their relation to human pathology. Brain 1984;107:619-36. PMID 6722520
63: Herrera DA, Ruge NO, Florez MM, Vargas SA, Ochoa-Escudero M, Castillo M. Neuroimaging findings in moebius sequence. AJNR Am J Neuroradiol 2019;40(5):862-5. PMID 30948378
64: Holmes G. Disturbances of visual orientation. Brit J Ophthalmol 1918;2:449-68, 506-16.
65: Holve S, Friedman B, Hoyme HE, et al. Athabascan brainstem dysgenesis syndrome. Am J Med Genet 2003;120:169-73. PMID 12833395
66: Hommell M, Gato J, Pollack P, et al. Magnetic resonance imaging and the “one-and-a-half syndrome”: a case report. J Clin Neuroopthalmol 1987;7:161-4. PMID 2958509
67: Incecik F, Elkay M, Herguner MO, Yerdelen D, Ozcan K, Atlunbasak S. Two siblings with familiar idiopathic scoliosis with conjugate gaze palsy. J Child Neurol 2005;20:81-3. PMID 15791929
68: Jackel R, Gittinger J, Smith T, Passarelli C. Metastatic adenocarcinoma presenting as a one-and-a-half syndrome. J Clin Neuroophthalmol 1986;6:116-9. PMID 2942568
69: Jain NR, Jethani J, Narendran K, Kanth L. Synergistic convergence and split pons in horizontal gaze palsy and progressive scoliosis in two sisters. Indian J Ophthalmol 2011;59(2):162-5. PMID 21350292
70: Jen JC, Chan WM, Bosley TM, et al. Mutations in a human ROBO gene disrupt hindbrain axon pathway crossing and morphogenesis. Science 2004;304:1509-13. PMID 15105459
71: Johansson M, Wentz E, Fernell E, Stromland K, Miller MT, Gillberg C. Autistic spectrum disorders in Mobius sequence: a comprehensive study of 25 individuals. Dev Med Child Neurol 2001;43:338-45. PMID 11368487
72: Johnston J, Sharpe J, Morrow M. Paresis of contralateral smooth pursuit and normal vestibular smooth eye movements after unilateral brainstem lesions. Ann Neurol 1992;31:495-502. PMID 1596085
73: Johnston J, Sharpe J, Ranalli P, Morrow M. Oblique misdirection and slowing of vertical saccades after unilateral lesions of the pontine tegmentum. Neurology 1993;43:2238-44. PMID 8232936
74: Kesler A, Stolovitch C, Hoffmann C, Avni I, Morad Y. Acute ophthalmoplegia and nystagmus in infants fed a thiamine-deficient formula” an epidemic of Wernicke encephalopathy. J Neuro-ophthalmol 2005;25:169-72. PMID 16148620
75: Kohno T, Oohira A, Hori S. Near reflex substituting for acquired horizontal gaze palsy: a case report. Jpn J Ophthalmol 2004;48:584-6. PMID 15592785
76: Lee SH, Lim GH, Kim JS, et al. Acute ophthalmoplegia (without ataxia) associated with anti-gq1b antibody. Neurology 2008;71(6):426-9. PMID 18678825
77: Lopez J, Reigosa R, Losada G, Facal S, Sanchez M, Muniz A. Bilateral infarction of the rostral pontine tegmentum as a cause of isolated bilateral supranuclear sixth nerve palsy related to hypertension. J Neurol Neurosurg Psychiatry 1996;40:238-9. PMID 8708673
78: Luna B, Thulborn K, Strojwas M, et al. Dorsal cortical regions subserving visually guided saccades in humans: an fMRI study. Cereb Cortex 1998;8:40-7. PMID 9510384
79: Luria A, Pravdina-Vinarskaya E, Yarbuss A. Disorders of ocular movement in a case of simultanagnosia. Brain 1963;86:219-28. PMID 13931765
80: MacDonald DB, Streletz LJ, Al-Zayed Z, Abdool S, Stigsby B. Intraoperative neurophysiologic discovery of uncrossed sensory and motor pathways in a patient with horizontal gaze palsy and scoliosis. Clin Neurophysiol 2004;115:576-82. PMID 15036053
81: MacKinnon S, Oystreck DT, Andrews C, Chan WM, Hunter DG, Engle EC. Diagnostic distinctions and genetic analysis of patients diagnosed with moebius syndrome. Ophthalmology 2014;121(7):1461-8. PMID 24612975
82: Magni R, Spadea L, Pece A, Durante A, Mansutti L, Brancato R. Electroretinographic findings in congenital oculomotor apraxia (Cogan’s syndrome). Doc Ophthalmol 1994;86(3):259-66. PMID 7813377
83: Marr JE, Green SH, Willshaw HE. Neurodevelopmental implications of ocular motor apraxia. Dev Med Child Neurol 2005;47:815-9. PMID 16288671
84: Matera E, Petruzzelli MG, Tarantini M, et al. Horizontal gaze palsy with progressive scoliosis with overlapping epilepsy and learning difficulties: a case report. Brain Sciences 2022;12(5):613. PMID 35625000
85: Meienberg O, Buttner-Ennever J, Kraus-Ruppert R. Unilateral paralysis of conjugate gaze due to lesion of the abducens nucleus. Neuroophthalmol 1981;2:47-52.
86: Milea D, Napolitano M, Dechy H, Le Hoang P, Delattre JY, Pierrot-Deseilligny C. Complete bilateral horizontal gaze paralysis disclosing multiple sclerosis. J Neurol Neurosurg Psychiatry 2001;70:252-5. PMID 11160480
87: Miller M, Ray V, Owens P, Chen F. Mobius and Mobius-like syndromes (TTV-OFM, OMLH). J Ped Ophthalmol Strabismus 1989;26:176-88. PMID 2668494
88: Miller NR, Biousse V, Hwang T, Patel S, Newman NJ, Zee DS. Isolated acquired unilateral horizontal gaze paresis from a putative lesion of the abducens nucleus. J Neuroophthalmol 2002;22:204-7. PMID 12352583
89: Morel E, Vernet-der-Garabedian B, Eymard B, Raimond F, Bustarret FA, Bach A. Binding and blocking antibodies to the human acetylcholine receptor: are they selected in various myasthenia gravis forms. Immunol Res 1988;7:212-7. PMID 3193008
90: Muni RH, Wennberg R, Mikulis DJ, Wong AM. Bilateral horizontal gaze palsy in presumed paraneoplastic brainstem encephalitis associated with a benign ovarian teratoma. J Neuroophthalmol 2004;24:114-8. PMID 15179063
91: Naqvi S, Greenwood R, D’Cruz O. Carbamazepine responsive epileptic oral motor and ocular motor apraxia. Neurology 1998;50:1475-7. PMID 9596012
92: Nazaryan-Petersen L, Oliveira IR, Mehrjouy MM, et al. Multigenic truncation of the semaphorin-plexin pathway by a germline chromothriptic rearrangement associated with Moebius syndrome. Hum Mutat 2019;40(8):1057-62. PMID 31033088
93: Newton H, Miner M. “One-and-a-half” syndrome after a resection of a midline cerebellar astrocytoma: case report and discussion of the literature. Neurosurg 1991;29:768-72. PMID 1961411
94: Nixon JN, Dempsey JC, Doherty D, Ishak GE. Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia. Neuroradiology 2016;58(2):179-87. PMID 26458891
95: Oommen K, Smith M, Labadie E. Pontine hemorrhage causing Fisher one-and-a-half syndrome with facial paralysis. J Clin Neuroophthalmol 1982;2:129-32. PMID 6226696
96: Orrison W, Robertson W. Congenital ocular motor apraxia. A possible disconnection syndrome. Arch Neurol 1979;36:29-31. PMID 420600
97: Otaduy MC, Leite Cda C, Nagae LM, et al. Further diffusion tensor imaging contribution in horizontal gaze palsy and progressive scoliosis. Arq Neuropsiquiatr 2009;67(4):1054-6. PMID 20069218
98: Parker D, Mitchell P, Holmes G. Poland-Mobius syndrome. J Med Genet 1981;18:317-20. PMID 7024548
99: Paus T. Location and function of the human frontal eye field: a selective review. Neuropsychologia 1996;34:475-83. PMID 8736560
100: Pedraza S, Gamez J, Rovira A, et al. MRI findings in Mobius syndrome: correlation with clinical features. Neurology 2000;55:1058-60. PMID 11061273
101: Pieh C, Lengyel D, Neff A, Fretz C, Gottlob I. Brainstem hypoplasia in familial horizontal gaze palsy and scoliosis. Neurology 2002;59:462-3. PMID 12177390
102: Pierrot-Deseilligny C, Chain F, Lhermitte F. Syndrome de la formation reticulaire pontique. Precisions physiopathologiques sur les anomalies des mouvements oculaires volontaires. Rev Neurol 1982;138:517-32. PMID 7156638
103: Pierrot-Deseilligny C, Chain F, Serdaru M, Gray F, Lhermitte F. The ‘one-and-a-half’ syndrome. Electroculographic analyses of five cases with deductions about the physiological mechanisms of lateral gaze. Brain 1981;104:665-99. PMID 7326563
104: Pierrot-Deseilligny C, Gautier JC, Loron P. Acquired ocular motor apraxia due to bilateral frontoparietal infarcts. Ann Neurol 1988;23:199-202. PMID 3270327
105: Pierrot-Deseilligny C, Goasguen J. Isolated abducens nucleus damage due to histiocytosis X. Brain 1984;107:1019-32. PMID 6334542
106: Pierrot-Deseilligny C, Gray F, Brunet P. Infarcts of both inferior parietal lobules with impairment of visually guided eye movements, peripheral inattention and optic ataxia. Brain 1986;10:81-97. PMID 3942858
107: Prasad P, Nair S. Congenital ocular apraxia: sporadic and familial. Support for natural resolution. J Neuroophthalmol 1994;14:102-4. PMID 7951926
108: Rappaport L, Urion D, Strand K, Fulton A. Concurrence of congenital ocular motor apraxia and other motor problems: an expanded syndrome. Dev Med Child Neurol 1987;29:85-90. PMID 2435594
109: Rivero-Gutierrez V, Nieto-Gomez Cristina, Hernandez-Galilea E. Horizontal gaze palsy following intraoral local anesthesia, a first manifestation of multiple sclerosis. Revista Mexicana de Oftalmologia 2017;91(6):327-30.
110: Robles J. Congenital ocular motor apraxia in identical twins. Arch Ophthalmol 1966;75:746-9. PMID 5937501
111: Rossi A, Catala M, Biancheri R, Di Comite R, Tortori-Donati P. MR imaging of brain-stem hypoplasia in horizontal gaze palsy with progressive scoliosis. AJNR Am J Neuroradiol 2004;25:1046-8. PMID 15205146
112: Rowe FJ, Wright D, Brand D, et al. Profile of gaze dysfunction following cerebrovascular accident. ISRN Ophthalmol 2013;2013:264604. PMID 24558601
113: Rucker JC, Webb BD, Frempong T, Gaspar H, Naidich TP, Jabs EW. Characterization of ocular motor deficits in congenital facial weakness: Moebius and related syndromes. Brain 2014;137(Pt 4):1068-79. PMID 24561559
114: Savitsky K, Sfez S, Tagle D, et al. The complete sequence of the coding region of the ATM gene reveals similarity to cell cycle regulators in different species. Hum Mol Genet 1995;4:2025-32. PMID 8589678
115: Saxena R, Menon V, Sinha A, Sharma P, Kumar DA, Sethi H. Pontine tuberculoma presenting with horizontal gaze palsy. J Neuroophthalmol 2006;26(4):276-8. PMID 17204922
116: Sharpe JA, Silversides JL, Blair RD. Familial paralysis of horizontal gaze. Associated with pendular nystagmus, progressive sclerosis, and facial contraction with myokymia. Neurology 1975;25(11):1035-40. PMID 1237821
117: Shawkat F, Harris C, Taylor D, Kriss A. The role of ERG/VEP and eye movement recordings in children with ocular motor apraxia. Eye 1996;10:53-60. PMID 8763304
118: Shawkat F, Kingslet D, Kendall B, Russell-Eggitt I, Taylor D, Harris C. Neuroradiological and eye movement correlates in children with intermittent saccade failure: “ocular motor apraxia.” Neuropediatrics 1995;26:298-305. PMID 8719744
119: Sicotte NL, Salamon G, Shattuck DW, et al. Diffusion tensor MRI shows abnormal brainstem crossing fibers associated with ROBO3 mutations. Neurology 2006;67(3):519-21. PMID 16894121
120: Smith M, Buchsbaum H, Masland W. One and a half syndrome. Occurrence after trauma with computerized tomographic correlation. Arch Neurol 1980;37:251. PMID 7362495
121: Spierer A, Barak A. Strabismus surgery in children with Mobius syndrome. J AAPOS 2000;4(1):58-9. PMID 10675874
122: Stavrou P, Willshaw HE. Familial congenital horizontal gaze palsy. J Pediatr Ophthalmol Strabismus 1999;36(1):47-9. PMID 9972517
123: Summers C, MacDonald J, Wirtschafter J. Ocular motor apraxia associated with intracranial lipoma. J Pediatr Ophthalmol Strabismus 1987;24:267-9. PMID 3681617
124: Their P, Bachor A, Faiss J, Dichgans J, Koenig E. Selective impairment of smooth pursuit eye movements due to an ischemic lesion of the basal pons. Ann Neurol 1991;29:443-8. PMID 1929215
125: Tomas-Roca L, Tsaalbi-Shtylik A, Jansen JG, et al. De novo mutations in PLXND1 and REV3L cause Mobius syndrome. Nat Comm 2015;6:7199. PMID 26068067
126: Tripp JH, Lake BD, Young E, Ngu J, Brett EM. Juvenile Gaucher’s disease with horizontal gaze palsy in three siblings. J Neurol Neurosurg Psychiatry 1977;40(5):470-8. PMID 408464
127: Tusa RJ, Hove MT. Ocular and oculomotor signs in Joubert syndrome. J Child Neurol 1999;14(10):621-7. PMID 10511333
128: Ucan B, Sahap SK, Bako D, Tiras ST, Ceylaner S, Fitoz S. Horizontal gaze palsy with progressive scoliosis in an infant diagnosed before developing scoliosis: MRI and DTI findings. Klin Padiatr 2022;234(1):52-5. PMID 34284498
129: Vassella F, Lutschg J, Mumenthaler M. Cogan’s congenital ocular motor apraxia in two successive generations. Dev Med Child Neurol 1972;14:788-96. PMID 4643438
130: Vauzelle C, Beghin D, Cournot MP, Elefant E. Birth defects after exposure to misoprostol in the first trimester of pregnancy: prospective follow-up study. Reprod Toxicol 2013;36:98-103. PMID 23207166
131: Verzijl HT, Padberg GW, Zwarts MJ. The spectrum of Mobius syndrome: an electrophysiological study. Brain 2005a;128:1728-36. PMID 15829555
132: Verzijl HT, Valk J, de Vries R, Padberg GW. Radiologic evidence for absence of the facial nerve in Mobius syndrome. Neurology 2005b;64:849-55. PMID 15753421
133: Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology 2003;61:327-33. PMID 12913192
134: Vincent A, Newsom-Davies J. Anti-acetylcholine receptor antibodies. J Neurol Neurosurg Psychiatry 1980;43:590-600. PMID 7400823
135: Wall M, Wray S. The one-and-a-half syndrome: a unilateral disorder of the pontine tegmentum: a study of twenty cases and a review of the literature. Neurology 1983;33:971-80. PMID 6683820
136: Washio N, Suzuki Y, Yamaki T, Kase M, Ohtsuka K. Vertical-torsional oscillations and dissociated bilateral horizontal gaze palsy in a patient with a pontine cavernous angioma. J Neurol Neurosurg Psychiatry 2005;76:283-5. PMID 15654054
137: Willison H, Veitch J, Paterson G, Kennedy P. Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside. J Neurol Neurosurg Psychiatry 1993;56:204-6. PMID 8437011
138: Xavier C, Vieira M, Duarte AF, Xavier A, Silva ED. Horizontal gaze palsy and progressive scoliosis in dizygotic twins. J Binocul Vis Ocul Motil 2022;72(2):105-10. PMID 35254960
139: Yang HK, Choi JY, Park KS, Kim JH, Hwang JM. Teaching Video NeuroImages: Horizontal gaze palsy with progressive scoliosis. Neurology 2019;92(8):e886-7. PMID 30777921
140: Yigit A, Bingol A, Mutluer N, Tascilar N. The one-and-a-half syndrome in systemic lupus erythematosis. J Clin Neuroopthalmol 1996;16:274-6. PMID 8956164
141: Zackon D, Sharpe J. Midbrain paresis of horizontal gaze. Ann Neurol 1984;16:495-504. PMID 6388487
142: Zaret C, Behrens M, Eggers H. Congenital ocular motor apraxia and brainstem tumour. Arch Ophthalmol 1980;98:328-30. PMID 7352886
143: Zee D, Optican L, Cook J, Robinson D, Engel W. Slow saccades in spinocerebellar degeneration. Arch Neurol 1976;33:243-51. PMID 1083233

Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Author

Yin Allison Liu MD PhD

Dr. Liu of University of California, Davis has no relevant financial relationships to disclose.
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Heather E Moss MD PhD

Dr. Moss of Stanford University has no relevant financial relationships to disclose.
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Former Authors

Heather E Moss MD PhD
Susiani Intan MD
Jason J S Barton MD PhD

Patient Profile

Age range of presentation: 0 month to 65+years
Sex preponderance: male=female
Heredity: heredity may be a factor
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-9: Palsy of conjugate gaze: 378.81

Deficiencies of saccadic eye movements: 379.57

Deficiencies of smooth pursuit movements: 379.58
ICD-10: Palsy of conjugate gaze: H51.0

Nystagmus and other irregular eye movements: H55

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Horizontal gaze palsy

Associated Disorders

Ataxia telangiectasia
Mobius syndrome

Differential Diagnosis

Graves ophthalmopathy
ocular myopathies
myasthenia gravis
ocular myasthenia
proximal limb or bulbar weakness
- botulism
- Miller-Fisher syndrome

Horizontal gaze palsy …

Horizontal gaze palsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Prevention

Differential diagnosis

Diagnostic workup

Management

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview.
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to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Neurogenetics and genetic and genomic testing

Acute traumatic spinal cord injury

Tumors of the skull base

Common maculopathies

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

Horizontal gaze palsy

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Presentation and course

Prognosis and complications

Clinical vignette

Biological basis

Etiology and pathogenesis

Prevention

Differential diagnosis

Diagnostic workup

Management

Media

References

Contributors

Author

Editor

Former Authors

Patient Profile

ICD & OMIM codes

This is an article preview. Start a Free Account to access the full version.

Questions or Comment?

Also in This Category

NF2-related schwannomatosis

Neurogenetics and genetic and genomic testing

Acute traumatic spinal cord injury

Tumors of the skull base

Common maculopathies

Toxic and nutritional deficiency optic neuropathies

Third nerve palsy

Nystagmus

This is an article preview.
Start a Free Account
to access the full version.