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Oculomotor deficits are common in hereditary ataxia, but disproportionally neglected in clinical ataxia scales and as outcome measures for interventional trials. Quantitative assessment of oculomotor function has become increasingly available and thus applicable in multicenter trials and offers the opportunity to capture severity and progression of oculomotor impairment in a sensitive and reliable manner. In this consensus paper of the Ataxia Global Initiative Working Group On Digital Oculomotor Biomarkers, based on a systematic literature review, we propose harmonized methodology and measurement parameters for the quantitative assessment of oculomotor function in natural-history studies and clinical trials in hereditary ataxia. MEDLINE was searched for articles reporting on oculomotor/vestibular properties in ataxia patients and a study-tailored quality-assessment was performed. One-hundred-and-seventeen articles reporting on subjects with genetically confirmed (n=1134) or suspected hereditary ataxia (n=198), and degenerative ataxias with sporadic presentation (n=480) were included and subject to data extraction. Based on robust discrimination from controls, correlation with disease-severity, sensitivity to change, and feasibility in international multicenter settings as prerequisite for clinical trials, we prioritize a core-set of five eye-movement types: (i) pursuit eye movements, (ii) saccadic eye movements, (iii) fixation, (iv) eccentric gaze holding, and (v) rotational vestibulo-ocular reflex. We provide detailed guidelines for their acquisition, and recommendations on the quantitative parameters to extract. Limitations include low study quality, heterogeneity in patient populations, and lack of longitudinal studies. Standardization of quantitative oculomotor assessments will facilitate their implementation, interpretation, and validation in clinical trials, and ultimately advance our understanding of the evolution of oculomotor network dysfunction in heredita

Purpose The aim of this study was to review the available scientific literature on the possible relationship between the visual system and motor development in children.MethodsThis study was performed according to the Preferred Reporting Items for Systematic Reviews (PRISMA) statement recommendations. The review protocol is available in PROSPERO (CRD42021245341). Four different databases, namely Scopus, PubMed, CINAHL and Web of Science, were assessed from April 2005 to February 2021. To determine the quality of the articles, we used the Critical Appraisal Skills Programme (CASP) Quality Appraisal Scale, and a protocol was followed to define the levels of evidence on the basis of the Centre for Evidence-Based Medicine Levels of Evidence. The search strategy included terms describing motor development in children and adolescents with visual disorders.Results Among the identified studies, 23 were included in the study. All selected articles examined the relationship between the visual system and development in children. The quality of most of the studies was moderate–high, and they were between evidence levels 2 and 4.ConclusionsOur systematic review revealed that all included studies established a relationship between the visual system and development in children. However, the methods for measuring the visual system and motor skills lacked uniformity.

Purpose The aim of this study was to review the available scientific literature on the possible relationship between the visual system and motor development in children.MethodsThis study was performed according to the Preferred Reporting Items for Systematic Reviews (PRISMA) statement recommendations. The review protocol is available in PROSPERO (CRD42021245341). Four different databases, namely Scopus, PubMed, CINAHL and Web of Science, were assessed from April 2005 to February 2021. To determine the quality of the articles, we used the Critical Appraisal Skills Programme (CASP) Quality Appraisal Scale, and a protocol was followed to define the levels of evidence on the basis of the Centre for Evidence-Based Medicine Levels of Evidence. The search strategy included terms describing motor development in children and adolescents with visual disorders.Results Among the identified studies, 23 were included in the study. All selected articles examined the relationship between the visual system and development in children. The quality of most of the studies was moderate–high, and they were between evidence levels 2 and 4.ConclusionsOur systematic review revealed that all included studies established a relationship between the visual system and development in children. However, the methods for measuring the visual system and motor skills lacked uniformity.

IntroductionClinical diagnosis of Parkinsonism is still challenging, and the diagnostic biomarkers of Multiple System Atrophy (MSA) are scarce. This study aimed to investigate the diagnostic value of the combined eye movement tests in patients with Parkinson's disease (PD) and those with MSA.MethodsWe enrolled 96 PD patients, 33 MSA patients (18 with MSA-P and 15 with MSA-C), and 40 healthy controls who had their horizontal ocular movements measured. The multiple-step pattern of memory-guided saccade (MGS), the hypometria/hypermetria of the reflexive saccade, the abnormal saccade in smooth pursuit movement (SPM), gaze-evoked nystagmus, and square-wave jerks in gaze-holding test were qualitatively analyzed. The reflexive saccadic parameters and gain of SPM were also quantitatively analyzed.ResultsThe MGS test showed that patients with either diagnosis had a significantly higher incidence of multiple-step pattern compared with controls (68.6%, 65.2%, and versus. 2.5%, p < .05, in PD, MSA, versus. controls, respectively). The reflexive saccade test showed that MSA patients showing a prominent higher incidence of the abnormal saccade (63.6%, both hypometria and hypermetria) than that of PD patients and controls (33.3%, 7.5%, respectively, hypometria) (p < .05). The SPM test showed PD patients had mildly decreased gain among whom 28.1% presenting "saccade intrusions"; and that MSA patients had the significant decreased gain with 51.5% presenting "catch-up saccades"(p < .05). Only MSA patients showed gaze-evoked nystagmus (24.2%), square-wave jerks (6.1%) in gaze-holding test (p < .05).ConclusionsA panel of eye movements tests may help to differentiate PD from MSA. The combined presence of hypometria and hypermetria in saccadic eye movement, the impaired gain of smooth pursuit movement with "catch-up saccades," gaze-evoked nystagmus, square-wave jerks in gaze-holding test, and multiple-step pattern in MGS may provide clu

Globular glial tauopathy (GGT) is a rare 4-repeat tauopathy characterized by the accumulation of tau globular inclusions in astrocytes and oligodendrocytes. Several clinical phenotypes have been associated with GGT, making the prediction of this rare pathological entity difficult. We report the case of a patient with eye-movement abnormalities and gait instability, reminiscent of progressive supranuclear palsy-Richardson's syndrome (PSP-RS), who later developed upper motor neuron symptoms suggestive of primary lateral sclerosis (PLS). Neuropathological assessment revealed GGT type III pathology. A theoretical framework is proposed to help clinicians predict GGT in subjects with coexistent features of PSP-RS and PLS.

BackgroundThe Movement Disorder Society criteria for progressive supranuclear palsy define diagnostic allocations, stratified by certainty levels and clinical predominance types. We aimed to study the frequency of ambiguous multiple allocations and to develop rules to eliminate them.MethodsWe retrospectively collected standardized clinical data by chart review in a multicenter cohort of autopsy-confirmed patients with progressive supranuclear palsy, to classify them by diagnostic certainty level and predominance type and to identify multiple allocations.ResultsComprehensive data were available from 195 patients. More than one diagnostic allocation occurred in 157 patients (80.5%). On average, 5.4 allocations were possible per patient. We developed four rules for Multiple Allocations eXtinction (MAX). They reduced the number of patients with multiple allocations to 22 (11.3%), and the allocations per patient to 1.1.ConclusionsThe proposed MAX rules help to standardize the application of the Movement Disorder Society criteria for progressive supranuclear palsy. © 2019 International Parkinson and Movement Disorder Society.

BackgroundThe Movement Disorder Society criteria for progressive supranuclear palsy define diagnostic allocations, stratified by certainty levels and clinical predominance types. We aimed to study the frequency of ambiguous multiple allocations and to develop rules to eliminate them.MethodsWe retrospectively collected standardized clinical data by chart review in a multicenter cohort of autopsy-confirmed patients with progressive supranuclear palsy, to classify them by diagnostic certainty level and predominance type and to identify multiple allocations.ResultsComprehensive data were available from 195 patients. More than one diagnostic allocation occurred in 157 patients (80.5%). On average, 5.4 allocations were possible per patient. We developed four rules for Multiple Allocations eXtinction (MAX). They reduced the number of patients with multiple allocations to 22 (11.3%), and the allocations per patient to 1.1.ConclusionsThe proposed MAX rules help to standardize the application of the Movement Disorder Society criteria for progressive supranuclear palsy. © 2019 International Parkinson and Movement Disorder Society.

Huntington disease (HD) is a neurodegenerative condition with prominent motor (including oculomotor), cognitive, and psychiatric effects. While neuropsychological deficits are present in HD, motor impairments may impact performance on neuropsychological measures, especially those requiring a speeded response, as has been demonstrated in multiple sclerosis and schizophrenia. The current study is the first to explore associations between oculomotor functions and neuropsychological performance in HD. Participants with impaired oculomotor functioning performed worse than those with normal oculomotor functioning on cognitive tasks requiring oculomotor involvement, particularly on psychomotor speed tasks, controlling for covariates. Consideration of oculomotor dysfunction on neuropsychological performance is critical, particularly for populations with motor deficits.

Huntington disease (HD) is a neurodegenerative condition with prominent motor (including oculomotor), cognitive, and psychiatric effects. While neuropsychological deficits are present in HD, motor impairments may impact performance on neuropsychological measures, especially those requiring a speeded response, as has been demonstrated in multiple sclerosis and schizophrenia. The current study is the first to explore associations between oculomotor functions and neuropsychological performance in HD. Participants with impaired oculomotor functioning performed worse than those with normal oculomotor functioning on cognitive tasks requiring oculomotor involvement, particularly on psychomotor speed tasks, controlling for covariates. Consideration of oculomotor dysfunction on neuropsychological performance is critical, particularly for populations with motor deficits.

OBJECTIVE: To delineate an algorithm of actuation designed for the attention to children and adolescents with acute torticollis and to offer them an efficient and opportune medical assistance.DEVELOPMENT: The attention of children and adolescents with acute torticollis requires that the physician may consider a group of disorders with different outcomes. Previously he should have a strategy for diagnosis that it may let him to arrive to quick and correct conclusions. Here is presented a proposition about the assistance to patients with acute torticollis. The proposed principles are organized and in the first line are considered evolution characteristic of events. Next, is important the inclusion of patients in groups with common characteristics that allow to carry out an approach to a cluster of illnesses. Lastly, should be included some suggestions about key investigations in occasion of suspicion of one specific disorder or even when there is not sufficient clear orientation of what happens.CONCLUSIONS: The delineated algorithm of actuation in children and adolescents with acute torticollis may to facilitate the correct and opportune action although this proposition only constitutes an orientation. With the professional experience and knowledge, the algorithm may be modified according different situations during the attention of one pediatric patient affected by acute torticollis., OBJETIVOS: Delinear un algoritmo de actuación ante un niño o adolescente con tortícolis aguda para facilitar la asistencia médica adecuada y oportuna.DESARROLLO: La atención de un niño o adolescente con tortícolis aguda, requiere que el médico considere un grupo de trastornos con diferentes pronósticos. Previamente debe poseer una estrategia diagnóstica que le permita llegar a conclusiones de una manera rápida y a la vez, adecuada. Se presenta una propuesta para la asistencia de estos pacientes. Los principios propuestos se organizan de manera que en primer lugar se establezcan las características evolutivas del evento. Luego la inclusión en grupos con características comunes que permitan realizar un acercamiento a un conjunto de enfermedades. Por último, se incluyen las sugerencias de los exámenes a indicar en caso de sospecha de un trastorno en específico o incluso cuando no hay una orientación suficientemente clara de lo que ocurre.CONCLUSIONES: El algoritmo de actuación delineado ante un niño o adolescente con tortícolis aguda puede facilitar la acción adecuada y oportuna, aunque constituye solo una orientación. Con la experiencia y los conocimientos de cada profesional dicho algoritmo puede ser modificado ante las múltiples situaciones que pueden presentarse en un paciente pediátrico con tortícolis aguda.

OBJECTIVE: To delineate an algorithm of actuation designed for the attention to children and adolescents with acute torticollis and to offer them an efficient and opportune medical assistance.DEVELOPMENT: The attention of children and adolescents with acute torticollis requires that the physician may consider a group of disorders with different outcomes. Previously he should have a strategy for diagnosis that it may let him to arrive to quick and correct conclusions. Here is presented a proposition about the assistance to patients with acute torticollis. The proposed principles are organized and in the first line are considered evolution characteristic of events. Next, is important the inclusion of patients in groups with common characteristics that allow to carry out an approach to a cluster of illnesses. Lastly, should be included some suggestions about key investigations in occasion of suspicion of one specific disorder or even when there is not sufficient clear orientation of what happens.CONCLUSIONS: The delineated algorithm of actuation in children and adolescents with acute torticollis may to facilitate the correct and opportune action although this proposition only constitutes an orientation. With the professional experience and knowledge, the algorithm may be modified according different situations during the attention of one pediatric patient affected by acute torticollis., OBJETIVOS: Delinear un algoritmo de actuación ante un niño o adolescente con tortícolis aguda para facilitar la asistencia médica adecuada y oportuna.DESARROLLO: La atención de un niño o adolescente con tortícolis aguda, requiere que el médico considere un grupo de trastornos con diferentes pronósticos. Previamente debe poseer una estrategia diagnóstica que le permita llegar a conclusiones de una manera rápida y a la vez, adecuada. Se presenta una propuesta para la asistencia de estos pacientes. Los principios propuestos se organizan de manera que en primer lugar se establezcan las características evolutivas del evento. Luego la inclusión en grupos con características comunes que permitan realizar un acercamiento a un conjunto de enfermedades. Por último, se incluyen las sugerencias de los exámenes a indicar en caso de sospecha de un trastorno en específico o incluso cuando no hay una orientación suficientemente clara de lo que ocurre.CONCLUSIONES: El algoritmo de actuación delineado ante un niño o adolescente con tortícolis aguda puede facilitar la acción adecuada y oportuna, aunque constituye solo una orientación. Con la experiencia y los conocimientos de cada profesional dicho algoritmo puede ser modificado ante las múltiples situaciones que pueden presentarse en un paciente pediátrico con tortícolis aguda.

ObjectiveFragile X premutation carriers (fXPCs) have an expansion of 55-200 CGG repeats in the FMR1 gene. Male fXPCs are at risk for developing a neurodegenerative motor disorder (FXTAS) often accompanied by inhibitory control impairments, even in fXPCs without motor symptoms. Inhibitory control impairments might precede, and thus indicate elevated risk for motor impairment associated with FXTAS. We tested whether inhibitory impairments are observable in fXPCs by assessing oculomotor performance.MethodParticipants were males aged 18-48 years asymptomatic for FXTAS. FXPCs (n = 21) and healthy age-matched controls (n = 22) performed four oculomotor tasks. In a Fixation task, participants fixated on a central cross and maintained gaze position when a peripheral stimulus appeared. In a Pursuit task, participants maintained gaze on a square moving at constant velocity. In a Prosaccade task, participants fixated on a central cross, then looked at a peripheral stimulus. An Antisaccade task was identical to the Prosaccade task, except participants looked in the direction opposite the stimulus. Inhibitory cost was the difference in saccade latency between the Antisaccade and Prosaccade tasks.ResultsRelative to controls, fXPCs had longer saccade latency in the Antisaccade task. In fXPCs, inhibitory cost was positively associated with vermis area in lobules VI-VII.ConclusionAntisaccades require inhibitory control to inhibit reflexive eye movements. We found that eye movements are sensitive to impaired inhibitory control in fXPCs asymptomatic for FXTAS. Thus, eye movements may be useful in assessing FXTAS risk or disease progression.

INTRODUCCIÓN: Usualmente los schwannomas del sistema nervioso central se originan del nervio vestibular y ocasionalmente del trigémino; aquellos originados dentro del seno cavernoso, son extremadamente raros y la literatura adolece de publicaciones al respecto. CASO CLÍNICO: Presentamos el reporte del primer caso de schwannoma esfeno–cavernoso manejado en el servicio de Neurocirugía del Hospital México de Costa Rica. Se hace una revisión de la anatomía pertinente, y se presentan los hallazgos de imagen pre y postoperatorios. CONCLUSIONES: Los schwannomas esfeno–cavernosos son raros. Este tipo de lesiones deberán de ser abordadas por personal formalmente entrenadas, con un amplio conocimiento de las técnicas de cirugía de base de cráneo, de la anatomía microquirúrgica y que también tengan acceso a un laboratorio de microcirugía., INTRODUCTION: Schwannomas of the central nervous system usually originate from the vestibular nerve and occasionally from the trigeminal nerve. Those that originate within the cavernous sinus are extremely rare and the literature is scanty. CASE REPORT: We present the report of the first case managed at our neurosurgery service of Hospital México (Costa Rica). We review the pertinent anatomy, and also the pre and post–operatory images. CONCLUSIONS: Spheno–cavernous sinus schwannomas are rare and should surgically approach by trained surgeons with a great knowledge of microsurgical anatomy and skull base surgery and also with the availability of a microsurgical lab.

ObjectiveFragile X premutation carriers (fXPCs) have an expansion of 55-200 CGG repeats in the FMR1 gene. Male fXPCs are at risk for developing a neurodegenerative motor disorder (FXTAS) often accompanied by inhibitory control impairments, even in fXPCs without motor symptoms. Inhibitory control impairments might precede, and thus indicate elevated risk for motor impairment associated with FXTAS. We tested whether inhibitory impairments are observable in fXPCs by assessing oculomotor performance.MethodParticipants were males aged 18-48 years asymptomatic for FXTAS. FXPCs (n = 21) and healthy age-matched controls (n = 22) performed four oculomotor tasks. In a Fixation task, participants fixated on a central cross and maintained gaze position when a peripheral stimulus appeared. In a Pursuit task, participants maintained gaze on a square moving at constant velocity. In a Prosaccade task, participants fixated on a central cross, then looked at a peripheral stimulus. An Antisaccade task was identical to the Prosaccade task, except participants looked in the direction opposite the stimulus. Inhibitory cost was the difference in saccade latency between the Antisaccade and Prosaccade tasks.ResultsRelative to controls, fXPCs had longer saccade latency in the Antisaccade task. In fXPCs, inhibitory cost was positively associated with vermis area in lobules VI-VII.ConclusionAntisaccades require inhibitory control to inhibit reflexive eye movements. We found that eye movements are sensitive to impaired inhibitory control in fXPCs asymptomatic for FXTAS. Thus, eye movements may be useful in assessing FXTAS risk or disease progression.

INTRODUCTION: Schwannomas of the central nervous system usually originate from the vestibular nerve and occasionally from the trigeminal nerve. Those that originate within the cavernous sinus are extremely rare and the literature is scanty. CASE REPORT: We present the report of the first case managed at our neurosurgery service of Hospital México (Costa Rica). We review the pertinent anatomy, and also the pre and post–operatory images. CONCLUSIONS: Spheno–cavernous sinus schwannomas are rare and should surgically approach by trained surgeons with a great knowledge of microsurgical anatomy and skull base surgery and also with the availability of a microsurgical lab., INTRODUCCIÓN: Usualmente los schwannomas del sistema nervioso central se originan del nervio vestibular y ocasionalmente del trigémino; aquellos originados dentro del seno cavernoso, son extremadamente raros y la literatura adolece de publicaciones al respecto. CASO CLÍNICO: Presentamos el reporte del primer caso de schwannoma esfeno–cavernoso manejado en el servicio de Neurocirugía del Hospital México de Costa Rica. Se hace una revisión de la anatomía pertinente, y se presentan los hallazgos de imagen pre y postoperatorios. CONCLUSIONES: Los schwannomas esfeno–cavernosos son raros. Este tipo de lesiones deberán de ser abordadas por personal formalmente entrenadas, con un amplio conocimiento de las técnicas de cirugía de base de cráneo, de la anatomía microquirúrgica y que también tengan acceso a un laboratorio de microcirugía.

ObjectiveTo identify the genetic cause of a syndrome causing cerebellar ataxia and eye movement abnormalities.MethodsWe identified 2 families with cerebellar ataxia, eye movement abnormalities, and global developmental delay. We performed genetic analyses including single nucleotide polymorphism genotyping, linkage analysis, array comparative genomic hybridization, quantitative PCR, and Sanger sequencing. We obtained eye movement recordings of mutant mice deficient for the ortholog of the identified candidate gene, and performed immunohistochemistry using human and mouse brain specimens.ResultsAll affected individuals had ataxia, eye movement abnormalities, most notably tonic upgaze, and delayed speech and cognitive development. Homozygosity mapping identified the disease locus on chromosome 4q. Within this region, a homozygous deletion of GRID2 exon 4 in the index family and compound heterozygous deletions involving GRID2 exon 2 in the second family were identified. Grid2-deficient mice showed larger spontaneous and random eye movements compared to wild-type mice. In developing mouse and human cerebella, GRID2 localized to the Purkinje cell dendritic spines. Brain MRI in 2 affected children showed progressive cerebellar atrophy, which was more severe than that of Grid2-deficient mice.ConclusionsBiallelic deletions of GRID2 lead to a syndrome of cerebellar ataxia and tonic upgaze in humans. The phenotypic resemblance and similarity in protein expression pattern between humans and mice suggest a conserved role for GRID2 in the synapse organization between parallel fibers and Purkinje cells. However, the progressive and severe cerebellar atrophy seen in the affected individuals could indicate an evolutionarily unique role for GRID2 in the human cerebellum.

Although encephalopathy, seizures, and changes in tone comprise the most common signs of neurologic disruption in neonates (and in older children and adults), careful neurologic examination can yield additional clues to assist with the management of underlying neurologic conditions.

Although encephalopathy, seizures, and changes in tone comprise the most common signs of neurologic disruption in neonates (and in older children and adults), careful neurologic examination can yield additional clues to assist with the management of underlying neurologic conditions.

Frontotemporal lobar degeneration (FTLD) often overlaps clinically with corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), both of which have prominent eye movement abnormalities. To investigate the ability of oculomotor performance to differentiate between FTLD, Alzheimer's disease, CBS and PSP, saccades and smooth pursuit were measured in three FTLD subtypes, including 24 individuals with frontotemporal dementia (FTD), 19 with semantic dementia (SD) and six with progressive non-fluent aphasia (PA), as compared to 28 individuals with Alzheimer's disease, 15 with CBS, 10 with PSP and 27 control subjects. Different combinations of oculomotor abnormalities were identified in all clinical syndromes except for SD, which had oculomotor performance that was indistinguishable from age-matched controls. Only PSP patients displayed abnormalities in saccade velocity, whereas abnormalities in saccade gain were observed in PSP > CBS > Alzheimer's disease subjects. All patient groups except those with SD were impaired on the anti-saccade task, however only the FTLD subjects and not Alzheimer's disease, CBS or PSP groups, were able to spontaneously self-correct anti-saccade errors as well as controls. Receiver operating characteristic statistics demonstrated that oculomotor findings were superior to neuropsychological tests in differentiating PSP from other disorders, and comparable to neuropsychological tests in differentiating the other patient groups. These data suggest that oculomotor assessment may aid in the diagnosis of FTLD and related disorders.

Frontotemporal lobar degeneration (FTLD) often overlaps clinically with corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), both of which have prominent eye movement abnormalities. To investigate the ability of oculomotor performance to differentiate between FTLD, Alzheimer's disease, CBS and PSP, saccades and smooth pursuit were measured in three FTLD subtypes, including 24 individuals with frontotemporal dementia (FTD), 19 with semantic dementia (SD) and six with progressive non-fluent aphasia (PA), as compared to 28 individuals with Alzheimer's disease, 15 with CBS, 10 with PSP and 27 control subjects. Different combinations of oculomotor abnormalities were identified in all clinical syndromes except for SD, which had oculomotor performance that was indistinguishable from age-matched controls. Only PSP patients displayed abnormalities in saccade velocity, whereas abnormalities in saccade gain were observed in PSP > CBS > Alzheimer's disease subjects. All patient groups except those with SD were impaired on the anti-saccade task, however only the FTLD subjects and not Alzheimer's disease, CBS or PSP groups, were able to spontaneously self-correct anti-saccade errors as well as controls. Receiver operating characteristic statistics demonstrated that oculomotor findings were superior to neuropsychological tests in differentiating PSP from other disorders, and comparable to neuropsychological tests in differentiating the other patient groups. These data suggest that oculomotor assessment may aid in the diagnosis of FTLD and related disorders.

Purpose: To study the frequency of different types of eye tracking disturbances in schizophrenia. Materials and Methods: Smooth pursuit eye movements were studied by electro-oculography (EOG) in 22 schizophrenic patients (ICD-10 criteria) and 15 age and sex-matched controls. The studied parameters included average pursuit gain, number of saccades, the frequency of different types of saccades (catch-up, back-up, anticipatory saccades), and disturbances during fixation. The results were analysed statistically. Results: The average pursuit gain was significantly affected in patients for target velocity of 30°/sec (p=0.007). The catch-up and back-up saccades were more common in cases than controls but the difference was not significant (p=0.39 and 0.36 respectively). The anticipatory saccades were significantly more frequent in cases than controls (p<0.0001) for both 15°/sec and 30°/sec target velocities. This was also correlated with the duration of illness. Conclusion: Anticipatory saccades are significantly more frequent during eye tracking in schizophrenia and appear to be an objective marker for the disease.

Significant contributions on the neuroophthalmologic manifestations of systemic disease have been published in the past year. The first part of this paper is devoted to practical guidelines that may help in the diagnosis and the management of oculomotor disorders, especially in connection with systemic diseases; the second part is focused on specific entities. Including idiopathic intracranial hypertension and ophthalmoplegic migraine in this review might be debatable; however, although the cause of these conditions is still unclear, relevant findings suggest that they may be the consequence of other adjacent processes rather than the cause itself. We believe that the diagnosis of idiopathic intracranial hypertension as well as that of ophthalmoplegic migraine remains a challenging issue for the ophthalmologist and may easily be overlooked.

We studied prospectively 105 unselected patients complaining of ptosis and/or diplopia due to extrinsic ophthalmic muscle palsies without other neurological signs. All patients underwent the same diagnostic protocol. The presenting symptoms were: ptosis, 35 patients (33%); diplopia, 27 patients (26%); ptosis and diplopia, 43 patients (41%). The oculomotor nerve was most frequently involved, followed by the abducens nerve. The final diagnoses were: ocular myasthenia, intracranial and/or orbital pathology, thyroid ophthalmopathy, diabetic ophthalmoplegia, mitochondrial myopathy, oculopharyngeal muscular dystrophy. In 26 patients (25%) the cause remained undetermined. Our study confirms the difficulty of establishing an aetiological diagnosis in patients with isolated ocular palsies.