Tonic Deviation Research Articles

A quantitative approach to study the adaptation of rhythmic eye movements and the resulting tonic eye deviation in larval zebrafish.

To optimize performance during vital tasks, animals are capable of tuning rhythmic neural signals that drive repetitive behaviors, such as motor reflexes under constant sensory stimuli. In the oculomotor system, animals track the moving image during slow phases while repetitively resetting the eye position from the eccentricity during quick phases. During optokinetic response (OKR), larval zebrafish occasionally show a delayed quick phase; thus, the eyes remain tonically deviated from the center. In this study, we scrutinized OKR in larval zebrafish under a broad range of stimulus velocities to determine the parametric property of the quick-phase delay. A prolonged stimulation revealed that the slow-phase (SP) duration-the interval between two quick phases-was tuned increasingly over time toward a homeostatic range, regardless of stimulus velocity. Attributed to this rhythm control, larval zebrafish exhibited a tonic eye deviation following slow phases, which was especially pronounced when tracking a fast stimulus over an extended time period. In addition to the SP duration, the fixation duration between spontaneous saccades in darkness also revealed a similar adaptive property after the prolonged optokinetic stimulation. Our results provide a quantitative description of the adaptation of rhythmic eye movements in developing animals and pave the way for potential animal models for eye movement disorders.

Toward Affirmation of Recovery of Deeply Embedded Autobiographical Memory with Background Music and Identification of an EEG Biomarker in Combination with EDA Signal Using Wearable Sensors

There is no disputing the role that background music plays in memory recall. Music has the power to activate the brain and trigger deeply ingrained memories. For dementia patients, background music is a common therapy because of this. Previous studies used music to recall lyrics, series of words, and long- and short-term memories. In this research, electroencephalogram (EEG) and electrodermal activity (EDA) data are collected from 40 healthy participants using wearable sensors during nine music sessions (three happy, three sad, and three neutral). A post-study survey is given to all participants after each piece of music to know if they recalled any autobiographical memories. The main objective is to find an EEG biomarker using the collected qualitative and quantitative data for autobiographical memory recall. The study finds that for all four EEG channels, alpha power rises considerably (on average 16.2%) during the memory “recall” scenario (F3: p = 0.0066, F7: p = 0.0386, F4: p = 0.0023, and F8: p = 0.0288) compared to the “no-recall” situation. Beta power also increased significantly for two channels (F3: p = 0.0100 and F4: p = 0.0210) but not for others (F7: p = 0.6792 and F8: p = 0.0814). Additionally, the phasic standard deviation (p = 0.0260), phasic max (p = 0.0011), phasic energy (p = 0.0478), tonic min (p = 0.0092), tonic standard deviation (p = 0.0171), and phasic energy (p = 0.0478) are significantly different for the EDA signal. The authors conclude by interpreting increased alpha power (8–12 Hz) as a biomarker for autobiographical memory recall.

Self-limited epilepsy of childhood with affective seizures: A well-defined epileptic syndrome?

ObjectiveHere we present cases of focal epilepsy with affective symptoms analyzing seizure characteristics, EEG pattern, treatment, and outcome. MethodsA multicenter, descriptive, retrospective study was conducted evaluating 18 patients with self-limited epilepsy who presented with seizures with affective symptoms seen between April 2000 and April 2018 at eight Argentinian centers. ResultsEighteen patients had focal seizures with affective symptoms; all of them had affective symptoms characterized by sudden fright or terror and screaming. Seizures started with manifestations of sudden fright or terror manifested by a facial expression of fear; consciousness was mildly impaired in 15/18 patients.Eleven of the patients also had autonomic manifestations, such as pallor, sweating, and abdominal pain. In addition, four of these 11 patients had ictus emeticus and one also presented with unilateral deviation of the eyes and head.Speech arrest, salivation, glottal noises, and chewing or swallowing movements were observed in 2/18 patients at the onset of the affective seizures. Two others also had mild asymmetric dystonic seizures involving both hands and arms. Three patients had tonic deviation of the mouth involving the lips and tongue as well pharyngeal and laryngeal muscles, resulting in anarthria and drooling. Two patients had brief hemifacial focal clonic seizures. ConclusionAffective manifestations associated or not with motor and/or autonomic manifestations and associated with typical EEG features of the idiopathic focal epilepsies of childhood is a particular presentation of self-limited focal epilepsy in childhood.

Spotlight on Oculogyric Crisis: A Review.

Background:Oculogyric crisis (OGC) is a form of acute dystonia characterized by sustained dystonic, conjugate, and upward deviation of the eyes. It was initially reported in patients with postencephalitic parkinsonism. But later, other factors such as medications, movement disorders, metabolic disorders, and focal brain lesions were also found to be associated with OGC.Methods:The literature regarding OGC was searched via PubMed, Google Scholar, and through citations in relevant articles till December 2019, with keywords including OGC, oculogyric eye movements, tonic eye movement, neuroleptics and OGC, antipsychotics and OGC, and all combinations of these. Only original articles (abstract or full text) that were published in the English language were reviewed.Results:Hypodopaminergic state is implicated in the pathogenesis of OGC. Common risk factors are younger age, male sex, severe illness, high neuroleptic dose, parenteral administration of neuroleptics, high potency of neuroleptic drugs, abrupt discontinuation of anticholinergic medication, and family history of dystonia.Conclusion:OGC is an acute dystonic reaction leading to tonic upward deviation of eyes. It is associated with various neurometabolic, neurodegenerative, and movement disorders and medications such as antipsychotics, antiemetics, antidepressants, antiepileptics, and antimalarials. OGC can adversely impact the compliance and prognosis of the primary illness. Hence, it needs to be managed at earlier stages with appropriate medication, primarily anticholinergics.

Clinical Characteristics of Functional Movement Disorders in the Stomatognathic System.

Background: Functional (psychogenic) movement disorders often have distinguishable clinical features in the orofacial region. Tonic mandibular deviation accompanying ipsilateral downward and lateral lip pulling is the most common phenotype seen in patients with facial functional movement disorders. However, functional movement disorders in the stomatognathic system are underrecognized.Objective: This study aimed to evaluate clinical characteristics and phenomenology in patients with functional movement disorders in the stomatognathic system.Methods: Ten-item inclusion criteria (point range: 0–10) for functional movement disorders in the stomatognathic system was produced, based on previously established criteria for functional movement disorders and general signs of functional facial dystonia, to determine subject inclusion. The criteria included inconsistency, incongruence, and paroxysm in symptoms; rapid onset; distractibility; suggestibility; static course; spreading to multiple sites; spontaneous remission; and lack of sensory tricks. Fifty-eight patients [42 women (72.4%), 16 men (27.6%); mean age: 46.2 years] scored over 7 points on the criteria and were included in further analyses. Characteristic features, including the pattern and site of abnormal movements, were assessed in clinical examination.Results: Frequent items in the scale were inconsistent symptoms (93.1%), incongruous symptoms (91.4%), spreading to multiple sites (89.7%), paroxysmal symptoms (86.2%), and lack of sensory tricks (81%). Sixty percent of patients exhibited a pattern resembling dystonia. Some patients had a combination of organic and functional disease. Common involuntary movements included jaw deviation (74.1%), jaw closing (50%), lip pulling (34.5%), and tongue movement (31%). A functional dystonia phenotype (unilateral lower lip pulling and jaw deviation) was observed in 26 patients (44.8%). Characteristic features of functional stomatognathic movement disorders were rapidly repeating mandibular (lateral or tapping) and tongue movements (27.6%), which fluctuated in speed and direction.Conclusion: In 58 patients with functional movement disorders in the stomatognathic system, the functional dystonia phenotype was observed in 44.8%. Furthermore, 27.6% of patients showed the most characteristic type of functional stomatognathic movement disorders: very fast repeated jaw and/or lingual movements.

“Mini” occipital seizures with accompanying ultra-slow waves (DC shift) on SEEG in a 14-year old child

Occipital lobe epilepsies can present with complex or elementary visual hallucinations and oculomotor symptoms like contraversive tonic or clonic deviation of eyes and head. Brief occipital seizures can be difficult to diagnose. We report a child with occipital plus epilepsy who was investigated with stereo-EEG.

Tonic Upward Eyeball Deviation Mimicking Non-Convulsive Occipital Lobe Status Epilepticus That Was Induced by Hydrocephalus

Several seizure-like symptoms are difficult to differentiate from epileptic convulsion, and then if they were misdiagnosed, they could be led to grave prognosis. A 41-year-old man was referred to the emergency room due to unconsciousness. Brain computed tomography (CT) revealed acute subdural hemorrhage along the left frontal lobe, and intraparenchymal hemorrhage in the left temporo-occipital lobe. After emergent decompressive craniectomy, he recovered an alert mental state but became progressively drowsy. Four days later, virtually continuous tonic upward eyeball deviation was observed. He had been taking antiepileptic drugs following an occipital lobectomy 20 years prior due to intractable epilepsy, and we assumed these upward eyeball deviations were symptoms of non-convulsive occipital lobe status epilepticus. Hence, doses and classes of antiepileptic drugs were modified, but clinical manifestations did not improve. Follow-up brain CT revealed newly developed hydrocephalus and compression of the mesencephalon. His symptoms fully resolved after a ventriculo-peritoneal shunt operation. In this case report, we describe the case of a patient exhibiting tonic upward eyeball deviation induced by hydrocephalus that was not associated with a seizure.

Ocular neuromyotonia

Ocular neuromyotonia is a rare, albeit treatable, ocular motor disorder, characterised by recurrent brief episodes of diplopia due to tonic extraocular muscle contraction. Ephaptic transmission in a chronically damaged ocular...

P03.16 Intracranial extraventricular neurocytoma: case report and literature review

Abstract Extraventricular neurocytomas are rare brain tumors with a reported worldwide incidence of only 0.13%. They originate from neuroepithelial tissue and may present throughout the central nervous system. They affect mostly young adults, and have a favorable prognosis. Due to their rarity, they are not well characterized and most features are derived from case reports. Extraventricular neurocytomas tend to be well circumscribed, contrast-enhancing and heterogeneously solid, often partly or mainly cystic. They share histological features with the more common central neurocytomas but are often more complex, less cellular, and more likely to contain ganglion cells. The primary differential diagnoses are oligodendroglioma, ganglioglioma, gangliocytoma, and dysembryoplastic neuropithelial tumor (DNET). Complete surgical resection is currently the optimal treatment choice. It is not clear whether tumors with anaplasia have a higher relapse rate or if they require adjuvant therapy. We report on a 48 year-old woman with a history of depression who was admitted for focal seizures characterized by left gaze tonic deviation over the last few months. The neurological examination was otherwise normal. Routine EEG showed no epileptic activity. MRI identified an intra-axial heterogeneous round mass in the right frontal lobe with faint heterogeneous enhancement after contrast administration and perilesional oedema. A low-grade glial tumor was suspected. A left frontal craniotomy was performed. Intraoperative findings revealed a grayish and friable mass without superficial expression and with well-defined limits. A gross total resection was achieved. Histological analysis revealed a lesion with rounded cells displaying a “salt-and-pepper” chromatin pattern, Homer Wright rosettes and perivascular pseudorosettes. Immunostaining was positive for GFAP, sinaptophysin and NSE. The Ki-67 labeling index of the tumor was 25%. Diagnosis was extraventricular neurocytoma with anaplastic features. Magnetic resonance imaging was performed at 3 months post-surgery and demonstrated no evidence of tumor recurrence. CONCLUSION: Although neurocytomas are often located in the lateral ventricles, they occur throughout the neuroaxis. This is the main reason why they are not always considered in the differential diagnosis of intraparenchymal cerebral tumors. The diagnosis is aided by radiologic and histologic features. If a gross total resection is achieved, one can expect a good prognosis.

Occipital lobe epilepsy or migraine headache

Introduction: Occipital lobe epilepsies are rarely met in clinical practice, but when they occur, they can be misdiagnosed as migraine-like headache. Their prevalence ranges from 5%to 10% of all epilepsies. Seizures can occur at any age; etiologically speaking they can be symptomatic, cryptogenic and idiopathic (most often onsetis in childhood). Clinical symptomatology is manifested by partial epileptic seizures in the sense of visual elementary and/or complex manifestations, palinopsia, amaurosis, tonic head deviation, bulbus, nistagmus and headache. Propagation discharge to neighbour areas (temporal, parietal and frontal) is a frequent occurrence appearing with complex partial seizures frequently finishing with secondary generalized tonic-clonic (GTC) seizures. Case report: We are presenting a17-year-old male patient who has suffered from attacks of visual problemswith headache since 10 years of age. All the time it is treated as a migraine headache. During the last attack of headache the patient also had a loss of consciousness, EEG that was performed for the first time evidenced epileptic discharges of the occipital area. The therapy also included treatment with antiepileptic drug pregabalin resulting in seizure withdrawal. Conclusion: The appearance of visual symptoms followed by headache is most frequently qualified as migraine triggered headache. However, when antimigraine therapy does not give favorable results epileptic headache should be suspected, with obligatory performance of EEG recording. Occipital lobe epilepsy often presents diagnostic dilemmas due to clinical manifestations that are similar to that of non-migraine headache.

Conservative management of Rasmussen's encephalitis in a Nigerian girl

Rasmussen's encephalitis (RE) is a rare disease of the brain characterized by focal seizures, unilateral cortical deficits, hemiparesis, and hemiatrophy of the contralateral cerebral hemisphere. The affected brain tissue shows a chronic inflammatory histopathology and an autoimmune reaction is suspected. Therapeutic strategies include anticonvulsants, immunomodulation and surgery. We present the first report of RE in a Nigerian. A 4-year-old girl presented with a 15-month history of recurrent involuntary jerky movements of the right upper limb and tonic deviation of the neck to the right side often associated with loss of consciousness. Seizures lasted 2 to 3 min and occurred every 2 to 3 h. She later developed right-sided weakness. On examination, she had right-sided spastic hemiparesis with astereognosis. The electroencephalograph showed theta and delta waves on the left cerebral hemisphere with epileptiform sharp and slow waves and phase reversal pattern. Brain computerized tomogram showed left hemispheric hemiatrophy with non-enhancing hypodense lesions in the left hemisphere. This was confirmed by brain magnetic resonance imaging, which showed left cerebral atrophy with associated left white matter hyperintensities on fluid-attenuated inversion recovery sequences. There was no enhancement with gadolinium. She improved on anticonvulsants. She fulfilled the European Consensus criteria for the diagnosis of RE. Electroencephalography and magnetic resonance imaging are important for the diagnosis of RE, a condition worth considering in patients with focal seizures and unilateral neurological deficits.

Les pathologies neurovisuelles chez les enfants cérébrolésés

Cerebral visual impairment is one of the main causes of child visual impairment in developed countries. The pattern of the brain damage is dependent on the stage on brain development at insult, the severity and the duration of the insult. Brain pathology caused by an insult during the first and second trimester will cause cerebral malformations. Between 24 and 34weeks, brain damage is referred to as periventricular leucomalacia, subcortical damage affecting particularly visual pathways. After 34weeks, hypoxic-ischaemic injury leads to cortical lesions in chronic injury and subcortical nuclei lesions in acute hypoxia. Visual dysfunction is caracterized by fixation troubles, subnormal acuity, visual fields defects, difficulty with perceiving depth, cognitive defects (dysgnosia [pictures, objects], prosopagnosia, visuospatial disorders, clumsiness to identify an element within a crowded scene and to bring about visually guided movement, difficulty with movement perception), ocular motility disorders (tonic gaze deviation, strabismus, nystagmus).

Brain SPECT Imaging of Ictal Smile Seizure

"Ictal smile" is defined as a facial expression during a seizure, which usually translates happiness, clearly distinct from a tonic deviation of the mouth or other abnormal tonic-clonic movements involving the face, not associated to any happiness emotion (Epilepsia. 1998;39:1357-1360). It is a rare condition (6%-10% of patients; Epilepsia. 1998;39:1357-1360) and seems to be related to seizures arising from temporal or frontal regions (Brain. 2003;126:2121-2138).Anecdotal reports of brain perfusion SPECT performed during ictal and interictal phases (Epilepsia. 1998;39:1357-1360) do not supply, unfortunately, imaging documentation. Thus, we report the first evidence of brain perfusion abnormalities induced by ictal smile seizure.

Non-convulsive status epilepticus presenting with catatonia and suicidal behavior

Non-convulsive status epilepticus (NCSE) is an uncommon clinical entity. An electroencephalogram (EEG) helps in diagnosis of NCSE, in cases without overt motor manifestations of seizures such as automatisms. We describe an 18-year-old patient with NCSE, who had presented with catatonia and suicidal attempts. There were no automatisms or eye signs indicative of seizure. He had an episode of tonic neck deviation followed by incontinence, which first alerted us to the possibility of epilepsy. An abnormal EEG and a clinical response along with correction of EEG abnormalities with diazepam clarified the diagnosis. Management and hospital course are described in brief. The patient had residual symptoms, suggestive of a postictal state at discharge. The case shows the importance of keeping NCSE as a differential diagnosis, in cases presenting with catatonic symptoms, perplexity, and confusion. The presence of fleeting hallucinations and behavioral disturbances indicate limbic seizures in such cases, presenting in a catatonic state.

Paroxysmal tonic upgaze in normal children: A case series and a review of the literature

The purpose of this review was to update the clinical characteristics of paroxysmal tonic upgaze in neurodevelopmentally normal children. We made the diagnosis (between 2008 and 2010) in 8 infants referred to us with suspected epilepsy. We found 38 further cases in the literature.In the 46 children (29 boys and 17 girls) tonic upward ocular deviation was first noticed between the age of 2 weeks and 90 months (median: 9 months). This tendency persisted for between 1 and 48 months (median: 7 months). The duration of paroxysmal events was highly variable: brief events lasted between 3 s and 10 min in 50% of the cases, intermediate events between 5 s and 30 min and long events between 10 s and 2 h. The frequency ranged from one every 3 months to 10 per day. In 15 children the episodes of upward deviation of the eyes were associated with an impaired movement coordination. In 2 further children an impaired movement coordination was noted during febrile illnesses.The results of this review in normal children characterize the entity as follows: onset under 2 years of age, a small predilection for the male gender, eventual improvement and recovery, and impaired movement coordination.

Parkinson's Disease Presenting with Oculogyric Crisis in the Off Period

We herein report the case of a 67-year-old Japanese man diagnosed with sporadic Parkinson's disease (PD) at 52 years of age who presented with oculogyric crisis (OGC) in the off period. Ordinarily, OGC is caused by postencephalitic parkinsonism or the chronic use of antidopaminergic medications. The OGC began at 65 years of age and was associated with the wearing-off of symptoms. The dominant OGC feature was tonic deviations in eye posture induced by looking upward with prominent retrocollis. The administration of control dopaminergic medications led to improvements in the wearing-off phenomenon and OGC. This observation confirms that sporadic PD can induce OGC in the off period.

What Does Galvanic Vestibular Stimulation Actually Activate: Response

2010; Hammam et al., 2011, 2012; Grewal et al., 2012). The sense of roll is consist-ent with a host of other studies using GVS (Fitzpatrick et al., 1994; Inglis et al., 1995; Day et al., 1997; Zink et al., 1997; Day and Cole, 2002; Scinicariello et al., 2002/2003; Wardman et al., 2003a,b; see Fitzpatrick and Day, 2004 for review). Modeled on this research, we used 2–3 mA currents in lightly anesthetized rats and found strong activation of the sympathetic nervous sys-tem and vasovagal responses, but not tonic deviations of the eyes (Cohen et al., 2011). We have also stimulated the vestibular nerve with trains of pulses to activate MSNA in humans (Voustianiouk et al., 2006), some-times utilizing currents of 5 mA. MSNA was facilitated, but ocular deviations were never induced.The semicircular canals can exert a powerful influence on eye movements through the vestibulo-ocular reflex. Head turns induce eye movements with veloci-ties of up to 400/s (Atkin and Bender, °1968), and with response characteristics up to 20 Hz ( Grossman et al., 1988; Tabak and Collewijn, 1994; Armand and Minor, 2001). Furthermore, a change in body temperature of only 7C during caloric °stimulation with either air or water read-ily induces nystagmus with slow phase velocities of 30–40/s in monkeys (° Arai et al., 2002) and 10–20°/s in humans (M. Dai, personal communication). Thus, at best, the semicircular canal activation induced using strong GVS by Curthoys and MacDougall, which produced average slow phase eye velocities of 5° /s, was weak and functionally inconsequential.The question of function is particularly relevant in this regard. The semicircular canals stabilize gaze in space during head

Can fast-component of nystagmus on caloric vestibulo-ocular responses predict emergence from vegetative state in ICU?

The aim of the study was to determine if bedside caloric vestibulo-ocular responses (VOR) are able to predict consciousness recovery from clinically determined vegetative state (VS) in the ICU. Twenty-six severely brain injured patients that were clinically in VS were included. Horizontal VOR were tested at bedside by cold-water irrigation of the external auditory canal. Visual inspection evaluated the presence of a slow drift toward the side of stimulation (slow-component of nystagmus or tonic deviation) as well as the presence of a rapid compensatory movement/jerk back to the midline (fast-component of nystagmus). Patients were then divided into two groups according to whether they eventually regained consciousness or not. Patients were 59 ± 21 years old. Thirteen out of 26 patients ultimately recovered consciousness and 13 remained unconscious. Thirteen patients (100%) presented a slow-component of nystagmus during VOR testing in the group that recovered consciousness and 11 (85%) in the group that remained unconscious. All the patients that recovered consciousness (13, 100%) presented a fast-component of nystagmus during VOR testing compared to only one of 11 patients (8%) in the group that remained unconscious. Sensitivity of fast-component of nystagmus during VOR testing to predict recovering of consciousness was 1.00, specificity was 0.92, positive predictive value was 0.93 and negative predictive value was 1.00. Bedside VOR testing in clinically VS patients seems able to predict consciousness recovery from vegetative state and could help to preclude active medical treatment withdrawal and to indicate the need for further complementary explorations, i.e. event-related potentials, functional MRI or PET-scan.

Neuroimaging of eye position reveals spatial neglect

Conjugate eye deviation describes the tonic horizontal deviation of the eyes in acute stroke patients. Here we investigate whether measuring patients' eye-in-head position in clinical magnetic resonance imaging or computed tomography scans obtained at admission shows a specific relationship to spatial neglect. We investigated 124 continuously admitted subjects with unilateral, first-ever left- or right-sided stroke. To control for the possibility that the degree of eye deviation is related to lesion size rather than spatial neglect, overall lesion volume was used as a covariate in the statistical analysis. Horizontal eye-in-head deviation on clinical brain scans appeared to be associated with spatial neglect rather than with brain damage per se. In contrast to the subject groups without the disorder, the patients with spatial neglect showed an eye-in-head position that was significantly deviated towards the ipsilesional right. Evaluation of eye-in-head position on clinical scans thus may be an additional helpful tool for diagnosing spatial neglect, particularly in the very early period of the stroke.

Internuclear and supranuclear disorders of eye movements: clinical features and causes

Eye movements bring visual stimuli to the fovea and also maintain foveal fixation on a moving target and during head movements. These movements are performed by the ocular motor system that consists of ocular motor nerves and nuclei in the brainstem originating in the cerebral cortex, cerebellum, vestibular structures, and the extraocular muscles. The ocular motor system is divided according to anatomic location into infranuclear, nuclear, internuclear, and supranuclear components. It is important to distinguish supranuclear and internuclear from nuclear and infranuclear disturbances affecting cranial nerves III, IV, and VI, because the disturbances are of highly varied causes and present different clinical pictures. Internuclear ophthalmoplegia is due to a lesion of the medial longitudinal fasciculus, caused by multiple sclerosis in younger patients, particularly when the ophthalmoplegia is bilateral, and usually of vascular origin in the elderly. Eye movement abnormalities of supranuclear origin are characterized by gaze palsies, tonic gaze deviation, saccadic and smooth pursuit disorders, vergence abnormalities, nystagmus, and ocular oscillations. Supranuclear disorders result from lesions above the level of the ocular motor nerve nuclei. If oculocephalic maneuvers move the eyes appropriately, the lesion causing the gaze palsy is supranuclear. Supranuclear disorders account for almost 10% of all patients with disorders of eye movements.