Oculogyric Crisis Research Articles

Aripiprazole-induced oculogyric crisis (acute dystonia) in 11 years old girl: a case report

Acute dystonic reaction which is described as involuntary muscle spasms and contractions including neck, chin, eye and mouth muscles is an extrapyramidal adverse effect. Acute dystonic reactions are rarely seen with atypical antipsychotics. In this study, we present a case of an aripiprazole-induced oculogyric crisis in an 11 years old girl who diagnosed with mild intellectual disability attention deficit and hyperactivity disorder, and conduct disorder. This case is important as aripiprazole-induced oculogyric crisis caused is not an expected side effect in the child and adolescent population.

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.

COVID-19 Catatonia—Would We Even Know?

COVID-19 Catatonia—Would We Even Know?

Clinical Profile and Outcome of Indian Children with Aromatic L-Amino Acid Decarboxylase Deficiency: A primary CSF Neurotransmitter Disorder Mimicking as Dyskinetic Cerebral Palsy.

Aromatic L-amino acid decarboxylase (AADC) deficiency is a disorder of neurotransmitter synthesis. It presents with psychomotor delay, dystonia, oculogyric crisis, and autonomic features. There is paucity of literature on this disorder. Hence, we are reporting this series with an objective to study profile and outcome of Indian children with AADC deficiency. In this retrospective review, all case records of genetically confirmed cases of AADC deficiency at the pediatric neurology department in a tertiary care hospital, from March 2014 to March 2020, were analyzed. The data were extracted in a predesigned proforma and analyzed. Out of seven cases, five were males. Median age of onset of symptoms was 4 months but median age of diagnosis was 12 months. All of them had developmental delay, oculogyric crisis, dystonia, increased sweating, intermittent fever, feeding and sleep disturbance, irritability, failure to thrive, axial hypotonia with dyskinetic quadriparesis, and normal magnetic resonance imaging (MRI) of brain and electroencephalogram (EEG). All of them were treated with pyridoxal 5-phosphate, trihexyphenidyl and pramipexole and six cases, in addition, were given bromocriptine. One case was additionally treated with selegiline. One case showed good improvement, five showed partial improvement, and one case expired. In conclusion, AADC deficiency should be suspected in any child with dyskinetic quadriparesis, oculogyric crisis, autonomic disturbances like increased sweating, intermittent fever, and sleep disturbance with normal neuroimaging.

AADC deficiency from infancy to adulthood: Symptoms and developmental outcome in an international cohort of 63 patients.

Aromatic l‐amino acid decarboxylase deficiency (AADCD) is a rare, autosomal recessive neurodevelopmental disorder characterized by impaired synthesis of dopamine, noradrenaline, adrenaline and serotonin, leading to a complex syndrome of motor, behavioral, and autonomic symptoms. This retrospective study assessed the symptoms and developmental outcome of a large international cohort of patients with AADCD via physician and/or caregiver responses to a detailed, standardized questionnaire. Sixty‐three patients (60% female; ages 6 months‐36 years, median 7 years; 58 living) from 23 individual countries participated. Common symptoms at onset (median age 3 months, range 0‐12 months) were hypotonia, developmental delay, and/or oculogyric crises. Oculogyric crises were present in 97% of patients aged 2 to 12 years, occurred in the majority of patients in all age groups, and tended to be most severe during early childhood. Prominent non‐motor symptoms were sleep disturbance, irritable mood, and feeding difficulties. The majority of subjects (70%) had profound motor impairment characterized by absent head control and minimal voluntary movement, while 17% had mild motor impairment and were able to walk independently. Dopamine agonists were the medications most likely to produce some symptomatic benefit, but were associated with dose‐limiting side effects (dyskinesia, insomnia, irritability, vomiting) that led to discontinuation 25% of the time. The age distribution of our cohort (70% of subjects under age 13 years) and the observation of a greater proportion of patients with a more severe disease phenotype in the younger compared to the older patients, both suggest a significant mortality risk during childhood for patients with severe disease.

The genetic and clinical characteristics of aromatic L-amino acid decarboxylase deficiency in mainland China

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare neurotransmitter metabolic disorder caused by DDC gene mutations, which leads to the metabolic disturbance of dopamine and serotonin. Most of the reported cases came from Taiwan China, but patients from mainland China were seldomly reported. The current study was the largest AADCD patient cohort from mainland China. Twenty-three patients with clinical features of AADCD and DDC gene variants were recruited. A total of 16 DDC variants were identified in this study, of which four variants (c.2T>C, c.277A>G, c.1021+1G>A, c.565G>T) were never reported previously. The intronic variant c.714+4A>T was the most common one, with an allele frequency of 45.7%. And patients carried this intronic variant presented with severe clinical manifestations, all of whom were bedridden. In this study, the average onset age was 3.61 ± 1.28 months and the average age of diagnosis was 12.91 ± 5.62 months. Early onset hypotonia, oculogyric crises, and autonomic symptoms such as excessive sweating, nasal congestion and profuse nasal, and oropharyngeal secretions, were common in our patients. Eighteen patients (78.3%) got various degree of improvement after using pyridoxine monotherapy or different combination of pyridoxine, dopamine agonists, and monoamine oxidase (MAO) inhibitors.

Paliperidone-Associated Acute Oculogyric Crisis.

Paliperidone-Associated Acute Oculogyric Crisis.

Tardive Oculogyric Crisis due to Aripiprazole treatment in an adolescent patient diagnosed with Bipolar Disorder

Antipsychotics usage in children and adolescents is becoming widespread. One of the extrapyramidal side effects associated with long-term antipsychotic drug use follow-up is an oculogyric crisis (OGC). OGC is defined as a dystonic reaction commonly observed after typical antipsychotic drug administration. However, there are rare cases which report OGC after atypical antipsychotic use. In this report, we present an adolescent with bipolar disorder who developed OGC after 2 years of aripiprazole and quetiapine use. To best of our knowledge, this case report has been the first case to present the clinical presentation of tardive OGC due to aripiprazole treatment in an adolescent patient. It is important to note that OGC might be observed in the acute phase and it might be as a result of chronic use detected during a clinical follow-up. Safety, tolerability and efficiency of aripiprazole in pediatric populations should be further analyzed in the future studies.

Methylphenidate-Induced Oculogyric Crisis in a Patient with Autism Spectrum Disorder and Attention Deficit Hyperactivity Disorder: A Case Report

Methylphenidate is one of the agents used in the treatment of attention deficit hyperactivity disorder. In the literature, the following side effects were reported due to the use of methylphenidate: sleep and appetite problems, blood pressure and heart rate changes, headache, nervousness, crying, tics, chorea, psychosis, and rarely dystonic reaction. Acute dystonia is a well-known extrapyramidal side effect of dopamine receptor antagonists. The oculogyric crisis, a specific form of acute dystonic reaction, refers to usually upward and sometimes lateral deviation of the eyes for a certain period due to contraction of the extraocular eye muscles. Despite the increase in dopamine-related activity, psychostimulants were also reported to cause dystonic reactions rather than blocking. In this study, a patient with oculogyric crisis after the initiation of methylphenidate treatment was analyzed. Although rarely observed, dystonic reactions that may occur after methylphenidate should be considered by clinicians.

Drug-induced oculogyric crisis – A rare presentation to the emergency department

Oculogyric crisis (OGC) is an acute dystonic reaction of the ocular muscles characterized by the bilateral dystonic elevation of visual gaze lasting from seconds to hours. OGCs are nonepileptic eye movements and therefore should be distinguished from more commonly occurring tonic eye deviation, seen in seizure disorders, paroxysmal tonic upward gaze, and encephalopathy, commonly encountered in the emergency department. This rare neurological condition may be easily misinterpreted or overseen as functional or as an exacerbation of psychotic illness.

Olanzapine-induced choreoathetoid movements in a young patient of schizophrenia

Atypical antipsychotics like olanzapine are used in the management of schizophrenia due to their lower propensity to cause extrapyramidal symptoms. Yet, few case reports have suggested that olanzapine may cause tardive dystonia, dyskinesia, and oculogyric crisis. However, olanzapine has been never reported to induce nonrhythmic choreoathetoid movements in an adolescent patient within short periods of consumption. Here, we report a novel case of choreoathetoid movements with history of olanzapine consumption for 2 years. The patient presented with involuntary bilateral movements affecting her entire body for the past 4 months, and a diagnosis of olanzapine-induced choreoathetoid movements was made after excluding other neurological and inherited causes by performing necessary investigations. After olanzapine was tapered and stopped, the patient improved and has been subsequently maintained on clozapine. Thus, constant monitoring for movement disorders is needed, particularly in patients with risk factors, even with atypical antipsychotics like olanzapine, and this must play a vital role in management.

Autoantibodies and eye movement disorders

AbstractIntroductionNew auto‐antibodies seem to be discovered every month and the field of eye movement disorders is fully part of the game: anti‐GAD, GM1, GQ1b, Hu, Ri, etc. What are their specificity? their practical use at the bedside? where and how to ask for their dosage? what to they tell us of their associated conditions?ObjectiveTo answer these questions in a synthetic way.MethodsLiterature review using Pubmed and Google Scholar; chosen clinical cases.ResultsVarious oculomotor presentations should prompt auto‐antibodies dosage: oculogyric crises associated with tics or chorea; progressive saccadic intrusions with associated ataxia, typically evolving into opsoclonus‐myoclonus syndrome; acquired neurological nystagmus with no obvious explanation, and non‐compressive unexplained ophthalmoparesis, especially when associated with unremarkable MRI ‐ or with MRI showing mild signs of encephalitis. The targets of these antibodies can be: the central nervous system, the peripheral nervous system, the neuromuscular junction or the extraocular muscles. Relevant auto‐antibodies can be classified into: antibodies directed against intracellular neuronal proteins, responsible for paraneoplastic neurological syndromes (Hu, Yo, Ri, CV2, amphiphysin, Ma1, Ma2) and antibodies directed against neural surface or synaptic antigens, responsible for autoimmune encephalitis syndromes, most frequent in children (AMPA, CASPR2, GABAR, GAD, GD1a, GD1b, GM1, GQ1b, GT1a, LGI1, NMDA).ConclusionsAuto‐antibodies are often the clue of ‘impossible’ oculomotor cases. They should be dosed in chosen and rare situations with specific techniques; the results should be interpreted according to the known specificity of each auto‐antibody.

Clinical and genetic analysis of two pedigrees affected with aromatic L-amino acid decarboxylase deficiency

To delineate the clinical and genetic features of two pedigrees affected with aromatic L-amino acid decarboxylase (AADC) deficiency. The clinical features, family history and results of genetic testing of 2 patients with AADC deficiency were retrospectively analyzed. Both patients featured hypotension, developmental delay and oculogyric crisis during infancy. Genetic testing confirmed that they have respectively carried c.714+ 4 (IVS6) A to T/c.175(exon2)G TO A compound heterozygous variants and c.714+ 4(IVS6)A to T homozygous variant. The clinical manifestation of children with AADC deficiency may include hypotonia, developmental delay and paroxysmal oculogyric crisis. The combination of 3-O-methyldopa testing and variant analysis is not only very useful for early diagnosis, but also important for the evaluation of treatment effect and prognosis of the disease. Discovery of the novel variants has enriched the variant spectrum of AADC deficiency.

METOCLOPRAMIDE-INDUCED ACUTE OCULOGYRIC CRISIS TREATED WITH BIPERIDEN: A CASE REPORT

Metoclopramide is an antiemetic prescribed in general medical practice. The drug can cause acute dystonic reactions. Here, we described the case of a 23-year-old young woman with a chief complaint of blurred vision and later dystonic, oculogyric-type crisis. She underwent laboratory tests and neuroimaging then was hospitalized in the Neurological Department. Treatment with intravenous biperiden interrupted the oculogyric crisis. Only a thorough drug history and correct clinical examination can discover the use of metoclopramide-induced adverse drug reaction such as oculogyric crisis.

Dystonic tics in patients with Gilles de la Tourette syndrome.

Gilles de la Tourette syndrome (GTS) is a childhood onset disorder characterised by motor and vocal tics. Different types of motor tics may occur in GTS, including dystonic tics (DTs). Although DTs have been recognised as part of GTS symptomatology, little is known about their risk factors or about how often and at what age they appear in affected individuals. The aim of our study was to investigate lifetime prevalence and clinical correlations of DTs in a Polish cohort of GTS patients. We performed a prospective, one-registration study in a cohort of 207 consecutive ambulatory patients (mean age: 16.5 ± 9.4 years, 131 children, 162 males) with GTS. Duration of GTS was 9.0 ± 8.0 years (range: 1-39 years). DTs were diagnosed during the interview. DTs were defined as slower and lasting longer than typical clonic tics, abnormal dystonia-like movements that led to a sustained, but not fixed, posture. DTs occurred at some point in the lifetime of 73.9% (n = 153) of patients. The prevalence of DTs in adults and children was almost the same (p = 0.963). Age at onset of DTs was 9.9 ± 5.2 years with the most frequent onset in children (7-11 years, 74.4%, n = 64), followed by adolescence (12-18 years; 17.4%, n = 15) and adulthood (≥ 18 years, 8.1%, n = 7). DTs occurred 3.7 ± 4.2 years after tic onset. On average, patients suffered from 1.8 ± 1.7 types of DTs. The most frequent manifestations of DTs were: eyes (tightening resembling blepharospasm 84.3%, n = 129 and oculogyric crisis 45.8%, n = 70), trunk (dystonic postures 59.5%, n = 91), jaw (bruxism 34.6%, n = 53), neck (30.7%, n = 47), upper limb (26.1%, n = 40), and foot (20.9%, n = 32). Multivariate logistic regression analysis showed significant associations of DTs with the total number of simple, and the total number of complex, tics. DTs are early and frequent symptoms of GTS. They tend to localise in the facial area. DTs occur more frequently in individuals with a higher number of tics and probably add to the global impairment caused by tics.

Eye movement disorders and neurological symptoms in late-onset inborn errors of metabolism.

ABSTRACTInborn errors of metabolism in adults are still largely unexplored. Despite the fact that adult‐onset phenotypes have been known for many years, little attention is given to these disorders in neurological practice. The adult‐onset presentation differs from childhood‐onset phenotypes, often leading to considerable diagnostic delay. The identification of these patients at the earliest stage of disease is important, given that early treatment may prevent or lessen further brain damage. Neurological and psychiatric symptoms occur more frequently in adult forms. Abnormalities of eye movements are also common and can be the presenting sign. Eye movement disorders can be classified as central or peripheral. Central forms are frequently observed in lysosomal storage disorders, whereas peripheral forms are a key feature of mitochondrial disease. Furthermore, oculogyric crisis is an important feature in disorders affecting dopamine syntheses or transport. Ocular motor disorders are often not reported by the patient, and abnormalities can be easily overlooked in a general examination. In adults with unexplained psychiatric and neurological symptoms, a special focus on examination of eye movements can serve as a relatively simple clinical tool to detect a metabolic disorder. Eye movements can be easily quantified and analyzed with video‐oculography, making them a valuable biomarker for following the natural course of disease or the response to therapies. Here, we review, for the first time, eye movement disorders that can occur in inborn errors of metabolism, with a focus on late‐onset forms. We provide a step‐by‐step overview that will help clinicians to examine and interpret eye movement disorders. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.

Oculogyric Crisis Due to Metronidazole Toxic Encephalopathy

Oculogyric crisis is an acute dystonia involving ocular muscles characterized by sustained conjugate upward or lateral deviation of the eyes. Metronidazole is a commonly used antimicrobial agent in treatment of anaerobic infections. However, its long-term use can cause toxic encephalopathy particularly in patients with hepatic dysfunction. Here, we describe a case of oculogyric crisis as a presenting manifestation of metronidazole-induced encephalopathy. Key Words: Metronidazole, Encephalopathy, Dystonia

Movement disorders in emergency settings: a prospective study.

Acute movement disorders (MD) are etiologically heterogeneous entities. Since studies on the relative frequency of different MD and their underlying diseases are limited, we performed a prospective study to investigate the spectrum of various MD and their causes in patients presenting with acute MD in an emergency room (ER) setting. To describe the spectrum and outcomes of acute MD in a prospective cohort and to guide its management. We investigated acute MD in 96 consecutive patients admitted to ERs between 2013 and 2017. Time of disease onset, type of MD according to published criteria, diagnostic workup, and outcome were collected. 73.9% of patients had hyperkinetic MD. Tremor was the most common symptom (19.8%), followed by myoclonus (17.7%), dystonia (15.6%), and chorea (11.4%). Other hyperkinetic MD (9.4%) included were gait disorders (imbalance due to involuntary movement), dyskinesia, akathisia, hemiballism, and oculogyric crisis. Hypokinetic MD included acute parkinsonism (15.6%), off-state (4%), akinesia (3%), and rigidity (3%). Co-occurrence of more than one MD was seen in 19.7% of patients. Time delay to medical consultation was between < 24h and 28days. Five etiological groups were recognized: drug-induced (29.2%), functional (19.8%), neurodegenerative diseases (15.6%), structural brain damage (11.5%), others (24.0%, metabolic, inflammatory, infective, undetermined). Outcome was better for neurodegenerative diseases and for drug-induced MD. Functional movement disorders (FMD) showed less favorable outcome. Acute MD is a distinct cause of ER admission, and a variety of treatable diseases may be the underlying cause of this symptom. Uncertain course is more probable in FMD and in structural brain lesions.

Successful Pregnancy in a Patient with L-Amino Acid Decarboxylase Deficiency: Therapeutic Management and Clinical Outcome.

Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare inherited disorder of biogenic amine metabolism presenting with developmental delay, hypokinetic movement disorders, hypotonia, and autonomic dysfunctions.1 We report on the successful pregnancy in a previously reported 26-year-old AADC patient with a mild phenotype (Table 1).2 Since the diagnosis at the age of 22, treatment with rotigotine, pyridoxine, and escitalopram resulted in a remarkable improvement of fluctuating muscular weakness, oculogyric crises, palpebral ptosis, balance, and gait.2 After, as consequence of behavioral disinhibition and reduced control of sexual impulses, rotigotine was replaced by a prolonged release formulation of pramipexole (up to 0.78 mg/day once a day). Selegine was added to improve diurnal fluctuation of motor symptoms (up to 7.5 mg/day after a very slow dose increasing). While MAO-inhibitors and SSRIs association is contraindicated in common neuropsychiatric disorders due to the risk of serotonin intoxication syndrome, their synergic effect may be exploited to magnify the residual enzymatic activity in the defect of serotonin synthesis, such as AADC deficiency. Compound heterozygous for the mutations p.Tyr37Thrfs*5 (c.105delC) and p.Phe237Ser (c.710 T>C) The pregnancy of our patient occurred naturally at the age of 26. Escitalopram was suspended to prevent the risk of low birth weight.3 Doses of pramipexole and selegiline could be decreased (Table 1) without obvious neurological worsening. No oculogyric crises or myoclonic jerks were observed, and limb rigidity, fatigability, and muscular weakness were well controlled. UPDRS-part III score did not change with respect the prepregnancy status (Table 1). Blood pressure remained below the normal range for the age. At the 31st week of gestation preeclampsia occurred. At the 38th week of gestation, a cesarean delivery (cardiac deceleration at cardiotocography) resulted in the birth of a vital (Apgar score 91 and 105) low birth weight (2.440 grams) and small (for gestational age) male newborn. The infant presented with transient tachypnea and hypoglycemia in the first day after birth. His growth was regular after the early introduction of artificial nursing. No significant health problems were observed in the following months. Pregnancy was previously reported in inherited neurotransmitter disorders with a less severe phenotype than AADC deficiency (35 women with autosomal dominant guanosine triphosphate cyclohydrolase 1 deficiency, one with dihydropterine reductase deficiency, and one with 6-pyrovoyl-tetrahydrobiopterin synthase deficiency).4, 5 The defect of monoaminergic neurotransmitters in AADC deficiency potentially impairs embryo-maternal interactions at preimplantation stages of early embryogenesis, which could be prevented by the treatment with drugs acting on dopaminergic axis.6 Efficacy and safety profile of pramipexole and selegiline during pregnancy and lactation are reported, in 24 and three women affected by Parkinson disease or restless legs syndrome, respectively.5, 7 Pramipexole was associated with spontaneous abortion in 3 cases (co-exposition with isotretinoin in one case) while selegiline was associated with a ventral septal defect in a twin newborn (co-exposition with L-DOPA/carbidopa and entacapone).5 The present case shows that in AADC deficiency: (a) fertility may be not impaired, (b) the course of pregnancy can be successfully managed in patients with mild phenotypes, and (c) low dosages of pramipexole and selegiline may have a good efficacy and safety profile during pregnancy. 1. Research project: A. Conception, B. Organization, C. Execution; 2. Statistical Analysis: A. Design, B. Execution, C. Review and Critique; 3. Manuscript Preparation: A. Writing of the first draft, B. Review and Critique. M.M.: 1A, 1B, 1C, 3A, 3B F.M.: 1B, 1C, 3A L.P.: 1A, 1B, 1C, 3A S.F.: 1A, 1B, 1C, 3A C.C.: 1C, 3B Cl.C.: 1C, 3B G.M.: 1A, 1B, 1C, 3B V.L.: 1A, 1B, 1C, 3A, 3B Ethical Compliance Statement: We confirm that we have read the journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. The authors confirm that the approval of an institutional review board was not required for this work and that they have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Informed written consent was obtained from the patient for the publication of her clinical data. Funding Sources and Conflicts of Interest: None of the authors have anything to declare. Financial Disclosures for previous 12 months: None of the authors have anything to declare.

Antipsychotic-Induced Oculogyric Crisis and Paroxysmal Perceptual Alteration

Antipsychotic-Induced Oculogyric Crisis and Paroxysmal Perceptual Alteration