Acute Movement Disorders in Children
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Abstract:
We describe acute movement disorders in 92 children, aged 5 days to 15 years, from an Indian tertiary hospital. Eighty-nine children had hyperkinetic movement disorders, with myoclonus in 25, dystonia in 21, choreoathetosis in 19, tremors in 15, and tics in 2. Tetany and tetanus were seen in 5 and 2 children, respectively. Hypokinetic movement disorders included acute parkinsonism in 3 children. Noninflammatory and inflammatory etiology were present in 60 and 32 children, respectively. Benign neonatal sleep myoclonus in 16 and opsoclonus myoclonus syndrome in 7 accounted for the majority of myoclonus cases. Vitamin B 12 deficiency in 13 infants was the most common cause of tremors. Rheumatic fever and encephalitis were the most common causes of acute choreoathetosis. Acute dystonia had metabolic etiology in 6 and encephalitis and drugs in 3 each. Psychogenic movement disorders were seen in 4 cases only, although these patients may be underreported.Keywords:
Choreoathetosis
Movement Disorders
Etiology
Psychogenic disease
Choreiform movement
Athetosis
HIBCH and ECHS1 genes encode two enzymes implicated in the critical steps of valine catabolism, 3-hydroxyisobutyryl-coenzyme A (CoA) hydrolase (HIBCH) and short-chainenoyl-CoA hydratase (ECHS1), respectively. HIBCH deficiency (HIBCHD) and ECHS1 deficiency (ECHS1D) generate rare metabolic dysfunctions, often revealed by neurological symptoms. The aim of this study was to describe movement disorders spectrum in patients with pathogenic variants in ECHS1 and HIBC.We reviewed a series of 18 patients (HIBCHD: 5; ECHS1D: 13) as well as 105 patients from the literature. We analysed the detailed phenotype of HIBCHD (38 patients) and ECHS1D (85 patients), focusing on MDs.The two diseases have a very similar neurological phenotype, with an early onset before 10 years of age for three clinical presentations: neonatal onset, Leigh-like syndrome (progressive onset or acute neurological decompensation), and isolated paroxysmal dyskinesia. Permanent or paroxysmal MDs were recorded in 61% of HIBCHD patients and 72% of ECHS1D patients. Patients had a variable combination of either isolated or combined MD, and dystonia was the main MD. These continuous MDs included dystonia, chorea, parkinsonism, athetosis, myoclonus, tremors, and abnormal eye movements. Patients with paroxysmal dyskinesia (HIBCHD: 4; ECHS1D: 9) usually had pure paroxysmal dystonia with normal clinical examination and no major impairment in psychomotor development. No correlation could be identified between clinical pattern (especially MD) and genetic pathogenic variants.Movement disorders, including abnormal ocular movements, are a hallmark of HIBCHD and ECHS1D. MDs are not uniform; dystonia is the most frequent, and various types of MD are combined in single patient.
Choreoathetosis
Athetosis
Paroxysmal dyskinesia
Movement Disorders
Choreiform movement
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Choreoathetosis
Athetosis
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One of the varieties of hyperkinesia, characterized by rapid, involuntary, disordered movements in the limbs and trunk, is chorea. With this disease, a nonrhythmic contraction of the different in location and function of muscle groups occurs. The term chorea is used to refer to both independent diseases in which choreic hyperkinesis is the leading manifestation, and a number of syndromes that arise in various diseases.Chorea as a syndrome can be in the composition of such hereditary extrapyramidal diseases as hepatocerebral dystrophy (Wilson Konovalov's disease), Lesch Nichin syndrome, and paroxysmal choreoathetosis. Therefore, in order to establish such diagnoses, attention should be paid to the presence of other clinico paraclinical manifestations inherent in these pathologies, that is, the patient needs differential diagnosis
Hyperkinesia
Hyperkinesis
Tetrabenazine
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Chorea is rapid, purposeless and random movement that flows from one body part to another. When chorea is proximal, it may lead to large flinging movements called ballism, and when it is distal, it may manifest as writhing movements called athetosis. Choreic movements result from structural abnormalities affecting the basal ganglia or its connections and may be due to metabolic abnormalities, effects of medications, or as a part of several recognized inherited disorders. Huntington disease and neuroacanthocytosis syndromes are the prototypical inherited degenerative disorders that cause chorea. Sydenham chorea is the most common acquired immune-mediated childhood chorea and is discussed in detail in this chapter. Management principles, including diagnostic considerations and pharmacotherapies commonly used in the management of chorea, ballism and athetosis, are discussed.
Athetosis
Movement Disorders
Choreiform movement
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To describe the case of a woman with poststroke choreoathetosis whose symptoms improved after treatment with tetrabenazine.A 48-year-old left-handed woman developed progressive involuntary dystonic choreoathetoid movements of her arm following a cerebrovascular event. Involuntary abnormal movements (IAMs) were treated with haloperidol, but they worsened 6 months later, also involving her mouth, tongue, and perioral area. Because of the suspected occurrence of drug-induced tardive dyskinesia, treatment was switched to tetrabenazine (titration up to 100 mg/daily), with rapid remission of the involuntary abnormal movements (Abnormal Involuntary Movement Scale score switching from 20 to 1). One month later, following discontinuation of tetrabenazine, the dystonic choreoathetoid movements rapidly reappeared. Subsequent rechallenge with tetrabenazine caused remission of her symptoms.Poststroke IAMs are rare, and lesions involving the basal ganglia and/or thalamus seem to be particularly implicated in such disorders. The exact pathogenic mechanism has not been clarified; however, it has been postulated that a central dopaminergic overactivity might play an important role in the IAM occurrence. Thus, antipsychotics are the main treatment option, but they are often associated with therapeutic failure or adverse effects, including extrapyramidal symptoms and metabolic complications. Interestingly, when our patient was treated with tetrabenazine for haloperidol-induced tardive dyskinesia, she demonstrated substantial improvement in the dystonic choreoathetoid movements of her left upper limb.The improved response of this case to tetrabenazine monotherapy suggests that tetrabenazine may be a pharmacologic alternative for patients with poststroke choreoathetosis that is intolerant or unresponsive to antipsychotic agents. Further studies are needed to better define the risk versus benefit profile of tetrabenazine.
Tetrabenazine
Choreoathetosis
Tardive dyskinesia
Choreiform movement
Athetosis
Movement Disorders
Stupor
Extrapyramidal symptoms
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Hyperkinesia
Choreiform movement
Athetosis
Irritability
Hyperkinesis
Girl
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Abstract Hyperkinetic movement disorders are characterized by an excess of movement. This chapter outlines the classification, causes, and treatment of chorea, tic disorders, and dystonia. The term chorea refers to dancelike irregular, arrhythmic, rapid, involuntary movements that appear to flow between muscle groups. Athetosis, a slower and twisting dystonialike movement, may be superimposed with chorea, leading to choreoathetosis. Ballism is a form of high-amplitude and proximal chorea that has the appearance of flailing movement.
Athetosis
Movement Disorders
Choreoathetosis
Hyperkinesis
Tetrabenazine
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Choreiform movement
Tetrabenazine
Hyperkinesia
Movement Disorders
Athetosis
Hyperkinesis
Animal model
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