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Information about movementdisorders

Published on January 12, 2009

Author: aSGuest10224


Movement disorders in children : Movement disorders in children Dr.E.M.Honey Department of Human Genetics and Paediatrics Involuntary movements : Involuntary movements Abnormalities in structural and biochemical function of the nuclear masses of the basal ganglia Not under voluntary control Patient can’t stop them Without apparent purpose Aggravated by physical, emotional and mental stress Classification : Classification 1. Hyperkinetic -- chorea myoclonus tics tremors 2. Hypokinetic -- akinetic rigid syndrome juvenile parkinsonism progressive pallidal degeneration 3. Dystonia -- post encephalitic parkinsonism extensor dystonia athetosis Hyperkinetic disorders more common in children Biochemistry - neurotransmitters : Biochemistry - neurotransmitters Dopamine Acetylcholine Serotonin Receptor Histamine GABA Glutamate Substance P Hypokinetic - dopamine decreased - treat with dopamine replacement or anticholinergic drugs Hyperkinetic - dopamine increased and acetylcholine decreased - treat with a dopamine antagonist or cholinergic drug Chorea : Chorea Brief, rapid irregular jerks Random in place and time From one part of the body to the other Involvement of the caudate nucleus or other subthalamic nuclei Accompanied with hypotonia Typical: Hyperpronation of the outstretched hands and wax and waning of the intensity of the hand grip Most common cause: Sydenham chorea - one of five signs of rheumatic fever chorea, emotional liability and hypotonia Other causes of chorea : Other causes of chorea Cerebral palsy Symptomatic chorea: Drug-induced: Huntington’s chorea *Thyrotoxicosis *Phenytoin Benign hereditary *SLE *Phenothiazine Choreo-acanthocytosis *Henoch-Schonlein *Alcohol Ataxia telangiectasia purpura *Lithium Encephalitis lethargica *Polycythaemia vera *Phenobarbitone * Hypernatraemia *Hypoparathyroidism *Subdural hematoma *Wilson’s disease *Post-asphyxial states Other dyskinesias : Other dyskinesias Hemiballism - form of chorea movements more coarse and ballistic accompaniment of Sydenham’s chorea, stroke, cerebral tumours and trauma Myoclonus - rapid shock-like muscle jerks Tics - rapid, repetitive and stereotyped movements 25 to 30% of children will experience transient developmental tics Gilles de la Tourette syndrome - multiple motor tics and one or more vocal tics - more than one year - onset before 21 years - location, number, frequency, complexity and severity of tics change over time Tremor Dystonias : Dystonias Sustained contraction of various muscle groups that’s repetitive in nature and distort parts of the body into dystonic positions Slow and laboured Either flexion or extension A number of diseases present with dystonia: Torsion dystonia Dystonia musculorum deformans Dystonia with marked duirnal variation Causes of dystonias : Causes of dystonias Hereditary Idiopathic Symptomatic - trauma infection vascular metabolic degeneration Ataxia : Ataxia Incoordination of postural control and gait as well as an incoordination of the skilled movements involved in fine hand movements and speech Extrapiramidal system and cerebellum involved “Physiological cerebellar signs” Clinical features: Truncal ataxia Volitional ataxia Hypotonia Nystagmus Ataxia - 3 broad categories : Ataxia - 3 broad categories Acute ataxia Infections: Cerebellar abscess, viral cerebellitis, bacterial Metabolic: Hartnup disease, maple syrup, organic acidurias, Leigh’s ecephalopaties, hypoglycaemia, hyperammonaemia Toxic: Alcohol, Phenytoin, phenobarbitone,lead,glue, Vit A Posterior fossa tumours Trauma Vascular Pseudo-ataxia Slide 12: Chronic non-progressive ataxias Perinatal insults Birth asphyxia Metabolic Intraventricular haemorrhage, meningitis Congenital malformations Primary cerebellar hypoplasia, hydrocephalus Foetal alcohol syndrome Joubert syndrome Cerebellar/ kidney associations Postnatally acquired - hypoxia, hypoglycaemia, chronic phenytoin, thiamine deficiency, trauma Slide 13: Chronic progressive Friedreich’s ataxia Onset before 20 years Autosomal recessive inheritance Progressive ataxia - gait difficulties, speech problems No nystagmus Weakness, a positive Babinski but absent ankle and knee reflexes - involvement of the corticospinal tract Loss of position and vibration sense and positive Romberg test - involvement of the posterior columns Bladder dysfunction Involvement of cranial nerves Scoliosis Cardiomyopathies Diabetes mellitis Slide 14: Ataxia telangiectasia( Louis-Bar disease) Progressive ataxia(1-4 years) Abnormal eye movements - oculomotor apraxia Telangiectasia(3years-adolescence) Cutaneous manifestations Higher risk for malignancies Abnormality in cellular and humoral immunity Elevated alpha feto protein References: : References: Coovadia and Wittenberg: Paediatrics and Child Health pp 490-495

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