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Movement Disorders in Children

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1 Movement Disorders in Children

2 Overview Childhood movement disorders occur secondary to a wide range of genetic and acquired disorders affecting brain development.   Classification by type of abnormal movement Bradykinetic disorders Hyperkinetic disorders Classification by Etiology Primary Secondary Fixed Structural lesion Degenerative Metabolic Drug Induced Infectious Important point Any disorder that affects the basal ganglia can cause a wide array of different movement disorders Static brain injury may nonetheless cause a changing movement disorder, as development and brain plasticity alter the brain’s response to injury.

3 Basal Ganglia Group of deep nuclei
Caudate nucleus Putamen Globus pallidus Substantia nigra dopamine-rich pars compacta pars reticularis Inputs: Corpus striatum (caudate nucleus and putamen) receives input from the cerebral cortex and the thalamus Outputs: projects by way of the thalamus to the cerebral cortex and then to the pyramidal system




7 Bradykinetic vs. Hyperkinetic
Bradykinetic disorders Very rare in children Parkinson disease is the most common bradykinetic disorder Hyperkinetic disorders Tic Disorders Dystonia Sterotypies Chorea Athetoses Ballismus Tremor Myoclonus Dyskinesia

8 Tic Disorders Tics are repeated, intermittent movements that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement Tourette syndrome : Multiple motor and vocal tics Etiology Primary: the vast majority Secondary: Huntingtons, encephalitis, medication induced, carbon monoxide poisoning, neuroacantocytosis

9 Tics Diagnosis: Associated with ADHD and OCD PANDAS Treatment
Typical movements Don’t occur in sleep Patient usually unaware of it occurring Patient can usually suppress for a short time But when they do, it is accompanied by a discomfort and a strong urge to do the tic (a compulsion) Wax and wane over time Worsen with stress Associated with ADHD and OCD Make sure to ask both of patient and family history PANDAS Treatment Reassurance Tics tend to wax and wane; most children outgrow them Medications (when necessary) Stimulants bring out tics; if the have ADHD, they can’t use stimulants Tenex Risperidone

10 Dystonia Involuntary sustained or intermittent muscle contractions that cause twisting and repetitive movements, abnormal postures, or both.


12 Dystonia

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15 Classification of Dystonia
By location Generalized dystonia affects most or all of the body. Focal dystonia is localized to a specific part of the body. Blepharospasm, Cervical Dystonia, Task Specific Dystonia (eg Writers cramp) Multifocal dystonia involves two or more unrelated body parts. Segmental dystonia affects two or more adjacent parts of the body. Hemidystonia involves the arm and leg on the same side of the body. By etiology Primary: by definition, no other neurologic impairment Secondary: Cerebral Palsy the most common cause in children Psychogenic

16 Primary Dystonias Genetic Dystonias DYT1 dystonia
dominantly inherited generalized dystonia typically begins in childhood, affects the limbs first, and progresses A great deal of phenotypic variability Dopa-responsive dystonia (Segawa’s disease) onset during childhood and have progressive difficulty with walking.   Symptoms characteristically fluctuate and are worse late in the day and after exercise.  Some forms are due to mutations in the DYT5 gene for GTP cyclohydrolase 1.    Patients with this disorder have dramatic improvements in symptoms after treatment with levodopa Many other genes that cause dystonic syndromes have been found


18 Secondary Dystonias Fixed Injury/Structural:
Kernicterus,, head trauma, encephalitis; tumors, stroke, congenital malformations Degenerative: Fahr's disease , pantothenate-kinase associate dneurodegenerative disease (Hallervorden-Spatz disease), Huntington's disease, spinocerebellar ataxias ; neuronal ceroid lipofuscinosis; Rett syndrome; Tay-Sachs disease; Sandhoff's disease; Niemann-Pick type C; metachromatic leukodystrophy; Leigh's disease; neuroacanthocytosis;;Pelizaeus-Merzbacher disease; ataxia-telangiectasia Chemical/Metabolic: Glutaric aciduria; mitochondrial disorders; Wilson's disease; homocystinuria; Lesch-Nyhan disease; methylmalonic aciduria; tyrosinemia, vitamin E deficiency Drug- or Toxin-induced: Neuroleptic and anti-emetic medications (e.g., haloperidol, thorazine, olanzapine, risperidone, quetiapine, compazine, prochlorperazine, metoclopramide, etc.); calcium channel blockers; stimulants , anticonvulsants (e.g., carbamazepine, phenytoin, ); thallium; manganese; carbon monoxide; ethylene glycol; cyanide; methanol; wasp sting Paroxysmal: Paroxysmal kinesogenic choreoathetosis; familial periodic paralysis; complex migraine; alternating hemiplegia; paroxysmal torticollis of infancy Psychogenic Disorders That Mimic Dystonia: Tonic seizures, syringomyelia; Arnold-Chiari malformation type II; posterior fossa mass; cervical spine malformation, Sandifer's syndrome; spasmus nutans; tics; self-stimulation; spasticity; myotonia; hyperexplexia; disorders

19 Work up of Dystonia Take careful history of medication, drug and supplement use Consider Genetic testing (especially DYT1) Consider empiric trial of levodopa Consider metabolic testing: amino acids, organic acis, Wilsons testing, lysosomal storage diseases Consider MRI

20 Treatment of Dystonia Botulinum toxin Medications
Particularly for focal dystonias Medications Anticholinergic agents: trihexyphenidyl and benztropine. GABAergic agents : benzodiazepines, baclofen Dopaminergic agents: tetrabenazine Levodopa for Dopa-responsive dystonia (DRD) Deep brain stimulation (DBS) Physical and other therapies

21 Sterotypies Repetitive, simple movements that can be voluntarily suppressed. Examples include repetitive chewing, rocking, twirling, or touching movements Most common in children with autism or mental retardation; can occur in otherwise normal children.

22 Rett Syndrome

23 Chorea, athetosis and ballismus
an irregular, rapid, uncontrolled, involuntary, excessive movement that seems to flow randomly from one part of the body to another. The affected child often appears fidgety or restless and can’t sit still Athetosis A slower writhing and twisting movement. Ballism (ballismus) chorea that affects proximal joints such as shoulder or hip, leading to large amplitude flailing movements of the limbs

24 Etiology of Chorea Fixed injury/Structural Degenerative
Cerebral Palsy (Kernicterus), Tumor, Trauma, Stroke Degenerative Huntingtons, Neuroacanthocytosis, Ataxia-telangiectasia; Fahr's disease; pantothenate kinase 2 deficiency ("Hallervorden-Spatz disease"); Rett syndrome; Niemann-Pick disease type C , Pelizaeus-Merzbacher disease; GM1 gangliosidosis, metachromatic leukodystrophy; Wilson's disease ,etc Drug Induced/Toxins: Neuroleptics, antiparkinson drugs, tricyclics , amphetamines, anticonvulsants, anticholinergics Metabolic: Hyperthyroid, hypoparathyroid, pregnancy, hyper and hypo natremia, hypomagnesemia, hypoclaciemia, nutrional deficiencies (beriberi, pellagra, B12 deficiency) Acyl-CoA dehydrogenase deficiency; Lesch-Nyhan disease;; methylmalonic aciduria; vitamin E deficiency; propionic acidemia; Infectious Sydenhams chorea Immunological SLE, HSP Migraine Psychogenic

25 Kernicterus

26 Sydenham chorea Sydenham chorea is a movement disorder characterized by chorea, emotional lability, and hypotonia. It is one of the major clinical manifestations of acute rheumatic fever (ARF). Symptoms of SC usually begin one to eight months after the onset of ARF. The symptoms typically improve gradually, with a mean duration of 12 to 15 weeks ( At least 30 percent of individuals have clinical carditis in association with SC. Diagnosis The diagnosis of SC is made clinically, based on characteristic neurological findings and a careful cardiac examination. If carditis is present, this confirms the diagnosis. The antistreptolysin O (ASLO) titer is of limited use in patients with SC, because titers generally peak before the onset of SC symptoms and children without rheumatic fever or SC often have low positive titers of ASLO. The antideoxyribonuclease (anti-DNAse) B titer is more useful for supporting the diagnosis of SC because it tends to remain elevated longer. If not clinicually definite, other causes of chorea should be excluded, including systemic lupus erythematosus, Huntington’s disease, and Wilson’s disease. Treatment Most patients with SC recover fully without treatment, with symptoms lasting from a few weeks to one year or more. For those with significant impairment of motor function and the possibility of self injury consider corticosteroids (prednisone 1 mg/kg daily for two weeks and then tapered over two to three weeks) Valproic acid if needed to treat chorea Up to 30 percent of individuals with SC experience a recurrence, usually within a few years of the initial episode. The risk is probably reduced, by chronic treatment with prophylactic antibiotics.

27 Sydenham Chorea

28 Athetosis

29 Work up Take careful history of medication, drug and supplement use
If acute onset: throat culture and streptococcal blood antigen test (ASLO, anti-DNAse), electrolytes, magnesium, calcium, thyroid function, CBC Consider amino and organic acid studies, ammonia, antinuclear antigen (ANA), antiphospholipid antibodies (APLA), work up for Wilsons disease (start with ceruloplasmin), evaluation of CBC for acanthocytes. Consider MRI

30 Treatment of Chorea May be difficult to treat.
Taper or discontinue any medications that can cause or worsen chorea In adults, the mainstay of treatment in adults is neuroleptics, including haloperidol and pimozide. In children the incidence of side effects in children is high. Therefore, treatment is usually Benzodiazepine, particularly clonazepam, diazepam, or clobazam Valproate, especially in Sydenham's chorea. Sydenham's chorea There is considerable debate about whether children with Sydenham's chorea due to streptococcal infection should be given long-term antibiotics. There is not yet scientific evidence to support this, although short-term treatment is certainly needed in order to prevent complications such as rheumatic fever.

31 Tremor A rhythmic back-and-forth or oscillating involuntary movement about a joint.

32 Classification of Tremor
Classification by type of tremor Rest Tremor Parkinsons, Wilson Disease, Severe essential tremor Action Tremor Postural Kinetic Intention: Cerebellar Tremor Task Specific Isomeric Classification by Etiology Physiologic tremor Essential tremor Associated w/ Peripheral Neuropathy: Charcot MarieTooth Psychogenic

33 Etiology of Tremor Primary Tremors:
Enhanced physiologic tremor Essential Tremor Static (fixed) injury: Stroke (particularly in the midbrain or cerebellum); multiple sclerosis Degenerative: Juvenile parkinsonism; Wilson's disease; Huntington's disease; Tay-Sachs disease Chemical/metabolic: Hyperthyroidism; hyper-adrenaline state (including anxiety or pheochromocytoma); hypomagnesemia; hypocalcemia; hypoglycemia; hepatic encephalopathy Drug-induced Valproate; lithium; thyroid hormone; albuterol, tricyclic antidepressants; stimulants, neuroleptics; cyclosporine; mercury; thallium; nicotine; lead; manganese; arsenic; cyanide; ethanol Psychogenic tremor Other causes of tremor: Peripheral neuropathy, cerebellar disease or malformation,spasmus nutans

34 Essential Tremor Tremor should be the only neurologic manifestation
Usually benign, but may progress to a disabling movement disorder. Hereditary ET can begin in infancy hereditary chin tremor and shuddering attacks.

35 Work up of Tremor Any medications that may worsen tremor should be avoided, if possible. Check electrolytes, including glucose, calcium and magnesium, thyroid function, copper in the urine (for Wilson's disease), and possibly the amount of adrenaline metabolites (for pheochromocytoma). Consider MRI if the tremor had sudden onset, Consider EEG if there is suspicion for seizures. If parkinsonian features are present, consider a trial of L-DOPA If there is a family history of tremor, it may be helpful to of alcohol ( in the affected family member). This suggests essential tremor.

36 Treatment of Tremor Often, mild tremor does not require treatment.
Medications: Propranolol Primidone benzodiazepines (i.e., clonazepam, diazepam, lorazepam).

37 Myoclonus Sudden, brief, jerky, shock-like involuntary movements.
May be triggered by attempts at voluntary movement, sensory stimulation or startle Myoclonus is not suppressible and is often activated by volitional movement. Negative myoclonus is a sudden involuntary relaxation of a muscle, rather than a contraction. Myoclonus is often associated with epilepsy. 

38 Classification and Etiology of Myoclonus
Physiological: e.g., sleep myoclonus, benign myoclonus of infancy Essential Myoclonus: familial essential myoclonus, essential myoclonus-dystonia, stimulus-sensitive myoclonus Epileptic: e.g., juvenile myoclonic epilepsy, progressive myoclonic epilepsies, epilepsia partialis continua, Rasmussen's encephalitis, early infantile myoclonic encephalopathy, infantile spasms, Lennox-Gastaut syndrome, benign familiar myoclonic epilepsy, Angelman syndrome Symptomatic Fixed injury: e.g., carbon-monoxide poisoning, hypoxic injury or near-drowning, heatstroke, trauma, stroke, electrocution Storage/Degenerative diseases: e.g., sialidoses, lipidosis, storage diseases, Wilson's disease, Rett syndrome, mitochondrial disorders, spinocerebellar ataxias Infections/Para-infectious: e.g., Creutzfeldt-Jacob disease, steptococcus, viral encephalitis Endocrine: e.g., hyperthyroidism, hyponatremia, hypoglycemia Structural: e.g., tumors that irritate brain in direct manner or release chemicals into the blood Drug-induced/Toxins: e.g., anti-seizure medications, antidepressants, stimulants, liver-toxic medications, respiratory depressants, corticosteroids, acyclovir, L-dopa Associated with systemic illness: e.g., dialysis, renal failure, liver failure, pulmonary disease, carbon dioxide intoxication








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