An approach to a child with abnormal movement

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An Approach to a Child with Abnormal Movement Sunil Agrawal 1 st year MD Pediatrics IOM

Transcript of An approach to a child with abnormal movement

Page 1: An approach to a child with abnormal movement

An Approach to a Child with Abnormal Movement

Sunil Agrawal1st year MD PediatricsIOM

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Contents

• Introduction• Pathophysiology• Classification• History • Examination • Investigations • Management

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Introduction

- Dysfunction in the implementation of appropriate targeting and velocity of intended movements,

- dysfunction of posture, - the presence of abnormal involuntary movements, -the performance of normal-appearing movements

at inappropriate or unintended times.

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Introduction- Contd…

• Can be the primary or secondary manifestation of numerous neurologic disorders

• Classification can be difficult

• Can resemble , sometimes difficult to distnguish from each other

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Pathophysiology

• The components typically implicated in disorders of movement are– the basal ganglia (caudate, putamen, globus pallidus,

subthalamic nucleus, substantia nigra) and – frontal cortex.

• The accomplishment of smooth, coordinated movement requires a multifaceted network of brain regions, including basal ganglia and frontal cortex, but also thalamus, cerebellum, spinal cord, peripheral nerve, and muscle.

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Movement Disorder according to lesionMovement Disorder according to lesion

. Lesion in globus pallidus – athetosis. Lesion in globus pallidus – athetosis

. Lesion in the subthalamic nucleus –. Lesion in the subthalamic nucleus –hemiballismus hemiballismus

. Multiple small lesion in putamen – s.chorea. Multiple small lesion in putamen – s.chorea

. Lesion in caudate nucleus- huntington chorea. Lesion in caudate nucleus- huntington chorea

. Lesion in substantia nigra –parkinson’s disease. Lesion in substantia nigra –parkinson’s disease

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MOVEMENT DISORDERS

PYRAMIDAL SYMPTOMS

BASAL GANGLIA DISORDERS

CERBELLAR DISORDERS

SPASTICITYATAXIA

HYPOKINESIAS HYPERKINESIAS MOTOR-SENSORY BEHAVIOUR

AKINESIA

RIGIDITY

TREMOR DYSTONIA MYOCLONU

SCHOREA/ ATHETOSIS

TICS/ STERIOTYPIES

COMPULSION

MANNERISM

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Fernandez alvarez, 2005684 patient< 18 years

• Tics - 43%• Dystonia- 23%• Tremor- 16%• Myoclonus 6%• Mixea- 4%• Chorea- 3%• Hypokinetic 3%

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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

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Definitions

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Tics• Spasmodic, involuntary, repetitive, stereotyped

movements that are nonrhythmic, often exacerbated by stress

• May affect any group of muscle

• Classification-– Transient tics of childhood ( < 1 year) 25 to 30% of children – Most common movement

abnormality of childhood– Chronic tics (> 1 year)– Tourette syndrome

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Chorea

• “Dance” in Greek• Irregular, rapid, uncontrolled, involuntary

movements• Worsen on rest, but remain or improve with

voluntary movement• Incorporated into semipurposeful acts to

modify the movement• Tone - normal

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Chorea-Contd..• Causes-• Parainfectious and autoimmune disorders-

– Syndenham’s chorea– SLE

• Structural basal ganglia lesions-– Vascular chorea in stroke– Mass lesions

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Chorea- causes Contd…

• Genetic-– Huntington’s disease– Ataxia telangiectasia

• Infectious chorea-– HIV encephalopathy– Cysticercosis– Toxoplasmosis– Diphtheria– Scarlet fever– Viral encephalitis( Mumps, measles, varicella)

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Chorea- causes Contd…

• Metabolic or toxic encephalopathies-– Hypo/ hypernatremia– Hypocalcemia– Hyperthyroidism– Hypoparathyroidism– Hepatic/ Renal failure– Carbon monoxide, Manganese, mercury, OP

poisoning

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Chorea- causes Contd…

• Drug induced chorea- – Dopamine receptor blocking agents-

• Phenothiazines– Antiparkinsonian drugs-

• L-dopa• Dopamine agonists• Anticholinergics

– Antiepileptic drugs-• Phenytoin• Carbamazepine

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Chorea- causes Contd…

• Drug induced chorea- – Dopamine receptor blocking agents-

• Phenothiazines– Antiparkinsonian drugs-

• L-dopa• Dopamine agonists• Anticholinergics

– Antiepileptic drugs-• Phenytoin• Carbamazepine

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Athetosis• Distal writhing movements of extremities• Choreoathetosis• Also has rigidity

• Causes-– Extrapyramidal CP- asphyxia, kernicterus or genetic

metabolic disorder like glutaric aciduria– CP due to prematurity– Post- infectious– Cirulatory arrest for complex cardiac surgery– Drugs like phenothiazines

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Tremor• Rhythmic oscillations of a part of the body around the central point

• Rest -• Intention -

Causes- . Physiological• Essential tremor• Drugs-

– Valproic acid– Neuroleptics– Caffeine

• Trauma- head injury• Metabolic disorder - hypoglycemia, thyrotoxicosis, neuroblastoma,

pheochromocytoma, Wilson disease

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Dystonia• Syndrome of sustained muscle contractions,

frequently causing twisting and repetitive movements or abnormal postures

hallmark - simultaneous contraction of agonist and antagonist muscle

• Focal• Segmental• Multifocal• Hemi dystonia• Generalized

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Dystonia- Contd..

• Causes-– Perinatal asphyxia– Kernicterus– Generalised primary dystonia– Drugs– Wilson disease- Dystonia most common

neurologic manifestation.Segmental- genetic, idiopathic or overuse

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Ballismus

• Form of chorea• Movements more coarse and ballistic• Hyper chorea• Extremity flailing• Causes

– Sydenham’s chorea– Stroke– Cerebral tumours and – Trauma

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Myoclonus

• Very brief, abrupt, involuntary, non-suppressible, jerky contraction involving a single muscle or muscle group- "shock like"

• Presence in normal (associated with sleep, exercise, anxiety) and numerous pathologic situations, both epileptic and nonepileptic

• Focal , segmental or generalized

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Myoclonus

• Causes-– Physiologic– Benign nocturnal myoclonus– Benign myoclonus of infancy– Essential myoclonus– Epileptic myoclonus( Juvenile myoclonic epilepsy) – Opsoclonus- myoclonus – Post CNS injury– Basal ganglia disorders– Drug induced

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Myoclonus

• Causes-– Physiologic– Benign nocturnal myoclonus– Benign myoclonus of infancy– Essential myoclonus– Epileptic myoclonus( Juvenile myoclonic epilepsy) – Opsoclonus- myoclonus – Post CNS injury– Basal ganglia disorders– Drug induced

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Ataxia• Inability to make smooth, accurate and coordinated

movements• Due to disorder of cerebellum,sensory pathway in posterior

column of spinal cord -Generalised or - primarily affect gait or hands and arms

. acute or chronic Causes:• Acute or Recurrent-

– Brain tumor– Drugs like alcohol, thallium, anticonvulsants– Postinfectious/ immune– Trauma– Vascular disorder

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Ataxia- Contd..

• Chronic or Progressive Ataxia-– Brain tumors– Congenital malformations-

• Cerebellar aplasias• Dandy- Walker malformation• Chiari malformation

– Hereditary ataxias

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Hypokinesia

• Parkinsonism : bradykinesia, rigidity, tremor or abnormal posture

• Is rare in childhood

• Causes-– Post head trauma– Post encephalitis– Genetic disorders- Juvenile Huntington chorea,

Wilson disease, ataxia telangiectasia

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Approach

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Key questions• Is the pattern of movements normal or abnormal?

• Is the number of movements excessive or diminished?

• Is the movement paroxysmal (sudden onset and offset), continual (repeated again and again), or continuous (without stop)?

• Has the movement disorder changed over time?

• Do environmental stimuli or emotional states modulate the movement disorder?

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Key questions- Contd..• Can the movements be suppressed voluntarily?

• Are there findings on the examination suggestive of focal neurologic deficit or systemic disease?

• Is there a family history of a similar or related condition?

• Does the movement disorder abate with sleep?

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History

• Age at onset-• full term neonate : jitteriness Infant : myoclonus, athetosis, transient dystonia Older child : chorea

• Sex- female: Sydenham’s chorea, thryrotoxicosis male : tics, tremors

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History- Contd..

Onset/duration Acute : Infection, trauma

Slowly progressive : Wilson’s disease, Tourette syndrome, Parkinson’s disease, Hungtington’s chorea

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History- Contd..

• Type of movement-• rapid jerky: chorea• slow movement : athetosis• sustained: dystonia

• Involvement of body parts:• distal limb : athetosis• all body parts : chorea• hand : writer’cramp ,focal dystonia

• Presence of movements in sleep : • seizure disorder • nocturnal myoclonus

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History- Contd..• H/o waxing and waning: Tics

• Aggravated with stress: tremor, tics, Tourette synd Generalised primary dystonia, Nocturnal myoclonus, Syndenham’s chorea

• Relieving factors-

• Behavioral abnormalities: chorea, Parkinsons disease

• Diurnal variation- with sleep : nocturnal myoclonus

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History- Contd..

• H/o fever : infective origin

• poisoning

• Associated with signs of hepatic failure- Wilson disease

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History- Contd..

• Sydenham chorea - Associated with hypotonia, emotionalability -Other features of rheumatic fever

• Joint pain , rashes : SLE

• Associated with presenile dementia- Huntington disease

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History- Contd..

• H/o heat intolerance, increased appetite with weight loss, increased stool frequency, palpitation- thyrotoxicosis

• Features of increased ICP- Brain tumors

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History- Contd..

• H/o intake of drugs

• Perinatal history- • Dystonia- Asphyxia, Jaundice• Athetosis- Asphyxia, jaundice and prematurity

• Cardiac surgery- Choreoathetosis

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History- Contd..

• Developmental history: -delayed milestones

• Immunization history : polio, diptheria , pertusis

• Family history- Huntington disease(AD) Wilson’s disease

Essential tremor• Consanguinuty

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On Examination• General look

unconscious – CNS infection

• Vitals :–• Raised temp - Infective• Pulse, BP, Pattern of respiration : ICSOL, CNS

infection, thyrotoxicosis • Ant fontanel

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• Icterus : Wilsons disease

• Eye :• opsoclonusmyoclonus syndrome• blepharospasm : tics• K-F ring

• Mask like face (Parkinsonism)

• Rash: Meningococal, Oculocutaneous Telangiectasias

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• Foreshortened occiput- Chiari malformation

• Prominent occiput- Dandy Walker malformation

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• Syndenham chorea- Milkmaid’s grip Choreic hand

Darting tongue Pronator sign

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CNS Examination GCS Speech- vocal tics, dysarthria- chorea

CRANIAL NERVE 3rd and 6th nerve palsy – raised ICT Motor exam- dystonia, hypotonia rigidity, bradykinesia:Parkinsons disease exaggerated reflex : thyrotoxicosis ataxic gait : cerebellar lesion, ataxia telangiectasis Tip toe walking-generalised primary dystonia

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CNS examination. Contd…• Presence of primitive reflexes- cerebral palsy

• Signs of meningeal irritation?

• any cerebellar signs?

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Other Systems• Musculoskeletal examinaion

-side of the body- hemichorea, hemiballismus

- which joint/limb- ballismus, dystonia - joint tenderness

• C.V.S: any murmurs

• Abdominal: hepatosplenomegaly , ascites

• Thyroid

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Investigations * CBC leucocytosis : infective raised ESR : SLE * Biochemical: RFT, LFT,RBS ,Electrolytes * Throat culture • Imaging: CT SCAN , MRI • USG, ECHO

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Investigations4)Electrophysiological studies

• EMG- dystonia • EEG• ECG

• 5)Special tests• Serological assay- ASO, antiDnase, ANA antiphospholipid Ab• Serum Cu/ceruloplasmin/24 hr urinary copper/ liver biopsy• Test for metabolic disorder• Toxins• Selective absence of IgA- Ataxia telangiectasia

*Other testing for rare disease- based in symptoms and clinical suspicion

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Principle of Management

• Symptomatic treatment• Treatment of the cause • Counselling

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Drug Treatment

• Dystonia :– Diphenhydramine iv may reverse drug related

dystonia – Trihexyphenidyl, carbamazepine levodopa,

bromocriptine, diazepam– Botilinum toxin injection– Deep brain stimulation for generalized dystonia– A trial of L-DOPA is indicated in all cases of chronic

dystonia.

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Drug Treatment

• Tics: Haloperidol, clonidine• Chorea:

Diazepam, valproic acid, phenothiazine, haloperidol

• Tremor: B blockers, anticholinergics

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References• Nelson Text book of pediatrics• Ghai ,Essential Pediatrics • Movement Disorders in Children -- Schlaggar and

Mink 24 (2) 39 -- Pediatrics in Review• Clinical pediatric neurology, Gerald.M.Fenichel 3rd

edition• Pediatrics in Review Vol.24 No.2 February 2003

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Thank you