an approach to a child with abnormal movement
TRANSCRIPT
An Approach to a Child with Abnormal Movement
Sunil Agrawal1st year MD PediatricsIOM
Contents
• Introduction• Pathophysiology• Classification• History • Examination • Investigations • Management
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.
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
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.
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
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
Fernandez alvarez, 2005684 patient< 18 years
• Tics - 43%• Dystonia- 23%• Tremor- 16%• Myoclonus 6%• Mixea- 4%• Chorea- 3%• Hypokinetic 3%
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
Definitions
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
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
Chorea-Contd..• Causes-• Parainfectious and autoimmune disorders-
– Syndenham’s chorea– SLE
• Structural basal ganglia lesions-– Vascular chorea in stroke– Mass lesions
Chorea- causes Contd…
• Genetic-– Huntington’s disease– Ataxia telangiectasia
• Infectious chorea-– HIV encephalopathy– Cysticercosis– Toxoplasmosis– Diphtheria– Scarlet fever– Viral encephalitis( Mumps, measles, varicella)
Chorea- causes Contd…
• Metabolic or toxic encephalopathies-– Hypo/ hypernatremia– Hypocalcemia– Hyperthyroidism– Hypoparathyroidism– Hepatic/ Renal failure– Carbon monoxide, Manganese, mercury, OP
poisoning
Chorea- causes Contd…
• Drug induced chorea- – Dopamine receptor blocking agents-
• Phenothiazines– Antiparkinsonian drugs-
• L-dopa• Dopamine agonists• Anticholinergics
– Antiepileptic drugs-• Phenytoin• Carbamazepine
Chorea- causes Contd…
• Drug induced chorea- – Dopamine receptor blocking agents-
• Phenothiazines– Antiparkinsonian drugs-
• L-dopa• Dopamine agonists• Anticholinergics
– Antiepileptic drugs-• Phenytoin• Carbamazepine
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
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
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
Dystonia- Contd..
• Causes-– Perinatal asphyxia– Kernicterus– Generalised primary dystonia– Drugs– Wilson disease- Dystonia most common
neurologic manifestation.Segmental- genetic, idiopathic or overuse
Ballismus
• Form of chorea• Movements more coarse and ballistic• Hyper chorea• Extremity flailing• Causes
– Sydenham’s chorea– Stroke– Cerebral tumours and – Trauma
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
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
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
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
Ataxia- Contd..
• Chronic or Progressive Ataxia-– Brain tumors– Congenital malformations-
• Cerebellar aplasias• Dandy- Walker malformation• Chiari malformation
– Hereditary ataxias
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
Approach
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?
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?
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
History- Contd..
Onset/duration Acute : Infection, trauma
Slowly progressive : Wilson’s disease, Tourette syndrome, Parkinson’s disease, Hungtington’s chorea
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
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
History- Contd..
• H/o fever : infective origin
• poisoning
• Associated with signs of hepatic failure- Wilson disease
History- Contd..
• Sydenham chorea - Associated with hypotonia, emotionalability -Other features of rheumatic fever
• Joint pain , rashes : SLE
• Associated with presenile dementia- Huntington disease
History- Contd..
• H/o heat intolerance, increased appetite with weight loss, increased stool frequency, palpitation- thyrotoxicosis
• Features of increased ICP- Brain tumors
History- Contd..
• H/o intake of drugs
• Perinatal history- • Dystonia- Asphyxia, Jaundice• Athetosis- Asphyxia, jaundice and prematurity
• Cardiac surgery- Choreoathetosis
History- Contd..
• Developmental history: -delayed milestones
• Immunization history : polio, diptheria , pertusis
• Family history- Huntington disease(AD) Wilson’s disease
Essential tremor• Consanguinuty
On Examination• General look
unconscious – CNS infection
• Vitals :–• Raised temp - Infective• Pulse, BP, Pattern of respiration : ICSOL, CNS
infection, thyrotoxicosis • Ant fontanel
• Icterus : Wilsons disease
• Eye :• opsoclonusmyoclonus syndrome• blepharospasm : tics• K-F ring
• Mask like face (Parkinsonism)
• Rash: Meningococal, Oculocutaneous Telangiectasias
• Foreshortened occiput- Chiari malformation
• Prominent occiput- Dandy Walker malformation
• Syndenham chorea- Milkmaid’s grip Choreic hand
Darting tongue Pronator sign
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
CNS examination. Contd…• Presence of primitive reflexes- cerebral palsy
• Signs of meningeal irritation?
• any cerebellar signs?
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
Investigations * CBC leucocytosis : infective raised ESR : SLE * Biochemical: RFT, LFT,RBS ,Electrolytes * Throat culture • Imaging: CT SCAN , MRI • USG, ECHO
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
Principle of Management
• Symptomatic treatment• Treatment of the cause • Counselling
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.
Drug Treatment
• Tics: Haloperidol, clonidine• Chorea:
Diazepam, valproic acid, phenothiazine, haloperidol
• Tremor: B blockers, anticholinergics
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
Thank you