An approach to a child with abnormal movement
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This document provides an overview of approaches to evaluating and diagnosing abnormal movements in children. It discusses the pathophysiology, classification, history taking, physical examination, investigations, and management of various movement disorders. The key components involved in movement are the basal ganglia and frontal cortex. Movement disorders are classified based on the type of abnormal movement and their underlying cause. A thorough history and physical exam aim to characterize the pattern, timing, exacerbating/relieving factors of the movements. Investigations include blood tests, imaging, and electrophysiological studies to identify underlying neurological or systemic conditions. Treatment involves addressing the specific cause, symptomatic relief of movements, and counseling.
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An approach to a child with abnormal movement
- 1. An Approach to a Child with Abnormal Movement Sunil Agrawal 1st year MD Pediatrics IOM
- 2. Contents • Introduction • Pathophysiology • Classification • History • Examination • Investigations • Management
- 3. 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.
- 4. 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
- 5. 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.
- 9. Movement Disorder according to lesionMovement Disorder according to lesion . Lesion in globus pallidus – athetosis. Lesion in globus pallidus – athetosis . Lesion in the subthalamic nucleus –hemiballismus. Lesion in the subthalamic nucleus –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
- 10. MOVEMENT DISORDERS PYRAMIDAL SYMPTOMS BASAL GANGLIA DISORDERS CERBELLAR DISORDERS SPASTICITY ATAXIA HYPOKINESIA S HYPERKINESIAS MOTOR-SENSORY BEHAVIOUR AKINESI A RIGIDITY TREMOR DYSTONI A MYOCLONU S CHOREA/ ATHETOSI S TICS/ STERIOTYPIE S COMPULSION MANNERISM
- 11. Fernandez alvarez, 2005 684 patient< 18 years • Tics - 43% • Dystonia- 23% • Tremor- 16% • Myoclonus 6% • Mixea- 4% • Chorea- 3% • Hypokinetic 3%
- 12. 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
- 13. Definitions
- 14. 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
- 15. 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
- 16. Chorea-Contd.. • Causes- • Parainfectious and autoimmune disorders- – Syndenham’s chorea – SLE • Structural basal ganglia lesions- – Vascular chorea in stroke – Mass lesions
- 17. Chorea- causes Contd… • Genetic- – Huntington’s disease – Ataxia telangiectasia • Infectious chorea- – HIV encephalopathy – Cysticercosis – Toxoplasmosis – Diphtheria – Scarlet fever – Viral encephalitis( Mumps, measles, varicella)
- 18. Chorea- causes Contd… • Metabolic or toxic encephalopathies- – Hypo/ hypernatremia – Hypocalcemia – Hyperthyroidism – Hypoparathyroidism – Hepatic/ Renal failure – Carbon monoxide, Manganese, mercury, OP poisoning
- 19. Chorea- causes Contd… • Drug induced chorea- – Dopamine receptor blocking agents- • Phenothiazines – Antiparkinsonian drugs- • L-dopa • Dopamine agonists • Anticholinergics – Antiepileptic drugs- • Phenytoin • Carbamazepine
- 20. Chorea- causes Contd… • Drug induced chorea- – Dopamine receptor blocking agents- • Phenothiazines – Antiparkinsonian drugs- • L-dopa • Dopamine agonists • Anticholinergics – Antiepileptic drugs- • Phenytoin • Carbamazepine
- 21. 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
- 22. 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
- 23. 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
- 24. Dystonia- Contd.. • Causes- – Perinatal asphyxia – Kernicterus – Generalised primary dystonia – Drugs – Wilson disease- Dystonia most common neurologic manifestation .Segmental- genetic, idiopathic or overuse
- 25. Ballismus • Form of chorea • Movements more coarse and ballistic • Hyper chorea • Extremity flailing • Causes – Sydenham’s chorea – Stroke – Cerebral tumours and – Trauma
- 26. 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
- 27. 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
- 28. 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
- 29. 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
- 30. Ataxia- Contd.. • Chronic or Progressive Ataxia- – Brain tumors – Congenital malformations- • Cerebellar aplasias • Dandy- Walker malformation • Chiari malformation – Hereditary ataxias
- 31. 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
- 32. Approach
- 33. 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?
- 34. 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?
- 35. 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
- 36. History- Contd.. Onset/duration Acute : Infection, trauma Slowly progressive : Wilson’s disease, Tourette syndrome, Parkinson’s disease, Hungtington’s chorea
- 37. 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
- 38. 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
- 39. History- Contd.. • H/o fever : infective origin • poisoning • Associated with signs of hepatic failure- Wilson disease
- 40. History- Contd.. • Sydenham chorea - Associated with hypotonia, emotionalability -Other features of rheumatic fever • Joint pain , rashes : SLE • Associated with presenile dementia- Huntington disease
- 41. History- Contd.. • H/o heat intolerance, increased appetite with weight loss, increased stool frequency, palpitation- thyrotoxicosis • Features of increased ICP- Brain tumors
- 42. History- Contd.. • H/o intake of drugs • Perinatal history- • Dystonia- Asphyxia, Jaundice • Athetosis- Asphyxia, jaundice and prematurity • Cardiac surgery- Choreoathetosis
- 43. History- Contd.. • Developmental history: -delayed milestones • Immunization history : polio, diptheria , pertusis • Family history- Huntington disease(AD) Wilson’s disease Essential tremor • Consanguinuty
- 44. On Examination • General look unconscious – CNS infection • Vitals :– • Raised temp - Infective • Pulse, BP, Pattern of respiration : ICSOL, CNS infection, thyrotoxicosis • Ant fontanel
- 45. • Icterus : Wilsons disease • Eye : • opsoclonusmyoclonus syndrome • blepharospasm : tics • K-F ring • Mask like face (Parkinsonism) • Rash: Meningococal, Oculocutaneous Telangiectasias
- 46. • Foreshortened occiput- Chiari malformation • Prominent occiput- Dandy Walker malformation
- 47. • Syndenham chorea- Milkmaid’s grip Choreic hand Darting tongue Pronator sign
- 48. 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
- 49. CNS examination. Contd… • Presence of primitive reflexes- cerebral palsy • Signs of meningeal irritation? • any cerebellar signs?
- 50. 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
- 51. Investigations * CBC leucocytosis : infective raised ESR : SLE * Biochemical: RFT, LFT,RBS ,Electrolytes * Throat culture • Imaging: CT SCAN , MRI • USG, ECHO
- 52. Investigations 4)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
- 53. Principle of Management • Symptomatic treatment • Treatment of the cause • Counselling
- 54. 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.
- 55. Drug Treatment • Tics: Haloperidol, clonidine • Chorea: Diazepam, valproic acid, phenothiazine, haloperidol • Tremor: B blockers, anticholinergics
- 56. 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
- 57. Thank you
Editor's Notes
- Stereotypies are intermittent, involuntary, repetitive, purposeless, patterned movements that are usually rhythmic Transient tic disorder- Most common movement abnormality of childhhood, lasts for weeks to <yr, M>F. +ve family history, Chronic tic- motor tic lasting for more than year Gilles de la tourette synd- AD, Atleast one vocal tic, OCD and ADHD Motor tics can be classified further by speed and quality as clonic (abrupt and fast) or dystonic/tonic (slow and sustained). Simple motor tics include blinking, nose twitching, grimacing, neck jerking, shoulder elevation, sustained eye closure, gaze shifts, bruxism, and abdominal tensing. Simple vocal tics include sniffing, throat clearing, grunting, squeaking, humming, coughing, blowing, and sucking sounds. Complex tics appear more "purposeful" than simple tics and may include combinations of movements of multiple body parts. Examples are head shaking, trunk flexion, scratching, touching, finger tapping, hitting, jumping, kicking, and gestures (obscene gestures are termed copropraxia). Complex vocal tics can encompass spoken syllables words or phrases; shouting of obscenities or profanities (coprolalia); repetition of the words of others (echolalia); and repetition of the final syllable, word, or phrase of one’s own words (palillalia)
- Hungtington’s disease- AD inheritance, expanded sequence of CAG repeats, Progressive chorea and presenile dementia
- Tardive dyskinesia- facial movement, late onset,
- Tardive dyskinesia- facial movement, late onset,
- Rigidity- muscle stiffness through out the range of motion in both flexors and extensors due to dysfn in BG D/d- Spasticity- increased tone is velocity dependent due to UMN dysfn
- Essential tremor- Autosomal dominant, esp upper extremities,
- Segmental- genetic form of torsion dystonia or idiopathic or acquired due to overuse Individuals who have dystonia often find that touching one part of the body relieves the dystonic spasms; this phenomenon is called a sensory trick or geste antagoniste . For example, rubbing the back of the hand may diminish writer’s cramp
- Generalised primary dystonia- torsion dystonia or dystonia musculorum deformans- Genetic disorder, tiptoe walking, aggravated by stress
- Opsoclonus- myoclonuc- conjugate eye movement, severe myoclonic jerking of head. Idiopathic, encephalitis, neuroblastoma
- Opsoclonus- myoclonuc- conjugate eye movement, severe myoclonic jerking of head. Idiopathic, encephalitis, neuroblastoma
- Thallium- used in home as pesticide Anticonvulsants like phenytoin Genetic disorder- Dominant recurrent ataxia, Episodic ataxia Type 1 and 2, Hartnup disease, MSUD, Pyruvate dehydrogenase disorder Postinfectious/ Immune- Varicella, Miller Fisher syndrome, Multiple sclerosis, Myoclonic encephalopathy/ Neuroblastoma Vascular disorder- Cerebellar hemorrhage, Kawasaki disease Ataxia telangiectasia- involves immunolgic, neurologic, endocronologic, hepatic and cutaneous abnormalities.
- Cerebellar hemangioblastoma- von Hippel-Lindau disease Autosomal dominant inheritance Autosomal recessive- Abetalipoproteinemia, ataxiao telangiectasia, Fredereich ataxia, MSUD, Ramsay Hunt syndrome Xlinked inheritance- Adrenoleukodystrophy, Leber optic neuropathy
- Juvenile idiopathic dystonia- sporadic or hereditary
- Generalised primary dystonia- torsion dystonia or dystonia musculorum deformans- Genetic disorder, tiptoe walking, aggravated by stress
- Rigidity- muscle stiffness through out the range of motion in both flexors and extensors due to dysfn in BG D/d- Spasticity- increased tone is velocity dependent due to UMN dysfn
- Milkmaid grip- relaxing and tightening hand shake Choreic hand- Spooning of the extended hand by flexion at the wrist and extension of finger Darting tongue- Tongue cannot be protruded for longer than a few seconds Pronator sign- The arms and palms turn outward when held above the head