An approach to a child with abnormal movement

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  • 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
  • An approach to a child with abnormal movement

    1. 1. An Approach to a Child withAbnormal MovementSunil Agrawal1styear MD PediatricsIOM
    2. 2. Contents• Introduction• Pathophysiology• Classification• History• Examination• Investigations• Management
    3. 3. Introduction- Dysfunction in the implementation of appropriatetargeting and velocity of intended movements,- dysfunction of posture,- the presence of abnormal involuntary movements,-the performance of normal-appearing movementsat inappropriate or unintended times.
    4. 4. Introduction- Contd…• Can be the primary or secondary manifestation ofnumerous neurologic disorders• Classification can be difficult• Can resemble , sometimes difficult to distnguishfrom each other
    5. 5. Pathophysiology• The components typically implicated in disorders ofmovement are– the basal ganglia (caudate, putamen, globus pallidus,subthalamic nucleus, substantia nigra) and– frontal cortex.• The accomplishment of smooth, coordinatedmovement requires a multifaceted network of brainregions, including basal ganglia and frontal cortex,but also thalamus, cerebellum, spinal cord,peripheral nerve, and muscle.
    6. 6. 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
    7. 7. MOVEMENT DISORDERSPYRAMIDALSYMPTOMSBASAL GANGLIADISORDERSCERBELLARDISORDERSSPASTICITYATAXIAHYPOKINESIASHYPERKINESIAS MOTOR-SENSORYBEHAVIOURAKINESIARIGIDITYTREMOR DYSTONIA MYOCLONUSCHOREA/ATHETOSISTICS/STERIOTYPIESCOMPULSIONMANNERISM
    8. 8. Fernandez alvarez, 2005684 patient< 18 years• Tics - 43%• Dystonia- 23%• Tremor- 16%• Myoclonus 6%• Mixea- 4%• Chorea- 3%• Hypokinetic 3%
    9. 9. Biochemistry - neurotransmittersDopamine AcetylcholineSerotonin Receptor HistamineGABA GlutamateSubstance PHypokinetic - dopamine decreased - treat with dopaminereplacement or anticholinergic drugsHyperkinetic - dopamine increased and acetylcholine decreased- treat with a dopamine antagonist or cholinergic drug
    10. 10. Definitions
    11. 11. Tics• Spasmodic, involuntary, repetitive, stereotypedmovements that are nonrhythmic, often exacerbatedby stress• May affect any group of muscle• Classification-– Transient tics of childhood ( < 1 year)25 to 30% of children – Most common movementabnormality of childhood– Chronic tics (> 1 year)– Tourette syndrome
    12. 12. Chorea• “Dance” in Greek• Irregular, rapid, uncontrolled, involuntarymovements• Worsen on rest, but remain or improve withvoluntary movement• Incorporated into semipurposeful acts tomodify the movement• Tone - normal
    13. 13. Chorea-Contd..• Causes-• Parainfectious and autoimmune disorders-– Syndenham’s chorea– SLE• Structural basal ganglia lesions-– Vascular chorea in stroke– Mass lesions
    14. 14. Chorea- causes Contd…• Genetic-– Huntington’s disease– Ataxia telangiectasia• Infectious chorea-– HIV encephalopathy– Cysticercosis– Toxoplasmosis– Diphtheria– Scarlet fever– Viral encephalitis( Mumps, measles, varicella)
    15. 15. Chorea- causes Contd…• Metabolic or toxic encephalopathies-– Hypo/ hypernatremia– Hypocalcemia– Hyperthyroidism– Hypoparathyroidism– Hepatic/ Renal failure– Carbon monoxide, Manganese, mercury, OPpoisoning
    16. 16. Chorea- causes Contd…• Drug induced chorea-– Dopamine receptor blocking agents-• Phenothiazines– Antiparkinsonian drugs-• L-dopa• Dopamine agonists• Anticholinergics– Antiepileptic drugs-• Phenytoin• Carbamazepine
    17. 17. Chorea- causes Contd…• Drug induced chorea-– Dopamine receptor blocking agents-• Phenothiazines– Antiparkinsonian drugs-• L-dopa• Dopamine agonists• Anticholinergics– Antiepileptic drugs-• Phenytoin• Carbamazepine
    18. 18. Athetosis• Distal writhing movements of extremities• Choreoathetosis• Also has rigidity• Causes-– Extrapyramidal CP- asphyxia, kernicterus or geneticmetabolic disorder like glutaric aciduria– CP due to prematurity– Post- infectious– Cirulatory arrest for complex cardiac surgery– Drugs like phenothiazines
    19. 19. 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
    20. 20. Dystonia• Syndrome of sustained muscle contractions,frequently causing twisting and repetitivemovements or abnormal postureshallmark - simultaneous contraction of agonist andantagonist muscle• Focal• Segmental• Multifocal• Hemi dystonia• Generalized
    21. 21. Dystonia- Contd..• Causes-– Perinatal asphyxia– Kernicterus– Generalised primary dystonia– Drugs– Wilson disease- Dystonia most commonneurologic manifestation.Segmental- genetic, idiopathic or overuse
    22. 22. Ballismus• Form of chorea• Movements more coarse and ballistic• Hyper chorea• Extremity flailing• Causes– Sydenham’s chorea– Stroke– Cerebral tumours and– Trauma
    23. 23. Myoclonus• Very brief, abrupt, involuntary, non-suppressible, jerky contraction involving asingle muscle or muscle group- "shock like"• Presence in normal (associated with sleep,exercise, anxiety) and numerous pathologicsituations, both epileptic and nonepileptic• Focal , segmental or generalized
    24. 24. 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
    25. 25. 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
    26. 26. Ataxia• Inability to make smooth, accurate and coordinatedmovements• Due to disorder of cerebellum,sensory pathway in posteriorcolumn of spinal cord-Generalised or- primarily affect gait or hands and arms. acute or chronicCauses:• Acute or Recurrent-– Brain tumor– Drugs like alcohol, thallium, anticonvulsants– Postinfectious/ immune– Trauma– Vascular disorder
    27. 27. Ataxia- Contd..• Chronic or Progressive Ataxia-– Brain tumors– Congenital malformations-• Cerebellar aplasias• Dandy- Walker malformation• Chiari malformation– Hereditary ataxias
    28. 28. Hypokinesia• Parkinsonism : bradykinesia, rigidity, tremoror abnormal posture• Is rare in childhood• Causes-– Post head trauma– Post encephalitis– Genetic disorders- Juvenile Huntington chorea,Wilson disease, ataxia telangiectasia
    29. 29. Approach
    30. 30. 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 (withoutstop)?• Has the movement disorder changed over time?• Do environmental stimuli or emotional states modulate themovement disorder?
    31. 31. Key questions- Contd..• Can the movements be suppressed voluntarily?• Are there findings on the examination suggestive offocal neurologic deficit or systemic disease?• Is there a family history of a similar or relatedcondition?• Does the movement disorder abate with sleep?
    32. 32. History• Age at onset-• full term neonate : jitteriness Infant : myoclonus, athetosis, transient dystonia Older child : chorea• Sex-female: Sydenham’s chorea, thryrotoxicosismale : tics, tremors
    33. 33. History- Contd..Onset/durationAcute : Infection, traumaSlowly progressive : Wilson’s disease,Tourette syndrome, Parkinson’s disease,Hungtington’s chorea
    34. 34. 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
    35. 35. History- Contd..• H/o waxing and waning: Tics• Aggravated with stress: tremor, tics, Tourette syndGeneralised primary dystonia, Nocturnalmyoclonus, Syndenham’s chorea• Relieving factors-• Behavioral abnormalities: chorea, Parkinsons disease• Diurnal variation- with sleep : nocturnal myoclonus
    36. 36. History- Contd..• H/o fever : infective origin• poisoning• Associated with signs of hepatic failure-Wilson disease
    37. 37. History- Contd..• Sydenham chorea- Associated with hypotonia, emotionalability-Other features of rheumatic fever• Joint pain , rashes : SLE• Associated with presenile dementia-Huntington disease
    38. 38. History- Contd..• H/o heat intolerance, increased appetite withweight loss, increased stool frequency,palpitation- thyrotoxicosis• Features of increased ICP- Brain tumors
    39. 39. History- Contd..• H/o intake of drugs• Perinatal history-• Dystonia- Asphyxia, Jaundice• Athetosis- Asphyxia, jaundice and prematurity• Cardiac surgery- Choreoathetosis
    40. 40. History- Contd..• Developmental history:-delayed milestones• Immunization history :polio, diptheria , pertusis• Family history- Huntington disease(AD)Wilson’s diseaseEssential tremor• Consanguinuty
    41. 41. On Examination• General lookunconscious – CNS infection• Vitals :–• Raised temp - Infective• Pulse, BP, Pattern of respiration : ICSOL, CNSinfection, thyrotoxicosis• Ant fontanel
    42. 42. • Icterus : Wilsons disease• Eye :• opsoclonusmyoclonus syndrome• blepharospasm : tics• K-F ring• Mask like face (Parkinsonism)• Rash: Meningococal, OculocutaneousTelangiectasias
    43. 43. • Foreshortened occiput- Chiari malformation• Prominent occiput- Dandy Walkermalformation
    44. 44. • Syndenham chorea- Milkmaid’s gripChoreic handDarting tonguePronator sign
    45. 45. CNS Examination GCS Speech- vocal tics, dysarthria- chorea CRANIAL NERVE3rdand 6thnerve 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
    46. 46. CNS examination. Contd…• Presence of primitive reflexes- cerebral palsy• Signs of meningeal irritation?• any cerebellar signs?
    47. 47. 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
    48. 48. Investigations* CBC leucocytosis : infectiveraised ESR : SLE* Biochemical: RFT, LFT,RBS ,Electrolytes* Throat culture• Imaging: CT SCAN , MRI• USG, ECHO
    49. 49. Investigations4)Electrophysiological studies• EMG- dystonia• EEG• ECG• 5)Special tests• Serological assay- ASO, antiDnase, ANAantiphospholipid 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 clinicalsuspicion
    50. 50. Principle of Management• Symptomatic treatment• Treatment of the cause• Counselling
    51. 51. Drug Treatment• Dystonia :– Diphenhydramine iv may reverse drug relateddystonia– 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 chronicdystonia.
    52. 52. Drug Treatment• Tics:Haloperidol, clonidine• Chorea:Diazepam, valproic acid, phenothiazine,haloperidol• Tremor:B blockers, anticholinergics
    53. 53. References• Nelson Text book of pediatrics• Ghai ,Essential Pediatrics• Movement Disorders in Children -- Schlaggar andMink 24 (2) 39 -- Pediatrics in Review• Clinical pediatric neurology, Gerald.M.Fenichel 3rdedition• Pediatrics in Review Vol.24 No.2 February 2003
    54. 54. Thank you

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