Unit 2 MSD Cerebral Palsy PDF

Unit 2 Cerebral Palsy 1) Developmental motor speech disorders: (i) Developmental dysarthria / anarthrias: due to congenital or genetic causes A] Cerebral palsy B] Others: Eg Muscular dystrophy, Craniostenosis, Congenital hydrocephalus etc (ii) Developmental apraxia of speech (DAS)...

Unit 2 Cerebral Palsy 1) Developmental motor speech disorders: (i) Developmental dysarthria / anarthrias: due to congenital or genetic causes A] Cerebral palsy B] Others: Eg Muscular dystrophy, Craniostenosis, Congenital hydrocephalus etc (ii) Developmental apraxia of speech (DAS) 2) Acquired motor speech disorders: various etiologies such as vascular pathologies, tumours, trauma etc. which is acquired in nature  In children, clinical manifestation of insult to nervous system falls under 6 major categories: Neurosensory Eg; Hearing impairment Neurointellectual Eg Mental retardation Neurobehavioral Eg; ADHD Seizural Eg Any type of epilepsy Neuroperceptual Eg LD, APD etc Neuromotor Eg: Cerebral palsy  Cerebral palsy/cerebral dysfunction First identified/described by John Little who was a physiotherapist (polio patient) and work with orthopaedic cases. He saw that there was one more condition which resembled polio but not actually polio and he called it Little’s disease. He found that one way to distinguish this condition from polio and myopathies was its non progressive nature. Term “Cerebral Palsy” first coined by Phelps (orthopaedic surgeon)  In 1947, a group of specialists (Neurosurgeons, neurologists, orthopaedic specialists, physiotherapists, occupational therapists, speech therapists) formed the AACP (American Academy for Cerebral Palsy) to form structured programs on diagnosis, treatment, technological development, perinatal care etc. of cerebral palsied children. In 1977- AACP retermed as AACPDM because they realized that there were other similar disorders which came under childhood disability conditions. DM-Developmental medicine means a branch of medicine that deals with developmental disabilities of children.  Definition of CP According to American Academy for Cerebral Palsy and Developmental Medicine (1977), cerebral palsy refers to a nonprogressive CNS deficit. The lesion may be in a single or multiple locations of the brain, resulting in definite motor and possible sensory abnormalities. It occurs as a result of inutero factors, events at the time of labor and delivery (congenital CP) or a variety of factors in the early developing years (acquired CP). In addition to motor deficit, other associated disorders may be found.  The definition identifies two varieties of CP: Congenital (85%) Acquired (15%) Congenital: (birth to 3-6 months, others say strictly birth to 15 days) conditions in which etiology is traced to intrauterine, natal and perinatal factors. Very common cause in CP Acquired: caused in the post natal period (6 mths to 2 years) controversial CNS infections, intracranial hemorrhage due to vascular pathologies (primary and secondary), late onset hydrocephalus or inadequately treated hydrocephalus, trauma, neoplastic growths in brain, toxicity, infections such as meningitis, encephalitis etc.  Sometimes anoxia due to following may lead to acquired variety Strangulation High altitude Deep pressure Hypoglycemia (reduced blood sugar) Poisoning Genetic: some well documented clients of spastic paraplegia with autosomal dominant trait CP is a static motor deficit with definite motor nature of the problem which distinguishes this from other disorders such as MR syndromes, organic brain deficits, autism or emotional disorders Incidence of CP ASHA statistics: 1 to 2 in 1000 M:F = 1.75 or 2:1 Etiologies of CP I Congenital A] Intrauterine factors 1) Prematurity: high risk factor ( may or may be at high risk depending on GA and BW) Small for gestational age (SGA- age of the foetus) and birthweight(BW) In premature children, if BW is appropriate for GA, lesser risk, but if BW less and not appropriate for GA, then high risk for acquiring developmental disabilities including CP  2) Asphyxia / Respiratory distress syndrome 3) Intracranial haemorrhage (especially MCA) 4) Intrauterine malnutrition 5) Intrinsic developmental disability or abnormal development 6) Disproportionate CNS maturation 7) Maternal prenatal condition: general health, effects of drugs, alcohol, tobacco, radiation, environmental pollutants and chemicals, malnutrition of placenta, maternal infection (Rubella, toxoplasmosis, cytomegalovirus, herpes)  B] Perinatal factors 1] Neonatal asphyxia: due to Obstetrical accidents (breach presentation, precipitated delivery, dry delivery, pre term, post term, forceps delivery, caesarian delivery etc) Anaesthesia Umbilical cord around neck or other mechanical problems Intrinsic pulmonary deficits of the infant 2] Infection: Eg; sepsis (illness in which the body has a severe response to bacteria or other germs, symptoms not caused by the germs themselves. Instead, chemicals the body releases cause the response, meningitis 3] Intracranial haemorrhage during delivery  4] Hyperbilirubinemia (increased levels of bilirubin in the blood) : RH factor 5] Blood group incompatibility: ABO groups 6] Fixed motor lesions due to: CNS insult: hydrocephaly, anencephaly (absence of skull or large part of brain), encephalocele (bones of the skull do not close completely, creating a gap through which cerebral spinal fluid, brain tissue and the membrane that covers the brain (the meninges) can protrude into a sac-like formation) etc Other causes such as neurofibromatosis (nerve tissue grows tumors), syndromes  Classification of CP 1] Physiologic (Motor) A] By Denhoff Spasticity Athetosis: Tension, Nontension, Dystonia and Tremor Rigidity Ataxia Tremor Atonia Mixed Unclassified  B] By Hardy (i) Disorders of muscle tone: extent of tension maintained in muscle group Hypotonia → Atonia, Flaccidity Hypertonia →Spasticity, Rigidity and Tension (ii) Disorders of movement (kinesia) a) Hyperkinesia (UMN lesion and excessive involuntary movement) I Dyskinesia: Athetosis, Chorea, Choreoathetosis II General variety Dystonia Tremors Myoclonus and spasmodic disorders  b) Hypokinesia: LMN lesion (reduced movements, rare in CP) (iii) Disorders of coordination: Ataxia 2]Topographic Monoplegia (symptoms are present only in one limb) Hemiplegia (one side of the body is affected – usually spastic and occasionally athetoid) Paraplegia (involvement of legs only - spastic and rigid types usually)  Triplegia (involvement of three extremities, more often both legs and one arm (usually spastic) Quadriplegia / Tetraplegia (involvement of all four limbs where legs are most involved (usually spastic with greatest involvement in arms, usually dyskinetic including athetoid) Diplegia (involvement of like parts on either side of the body i.e. bilateral paralysis; both hands or both legs, arms are affected to a lesser degree than legs) Double hemiplegia (arms are more involved than legs. Usually spastic in type) 3] Etiological: Genetic, congenital, acquired etc  4] Neuroanatomical: Cortical, subcortical, UMN, LMN, central, peripheral etc. 5] Supplementary: (i) Psychological status: cognition, degree of MR etc (ii) Physical status: (assessed by a paediatrician) Physical growth evaluation- height, weight etc. Developmental level- in different doamins like sensory, sp-lang Bone age (By biopsy) Contractures (permanent fixed postures in body when spasticity /rigidity is not reduced through physiotherapy) (iii) Convulsions/ seizures Grandmal (tonic-clonic), petitmal (absence), focal etc  (iv) Posture and locomotive behavior pattern (v) Eye hand behavior pattern Eye dominance Eye movements Eye posture Fixation (eye gaze) Convergence Prehensory approach (simultaneous eye hand coordination exhibited on both sides) Grasp Manipulation Hand dominance (vi) Visual status a) Sensory: amblyopia (astigmatism-a type of squint where there is cross over of visual fields), field defects, myopia- (distance object not clear, near object clear)  b) Motor: Conjugate deviations Fixation defects Spasmus fixus (fixation deficit due to sudden spasm in extra and intraocular muscles) Strabismus fixus (fixation deficit due to squint) Esotropia (type of squint when eyeballs deviate towards midline) Exotropia (deviate away from midline) Hypertropia (deviate towards head) Hypotropia (deviate towards mouth)  Nystagmus (involuntary movements of eyeball) Pseudopalsy of external eye muscles (appears as palsy of eyeballs, severe spasticity in and around eyeballs , but not eyeballs) (vii) Auditory disorders (e.g. in athetoid due to kernicterus, the could be high frequency sloping SNHL) (viii) Speech / language and communication disorders  6] Functional capacity Class I: no practical limitation of capacity Class II: Mild to moderate limitation of capacity Class III: Moderate to severe limitation of capacity Class IV: Non ambulatory 7] Therapeutic classification Class A: Not requiring treatment Class B: Requires minimal therapy Class C: Requires team approach Class D: Requires long term institutionalization and treatment Motor development in typically developing children and in children with cerebral palsy Typically developing children Based on neural commands from motor areas, the structures of the body act-i.e. muscles, tendons and joints Various degrees of (a) flexion and extension (b) contraction (c) force physiology possible (precision, range, speed, force) CP children: lesion and topography of disorder affects the motor movement to various degrees difficulty to initiate movement total arrest of movements stereotyped movements involuntary movements incoordinated movements  abnormal posture patterns Contractures Definition of : A] Milestones-motor, sensory, social and adaptive, speech and language, visual, auditory etc. B] Critical period (embryogenesis and fetal developments) C] Sensitive period (periods when a child may learn particular skills more easily than others) Motor milestones 1] Gross motor development 2] Fine motor development  The functional system for speech is not present at birth. The neural functional system for speech is not in place until the end of second year of life Neural correlates of milestone acquisition: maturation of nervous system 1] Synaptic connections 2] Myelination (increase in thickness for faster conduction) 3] Neurotransmitters (quality and function change for faster conduction) 4] Cell morphology (increase in cell size and morphology) 5] Dendritic arborization (branching of dendrites- faster conduction)  Motor development/ control in normal children The direction of motor development is always proximodistal (arms first control-> fingers) and cephalocaudal (head control first->arms->legs) Initially arms are flexed/adductedabducted/extension Initially only reflexive motor activities/behaviour (primitive reflexes) which is later modified into voluntary activities (becoz cortex not mature in new born, movements controlled by lower centres. As maturation occurs, function taken over by brainstem, midbrain and then cortex by one to one and a half years) Lower order nervous system (age of onset and completion of neural correlates faster than higher order nervous system) Higher order nervous system  Jackson’s concept Evolution of the nervous system Dissolution of the nervous system Dr. Jackson regarded the CNS as an hierarchy in which each grade controls the grade below and is controlled by grade above Each centre in the lowest grade represents a limited portion of the organism and each centre in the highest grade represents the whole of the organism  Principle of evolution: Because the higher structures are immature and the lower structures are responsible for the control of motor actions and as and when the higher structures mature the function of the lower centers are controlled by the higher centers. Principle of dissolution: When an individual incurs damage to the cortical structures, the cortical structure loses its control over the lower structures and the function of the cortical structures are taken over by the lower structures.  Duplex’s symptomatology- explained the same concept as symptoms When disease affects the nervous system, there are two sets of manifestations, negative and positive: loss of function and excess of function The functions lost are those of the centers diseased The functions in excess are those of the centers subordinate to centres diseased Postive symptoms→gain of functions of the higher centers by the lower order nervous system Negative symptoms→loss of function of higher order nervous system  Reflexes Definition: stereotyped involuntary motor response to a stimulus which is mediated through the nervous system Types: 1] Superficial: from skin or cutaneous layer 2] Visceral: from visceral systems 3] Deep: from tendons, ligaments etc. [ Neuromuscular units-muscles (striated and unstriated), tendons (muscles to bones), ligaments (bones to bones)  Receptors 1] Exteroceptors (placed externally to body structure- skin, hair follicles etc) 2] Interoceptors (intestine, heart) 3] Proprioceptors (its relation to deep tendon reflexes- status of given joint structure) Reflex arcs/paths 1) Monosynaptic reflex arcs: Receptors/ Afferent/ Sensory→internuncial/integrating neurons→effector/ efferent/ motor 2) Multisynaptic reflex arcs  Reflexes are controlled at 4 levels – from the lowest level i.e. spinal cord, then pons and MO, then midbrain and then the cortex. i.e. internuncial neurons are located at each of these levels for different reflexes. Most primitive reflexes-controlled at the spinal cord level (crude level of functioning)  Types of reflexes: 1] Primitive reflexes: some reflexes appear during specific age range and disappear after a period, e.g., sucking, hand grasp etc. These reflexes following this pattern are called primitive reflexes 2] Postural reflexes: As a child matures there is coordination in the child’s movements. 2 types of development seen (a) Righting reactions (b) Equilibrium reactions Righting reactions- ability of the body to maintain or stick on to the axis of the body. Can be elicited in all positions Equilibrium reactions- ability to maintain equilibrium, i.e. balance the body against gravity Both these give postural stability to the child. Occurs because of increase in myelination of increasing maturation of CNS These two reactions if are appropriately developed gives rise to appropriate postures and perfect voluntary movements  Most “infant” reflexes do not last beyond the first year. Infant reflexes may not completely disappear. – May be inhibited by maturing CNS. – May be integrated into new movements. Primitive reflexes critical for human survival. Concept of pathological reflexes: Late appearance and late disappearance or persistence of primitive reflex beyond a specified age range –gives clue reg. possible neuromotor involvement Keen observation of reflexes necessary  -Reflexes can determine level of neurological maturation. – Reflexes are age-specific in normal, healthy infants – Severe deviations from normal time frame may indicate neurological immaturity or dysfunction. – minor deviation in reflexes-suspicion Each reflex has to be elicited in a particular manner- a particular pattern of movement is seen which is common across all normal children What happens in cerebral palsies? Postural reflexes disturbed in CP, because of damage to higher centers-movements are incoordinated, reflexive and crude, nonsegmental (mass reflex action without fine control in the movements  Babinski:till 4m S: Stroke bottom or lateral portion of foot R: Great toe turns downward Sl. Reflex Stimulus Response Normal No reaction Primary / primitive reflexes 1. a) Suckling Introduce a Sucking action of the lips and jaw Upto 3 b) Sucking finger into the month mouth s 2 Rooting Touch baby’s Head turns towards stimulus Upto 3 cheek month s 3 Cardinal a) Touch a) Lower lip of this corner lowers and Upto 2 points corner of tongue moves towards the point of month reaction mouth stimulation. When finger slides away s from the corner, head also turns in this direction and follows the finger b) Central b) Upper lip elevates, tongue moves portion of towards the point of stimulation. If upper lip is finger slides along the oronasal stimulated groove, the head extends upwards c) Central c) Lower lip is lowered and tongue moves portion of towards the point of stimulation. If lower lip is finger moves towards chin, the stimulated mandible is lowered and head flexes 4 Hand Stroke ulnar Automatic opening of hand Upto 2 openi border of palm months ng and little finger 5 Hand Hand grasp Press finger or other suitable Upto 3 grasp object into the palm from ulnar months side 6 Moro Baby in supine There is abduction and Upto 4 reflex position. Head extension of arms and fingers. months and shoulders are This reaction is followed by raised from the adduction of arms and fingers table and head is (as if in an attempt to embrace) dropped back suddenly 7 Startle Obtained by Elbow is flexed (not extended as Remains reflex sudden noise or in moro) and the hand remains by tapping the closed through sternum out life 8 Foot Press the sole of a Fingers curl and there is Upto 9 grasp foot behind the grasping response of feet months reflex toes 9 Placin Brush the anterior Child lifts the respective limb Upto 5 g aspect of foot or upto place / step onto table to 7 reflex hand against the months Palmar/HandGrasping:7- 11m MORO:4m  Plantar Grasp :till 9 m S: Touching the ball of foot R:Toes grasp 10 Primary Hold the baby Reciprocal action of Upto 5 walking or upright and tilt flexion and to 7 automatic him/her forward, extension of legs is months walk or with the sole of seen (which reflex foot pressing resembles walking) stepping against the table 11 Galant’s Stroke the back of Trunk flexes towards Upto 2 trunk the child (lateral to the side of the months incurvation the spine) stimulus 12 Automatic Child in supine Child pulls to sitting Upto 2 sitting position. Pressure is position from supine months placed on the thighs and the head is held in flexion 13 Flexor Child in supine Uncontrolled flexion Upto 2 withdrawal position. Head in response of months reflex midline, legs stimulated leg extended. Stimulate the sole of foot 14 Extensor Child in supine Uncontrolled Upto 2 thrust reflex position. Head in extension of months midline, one leg stimulated leg extended and Gallant’s trunk incurvation: till 2m 1 Crossed Child in supine Opposite leg Upto 2 5 extensio position. Head in adducts months n midline and legs internally, extended. Stimulate rotates and foot medial surface of plantar reflexes one leg by tapping (typical scissor position) II Postural reflexes 1 Tonic Put the child in The child will be unable to Upto 2 to 5 labyrinthi prone (face dorsiflex head, retract months ne reflex down) position shoulders, extend trunk, (a)Reactio arms and legs. That is, n to prone normal flexor tone is noted position in newborn (fetal position). Begins to disappear with development of extensor 2 Reaction Put the child in tone Upto 2 to 5 to supine supine (face months position up) position Extensor tone dominates when arms and legs are passively flexed. That is, development of extensor tone is seen for flexion in supine, or total extension (TLR)Tonic Labyrinthine: 5- 6m 2 Asymmetr Patient in supine Extension of arm Upto 3 to 4 ical tonic position. Head in and leg on face months (it neck midline and arms side (i.e., is a rare reflex and legs increase in reflex and (ATNR) extended. In this extensor tone) usually (Fencer’s position, turn the and flexion of arm pathologica reflex) head of the and leg on skull l) patient to one side (i.e. increase side in flexor tone). This resembles Fencer’s posture 3 Symmetric Position: patient in al quadruped position Tonic neck (on all fours) or on reflex the testers knees (STNR) (a) In the above Arms flex (or flexor It is a rare position, when tone dominates) reflex and the head is and legs extend (or usually ventroflexed extensor tone pathological dominates) (b)In the above Arms extend (or position, when extensor tone (ATNR)Asymmetric tonic neck reflex: 8-9m (STNR)Symmetric tonic neck reflex:5-6m 4 Positive Child is In first 2 to 4 months, Upto3 to 5 support held there is reflexive months ing vertically extension of legs and reflex under arms some weight bearing is and noted. bounced on balls of feet Between 5 to 7 months, extension of legs is followed by flexion and kicking responses. This becomes a more voluntary standing response from 6 months onwards 5 Negative Child is held Child sinks (astasia) Upto 3 to 5 supporti vertically months ng reflex under arms (in weight bearing position) Positive support:4m  III Secondary reflexes (righting and equilibrium) 1 Automatic (a)When the Head extends Newborn to 2 head/neck child is held atleast in line months righting supported under with the rest of reflex stomach the body Between 2 to 4 (b) When the Infant holds head months child is held in steadily sitting position Between 4 to 6 (c) When the Head returns to months child is held in midline to correct vertical position for body position under arms and tilted sideways 2 Landau Suspend the child 1st stage: extension 1-3 months reaction by holding him of head above face down under shoulder level his upper thorax 2nd stage: extension 3-6 months of head and thorax 3rd stage: extension 6-12 months of head, trunk, hips 3 Protective (a)Supine and prone: Lateral 6 months. / tilt patient on tilt board. curving of reactions Arms and legs head and (protectiv extended, tilt the thorax, e board to one side protective reactions reaction in appear in limbs response (b)Four point kneeling: accompany to a (i) Patient in trunk sudden quadruped position, reaction displacing tilt towards one side 7 to 12 force and Lateral months are in the curving of same head and direction thorax. There as the is abduction force) (extension) (ii) In the same position of arm and as above tilt the leg on raised board forward and side and backward protective (anterior/posterior) reaction on lowered side When tilted (c) Sitting: Arms will immediately 4 to 6 (i) Childs balance in move forward and the months sitting is suddenly child will prop on disturbed arms. This response forwards by will improve with age pushing child from back Arm will reach 8 to 12 (ii) Childs balance in backwards to break months sitting is suddenly the anticipated fall, disturbed with rotation of the backwards by trunk and forward pushing child from flexion of the legs. front This response usually is present before walking can be anticipated 6 to 8 (iii)Lateral: childs months balance in sitting is suddenly disturbed by pushing the child 18 months sideways Head and thorax curve. There is (d) (i) Kneel standing: abduction-extension of (ii) In the same Head and back flex position as above , and arms flex and tilt the patient extend backwards forward (iii)In the same Head and back position as above, extend and arms tilt the patient extend forward backwards 12 to 18 months (e) Standing: in Similar reactions as standing position, above are seen push the child depending on the forward, backward position and sideways Partial reactions (f) Parachute or Childs upper limbs by 4 to 5 protective and fingers abduct months. extension forward: and extend as if to Fully child is held by protect his head developed waist upended and parachute moved quickly reaction toward the table anticipated or floor by 9 months 4 Staggering a) Standing reactions (saving from (i) A sudden Hopping or 12 to 18 falling) push to left stepping months or right by sideways is seen holding the inorder to upper arm maintain equilibrium Hopping or stepping forward (ii) A sudden is seen inorder to push forward maintain by holding equilibrium the upper arm Hopping or stepping backwards to maintain equilibrium or (iii)A sudden dorsiflex the feet push (going on to backwards heels) backwards by holding by holding the the upper upper arm  Early signs of cerebral palsy 1) Delay and deviance in motor development Gross motor development Fine motor development Oral vegetative skills Speech motor skills 2) Postural control: Persistence of primitive reflexes poor postural reflex control 3) Associated and comorbid disorders 4) Feeding and related problems 5) Sleep and general behavior 6) Presence of syndromes 7) Secondary effects on development of other related skills such as communication, speech, social and adaptive etc  Type of motor disorders in cerebral palsy Spastic cerebral palsy Lesion → Pyramidal pathway (UMN) –which controls voluntary movements Motor characteristics 1) Increased tone – hypertone Hypertonia is caused by breakdown in Alpha-gamma loop. When extrafusal expand, intrafusal also expand. The limit to which they stretch is controlled by stretch receptors. If stretch overshoots or undershoots, inf passed on to GMN which further passes to AMN which will correct the stretch. Proper coordination by the GMN and AMN results in maintenance of normal tone. Further even if there is increased discharge by AMN, inhibition is brought about by AMN.  2) Exaggerated reflexes – hyperreflexia 3) Loss of voluntary control: inability to execute voluntary movements in an adequate manner. Antigravity muscles (flexors) are more affected, affecting agonist antagonist reciprocal activity (eg: lifting of hand) Hyperreflexia and reduced voluntary control-leads to abnormal postures and movements  Normally, muscles coordinate in pairs; when one group of muscles contract (tighten), the other group relaxes. Agonists contract and increase the tone of the muscles. Antagonists do the opposite. This allows free movement. When complications in brain-to-nerve-to-muscle communication occur, the balanced degree of muscle tension is disrupted. Muscles affected by spastic cerebral palsy become active together which effectively blocks coordinated movement. Thus, the muscles in spastic cerebral palsy patients are constantly stiff, or spastic.  In severe cases, Mass reflex action seen due to hyperreflexia– entire body acts like a mass In prone position- total flexion of the body (trunk curved, head fixed inwards, knee, foot and hip flexed, arms flexed kept close to chest) In supine position-total extension of the body, head and shoulder extends backwards, trunk and hips extended, foot, ankle & arms extended Postural abnormalities Voluntary movements-jerky and uncontrolled  ATNR seen (arm towards the side of face is extended) If increased spasticity in one of upper limb, then thumb in palm sign is seen and palm fisted. Arms flexed at elbow, wrist. Fingers flexed and fisted  5) Early signs Positive moro reflex (stiffening of body, excessive arching of body and excessive startling) Difficulty in feeding, swallowing regurgitation Drooling Vomiting-becoz of spasm in alimentary canal Fitful/irregular sleeping habits Excessive crying Hyperirritability Strabismus of eyes  6] Gait abnormalities (lower limb signs) Scissoring: Evident in standing position and in walking. This is characterized by: Thighs in adduction position Internal rotation of hips Crossing of legs Feet turned inwards Occurs because of increased spasticity in lower limbs. If not corrected and learns to walk, he/she has scissoring of gait If increased spasticity in extreme end of lower limbs, toe walking present. Toe walking (becoz of severe extension in  7] Topographic distribution: Spasticity can affect entire body or it may be restricted to certain areas and hence topographically depending on the involvement spastics may be divided into Monoplegics Di Para Hemi Tri Quadri Speech and language functions are intact when the upper part of the body is not affected. So in paraplegics, no sp & lang abnormalities Easy to recognize hemiplegia because of few signs: arms pressed against the body with the forearm bent at right angles to the upper arm and the hand is bent against forearm, fist clenched tightly with the finger pressed into the palm, toe walking & / or walk on outer part of the ball of the foot and toes &/or rotate leg inwardly and limited bending of knees  8] Overflow/synkinesia: body parts other than the one intended also participates 9] Emotional incontinence (uncontrolled crying, laughing →by UMN lesion) 10] Intellectual impairment (MR present…) 11] Visual signs (strabismus, nystagmus, field effects) 12] Epilepsy (Grandmal type of epilepsy) 13] communication impairment Speech deviation-spastic dysarthria with language delay/deviance  14] Response to passive movement (physiotherapist) Clasp knife phenomenon (increased tone in either flexion or extension followed by sudden relaxation) (by neurologist) When passive movement is induced in the limbs, initially the structure gives in or there is less resistance to passive movement induced but at certain angles increased resistance is offered to passive movement becoz of abnormally high discharge of extensor action in the joint concerned. But if passive resistance is continued the extensor reaction is broken up and the resistance decreases, and the limb can be moved to whatever angle required.  Clonus or ankle clonus phenomena(alternate involuntary muscular contraction and relaxation in rapid succession) (by neurologist) When passive movement is induced at the level of the foot and the foot is bent backwards towards the knees, there is resistance offered at certain angles. But if passive resistance is continued there will be sudden tremor like or reverberating movement of the ankle.  Rigidity Considered as extreme form of spasticity Extrapyramidal involvement Equal involvement of agonist and antagonist, all muscle spindles equally involved unlike spasticity Hypertone Normal or diminished reflexes No clonus, stretch reflex and involuntary movements Poor voluntary movements Response to passive movements (a) Lead pipe rigidity (b) Cogwheel rigidity  Cogwheel rigidity: On application of passive movement, the affected structure shows intermittent phases of resistance followed by relaxation in sequential manner. Since this mechanism resembles the mechanism of cogwheel, it is called as cogwheel rigidity. (Rigidity in which the muscles respond with cogwheel-like jerks to the use of force in bending the limb, as occurs in Parkinson's disease). Lead pipe rigidity: On application of passive movements, since the agonist and antagonist are equally involved, the affected structure does not yield to passive movements. Even if passive movements are continued, resistance persists. Limb is like a lead pipe. This is called lead pipe rigidity. Like in spastics, rigidity can be restricted to 1/2/3 limbs  Differentiating factors of rigidity and spasticity Rigidity Spasticity Equal involvement Antagonist more of agonist and affected than antagonist agonist Symptoms increase Symptoms with slow passive decrease with slow movements passive movements Symptoms Symptoms increase decrease with fast with fast passive passive movements movements  Atonia Lesion in LMN pathways / peripheral nerves - Hypotone or lack of tone - Failure of muscles to respond to volitional stimulation -Muscles lack turger or firmness of the normal relaxed muscles -Absence of reflexes or weak reflexes -Exists mostly as an outstanding initial symptom of any other form of CP -Atonia seen in children below 2 years of age. Exhibition of characteristics of another form of CP Speech deviation: Flaccid dysarthria along with language delay and deviations  Tension According to Hardy’s classification, it is another type of CP. It is not a permanent subtype of CP, but a temporary condition-symptoms last for a short duration or can be ‘shaken away’ by inducing passive movements Varies in severity according to the emotional state of the individual Usually associated with movement disorder, not with disorders of tone, where tension cannot be shaken away Assumed that EPS is involved, but neuroanatomical base not known  Hyperkinetic disorders Extrapyramidal system- Inhibitory/ Excitatory Hyperkinesia/Dyskinesia- Chorea, athetosis, choreoathetosis Quick/ slow Slow: Athetosis, chorea, dystonia Quick:Ballismus, tremor, myoclonus  Athetosis Movement disorder-involuntary movements Lesion: Basal ganglia /extrapyramidal Characteristics: 1] Tone: Fluctuating muscle tone-hyper to hypo and viceversa 2] Reflexes: Hyper and primitive. Reflex actions and subsequent postural abnormality similar to that of spastics 3] Involuntary movements: due to lack of inhibitory and excitatory influence on the pyramidal output. Twisting, writhing (snake like movements), worm like movements progressing from proximal to distal areas in wave like fashion Increase in stressful or excited conditions (true for all types of hyperkinesia), Disappear during sleep 4] Posture and movements: Arms drawn back or forearm held with palm downwards, the fingers are overextended and spread outwards. Head held back, neck stiffened, mouth open and tongue protruded with or without drooling, facial grimaces (all these may be seen in quadriplegia / upper limb diplegia) Speech characteristics depend on whether any involvement of speech apparatus 5] Sensory problems Kernicterus athetosis: due to Rh incompatibility, bile pigment deposits on basal ganglion Triad symptoms: High frequency sensorineural hearing loss, athetosis, difficulty of occular movements in horizontal and vertical direction 6] Cognition: not affected 7] Gait: writhing, lurching (staggering) and stumbling with overflow of arms when he moves 8] Topographic distribution (monoplegia to quadriplegia) 9] Early signs: similar to spastic  10] Denhoff-12 subtypes of athetosis- criticized for its practical applicability while diagnosing athetoid varieties of CP Rotary athetosis: slow, circular movements of the extremities Tremor athetosis: irregular and uneven involuntary movement characterized by flexion-extension, adduction-abduction like movements Dystonic athetosis: distorted positions held involuntarily for some seconds or minutes. May involve neck, trunk, arms and legs. Shudder athetosis: momentary tremor of mild to violent intensity  Flailing athetosis: violent thrashing of arms and legs from shoulder and hip joints. Usually, there is little involvement of hands, fingers, wrists and knees Tension athetosis: associated with hypertone state Nontension athetosis: associated with hypotone state Hemi athetosis: one half of the body Neck and arm athetosis- Athetoid characters seen in neck and arms  Deaf athetosis / Kernicterus athetosis: (a) Athetosis (b) High frequency SN hearing loss (c) limited vertical and horizontal eye movements Balance – release athetosis: sudden loss of balance without falling due to involuntary movements Emotional-release athetosis: increased signs and symptoms during emotional state Speech deviation: hyperkinetic dysarthria along with language delay and deviations  Dystonia Lesion: Extrapyramidal system - Considered extreme form of athetosis - Involuntary movements: typically characterized by involuntary contractions of muscles of torso, neck and face. The contractions increase relatively slowly in intensity, and result in grotesque posture due to extremely forceful contraction of the involved muscles before relaxation. Types: focal and nonfocal Speech deviation: Hyperkinetic dysarthria  Tremor Lesion: Extrapyramidal system Characterized by involuntary movement: back and forth, pendular movement of the extremities and or the head Types:  Physiological tremor/ normal tremor  Pathological tremor: - Tremor at rest/ akinetic tremor / static (basal ganglia lesion)-Parkinson disease - Tremor in action / kinetic tremor (cerebellar lesion) - Usually seen with other types of CP. Pure form is rare. If only tremor is seen, it is an indication for the onset of other diseases. - Speech deviation: Hyperkinetic dysarthria  Ballismus Lesion: subthalamic nucleus (Nucleus of Luys) -Involuntary movements: rapid, violent, flailing movements of the extremities -Hemiballismus: lesion in the contralateral side -Seen associated with dyskinesia or other types of CP - Speech deviation: Hyperkinetic dysarthria  Myoclonus and other spasmodic disorders Lesion: Extrapyramidal system -Involuntary movements: due to rapid, abrupt, twitching unsustained muscle contractions in large and small groups of muscles -Types: (a) Synchronous myoclonus: a number of muscle groups jerk or contract at the same time or one after the other (b) Asynchronous myoclonus: a number of muscle groups contract at different times - Muscles of the facial structures may be involved: Palatal myoclonus: rhythmic continued elevation of the palate, leads to resonance disorders Laryngeal myoclonus: rhythmic contraction of laryngeal muscles Pharyngeal myoclonus etc. May be associated with convulsive disorders etc Speech deviation: Hyperkinetic dysarthria  Chorea Lesion: Extrapyramidal system Involuntary movements: Choreic movements are described as quasi purposive. That is, these movements resemble movements of a high order (although they achieve no purpose). They resemble fragments of purposive movements following one another in a disorderly fashion. Eg:, the eyes may be rolled from one side to another, the head turning in the same direction. Tone: fluctuates between hyper and hypo Speech deviation: Hyperkinetic dysarthria  Sydenham’s chorea Chorea seen in children Also called as Saint Vitus dance. Definition: Sydenham’s chorea is a disorder of the nervous system occurring due to the toxic effect of acute rheumatism characterized chiefly by involuntary movements. Occurs in childhood and adolescence. Pathology: Macroscopic: edema and congestion Microscopic: more changes in corpus striatum, substantia nigra and subthalamic nucleus Cortical abnormalities also seen Abnormality is usually in the form of degeneration of ganglion cells Etiology Due to rheumatism Sometimes may occur after scarlet fever and diphtheria Incidence Between 5 to 20 years Females more affected than males. Ratio 3:1  Symptoms 1. Mode of onset: usually insidious. Child clumsy and drops things. Described as restless, fidgety, unable to keep still. Sometimes symptoms are abrupt and may be described as fright 2. Involuntary movements: choreic movements Upper limb movements are more conspicuous than lower limbs (dance like) Respiration: often jerky and irregular Choreic movements are intensified on voluntary effort and excitement. They disappear during sleep.  In mild cases speech not affected. In severe cases dysarthria In severe cases, swallowing and mastication may also be affected and hence they have to be artificially fed 3. Associated movements: when voluntary acts are performed, it is associated with other movements. Eg making a fist may be associated with tongue movements, lips etc 4. Voluntary movements: In initial stages not affected, but later stages muscle power affected 5. Hypotonia 6. Posture: Choreic posture of the hand- the thumb and fingers are hyperextended at the metacarpophalyngeal joints and wrist is flexed. This is due to hypotonia, resulting from loss of tone in the antagonistic muscles 7. Mental state: might exhibit emotional instability due to their condition. But intellectually above average. In severe cases, there may be a persistent state of excitement associated with insomnia- maniachal chorea 8. In most cases, chorea is due to rheumatism and hence cardiac abnormalities associated  Prognosis: Death is very rare. Most patients recover within 2 to 3 months. Interval between attacks one or two years Speech disorder: Hyperkinetic dysarthria (slow variety due to chorea) Dysarthria could be temporary and variable in terms of severity, depending on the severity of the condition. If there is recurrence of the condition, then dysarthria may also recur. Treatment Should be kept in bed till symptoms disappear Medication-chiefly sedatives Supportive therapies such as physiotherapy, occupational therapy and speech therapy may be required when the choreic symptoms are active  Hypokinesia Lesion: extrapyramidal Manifested as delay in initiation of movement, slow movements (Bradykinesia) and reduced associated movements such as swinging of arms while walking etc Speech deviation: Hypokinetic dysarthria No cerebral palsy is classified under hypokinesia  Moebius syndrome Characterized by lifetime facial paralysis Cannot smile or frown; seems like person has a mask on Cannot blink and have no lateral eye movement (Inability to move eyes from side to side), but they will be able to move them up and down Sixth and seventh cranial nerves are not fully developed, resulting in eye muscle and facial paralysis (blinking, lateral eye movements and facial expression affected) Incidence and etiology Rare disorder Recurrence risk is 2% Precise cause remains unknown Affects boys and girls equally Diagnosis made by clinical examination (No prenatal test or diagnostic test to detect the condition) Cranial nerve involvement Paralysis of bilateral facial nerves The sixth and seventh cranial nerves do not fully develop (paralysis and underdeveloped nerves – unilateral or bilateral paralysis)  Cranial nerves 3,4,5, 9, 10 and 12 may also be involved Table shows functions of cranial nerves that can be involved Cranial Functions nerve VI Side to side eye movement VII Facial expressions, taste, blinking III Eyelid and eyeball movement IV Eyeball movement V Chewing, facial sensations IX Taste, helps with swallowing X Conveys sensory and motor information to and from several organs in the chest and abdomen  Parents may notice the following in their baby because of cranial nerve involvement Poor sucking As children start to learn to speak, they may have some difficulty making sounds that involve using the lips and tongue, and making facial expressions , such as smiling  Symptoms Related to facial expression and function Excessive drooling and crossed eyes (Strabismus) may be present There can be deformities of tongue and jaw, some limbs, including club foot and webbed fingers. Low muscle tone particularly of upper body Lack of facial expression, inability to smile Feeding, swallowing and choking problems (more difficulty with solid foods)  Keeps head back to swallow Motor delays due to upper body weakness High palate Short or deformed tongue Limited movement of tongue Submucous cleft palate Teeth problems Hearing problems (due to fluid in ears) Speech difficulties (especially with closed mouth sounds and sounds involving the lips) Minor Mid-line anomalies like cleft lip and palate Developmental features Although they crawl and walk later, they eventually catch up. Speech problems respond to therapy, but may persist due to impaired mobility of tongue and lips. As children get older, lack of facial expression and inability to smile are the predominant features Associated problems Accompanied by Pierre Robin syndrome  Treatment Infants sometimes require special bottles or feeding tubes like Habermann feeder to maintain sufficient nutrition Haberman feeder: special feeding bottle/device invented by the mother of a little girl with Pierre Robin syndrome, nipple rewards even the slightest effort from the baby’s tongue or gums. If the baby cannot nurse at all, one can squeeze and release a limited volume of milk from the reservoir into the baby’s mouth. The holes are also enlarged and more in no. Strabismus is usually correctable with surgery  Benefits from PT/OT, speech therapy Limb, jaw, hand and foot deformities may be improved through surgery Nerve and muscle transfers to the corners of the mouth have been performed to provide and ability to smile Tracheostomy (tube in the windpipe) for severe breathing problems Symptoms doesn’t get worse with time. Symptomatic treatment  Dystonia musculorum deformans Rare dystonic disorder in childhood Dysarthria may be seen in later stage Spastic dysphonia/ Spasmodic dysphonia Type of focal dystonia, since physiology of muscular spasms is similar Linguo-facial-buccal dyskinesia / orofacial dyskinesia / Tardive dyskinesia Bizarre movements are limited to the mouth, face, jaw and tongue. There is grimacing, pursing of the mouth and lips and writhing of tongue-hence articulation is affected Motor signs usually develop after prolonged use of neuroleptic drugs (antipsychotic drugs)/ powerful tranquilizing drugs The common class of these are phenothiazenes. Drug induced dyskinesia associated with phenothiazine and related medication may even produce athetoid movements or dystonic movements  Levodopa induced dyskinesia: Levodopa is a traditional drug administered to Parkinsons subjects to overcome, rigidity and hypokinesia. Extensive use or overdosage of this drug can lead to dyskinesia Orofacial dyskinesia also occurs in elderly patients without drug use. A rare disorder that includes dyskinesia of the eyelids, face, tongue and other muscles is called Meige syndrome Many other varieties of dyskinesia are included in the spectrum of extrapyramidal disorders  Ataxia (disorder of coordination) Primary function of cerebellum: to bring about synergistic coordination (smooth synchronisation to achieve a particular target) of muscles and muscle groups. That is, coordination in agonist and antagonist groups of muscles. It receives sensory information through peduncles and controls the activity of the body in space (maintains equilibrium). It analyses and compares the incoming afferent information to make suitable adjustments to the target specification of the motor act to be executed. It also discharges inhibitory and excitatory influences through extrapyramidal system to final common pathway. It helps maintain muscle tone. Lesion to the cerebellum is characterized by asynergia or dysynergia. There is incoordination of volitional movement and volitionally maintained postures. Clinical signs usually appear on the same side (ipsilateral) of the body as the cerebellar lesion.  Ataxic movements are incoordinated movements characterized by what can be described as undershooting (hypometria) and overshooting (hypermetria) during the approach and attempt to accomplish the motor act Signs of cerebellar lesion: 1] Ataxia: Taxis (lack of taxia or ranking/ordering): The order of the motor acts are incoordinated. This leads to staggering gait and abnormal posture. Patient tries to compensate for this by standing and walking with feet wide apart i.e. broad based gait 2] Decomposition: patient will break a complex motor act into its components and execute the act movement by movement as if it were being performed by a robot  3] Dysmetria: inability to gauge the distance, speed and power of movement. There may be undershoot or overshoot of a motor act 4] Adiadochokinesis or dysdiadochokinesis: is the inability to perform rapid alternating movements. DDK is a most sensitive measure in ataxia. Neurologists test for alternating movements in the muscle groups Eg: successive pronation and supination of the hands, rapid opening and closing of fists, rapid tapping of the fingers. There will be awkwardness or clumsiness of alternate movements  5] Rebound phenomenon: also known as the loss of the check reflex. For eg, when a patient is flexing his or her elbow isometrically against a resistance. When the resistance is suddenly removed without warning, the patient's arm may swing up and even strike themselves. With an intact check reflex, the patient will check and activate the opposing  6] Hypotonia: decrease in resistance to passive movement, is seen in cerebellar dysfunction. The muscles are flabby and lack normal tone. 7] Tremor: Intension or kinetic tremor (tremor occurs only when movement occurs) 8] Nystagmus: oscillatory abnormalities of the pupil of the eyes. Nystagmus may be vertical, horizontal etc. 9] Muscle stretch reflex: normal or diminished. Pendular reflexes observed. Series of smooth to and fro movements of limbs before it comes to rest – like a pendulum (eg in knee jerk reflex, limb movements) Type of dysarthria: ataxic dysarthria  Sensation and its deficits Senses are classified into general and special. Special senses include hearing, vision, taste, smell and tactile sense. General consists of rest excluding the special senses. Multisensory functions are called higher sensations. They include recognition of form, size, texture, weight and 2 point discrimination. Receptors and what they mediate Exteroceptors: mediate sound, sight, smell and cutaneous sensation (touch, superficial pain, temperature, itching and tickling) Proprioceptors: mediate deep somatic sensations from receptors beneath the skin, in muscles ad joints and inner ear. These sensations include pressure, movement, vibration, position, deep pain and equilibrium. Interoceptors: include sensation from viscera (visceral pain, pressure and distension  Neuroanatomy: Those tracts related to body sensations are in the posterior column of spinal cord. Cranial nerve pathways supply the head, larynx, pharynx, tongue etc. Eg. Sensory innervation of speech mechanism Structure Cranial nerves Face V: pain, temperature, touch to face VII: proprioception to face Tongue V: touch to anterior 2/3rd of the tongue IX: touch to posterior 1/3rd of tongue Palate IX: sensory to soft palate Pharynx IX: sensory to lateral and posterior pharyngeal wall X: sensory to pharynx Larynx X: sensory to most of the laryngeal muscles  Tests for sensory abilities 2 point discrimination: Two simultaneous stimulation on both sides of the body or different areas of the same part with suitable stimuli. Inability to perceive the two points of stimulation is called as loss of 2 point discrimination. Suggests cortical lesion (parietal) Pain: A sharp edged stimuli placed on the organ/ area to be tested  Reduced sensation of pain: hypoalgesia  Increased sensation of pain: hyperalgesia  Loss of pain sensation: analgesia  Temperature: test for ability to sense hot or cold Loss of ability to recognize limb position (when eyes are closed) eg.agnosia Astereognosis: loss of recognition of form, shape etc. with eyes closed Loss of vibration sense: tested with a vibrating stimulus, E.g. use of tuning fork  Associated problems in cerebral palsy I. Sensory problems: More prevalent in CP - In spastic hemiplegia-reduced sensation in the affected limb (due to parietal lobe dysfunction - Disorders of stereognosis, 2 point discrimination, position sense, sharp dull discrimination, pain, light, touch and temperature sense, vibration sense, texture recognition etc. - Sensory defects may include right-left asymmetry, visual field neglect - severity of sensory impairments may not be related to motor deficits II. Sensory disorders A. Visual problems: -cortical blindness – pupillary reaction present but they may not respond to threatening gesture - Strabismus (squint): Esotropia and exotropia - Refractive errors-(a) myopia (short), (b) long sightedness and (c) astigmatism-unequal curvature of the refractive surface of the eye leading to diffused image on retina - Visual field defects-homonymous hemianopia (usually in hemiplegics)-left nasal and right temporal field defect or right nasal and left temporal field defect  occurs when there is hemianopic visual field loss on the same side of both eyes. Hemianopias occur because the right half of the brain has visual pathways for the left hemifield of both eyes, and the left half of the brain has visual pathways for the right hemifield of both eyes. Other peripheral and central visual defects may be noticed - Ocular defects such as nystagmus, congenital cataracts, optic atrophy, conjugate deviations (hypertone deviations occurring in both eyes), which are usually absent in sleep Right-dextroversion Left-Levoversion Up-Supraversion Down- Infraversion B. Auditory problems Any type of hearing impairment, especially SN loss is common. One exclusive variety associated with dyskinetic CP due to Kernicterus (triad symptoms) III. Cognitive dysfunctions Mental retardation-more in spastics than athetoids More extensive (multiple) the lesion, greater the intellectual impairment When CP is associated with multiple disabilities, there could be an intellectual impairment, Eg. i) Metabolic disorders such as Hurlers syndrome. Galactosemia etc. ii) Glandular dysfunctions such as cretinism, myxedema, pituitary gland errors etc. iii) progressive disorders such as destruction of myelin sheath, dystrophy etc. IV. Seizural disorders: seizural disorders (epilepsy) of all varieties is very common in CP Types may include: (1) General – Grandmal (intermittent and continuous), Petitmal and myoclonus (2) Focal-simple or complex (3) Neonatal (4) Febrile or afebrile More common in ‘difficult to thrive’ children or children with degenerative conditions, becoz they have weaker physical abilities becoz of poor eating habits, poor development of muscle strength, power etc. V. Emotional and behavioral disorders Common behavioral problems in CP include hyperactivity or overactivity (this should be discriminated from the symptoms of involuntary movement disorders in hyperkinetic disorders), attentional aberrations, impulsivity and distractability. In adults secondary behavioural dysfunctions could be due to other complications. Observations should also be made in stressful and unstressed situations VI. Pseudo degeneration: Due to reduced use of a structure which has neuromotor impairment, there is functional overlay. That is although the physical movements can be executed to a certain extent, the person will not be doing appropriate action. Reasons can be many such as overprotection by parents, lack of focused goal in therapy to tap the residual potential, lack of motivation and drive on the part of the child, psychological gain etc. Due to these there will be degeneration in functional capacity (which is actually pseudo) VII. Orthopedic problems: Common problems may include contractures, imbalance, instability, malalingment of bony framework etc. Test specifically for hand functions and co- ordination such as range of motion, strength, co-ordination and dexterity (for manipulative skills) VII. Perceptual problems Agnosia and allied disorders, CAPD etc. may coexist with CP IX. Speech, language and communication problems Depends on the type, severity and topography of different types of CP Team approach in the rehabilitation of cerebral palsy Team of professionals include: A. Medical team: - Paediatrician -General practitioners - Neurologist - Neurosurgeons - Orthopaedic surgeon B. Nonmedical team: - Speech-Language Pathologist - Occupational therapist - Physiotherapist - Special Educator - Social worker - Vocational placement officer  Others (depending on the associated problems) -Audiologist -Ophthalmologist -ENT -Clinical Psychologist Role of the professionals Medical a) General practitioners: Identification of early signs Appropriate referrals to different professionals Physical well being of the child in terms of b) Paediatrician: Diagnosis of the condition based on the examination of the physical status of the child Monitoring growth and development of the child Identify symptom cluster and classify the disorder (especially in syndrome conditions) c) Neurologist: Assess neurological status based on hard and soft neurological signs In gross deformities, check for hard neurological signs (lesion sites in brain and other structures) and reflexes, physiological nature of the disorder (tremor, dykinesia etc) Supplement finding by neuroimaging and lab investigations check for signs and symptoms In milder cases, check for subclinical manifestations and soft neurological signs (eg. Lack of fine motor control, memory lapses, hyperactivity, impulsiveness etc) Prescribe medication for control of disorder or alleviation of symptoms  d) Neurosurgeons: In conducting neurosurgical procedures when required e) Orthopaedic surgeon: - conduct surgery to rectify orthopaedic defects or reduce contractures. Some techniques include ligament sectioning, bone carving, positioning bones using internal prosthesis etc - these procedures improve postures and movement of the deformed structures - prescribe orthotic aids and devices with the help of orthotic specialists, physiotherapist (PT) and occupational therapist (OT) to improve posture and mobility of structures. Example of these aids include special shoes, splints, braces etc.  Non Medical: Speech Language Pathologist: To improve speech, language and communication behavior The improvement depends on the degree of the disorder and comorbid disorders principle is to improve physiological support for speech, overcome functional overlays and work within the individuals residual capacity Assess the speech and language functions thoroughly through identified methods In communication, work for nonverbal modes through AAC  Physiotherapist: continuous role – identify and intervene in the physical developmental stages of child. In adults, for post surgical consequences to improve/normalize tone, build muscle strength, reduce pathological/primitive reflexes use strategies and aims to exercise the disordered group of muscles and use assistive devices to improve the function of the given group of muscles Different types of exercises by various schools of thought are:  passive stretching  brushing  sensory stimulation  sensory motor exercises  vibration etc  They also use assistive devices to achieve their goals (eg. Adaptive furniture, adaptive tools (calipers, braces etc) All these are used to improve posture of the part of the body, improve mobility of the structure and coordination of the structure Other devices also used to facilitate motor movements such as parallel bars for walking, bolsters and tilt boards for balancing etc. Many approaches or schools of thought in physiotherapy (Eg Denvers, Roods, Bobath etc). Some of these are adaptable not only for improvement of general motor skills but also the oral motor abilities (Eg. Roods, Bobaths, Phelps etc). Some are exclusively for the improvement of general physical abilities.  Occupational therapist More or less the same as that of PT, but specific physical skills required for improving certain academic, vocational or social activity is focused on. Principle is to work on strengthening of perceptual skills strengthening of sensory motor skills strengthening of skills related to academic activities strengthening of skills related to related to vocational activities strengthening of skills related to related to daily living  Just like a PT works on improvement of tone, coordination, strength etc. but all related to the execution of the skill for which the individual is being trained, e.g., writing skill, throwing ball etc. They also prescribe aids and devices, Eg. For feeding, special angled spoons, specially deviced bowls, control of type of food etc  Special educator: Comes into picture when the CP child is in special school, semiintegrated setups and requires supportive services to do well in academic domains Focus on reading and writing skills Coordinate with other team members to prescribe class room requirements such as special furniture, writing devices, writing pens, adaptations in symbols and alphabet systems, establishment of figure ground discrimination, adaptation of visual defects, instruments etc.  Social worker To liaison between the disabled individual and the various authorities in the society such as school authorities, vocational/job placement officers or between family and peer groups or friends They counsel, orient or demonstrate to the selected group of people regarding the strengths and weaknesses of the given individual, or regarding the attention to be given or placement to be considered in schools or jobs etc.  Vocational placement officer Is a social worker in all respects but is concerned with placement of the individual in sheltered workshops, prevocational centers, or actual jobs in various setups Selects suitable job (less physical orientation) and works on adaptation/ modifications required in the setup or work schedule etc. Mediates between the employer, prospective employer, peer group, family etc.  Variation seen in speech and language problems CP is a nonprogressive disorder caused due to single etiology during prenatal/ postnatal period. The degree of insult can vary from mild to severe. It varies also in terms of the (1) type and no: of associated problems (2) mixture/combination of disorder (eg., Athetoid combined with spastic etc) (3) the distribution of symptoms in the body (topography). Given these factors speech-language acquisition in C.P children can be expressed in varying degrees.  In mild cases / clients with limited topographic distribution / clients with pure form of CP without any associated problem, it is possible that the language may be normal / slightly delayed with only speech impairment being predominant. On the other hand, in severe cases with the combined CP varieties / Quadriplegia with increased tone or more associated problems, language development will be severely retarded along with deviations in speech. When speech is impaired, since it is caused due to neurological insult, the speech disorder  Assessment of developmental dysarthria: is traditionally/ commonly carried out using perceptual methods. The other methods of evaluation of dysarthria includes 1)acoustic analysis of speech (2) kinematic analysis of speech (how speech articulators move) (3) physiological analysis of speech (muscle potentials). These are commonly adapted in adults Instrumental analysis: poor accessibility, practical problems, lack of norms, the cost involved in procuring instrument etc and hence perceptual evaluation is used as a common tool to describe the speech deviancies in CP children  Assessment of motor speech disorders in children Dys+arthria = dysarthria (Greek word) Dysarthria- all speech systems involved or unitary systems involved Assessment- 1] perceptual 2] objective – a] acoustic b] aerodynamic c] kinematic d] muscular Dysfunction in the neuromuscular control may be in the form of weakness of structures, slowness of movement, reduced or lack of coordination of structures because of spasticity, flaccidity or involuntary movements etc  Specific features: 1] immature speech system-changing picture with age-repeated assessments necessary 2] primitive and pathological reflexes – specifically in CP 3] specific clusters of symptoms in syndromic and metabolic conditions 4] implications on language development- delay and deviancies 5] nonprogressive lesions –in CP, but physiological effects due to neural maturation may be changing 6] presence of associated problems – requiring intensive therapeutic intervention and more likely for language to be delayed 7] reduced prelinguistic experiences and abilities 8] unlike adult dysarthria, mostly nonreversible conditions and permanent conditions 9] More localized lesions, specific oral involvement eg. Minimal motor dysfunction and isolated dysarthria. Generalized lesion-as in CP 10] idiosyncratic influences-family, environment, needs etc  Salient features in developmental dysarthria 1] Delayed onset of speech and language functions-severe impairment in verbal language. Comprehension and expression of language may be equally or differentially impaired-depending on the associated problems, severity of disorder, stimulation, early intervention and management techniques etc. 2] If speech develops – characterized by different types of dysarthria On perceptual dimension: spastic, flaccid, hyperkinetic, hypokinetic, ataxia and mixed In each type of dysarthria, specific clusters of 3] Oral motor characteristics: a] movement of articulators for nonspeech movements such as feeding and nonspeech functions b] poor on repetitive tasks (DDK) c] dominated by primitive reflexes d] controversy between vegetative and speech skills Specific errors of speech system Respiratory errors: Cortical respiratory errors (BS nuclei  poor speech breathing dynamics poor aerodynamics (subglottal pressure, intraoral pressure, mean airflow rate etc)-aeroview-instumental poor respiratory reservoir-spirometer- vital capacity poor rhythm of respiration interrupted respiration (stridors, spasms etc) Pathological patterns (reverse breathing/paradoxical breathing, belly breathing/ diaphragmatic breathing, clavicular)  Poor physiology of breathing (inspiratory expiratory cycles-volume, time, frequency, quality etc). In normal infants – quiet breathing- nasal. Predominantly abdominal – thoracic by 6-7 years of age. Inspiratory expiratory cycle: 1:1 to 1:6 –nasal symmetrical to oral asymmetrical – required for development of vocalization and speech  In CP, regression or delay in the development of respiratory cycles Infantile breathing pattern- shallow or irregular respiratory cycles, increased frequency, diaphragmatic or abdominal Pathological breathing pattern- due to spasms in the vocal folds or posterior tongue or velopharynx or trunk dystonia, palsy in thoracic muscles, improper standing and sitting postures or prolonged use of chest and stomach bands in some children etc  All these will have implication on phonation pattern Eg. Delay in acquisition of oral asymmetrical breathing pattern – difficulty in initiating phonation voluntarily Shallow breathing pattern - only 1 or 2 syllables per breath Spasms- inspiratory voice quality, forced voice, interrupted voicing, laryngeal stridor, sudden arrest of phonation, uncontrolled loudness, asynchrony of respiratory and laryngeal movements  Laryngeal errors Poor pitch control (high pitch, low pitch, fluctuating pitch etc) Poor loudness control (weak voice, abrupt changes in loudness) Poor quality (breathiness, spasms- adductor, abductor, monotonous, forced and throaty, tremulous, uncontrolled variations, intermittent dysphonia, breathy, strained and stangled etc)  Flaccid paralysis – breathy voice Spasms in intrinsic and extrinsic laryngeal muscles – hoarseness Adductor spasms – inability to phonate Abductor spasms –inability to phonate Variation in laryngeal muscle tension- shifts in pitch, intensity and quality Athetoid Spastic Ataxic Pitch Low High - Loudne Weak and Weak and - ss forced forced Quality Grunty and Breathy, Monotone, forced hypernas dysprosody al Prosody Fluctuating - Abnormal prosody  Resonance errors Inconsistent and incoordination in resonance control nasal air escape etc Difficulty in quick alternating movements of the velopharyngeal mechanism Closure adequate for back consonants and high back vowels but inadequate for front consonant and neutral vowels Better closure for voiceless consonants (due to overall pressure built up in the oral cavity) Dorsiflexed position of head – VPI open. Ventroflexed position-VPI closed  Articulation errors Delayed but like normal in terms of development of sounds Specific sound misarticulation are rare, generally all phonemes affected because of poor strength, precision of place and manner control, abnormal timing for coarticulation Alternating movements difficult (DDK-AMR Alternating motion rate /ppp../ and SMR- sequential motion rate /ptk…/) Asynchronous in movement Vowels generally more easy than consonants Tongue tip sounds more involved Voiceless affected more than voiced  Back consonants difficult compared to front consonants Fricatives and glides easier than nasals but difficult in any position of words Nasals easy in initial position than final Stops least difficult in medial but very difficult in initial and final position Distortions and omissions more than additions or substitutions Errors in spastic and flaccid more consistent compared to dyskinetics Temporal measures of sounds often  Articulatory errors directly proportionate to the number of articulators involved in the oral cavity, unilateral or bilateral involvement and severity of impairment Articulation more affected as the length of linguistic elements increase Discoordination in the movements of lips, jaw and tongue because of lack of independent control on these structures  Primitive oral reflexes such as snout reflex (orofacial reflex elicited by stimulating nasolabial furrow; Response: lip protrusion, wrinkling of nose), biting, sucking, jaw thrust etc. could interfere with articulation Focal varieties of dystonia, myoclonus etc in the oral structures can affect articulation Tongue moves as a whole in severe cases (tongue thrust, drooling and others)  Prosodic errors Specific in different types (eg. Spasmodic in spastic, monotonous in flaccid, staccato and scanned in ataxia etc) Stress, rhythm, intonation, juncture and pause, hyper and hypofunctional Slow rate Inappropriate rhythm Labored speech production Spasmodic and broken rhythm and prosody  Explosive and jerky Prolonged and drawling Reduced stress consonants Atypical intonation patterns Monotonous Inappropriate voice stoppages etc  Language development Depending on the type, distribution and associated problems Language delay in verbal and nonverbal CP (use of hand gestures, eye gaze etc.) Receptive and expressive language abilities – since there are no standard scales to test these in CP, can use general language scales, but make sure that non performance based items should only be evaluated Evaluation of neuromotor disorders in children Difficult to identify neuromotor disorders in young children since the abnormality due to NS insult appears only later as and when nerve pathways become more functional. Usually, in spasticity – 7 to 9 months, athetosis -