Cerebral Palsy Margie Ream MD, PhD Assistant Professor, Pediatric Neurology Objectives: stuff to know about cerebral palsy Definition, incidence and timing of insult Diagnosis, exam findings Classification of CP Differential Diagnosis and comorbidities Treatment Prognosis Quiz Cerebral Palsy - definition “Non-progressive disorder of posture or movement caused by a lesion in the developing brain.” The diagnosis requires three conditions: 1) 2) 3) Motor difficulty Not progressive Injury to the immature brain (prenatal up to ≈2 yr) Notice that the definition does not comment on intellectual ability. Incidence and prevalence 2-5/1,000 births At 12 months old 5/1000 At 7 years old 2/1000 Some kids “grow out of it” and some kids don’t get diagnosed until 2-3 years old Timing of insult leading to CP Term babies Preterm Examples Prenatal 24% 6% Maternal and placental factors Perinatal 20% 50% HIE, trauma, cerebral hemorrhage, hyperbili Postnatal 8% 2% combination 20% 23% No known cause 30% 17% <32 weeks, <2500g CNS infection, accident, abuse Taft 1995 Diagnosis Delayed motor development. May present as “floppy baby” and later develop increase tone. Increased tendon reflexes sustained ankle clonus – always abnormal any ankle clonus after the first few months increased sensory input zone for reflexes (percuss over deltoid and get biceps) cross adduction at the patellar reflexes. Normal Developmental Milestones Age Gross Motor Fine Motor & Vision Hearing and speech Social behavior Turn head & eye Sleep most of the time. Primitive reflex, marked towards light. Startled by sudden loud Stop crying when picked 1 mth head lag. Watches mom's face. noises. up/spoken to. Head & body in same Hands mostly open. 2 mth horizontal plane Grasp reflex weak. Vocalizes when spoke to.Smile readily (6 wk) Watches own hands. Respond with pleasure Minimal head lag. Supine - Clasps & unclasps Turn to soft sound at ear to friendly handling. 3 mth - lies with head in midline. hands. level. Squeals in delight. Hold rattle momentarily. Hands come together Spontaneous vocalizing No head lag. Rolls from in midline. Reaches to self, toy and people. Initiates social contact 4 mth front to back. towards object. Laugh. with smile. Reach with both Turn to voice and Grasp feet and put toes hands to grasp babbling, tuneful. Laugh in mouth. Smile at mirror 5 mth Rolls from back to front. object. & squeal in play & vocalizes. Sit with support. Prone - Palmar grasp. lift chest, extend arms. Transfer objects. Respond to name. Turn Mouthing. Still friendly Take weight on legs when Follow adult toward sound at 40-50 with strangers. Laugh, 6 mth standing. movements. cm at ear level. squeal and chuckle. Look for toys. Picks Localize sound at 1 m Look for object fallen out Sit without support. up object but can't above and below ear of sight. Stranger Crawling, cruising (10 place down. Pincer level. Say mama and anxiety. Waves bye bye 9 mth mth). grasp. papa. and peek a boo Point with index Drinks from a cup. Put Walk with 1 hand held. finger. Pincer grasp. Undestand simple blocks in and out of cup. Can rise from lying to Repeatedly throw commands. Say 2-3 Knows and turns to own 12 mth sitting. object. words with meaning. name. Exam findings Asymmetri c tonic neck reflex. Taft, 1995 Persistent primitive reflexes: Asymmetric tonic neck – should never be obligatory (held >30 sec) and should disappear by 6 months. Crossed extensor reflex – stimulate foot of extended leg. Contralateral leg will flex then extend and adduct. Normal up to 4 months. Crossed extensor reflex. Intranet.tdmu.edu Positive supporting reaction – baby looks like he is stepping, should disappear by 4 months. Moro should disappear by 6 months. Moro reflex. library.med. utah.edu/ Stepping reflex. Artship.org Classification of CP Spastic (70-80%) Diplegia (legs) - #1 Hemiparesis Quadriparesis Dyskinetic (10-15%) Athetosis, chorea, ballismus, tremor Ataxic (1%) Mixed (10-15%) Rigid (5%) Di = 2 Hemi = half Quad = four Paresis = weak Plegia = paralyzed Spastic Diplegia Most common form. Affects legs >>> arms. Delayed sitting and crawling. May prefer to “army crawl” Leg scissoring with increased tone, increased reflexes in legs. May not be obvious until 1 year old Risk of hip subluxation or dislocation Studyblue.com Spastic diplegia video Upper body strength/coordination far exceeds that in her lower body. www.youtube.com/watch?v=mHXNjZS74s Spastic diplegia video Child with spastic diplegia undergoing gait analysis using a walker. Notice toe walking. www.youtube.com/watch?v=TP37l54UqTE Spastic Diplegia Common cause is IVH in premature babies leading to periventricular leukomalacia affecting the motor tracks for legs. Spastic Quadriplegia All limbs affected, legs > arms Double hemiplegia if arms>legs Hypotonia early after injury Profound motor delay Dysphagia/dysarthria common Seizures in 50% Abc.net.au Hemiplegic Cerebral Palsy Affects one side of the body, arm>leg. First noticed around 4 months as hand fisting and contralateral hand preference. Increased tone and reflexes noted by 15 months tight elbow flexors, wrist pronators, gastrocnemius Causes: Stroke IVH Cerebral malformations Physio-pedia.com Hemiplegic CP video Listen to narration then turn down volume and play video – we weren’t able to remove the sound. Notice the upper motor neuron pattern of weakness in which she keeps right arm flexed and has decreased arm swing. Also she doesn’t flex at the hip as much on the right when walking. https://www.youtube.com/watch?v=3w2IhGqIPAU Less common forms of CP Athetoid dystonic CP: Prior to Rhogam was caused by hyperbilirubinemia/kernicterus. Basal ganglia and auditory centers are sensitive to bilirubin toxicity. Often comorbid hearing loss. Typical course was initial extensor posturing/opisthotonis, then hypotonia with obligatory tonic neck reflex. Athetosis and dystonia at 12-18 months, then rigidity by 2 yrs. Now most commonly caused by hypoxiaischemia in basal ganglia (HIE) Quizlet.com Athetoid CP video Athetoid movements are slow and writhing, usually distal. https://www.youtube.com/watch?v=NXuwX7UBOlc Less common forms of CP Ataxic-spastic CP Low tone with normal DTR, then develop wide based support and hyperreflexia. Can be due to hydrocephalus. Ataxia can represent early signs of metabolic disorder so watch for progression and be suspicious. Differential diagnosis of CP Comorbidities of CP Intellectual disability in 50-60% Seizures in up to 1/2 Vision, hearing, sensory impairments Feeding/secretion difficulty Constipation, incontinence Pain and dislocation from contractures Osteopenia Communication difficulties even with normal IQ Spastic bladder Depression Skin breakdown Treatment of Spasticity Medical management Oral meds (Baclofen, Benzodiazepines) Botulinum toxin injections Baclofen pumps Surgical management Dorsal rhizotomy (cut dorsal root from L1-S2) Bracing or osteotomy for hip dislocation Tendon release Rch.org.au Treatment of comorbidities Monitor feeding difficulty and airway protection Consider g-tube in some patients Assistive communication devices Antiepileptics if needed PT, OT, ST Educational and social support Uakron.edu Prognosis of CP It is difficult to give prognosis, especially based on <12 mo exam 20-30% of children with CP at 12 months outgrow the diagnosis. Some deficits may not be apparent until 2-3 years old. Good prognosis for ambulation if sitting independently by 1.5-2 years If can sit by 2-4 years, most can walk. Not sitting by 4 years no ambulation later. References Taft, Lawrence. Cerebral Palsy. Pediatrics in Review. 1995. Krigger, Karen. Cerebral Palsy: An Overview. American Family Physician. 2006. Clinical Pediatric Neurology: A Signs and Symptoms Approach, Gerald Fenichel eMedicine Medscape Bailer and White. Nature Reviews Drug Discovery 9, 68-82 (January 2010) Thank you! For questions please contact Dr. Ream at Margie.Ream@nationwidechildrens.org Survey We would appreciate your feedback on this module. Click on the button below to complete a brief survey. Your responses and comments will be shared with the module’s author, the LSI EdTech team, and LSI curriculum leaders. We will use your feedback to improve future versions of the module. 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