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Cerebral Palsy

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CP definition   A clinical set of static encephalopathies linked by their expression of variable disabilities of movement/posture (*Not progressive)  
Final Common neurologic pathways   The concept that the brain has a limited number of ways of expressing itself (phenotype) when something goes wrong  
Common neurologic phenotypes   1. Disorders of mentation 2. Eplileptiform/paroxysmal, 3. Neurobehavioral 4. Neuromotor  
Neuromotor Disorders   1. Cortical 2. Basal ganglia 3. Cerebellum 4. Long tracts 5. Anterior horn cells 5. Spinal cord 6. Peripheral nerves 7. muscles  
CP Co-Morbidity   1. MR 2. Epilepsy 3. Behavioral changes 4. Movement disorders  
CP Epidemiology   Uncommon, prevalence 2/1000  
Cases of CP in extreme prematurity   86/1000  
Cases of CP in term infants   1.2/1000  
Incidence stability of CP...   has not decreased despite decades of improvement in prenatal/perinatal care  
Cerebral Palsy Classification   Spastic quadraparesis (6%), Spastic diplegia (44%), Spastic monoplegia/hemiplagia (33%), ataxic/dyskinetic (12%)  
Spastic quadraparesis   Only type associated with asphyxia in term infants (6%)  
Spastic diplegia   Most common in premies, affects just the legs (44%)  
Spastic monoplegia/hemiplegia   A single limb is affected, not associated with asphyxia (33%)  
Ataxic/dyskinetic   more discordinated movements, around 26 weeks HEI  
CP Occurrence of insult   20% prenatal, 35% prenatal and perinatal, 35% perinatal, 10% postnatal *Can acquire CP at any point in life  
CP Etiology: Premature infants   Can identify etiology 75% of time (10% prenatal, 605 perinatal/neonatal, 30% not clear)  
CP Etiology: Term infants   Can identify etiology 80% of time (50% prenatal, 35% perinatal/neonatal, 15% not clear)  
CP is due to prematurity...   70% of time, with only 20% being "medically preventable"  
CP is found in term births...   30% of the time, with only 6-10% being "medically preventable"  
What percentage of CP is "socially preventable"? (ie. FAS)   4  
Why is the pattern of CP seen in premature infants notably different than that seen in term babies?   The brain, cerebellum, is particularly vulnerable in extremely premature infants (under 28 weeks)  
Premature viability is now seen at...   23-24 weeks  
Cerebellar anomalies...   "Pancake cerebellum" no periventricular white damage changes  
Colpocephaly   -A developmental disruption, -<1000g <26 weeks -Enlargement of posterior ventricles  
Possible causes of CP   1. Hypoxic-ischemic encephalopathy 2. Genetic syndromes 3. Chromosome anomalies 4. MCA without unifying diagnosis 5. Cerebral dysgenesis 6. Teratogenic 7. Metabolic disorders 8. Coagulopathies 9. Trauma 10. Slowly progressive neuromotor disorders  
HIE   Hypoxic-Ischemic Encephalopathy - disturbed brain function secondary to decreased oxygen or blood supply.  
HIE must be associated with all 3 of the following perinatal events:   1. Perniatal asphyxia 2. Neonatal neurologic syndrome 3. Multiorgan system dysfunction  
Perinatal Asphyxia   1. Defined as decreased APGAR's, fetal acidosis or both 2. Occurs in only 20% of all cases of CP (motor dysfunctions)  
Asphyxia can occur...   as a secondary result of an already compromised fetus  
Neonatal Neurologic Syndrome   its occurrence is a sine qua non for attitributing subsequent brain injury to intrapartum events  
Neonatal neurologic syndrome (birth to 12 hours)   1. Depressed level of consciousness (usually deep stupor or coma) 2. Ventilatory dependence (periodic breathing) 3. Intact pupullary responses 4. Intact oculomotor responses 5. Hypotonia with minimal movement 6. Seizures  
NNS (24-72 hours)   1. Stupor or coma 2. Respiratory arrest 3. Brainstem oculomotor and pupillary disturbances 4. Catastrophic deterioration with intraventricular hemorrhage (premature)  
NNS >72 hours   Persistent, yet dimished stupor; disturbed sucking, swallowing, gag, and tongue movements; hypotonia> hypertonia; weakness (proximal limbs> lower (full term); hemiparesis (full term); Lower limbs (Premature))  
NNS, when associated with clinically significant encephalopathy...   Not subtle; indicative of recent (itrapartum) insult' prenatal insults may also have occurred; absence rules out intrapartum insult capable of causing major brain injury  
Multi-organ system dysfunction   Renal (ATN), Hepatic (elevated LFTs), Cardiac (elevated MB fraction of CK), Pulmonary  
Three features necessary to consider intrapartum insult as a possible cause of neonatal brain injury...   1. Evidence of fetal distress 2. Depression at birth 3. Neonatal Neurologic syndrome  
Asphyxia can occur as a secondary result of an ...   already compromised fetus  
Sotos syndrome: brain changes   Macrocrania without megalencephaly - Increased sizes of fluid filled spaces in the brain - subtle markers of cerebral dysgenesis - hypoplasia of the cerebellar vermis  
Sotos syndrome: Secondary CNS changes   -Higher incidence of periventricular leukomalacia, (white matter); neonates with sotos at higher risk for brain injury (large infants, macrocephaly, hypotonia)  
Etiology of CP   Rett Syndrome, ARX mutations, inverted dup 7q (Williams)  
Rett Syndrome: Expanded phenotype/females   Normal, pedigrees ~XLR, skewed X-inactivation, learning disabilities, MR, non-progressive encephalopathy with spasticity  
Rett Syndrome: Expanded phenotype/ males   Progressive encephalopathy, spasticity and acquired microcephaly; non-progressive (static) encephalopathy  
Recently recognized conditions that mimic HIE   ARX mutations (esp. if infantile spasms) gene sequencing  
ARX mutations... phenotype spectrum   1. Hydrocephalus 2. Lessencephaly (XLAG) 3. ACC 4. West syndrome 5. MR with epilepsy 6. Mr with dystonia 7. Not, isolated MR  
Individuals with neurologic disorders and pigmentary abnormalities should have a...   fibroblast karyotype if the lymphocytic karyotype is normal  
Minor malformations are common in what % of CP...   50  
Increased incidence of what in children with CP   minor malformations  
Major predictors of CP   1. Fetal malformations, 2. Maternal MR, 3. Low Birth weight  
Subtle markers of Cerebral Dysgenesis   1. Hypogenesis of the corpus callosum 2. Mega cisterna magna 3. Wide septum pellucidum 4. Persistence of the cavum septum pellucidum 5. Open operculum 6. Mega cerebellum 7. Focal cortical dysplasia  
Standard metabolic screening   1. Serum 2. Urine  
Etiology of CP: Coagulopathies...Genetic "Clot Panel"   Common: Factor V Leiden, Prothrombin (factor 2), hperhomocysteinemia, MTHFR Rare: Protein S or C deficiency, Abnormal antithrombin III, Dysfibrinoginemia... May account for 70-80% of all pathologic thrombi  
Coagulopathies: Clear relationship between hemiplegic CP and...   Factor V Leiden  
Full coagulopathy workup if neuroimagining indications of   vascular disruption  
Associated with increased incidence of adverse pregnancy outcomes...   1. Miscarriage 2. Stillborn 3. Congenital anomalies 4. CP (?)  
For coagulopathies, test...   both parents and the child!  
Etiology of CP: Trauma   1. Shaken infant 2. Spinal cord injury  
Etiology of CP: Progressive neuromuscular disease   Ie. Spinocerebellar ataxia X-linked OPCA  
Etiology of CP: CP genes   Several CP Genes recently identified by linkage but there is currently no clinical testing available  
Proposed work-up for CP: (3 tiers) First...   1. Neurologic exam 2. Ophthalmologic/neuro-ophthalmologic exam 3. Family hx highlighting neurobehavioral and vascular questions 4. pregnany hx 5. dysmorphology eval (w. woods lamp) 6. brain mri 7. placental pathology  
Proposed work-up for CP: (3 tiers) Second...   1. Karyotype 2. CGH 3. MECP-2 gene testing 4. Coagulopathy panel 5. Metabolic screening 6. Infectious titers **First two tiers give an 80% diagnostic yield***  
Proposed work-up for CP (3 tiers) Third...   *Done infrequently ... unknown yield 1. ARX gene testing (particularly with infantile spasms or dystonia), Spinal Tap, Advanced metabolic testing  
Diagnosis a disorder based on...   Phenotype!  
Do not dx a disorder based on...   Genotype!  


   





 
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Created by: Kali Chatham Kali Chatham on 2010-04-26



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