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Cerebral Palsy

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Question
Answer
CP definition   show
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show The concept that the brain has a limited number of ways of expressing itself (phenotype) when something goes wrong  
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Common neurologic phenotypes   show
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Neuromotor Disorders   show
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show 1. MR 2. Epilepsy 3. Behavioral changes 4. Movement disorders  
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CP Epidemiology   show
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show 86/1000  
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Cases of CP in term infants   show
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Incidence stability of CP...   show
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show Spastic quadraparesis (6%), Spastic diplegia (44%), Spastic monoplegia/hemiplagia (33%), ataxic/dyskinetic (12%)  
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show Only type associated with asphyxia in term infants (6%)  
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show Most common in premies, affects just the legs (44%)  
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show A single limb is affected, not associated with asphyxia (33%)  
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show more discordinated movements, around 26 weeks HEI  
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CP Occurrence of insult   show
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show Can identify etiology 75% of time (10% prenatal, 605 perinatal/neonatal, 30% not clear)  
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show Can identify etiology 80% of time (50% prenatal, 35% perinatal/neonatal, 15% not clear)  
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show 70% of time, with only 20% being "medically preventable"  
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show 30% of the time, with only 6-10% being "medically preventable"  
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What percentage of CP is "socially preventable"? (ie. FAS)   show
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show The brain, cerebellum, is particularly vulnerable in extremely premature infants (under 28 weeks)  
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show 23-24 weeks  
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show "Pancake cerebellum" no periventricular white damage changes  
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show -A developmental disruption, -<1000g <26 weeks -Enlargement of posterior ventricles  
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Possible causes of CP   show
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HIE   show
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show 1. Perniatal asphyxia 2. Neonatal neurologic syndrome 3. Multiorgan system dysfunction  
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Perinatal Asphyxia   show
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Asphyxia can occur...   show
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show its occurrence is a sine qua non for attitributing subsequent brain injury to intrapartum events  
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show 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  
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show 1. Stupor or coma 2. Respiratory arrest 3. Brainstem oculomotor and pupillary disturbances 4. Catastrophic deterioration with intraventricular hemorrhage (premature)  
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show 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))  
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show Not subtle; indicative of recent (itrapartum) insult' prenatal insults may also have occurred; absence rules out intrapartum insult capable of causing major brain injury  
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show Renal (ATN), Hepatic (elevated LFTs), Cardiac (elevated MB fraction of CK), Pulmonary  
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Three features necessary to consider intrapartum insult as a possible cause of neonatal brain injury...   show
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show already compromised fetus  
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Sotos syndrome: brain changes   show
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Sotos syndrome: Secondary CNS changes   show
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show Rett Syndrome, ARX mutations, inverted dup 7q (Williams)  
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show Normal, pedigrees ~XLR, skewed X-inactivation, learning disabilities, MR, non-progressive encephalopathy with spasticity  
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Rett Syndrome: Expanded phenotype/ males   show
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Recently recognized conditions that mimic HIE   show
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show 1. Hydrocephalus 2. Lessencephaly (XLAG) 3. ACC 4. West syndrome 5. MR with epilepsy 6. Mr with dystonia 7. Not, isolated MR  
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Individuals with neurologic disorders and pigmentary abnormalities should have a...   show
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show 50  
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Increased incidence of what in children with CP   show
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show 1. Fetal malformations, 2. Maternal MR, 3. Low Birth weight  
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show 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  
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show 1. Serum 2. Urine  
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show 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  
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Coagulopathies: Clear relationship between hemiplegic CP and...   show
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Full coagulopathy workup if neuroimagining indications of   show
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Associated with increased incidence of adverse pregnancy outcomes...   show
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show both parents and the child!  
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Etiology of CP: Trauma   show
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show Ie. Spinocerebellar ataxia X-linked OPCA  
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Etiology of CP: CP genes   show
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show 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  
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Proposed work-up for CP: (3 tiers) Second...   show
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show *Done infrequently ... unknown yield 1. ARX gene testing (particularly with infantile spasms or dystonia), Spinal Tap, Advanced metabolic testing  
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show Phenotype!  
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Do not dx a disorder based on...   show
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