Cerebral Palsy
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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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Created by:
KChatham
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