Cerebral Palsy “uasday, ecember 22,2029 696m Cerebral palsy (CP) is a diagnostic term used to describe a group of permanent disorders of movement and posture causing activity limitation that are attributed to nonprogressive disturbances in the developing fetal or infant brain. The motor disorders are often accompanied by pain cognitive disability - MR hip displacement behavioral disorders sleep disturbances visual impairment: Strabismus, nystagmus, and optic atrophy hearing impairment ETIOLOGY © antenatal factors causing abnormal brain development © 10% evidence of intrapartum asphyxia, Intrauterine exposure to maternal infection (chorioamnionitis, inflammation of placental membranes, umbilical cord inflammation. foul-smelling amniotic fluid. maternal sepsis. temperature > 38°C during labor ‘urinary tract infection) ‘© ‘The major lesions that contribute to CP in preterm infants are intracerebral hemorrhage and periventricular leukomalacia (PVL) © imultiple pregnancy-- twins 5-8 times and triplets 20-47 times greater SPASTIC Babinski sign may be present, the deep tendon reflexes are increased. and wealmess of the hand and foot dorsiflexors Hemiplegia _-Difficulty in hand manipulation is obvious by lyr - Walking at 18-24 mo ~ Cireumduetive git ~ Equinovarus walls on tiptoe because of the increased tone in the antigravity guetrecmemsur muscles Diplegia——- commande aawi ~ unable to sit-parsepinal muscle mvelvement “brisk reflexes, ankle clonue, anda bilateral Babine sign Scissor Gait & Equinovarus Disproportionate growth b/n upper and lower extremitiesQuadziplegia sntellectual disability and seizures ~supranuclear bulbar palsies- Swallowing dificultes sspiration pneumonia and growth fidlure plantar extensor responses and Flexion contractures DYSKINETIC/ATHETOID CP * Basal ganglia and Thalamus, extrapyramidal ‘* hypotonic with poor head control and marked head lag and develop variably increased tone with rigidity and dystonia * Unlike spastic diplegia. the upper extremities are generally more affected than the lower extremities * Feeding may be difficult. and tongue thrust and drooling may be prominent. Speech is absent or sentences, are slurred, and voice modulation is impaired due to the oropharyngeal muscles involvement DIAGNOSIS ‘© MRI scan of the brain is indicated to determine the location and extent of structural lesions or associated congenital malformations: Tests of hearing and visual function * Genetic evaluation- for congenital malformations (chromosomes) or evidence of metabolic disorders Tests to detect inherited thrombophilic disorders TREATMENT © cooling term infants with hypoxic-ischemic encephalopathy to 33.3°C for 3 days, starting within 6 hr of birth. reduces, the risk of the dyskinetic or spastic quadriplegia form of CP © a team of physicians is important to reduce abnormalities of movement and tone and to optimize normal psychomotor development including - neurodevelopmental & neurologists pediatric ~ physical medicine and rehabilitation specialists. as well as - occupational therapists, social workers ~ speech pathologists and developmental psychologists. * Exercises designed to prevent the development of contractures. especially a tight Achilles tendon. © Adaptive equipment, suchas orthoses, walkers. poles. and standing frames for spastic diplegia © Surgical soft-tissue procedures adductor tenotomy or psoas transfer and release- hip girdle rhizotomy procedure in which the roots of the spinal nerves are divided produces considerable improvement in selected patients with severe spastic diplegia and little or no basal ganglia involvementtenotomy of the Achilles tendon © constraint induced movement therapy for hemiplegic CP © Deep brain stimulation has been used in selected refractory patients MEDICATIONS. FOR SPATICITY © Botulinum toxin injected into specific muscle groups for the management of spasticity. NB- can even be injected into salivary glands to reduce the severity of drooling © oral diazepam (0.01-0.3 mg/kg/ day. divided bid or gid) * baclofen (0.2-2 mg/kg/day. divided bid or tid) © Intrathecal baclofen delivered with an implanted pump- directly delivers to the spinal cord where it reduces neurotransmission of afferent nerve fibers and reduce CNS SE caused by PO meds © dantrolene (0 5-10 mg/kg/day. bid) DYSTONIA or DOPA responsive dystonia. © levodopa (0.5-2 mg/kg/day) © Artane (trihexyphenidyl, 0.25 mg/day. divided bid/tid and titrated upward) Can be useful for treating dystonia and can increase the use of the upper extremities and vocalizations ATHETOSIS or CHOREA © Reserpine (0.01-0.02 mg/kg/day. divided bid to a maximum of 0.25 mg daily) or © Tetrabenazine (12.5- 25.0 mg divided bid or tid) MOTOR SYNDROME (APPROX. % OF CP) NEUROPATHOLOGY/MRI MAJOR CAUSES Spastic diplegia (35%) Periventricular leukomalacia Prematurity Ischemia Infection Endocrine/metabolic (e.g.. thyroid) Spastic quadriplegia (20%) Periventricular leukomalacia, Ischemia infection Endocrine/metabolic genetic/ developmental Hemiplegia (25%) Stroke: in utero orneonatal _Thrombophilic disorders Infection Genetic/ developmental (athetoid dyskinetic) (15%) Asphysxia: symmetric scars in putamen and thalamus Kernicterus Mitochondrial Genetic/ metabolic