CEREBRAL PALSY AN OVERVIEW BY: RISHAN JEYAKUMAR MS-4 CARIBBEAN MEDICAL UNIVERSITY SOM 09/2020
CEREBRAL PALSY (CP) Most common physical disability of childhood. It is a group of permanent disorders of the development of movement and posture attributed to non-progressive injury to the fetal or infant brain. It is clinically diagnosed usually between 12 and 24 months. The incidence as of 2020 1.5-3.0 per 1000 live births.
TYPES OF CEREBRAL PALSY (SCPE CLASSIFICATION) Distribution Dyskinetic Ataxic 6% 5% Spastic 89% Type will emerge and change over the first 2 years of life.
CLINICAL PRESENTATION The type they present as is dependant upon the location of the injury. In addition to motor disabilities, disturbances can be seen in: • Cognition • Perception • Communication • Behaviour Comorbid Conditions: • Epilepsy • Secondary musculoskeletal problems
CLINICAL PRESENTATION Spastic Cerebral Palsy: • Spasticity is defined as velocity dependant resistance to stretch. • Limb involvement can be hemiplegia, diplegia, or quadriplegia. • Affected limbs will have hyperactive DTRs, hypertonicity, tremors, weakness. • There is a characteristic “scissor gait” with toe walking. https://www.youtube.com/watch?v=d0Lm aJnAxfY&ab_channel=prohealthsys
CLINICAL PRESENTATION Dyskinetic Cerebral Palsy • Abnormally slow writhing movements of the hands, feet, or legs. • Exacerbated during periods of stress. • Absent during sleep. • Dystonic CP: Irregular posture + enhanced muscle tension • “hypertonic - hypokinetic” • Athetoid CP: Quick, uncontrolled, fragmented movements overlapped with dynamic twisting movement + predominantly diminished muscle tension • “hypotonic - hyperkinetic”
CLINICAL PRESENTATION Ataxic Cerebral Palsy: • Impaired balance and coordination. • Wide-based gait. • Intention tremors that impair fine motor function. • Predominantly lowered muscle tension.
ETIOLOGIES Historically CP was thought to be due to hypoxia during labour, delivery, and perinatal periods; however, treatment measures targeted in these areas did not change the incidence. Risk factors for developing cerebral palsy are now divided as: Preconception, Prenatal, Perinatal, and Neonatal/infant period.
DIAGNOSIS Warning signs for Cerebral Palsy • Delayed development (largely motor) • Abnormal muscle tone • Unusual posture • Persistent infantile reflexes • Hand preference before the age of 1
DIFFERENTIAL DIAGNOSIS
DIAGNOSTICS • Based on clinical presentation and history. • Precise interview concerned with pregnancy, labour, neonatal and infant period. Imaging Tools: • Brain ultrasonography in infants. • CT of the brain in older children. • MRI of the brain; Can be conducted on foetuses. • Abnormalities demonstrated in more than 80% of CP patients. • MRI can reveal anatomic abnormalities characteristic of particular CP types Additional tests: Psychological tests, vision evaluation, audiometric tests and Video EEG
MAGNETIC RESONANCE IMAGING CLASSIFICATION SYSTEM (MRICS) 5 Main Groups: A. Maldevelopments B. Predominant white matter injury C. Predominant grey matter injury D. Miscellaneous E. Normal Mixed lesions may also present
MRICS: A – MALDEVELOPMENTS A1: disorder of cortical formation A2: other maldevelopments
MRICS: B – PREDOMINANT WHITE MATTER INJURY B1: periventricular leukomalacia (PVL) B2: sequelae of Intraventricular hemorrhage (IVH) or periventricular hemorrhagic infarction B3: combination of PVL and IHV sequelae
MRICS: C – PREDOMINANT GREY MATTER INJURY C1: basal ganglia/thalamus lesion C2: cortico-subcortical lesion C3: arterial infarction
MRICS: D – MISCELLANEOUS D: miscellaneous
MRICS: E – NORMAL E: normal imaging
ASSESSMENT
GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM GMFCS I Children walk at home, school, outdoors and in the community. They can climb stairs without the use of a railing. Children perform gross motor skills such as running and jumping, but speed, balance and coordination are limited.
GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM GMFCS II Children walk in most settings and climb stairs holding onto a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces. Children may walk with physical assistance, a handheld mobility device or used wheeled mobility over long distances. Children have only minimal ability to perform gross motor skills such as running and jumping.
GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM GMFCS III Children walk using a hand-held mobility device in most indoor settings. They may climb stairs holding onto a railing with supervision or assistance. Children use wheeled mobility when traveling long distances and may self- propel for shorter distances.
GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM GMFCS IV Children use methods of mobility that require physical assistance or powered mobility in most settings. They may walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned. At school, outdoors and in the community children are transported in a manual wheelchair or use powered mobility.
GROSS MOTOR FUNCTION CLASSIFICATION SYSTEM GMFCS V Children are transported in a manual wheelchair in all settings. Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements.
MANAGEMENT Goal: Increase functionality, improve capabilities, sustain health • Physical and Occupational Therapy • Speech and Language Therapy • Assistive Technologies • Medications • Botulinum toxin type A • Baclofen • Benzodiazepine • Surgery • Selective dorsal rhizotomy • Selective peripheral neurotomy • Supportive surgical procedures
MANAGEMENT OF EPILEPSY IN CEREBRAL PALSY • Antiepileptic drugs have a lower efficacy and patients are often drug resistance. • Drug resistant epilepsy occurs more often in spastic quadriplegia type CP. • Risk factors: • Neonatal convulsions • Intense neuropathological changes in cerebrum • Mental retardation. • Consider non-pharmacological methods of treatment for drug resistant epilepsy • Surgeries (Causal vs. Supportive) • Neuromodulation • Ketogenic diet
ADDITIONAL MANAGEMENT AND PROGNOSIS • Oral-motor function is commonly impaired. • May require long-term nasogastric tube feeding or gastrostomy. • Associated with decreased survival. • Marked reduction in bone mass in non-ambulatory patients. • Mental health can be affected by chronic pain, social isolation, and loss of functionality/independence. • Patients are also have higher mortality for ischemic heart disease, cerebrovascular disease, and digestive disorders. • Increased risk of breast and brain cancer. • Increased risk from preventable deaths (drowning, motor vehicles crashes) • Prognosis is based on disease severity and functionality.
REFERENCES Krigger KW. Cerebral palsy: an overview. Am Fam Physician. 2006;73(1):91-100. Sadowska M, Sarecka-Hujar B, Kopyta I. Cerebral Palsy: Current Opinions on Definition, Epidemiology, Risk Factors, Classification and Treatment Options. Neuropsychiatr Dis Treat. 2020;16:1505-1518. Published 2020 Jun 12. doi:10.2147/NDT.S235165 Novak I, Morgan C, Adde L, et al. Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment [published correction appears in JAMA Pediatr. 2017 Sep 1;171(9):919]. JAMA Pediatr. 2017;171(9):897- 907. doi:10.1001/jamapediatrics.2017.1689 Mathewson MA, Lieber RL. Pathophysiology of muscle contractures in cerebral palsy. Phys Med Rehabil Clin N Am. 2015;26(1):57-67. doi:10.1016/j.pmr.2014.09.005 Yin R, Reddihough D, Ditchfield M, Collins K. Magnetic resonance imaging findings in cerebral palsy. J Paediatr Child Health. 2000;36(2):139-144. doi:10.1046/j.1440- 1754.2000.00484.x
REFERENCES MacLennan AH, Lewis S, Moreno-De-Luca A, et al. Genetic or Other Causation Should Not Change the Clinical Diagnosis of Cerebral Palsy. J Child Neurol. 2019;34(8):472-476. doi:10.1177/0883073819840449 GMFCS descriptors: Palisano et al. (1997) Dev Med Child Neurol 39:214 – 23 “Types of CP.” CEREBRAL PALSY PROJECT, www.cerebralpalsyproject.org/types- of-cp.html. Cans C, Dolk H, Platt MJ, Colver A, Prasauskiene A, Krageloh-Mann I; SCPE Collaborative group. Recommendations from the SCPE collaborative group for defining and classifying cerebral palsy. Dev Med Child Neurol Supp. 2007;109:35 – 38. doi:10.1111/j.1469-8749.2007.tb12626.x Mlodawski J, Mlodawska M, Pazera G, et al. Cerebral palsy and obstetric- neonatological interventions. Ginekol Pol. 2019;90(12):722-727. doi:10.5603/GP.2019.0124 Weyant , Curtis. “Cerebral Palsy Symptoms.” ConsumerSafety.org , ConsumerSafety.org, 20 Aug. 2020, www.consumersafety.org/news/how-does- cerebral-palsy-affect-development/.
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