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Cerebral Palsy

Cerebral Palsy

Cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies (CP) refers to a group of conditions resulting in motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology impairment affecting tone and posture and limiting physical activity. Cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies is the most common cause of childhood disability Disability Determination of the degree of a physical, mental, or emotional handicap. The diagnosis is applied to legal qualification for benefits and income under disability insurance and to eligibility for social security and workman's compensation benefits. ABCDE Assessment. It is caused by a nonprogressive CNS injury to the fetal or infant brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification. It is classified according to muscle tone Muscle tone The state of activity or tension of a muscle beyond that related to its physical properties, that is, its active resistance to stretch. In skeletal muscle, tonus is dependent upon efferent innervation. Skeletal Muscle Contraction, its distribution, and the presumed time of injury (spastic diplegia is the most common), and it is present in around 3 per 1,000 live births. Diagnosis is made by a detailed history and physical exam with an MRI scan confirming CNS insult. Interventions are multidisciplinary and prognosis Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas depends on the degree of disability Disability Determination of the degree of a physical, mental, or emotional handicap. The diagnosis is applied to legal qualification for benefits and income under disability insurance and to eligibility for social security and workman's compensation benefits. ABCDE Assessment.

Last updated: 25 Oct, 2021

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Contents

Overview

Definition

Cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies (CP) is a syndrome of permanent motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology impairment (posture and movement) that results from nonprogressive lesions to the developing brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification.

Epidemiology

  • 2.0–3.5 per 1,000 live births
  • Boy-to-girl ratio of 1.4:1
  • Prenatal events cause the majority of cases.
  • Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency is inversely proportional to gestational age Gestational age The age of the conceptus, beginning from the time of fertilization. In clinical obstetrics, the gestational age is often estimated as the time from the last day of the last menstruation which is about 2 weeks before ovulation and fertilization. Pregnancy: Diagnosis, Physiology, and Care and birth weight:
    • Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency is higher in preterm children.
    • Infants < 28 weeks at highest risk
    • Infants < 1,500 grams have 5%–15% risk.
  • The most common form is spastic diplegia (35%), a form of paralysis that affects symmetrical Symmetrical Dermatologic Examination body parts (i.e., both legs or both arms).
  • Risk factors for CP:
    • Prematurity Prematurity Neonatal Respiratory Distress Syndrome
    • Placental abnormalities Placental abnormalities Normal placental structure and function are essential for a healthy pregnancy. Some of the most common placental abnormalities include structural anomalies (such as a succenturiate lobe or velamentous cord insertion), implantation anomalies (such as placenta accreta and placenta previa), and functional anomalies (such as placental insufficiency). Placental Abnormalities
    • Major and minor birth defects
    • Meconium aspiration Meconium Aspiration A condition caused by inhalation of meconium into the lung of fetus or newborn, usually due to vigorous respiratory movements during difficult parturition or respiratory system abnormalities. Meconium aspirate may block small airways leading to difficulties in pulmonary gas exchange and aspiration pneumonia. Physical Examination of the Newborn
    • Emergency cesarean section
    • Birth asphyxia Asphyxia A pathological condition caused by lack of oxygen, manifested in impending or actual cessation of life. Drowning
    • Neonatal seizures Neonatal Seizures Seizures in Children
    • Respiratory distress syndrome
    • Intrauterine and neonatal infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
    • Multiple-gestation births (twins, triplets, etc ETC The electron transport chain (ETC) sends electrons through a series of proteins, which generate an electrochemical proton gradient that produces energy in the form of adenosine triphosphate (ATP). Electron Transport Chain (ETC).)
    • Intrauterine growth restriction/low birth weight
    • Maternal substance abuse
    • Preeclampsia Preeclampsia A complication of pregnancy, characterized by a complex of symptoms including maternal hypertension and proteinuria with or without pathological edema. Symptoms may range between mild and severe. Pre-eclampsia usually occurs after the 20th week of gestation, but may develop before this time in the presence of trophoblastic disease. Hypertensive Pregnancy Disorders
    • Perinatal hypoglycemia Hypoglycemia Hypoglycemia is an emergency condition defined as a serum glucose level ≤ 70 mg/dL (≤ 3.9 mmol/L) in diabetic patients. In nondiabetic patients, there is no specific or defined limit for normal serum glucose levels, and hypoglycemia is defined mainly by its clinical features. Hypoglycemia
    • Genetic susceptibility

Etiology

  • CNS injury of many types can lead to CP.
  • CP is nonprogressive.
  • Can occur in any of the following developmental periods:
    • Prenatal:
      • Congenital Congenital Chorioretinitis brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification malformations
      • Intrauterine infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
      • Intrauterine stroke
      • Chromosomal abnormalities
    • Perinatal:
      • Hypoxic-ischemic insults
      • CNS infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
      • Stroke
      • Kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn
    • Postnatal: 
      • Head trauma Head trauma Head trauma occurs when external forces are directed to the skull and brain structures, resulting in damage to the skull, brain, and intracranial structures. Head injuries can be classified as open (penetrating) or closed (blunt), and primary (from the initial trauma) or secondary (indirect brain injury), and range from mild to severe and life-threatening. Head Trauma
      • CNS infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
      • Stroke
      • Anoxic insults
      • May be accompanied by:
        • Sensory Sensory Neurons which conduct nerve impulses to the central nervous system. Nervous System: Histology, cognitive, communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence, and behavioral disturbances
        • Epilepsy Epilepsy Epilepsy is a chronic brain disorder marked by recurrent and unprovoked seizures. These seizures can be classified as focal or generalized and idiopathic or secondary to another condition. Clinical presentation correlates to the classification of the epileptic disorder. Epilepsy
        • Musculoskeletal impairment

Classification

  • According to muscle tone Muscle tone The state of activity or tension of a muscle beyond that related to its physical properties, that is, its active resistance to stretch. In skeletal muscle, tonus is dependent upon efferent innervation. Skeletal Muscle Contraction:
  • According to distribution:
    • Diplegia (paralysis of both legs or both arms)
    • Hemiplegia (1 side of the body is affected)
    • Quadriplegia Quadriplegia Severe or complete loss of motor function in all four limbs which may result from brain diseases; spinal cord diseases; peripheral nervous system diseases; neuromuscular diseases; or rarely muscular diseases. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper brain stem which injures the descending corticospinal and corticobulbar tracts. Locked-in Syndrome (all extremities are affected)
  • According to presumed time of injury:
    • Prepartum
    • Intrapartum
    • Postneonatal

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Cerebral Palsy, Intellectual Disability and Autism Spectrum Disorder

Pathophysiology

The ultimate cause of CP is injury to a child’s CNS during its embryological or perinatal development. The pathophysiology of this insult varies based on its etiology.

Table: CP subtypes, etiologies, and MRI findings
CP subtype Etiologies MRI findings
Hemiplegia (25%)
  • Thrombophilic disorders
  • Infection
  • Genetic/developmental
  • Periventricular hemorrhagic infarction
  • Stroke: in utero or neonatal
  • Focal infarct Infarct Area of necrotic cells in an organ, arising mainly from hypoxia and ischemia Ischemic Cell Damage or cortical, subcortical damage
  • Cortical malformations
Spastic quadriplegia Quadriplegia Severe or complete loss of motor function in all four limbs which may result from brain diseases; spinal cord diseases; peripheral nervous system diseases; neuromuscular diseases; or rarely muscular diseases. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper brain stem which injures the descending corticospinal and corticobulbar tracts. Locked-in Syndrome (20%)
  • Ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage, infection
  • Endocrine/metabolic, genetic/developmental
  • Periventricular leukomalacia
  • Multicystic encephalomalacia
  • Cortical malformations
Spastic diplegia (35%)
  • Prematurity Prematurity Neonatal Respiratory Distress Syndrome
  • Ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage
  • Infection
  • Endocrine/metabolic (e.g., thyroid Thyroid The thyroid gland is one of the largest endocrine glands in the human body. The thyroid gland is a highly vascular, brownish-red gland located in the visceral compartment of the anterior region of the neck. Thyroid Gland: Anatomy)
  • Periventricular leukomalacia
  • Periventricular cysts Cysts Any fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues. Fibrocystic Change or scars in white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome, ventricles enlarged, squared-off posterior ventricles
Extrapyramidal (athetoid, dyskinetic) (15%)
  • Asphyxia Asphyxia A pathological condition caused by lack of oxygen, manifested in impending or actual cessation of life. Drowning
  • Kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn
  • Mitochondrial
  • Genetic/metabolic
  • Asphyxia Asphyxia A pathological condition caused by lack of oxygen, manifested in impending or actual cessation of life. Drowning: symmetric scars in putamen and thalamus Thalamus The thalamus is a large, ovoid structure in the dorsal part of the diencephalon that is located between the cerebral cortex and midbrain. It consists of several interconnected nuclei of grey matter separated by the laminae of white matter. The thalamus is the main conductor of information that passes between the cerebral cortex and the periphery, spinal cord, or brain stem. Thalamus: Anatomy
  • Kernicterus Kernicterus A term used pathologically to describe bilirubin staining of the basal ganglia; brain stem; and cerebellum and clinically to describe a syndrome associated with hyperbilirubinemia. Clinical features include athetosis, muscle spasticity or hypotonia, impaired vertical gaze, and deafness. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the blood-brain barrier (e.g., sepsis). This condition occurs primarily in neonates, but may rarely occur in adults. Hyperbilirubinemia of the Newborn: scars in globus pallidus Globus pallidus The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus. Basal Ganglia: Anatomy, hippocampus
  • Mitochondrial: scarring Scarring Inflammation of globus pallidus Globus pallidus The representation of the phylogenetically oldest part of the corpus striatum called the paleostriatum. It forms the smaller, more medial part of the lentiform nucleus. Basal Ganglia: Anatomy, caudate, putamen, brainstem
  • No lesions: dopa-responsive dystonia Dystonia Dystonia is a hyperkinetic movement disorder characterized by the involuntary contraction of muscles, resulting in abnormal postures or twisting and repetitive movements. Dystonia can present in various ways as may affect many different skeletal muscle groups. Dystonia

Clinical Presentation

History

  • Perinatal:
    • Preterm birth Preterm birth Preterm labor refers to regular uterine contractions leading to cervical change prior to 37 weeks of gestation; preterm birth refers to birth prior to 37 weeks of gestation. Preterm birth may be spontaneous due to preterm labor, preterm prelabor rupture of membranes (PPROM), or cervical insufficiency. Preterm Labor and Birth 
    • Low birth weight
    • Complications during pregnancy Pregnancy The status during which female mammals carry their developing young (embryos or fetuses) in utero before birth, beginning from fertilization to birth. Pregnancy: Diagnosis, Physiology, and Care
    • Infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease or exposure Exposure ABCDE Assessment to infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease during pregnancy Pregnancy The status during which female mammals carry their developing young (embryos or fetuses) in utero before birth, beginning from fertilization to birth. Pregnancy: Diagnosis, Physiology, and Care
  • Developmental/cognitive:
    • Cognitive impairment (seen in approximately 50%): degree of disability Disability Determination of the degree of a physical, mental, or emotional handicap. The diagnosis is applied to legal qualification for benefits and income under disability insurance and to eligibility for social security and workman’s compensation benefits. ABCDE Assessment correlates with severity of motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology handicap
    • Behavioral abnormalities: 
      • Excessive irritability or docility
      • Autism spectrum disorder Autism spectrum disorder Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder
      • Obsessive compulsive behavior 
      • ADHD ADHD Attention deficit hyperactivity disorder is a neurodevelopmental disorder characterized by a pattern of inattention and/or hyperactivity-impulsivity that occurs in at least 2 different settings for more than 6 months. Although the patient has normal intelligence, the disease causes functional decline. Attention Deficit Hyperactivity Disorder
  • Neurological:
    • Disability Disability Determination of the degree of a physical, mental, or emotional handicap. The diagnosis is applied to legal qualification for benefits and income under disability insurance and to eligibility for social security and workman’s compensation benefits. ABCDE Assessment is static (progressive CNS lesions must be excluded).
    • Visual and/or hearing impairment Hearing impairment Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
    • 35% have a visual problem.
    • Delay in disappearance of primitive reflexes Primitive Reflexes Primitive reflexes are involuntary motor responses that can be elicited after birth. Although these reflexes are important for survival, they gradually disappear within the 1st year of life due to their inhibition by the developing frontal lobe. Primitive Reflexes
    • Epilepsy Epilepsy Epilepsy is a chronic brain disorder marked by recurrent and unprovoked seizures. These seizures can be classified as focal or generalized and idiopathic or secondary to another condition. Clinical presentation correlates to the classification of the epileptic disorder. Epilepsy
    • Chronic pain Chronic pain Aching sensation that persists for more than a few months. It may or may not be associated with trauma or disease, and may persist after the initial injury has healed. Its localization, character, and timing are more vague than with acute pain. Pain Management
    • Sleep Sleep A readily reversible suspension of sensorimotor interaction with the environment, usually associated with recumbency and immobility. Physiology of Sleep dysfunction 
  • Pulmonary:
    • Frequent lung infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease ( aspiration pneumonia Aspiration pneumonia A type of lung inflammation resulting from the aspiration of food, liquid, or gastric contents into the upper respiratory tract. Pneumonia)
    • Restrictive lung disease due to poor tone and scoliosis Scoliosis Scoliosis is a structural alteration of the vertebral column characterized by a lateral spinal curvature of greater than 10 degrees in the coronal plane. Scoliosis can be classified as idiopathic (in most cases) or secondary to underlying conditions. Scoliosis
  • Gastrointestinal:
    • Feeding intolerance (often leads to failure to grow)
    • Chronic constipation Constipation Constipation is common and may be due to a variety of causes. Constipation is generally defined as bowel movement frequency < 3 times per week. Patients who are constipated often strain to pass hard stools. The condition is classified as primary (also known as idiopathic or functional constipation) or secondary, and as acute or chronic. Constipation
    • Gastroesophageal reflux
  • Urological: 
  • Orthopedic:

Physical examination

Neurological:

  • Spasticity (scissoring of the legs)
  • Ataxia
  • Dystonia
  • Choreoathetosis (movement disorder causing involuntary twitching or writhing)
  • Diplegic gait in cases with a diplegic distribution
  • Early sign of spastic diplegia, dragging legs when crawling (e.g., “commando crawl”)
  • Decreased spontaneous movements on the affected side
  • Feet in a position of equinovarus:
    • Also known as clubfoot Clubfoot Clubfoot, also called talipes equinovarus, is a complex condition with a plantar flexed foot (equinus), adductus of the forefoot, and an inversion deformity of the heel (varus). Foot Deformities
    • Hindfoot in varus, a forefoot that is adducted, and an ankle in equinus
Photograph of a child with spastic diplegia

Photograph of a child with spastic diplegia:
Notice the scissoring of the legs, a key finding in the spastic diplegic form of cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies (CP).

Image: “Practical diagnosis- the use of symptoms in the diagnosis of disease (1899) (14785191513)” by H.A. Hare. License: Public Domain, cropped and edited by Lecturio.

Diagnosis

  • History and clinical findings are sufficient for initial diagnosis.
  • Further investigation to define subtype/severity and comorbid conditions:
    • MRI to determine:
      • Location and extent of structural lesions/ congenital malformations Congenital malformations Congenital malformations or teratogenic birth defects are developmental disorders that arise before birth during the embryonic or fetal period. The rate of incidence for children born alive is approximately 3%. Teratogenic Birth Defects
      • Presence of spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord: Anatomy pathology 
    • Hearing and visual function
    • Genetic evaluation in patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship with: 
      • Congenital malformations Congenital malformations Congenital malformations or teratogenic birth defects are developmental disorders that arise before birth during the embryonic or fetal period. The rate of incidence for children born alive is approximately 3%. Teratogenic Birth Defects ( chromosomes Chromosomes In a prokaryotic cell or in the nucleus of a eukaryotic cell, a structure consisting of or containing DNA which carries the genetic information essential to the cell. DNA Types and Structure)
      • Suspected metabolic disorders (e.g., amino acids Amino acids Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins. Basics of Amino Acids, organic acids Acids Chemical compounds which yield hydrogen ions or protons when dissolved in water, whose hydrogen can be replaced by metals or basic radicals, or which react with bases to form salts and water (neutralization). An extension of the term includes substances dissolved in media other than water. Acid-Base Balance, magnetic resonance ( MR MR Calculated as the ratio of the total number of people who die due to all causes over a specific time period to the total number of people in the selected population. Measures of Health Status) spectroscopy)
Different types of structural brain abnormalities in cerebral palsy

Different types of structural brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification abnormalities in cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies (CP):
All images are axial Axial Computed Tomography (CT) T2-weighted MRI scans. Each image is subtitled by its presumed etiology and timing during gestation.
A: a child with lissencephaly showing cortical thickening and agyria
B: a child with congenital Congenital Chorioretinitis cytomegalovirus Cytomegalovirus CMV is a ubiquitous double-stranded DNA virus belonging to the Herpesviridae family. CMV infections can be transmitted in bodily fluids, such as blood, saliva, urine, semen, and breast milk. The initial infection is usually asymptomatic in the immunocompetent host, or it can present with symptoms of mononucleosis. Cytomegalovirus infection showing an overfolded cortex (polymicrogyria), thin white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome, and dilated lateral ventricles Lateral ventricles Cavity in each of the cerebral hemispheres derived from the cavity of the embryonic neural tube. They are separated from each other by the septum pellucidum, and each communicates with the third ventricle by the foramen of monro, through which also the choroid plexuses (choroid plexus) of the lateral ventricles become continuous with that of the third ventricle. Ventricular System: Anatomy
C: an ex- premature Premature Childbirth before 37 weeks of pregnancy (259 days from the first day of the mother’s last menstrual period, or 245 days after fertilization). Necrotizing Enterocolitis child showing cystic Cystic Fibrocystic Change white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome injury (arrows) consistent with periventricular leukomalacia
D: a child who suffered a hemorrhagic stroke Hemorrhagic stroke Stroke due to rupture of a weakened blood vessel in the brain (e.g., cerebral hemispheres; cerebellum; subarachnoid space). Subarachnoid Hemorrhage in the newborn Newborn An infant during the first 28 days after birth. Physical Examination of the Newborn period. There is cortical and white matter White Matter The region of central nervous system that appears lighter in color than the other type, gray matter. It mainly consists of myelinated nerve fibers and contains few neuronal cell bodies or dendrites. Brown-Séquard Syndrome loss in the right frontal Frontal The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face. Skull: Anatomy and parietal Parietal One of a pair of irregularly shaped quadrilateral bones situated between the frontal bone and occipital bone, which together form the sides of the cranium. Skull: Anatomy lobes (arrowheads) consistent with previous ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage.

Image: “Examples of different types of structural brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification abnormalities in cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies” by Boyd, R.N., et al AL Amyloidosis. License: CC BY 2.0
Table: Gross motor Gross Motor Developmental Milestones and Normal Growth function classification system (GMFCS)
GMFCS level Characteristics
I
  • Is able to walk over more challenging terrain, and climb stairs without using hands for support
  • Is able to run and jump
  • Speed, balance, and coordination Coordination Cerebellar Disorders may be decreased
II
  • Is able to climb stairs using railing
  • Difficulty navigating challenging terrain (e.g., uneven surfaces, inclines, and crowds)
  • Is mostly unable to run or jump
III
  • Has physical impairments limiting purposeful movement
  • Struggles to maintain fixed head and neck Neck The part of a human or animal body connecting the head to the rest of the body. Peritonsillar Abscess position even only against gravity
  • Is impaired in all areas of motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology function
  • Cannot sit or stand independently, even with adaptive equipment
  • Walks with powered mobility Mobility Examination of the Breast aids AIDS Chronic HIV infection and depletion of CD4 cells eventually results in acquired immunodeficiency syndrome (AIDS), which can be diagnosed by the presence of certain opportunistic diseases called AIDS-defining conditions. These conditions include a wide spectrum of bacterial, viral, fungal, and parasitic infections as well as several malignancies and generalized conditions. HIV Infection and AIDS only
IV
  • Cannot walk even with assistive devices
  • Requires wheelchairs for ambulation, may control power Power The probability that a test will correctly reject a false null hypothesis. Statistical Tests and Data Representation wheelchair
  • Can perform standing transfers, with or without assistance
V
  • Is able to walk with assistive mobility Mobility Examination of the Breast on flat surfaces both indoors and outdoors
  • May be able to climb stairs using a railing
  • May ambulate with manual wheelchair, but requires help over long distances or uneven surfaces

Management

There is no curative treatment at this time. Management focuses on improving symptoms and increasing function while addressing comorbidities Comorbidities The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival. St. Louis Encephalitis Virus. Early multidisciplinary intervention improves patient outcomes.

  • Physical and occupational therapy Occupational Therapy Skilled treatment that helps individuals achieve independence in all facets of their lives. It assists in the development of skills needed for independent living. Fetal Alcohol Spectrum Disorder: 
    • Stretching exercises as early as possible (contracture prevention)
    • Adaptive equipment (e.g., walkers, poles)
    • Individualized evaluation/intervention for educational needs
  • Medical therapy:
    • Aimed at the treatment of:
      • Pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways
      • Seizures Seizures A seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized. Seizures
      • Spasticity Spasticity Spinal Disk Herniation
      • Sialorrhea
      • Behavioral disorders
    • Valium ( diazepam Diazepam A benzodiazepine with anticonvulsant, anxiolytic, sedative, muscle relaxant, and amnesic properties and a long duration of action. Its actions are mediated by enhancement of gamma-aminobutyric acid activity. Benzodiazepines) and other benzodiazepines Benzodiazepines Benzodiazepines work on the gamma-aminobutyric acid type A (GABAA) receptor to produce inhibitory effects on the CNS. Benzodiazepines do not mimic GABA, the main inhibitory neurotransmitter in humans, but instead potentiate GABA activity. Benzodiazepines 
    • Dantrolene Dantrolene Skeletal muscle relaxant that acts by interfering with excitation-contraction coupling in the muscle fiber. It is used in spasticity and other neuromuscular abnormalities. Although the mechanism of action is probably not central, dantrolene is usually grouped with the central muscle relaxants. Spasmolytics
    • Baclofen Baclofen A gamma-aminobutyric acid derivative that is a specific agonist of gaba-b receptors. It is used in the treatment of muscle spasticity, especially that due to spinal cord injuries. Its therapeutic effects result from actions at spinal and supraspinal sites, generally the reduction of excitatory transmission. Spasmolytics
    • Anticholinergics Anticholinergics Anticholinergic drugs block the effect of the neurotransmitter acetylcholine at the muscarinic receptors in the central and peripheral nervous systems. Anticholinergic agents inhibit the parasympathetic nervous system, resulting in effects on the smooth muscle in the respiratory tract, vascular system, urinary tract, GI tract, and pupils of the eyes. Anticholinergic Drugs (e.g., atropine Atropine An alkaloid, originally from atropa belladonna, but found in other plants, mainly solanaceae. Hyoscyamine is the 3(s)-endo isomer of atropine. Anticholinergic Drugs)
    • Botulinum toxin Botulinum toxin Toxic proteins produced from the species Clostridium botulinum. The toxins are synthesized as a single peptide chain which is processed into a mature protein consisting of a heavy chain and light chain joined via a disulfide bond. The botulinum toxin light chain is a zinc-dependent protease which is released from the heavy chain upon endocytosis into presynaptic nerve endings. Once inside the cell the botulinum toxin light chain cleaves specific snare proteins which are essential for secretion of acetylcholine by synaptic vesicles. This inhibition of acetylcholine release results in muscular paralysis. Botulism (Botox) injections in spastic areas
  • Surgical therapy:
  • Prevention:
    • Proper prenatal care Prenatal care Prenatal care is a systematic and periodic assessment of pregnant women during gestation to assure the best health outcome for the mother and her fetus. Prenatal care prevents and identifies maternal and fetal problems that adversely affect the pregnancy outcome. Prenatal Care and screening Screening Preoperative Care 
    • Administration of magnesium sulfate Magnesium Sulfate A small colorless crystal used as an anticonvulsant, a cathartic, and an electrolyte replenisher in the treatment of pre-eclampsia and eclampsia. It causes direct inhibition of action potentials in myometrial muscle cells. Excitation and contraction are uncoupled, which decreases the frequency and force of contractions. Laxatives in cases of preterm labor Labor Labor is the normal physiologic process defined as uterine contractions resulting in dilatation and effacement of the cervix, which culminates in expulsion of the fetus and the products of conception. Normal and Abnormal Labor before 32 weeks’ gestation
    • Genetic counseling Genetic Counseling An educational process that provides information and advice to individuals or families about a genetic condition that may affect them. The purpose is to help individuals make informed decisions about marriage, reproduction, and other health management issues based on information about the genetic disease, the available diagnostic tests, and management programs. Psychosocial support is usually offered. Myotonic Dystrophies for future offspring in select situations
  • Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas:
    • Dependent on the degree of motor Motor Neurons which send impulses peripherally to activate muscles or secretory cells. Nervous System: Histology, cognitive, and sensory deficit Sensory Deficit Anterior Cord Syndrome
    • Most patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship survive into adulthood.
    • Life expectancy Life expectancy Based on known statistical data, the number of years which any person of a given age may reasonably expected to live. Population Pyramids has improved significantly in the last 10–20 years.
    • Most common cause of early death is a respiratory failure Respiratory failure Respiratory failure is a syndrome that develops when the respiratory system is unable to maintain oxygenation and/or ventilation. Respiratory failure may be acute or chronic and is classified as hypoxemic, hypercapnic, or a combination of the two. Respiratory Failure as a result of aspiration pneumonia Aspiration pneumonia A type of lung inflammation resulting from the aspiration of food, liquid, or gastric contents into the upper respiratory tract. Pneumonia.

Clinical Relevance

The following conditions are etiological and/or risk factors for CP:

  • Neonatal respiratory distress syndrome Neonatal Respiratory Distress Syndrome Respiratory distress syndrome (RDS), also known as hyaline membrane disease, is caused by the lack of adequate pulmonary surfactant production in an immature lung. The syndrome is most commonly seen in preterm infants. Neonatal Respiratory Distress Syndrome: a syndrome of respiratory distress caused by lack of pulmonary surfactant Surfactant Substances and drugs that lower the surface tension of the mucoid layer lining the pulmonary alveoli. Acute Respiratory Distress Syndrome (ARDS) in the preterm infant. The syndrome is a known risk factor for the development of intraventricular hemorrhage Intraventricular hemorrhage Bleeding within the cerebral ventricles. It is associated with intraventricular trauma, aneurysm, vascular malformations, hypertension and in very low birth weight infants. Intracerebral Hemorrhage and periventricular leukomalacia, lesions frequently associated with CP.
  • Preterm delivery: delivery of the fetus before the pregnancy Pregnancy The status during which female mammals carry their developing young (embryos or fetuses) in utero before birth, beginning from fertilization to birth. Pregnancy: Diagnosis, Physiology, and Care reaches 37 weeks’ gestation. Etiology of preterm delivery is multifactorial, including maternal and fetal factors, some of which are treatable/preventable, while others are not. Preterm birth Preterm birth Preterm labor refers to regular uterine contractions leading to cervical change prior to 37 weeks of gestation; preterm birth refers to birth prior to 37 weeks of gestation. Preterm birth may be spontaneous due to preterm labor, preterm prelabor rupture of membranes (PPROM), or cervical insufficiency. Preterm Labor and Birth has been identified as a major risk factor for the development of CP.
  • Gestational infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease ( chorioamnionitis Chorioamnionitis Chorioamnionitis, commonly referred to as intraamniotic infection (IAI), is a common obstetric complication involving infection and inflammation of the fetal membranes, amniotic fluid, placenta, or the fetus itself. Chorioamnionitis is typically caused by a polymicrobial infection that ascends from the lower genitourinary tract. Chorioamnionitis, congenital Congenital Chorioretinitis TORCH infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease): chorioamnionitis Chorioamnionitis Chorioamnionitis, commonly referred to as intraamniotic infection (IAI), is a common obstetric complication involving infection and inflammation of the fetal membranes, amniotic fluid, placenta, or the fetus itself. Chorioamnionitis is typically caused by a polymicrobial infection that ascends from the lower genitourinary tract. Chorioamnionitis (the inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of the chorion Chorion The outermost extraembryonic membrane surrounding the developing embryo. In reptiles and birds, it adheres to the shell and allows exchange of gases between the egg and its environment. In mammals, the chorion evolves into the fetal contribution of the placenta. Placenta, Umbilical Cord, and Amniotic Cavity and amnios) and fetal infection by TORCH (Toxoplasmosis, Other agents, Rubella, Cytomegalovirus, and Herpes simplex) microorganisms are well-known risk factors for CP. Infection can cause direct CNS injury or complications with the delivery/perinatal course, or induce premature Premature Childbirth before 37 weeks of pregnancy (259 days from the first day of the mother’s last menstrual period, or 245 days after fertilization). Necrotizing Enterocolitis labor Labor Labor is the normal physiologic process defined as uterine contractions resulting in dilatation and effacement of the cervix, which culminates in expulsion of the fetus and the products of conception. Normal and Abnormal Labor.
  • Meningitis Meningitis Meningitis is inflammation of the meninges, the protective membranes of the brain, and spinal cord. The causes of meningitis are varied, with the most common being bacterial or viral infection. The classic presentation of meningitis is a triad of fever, altered mental status, and nuchal rigidity. Meningitis: an inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of the meninges Meninges The brain and the spinal cord are enveloped by 3 overlapping layers of connective tissue called the meninges. The layers are, from the most external layer to the most internal layer, the dura mater, arachnoid mater, and pia mater. Between these layers are 3 potential spaces called the epidural, subdural, and subarachnoid spaces. Meninges: Anatomy, usually caused by an infectious Infectious Febrile Infant pathogen. Cerebral palsy Palsy paralysis of an area of the body, thus incapable of voluntary movement Cranial Nerve Palsies is among the neurological sequelae that can develop in children under 5 of age who undergo the CNS inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation seen in meningitis Meningitis Meningitis is inflammation of the meninges, the protective membranes of the brain, and spinal cord. The causes of meningitis are varied, with the most common being bacterial or viral infection. The classic presentation of meningitis is a triad of fever, altered mental status, and nuchal rigidity. Meningitis.
  • Hyperbilirubinemia Hyperbilirubinemia A condition characterized by an abnormal increase of bilirubin in the blood, which may result in jaundice. Bilirubin, a breakdown product of heme, is normally excreted in the bile or further catabolized before excretion in the urine. Jaundice: elevated bilirubin Bilirubin A bile pigment that is a degradation product of heme. Heme Metabolism levels during the neonatal period (first 28 days of life). Hyperbilirubinemia Hyperbilirubinemia A condition characterized by an abnormal increase of bilirubin in the blood, which may result in jaundice. Bilirubin, a breakdown product of heme, is normally excreted in the bile or further catabolized before excretion in the urine. Jaundice and its neurological complications ( bilirubin Bilirubin A bile pigment that is a degradation product of heme. Heme Metabolism-induced neurological dysfunction ( BIND BIND Hyperbilirubinemia of the Newborn)) are risk factors for the development of CP.
  • Preeclampsia Preeclampsia A complication of pregnancy, characterized by a complex of symptoms including maternal hypertension and proteinuria with or without pathological edema. Symptoms may range between mild and severe. Pre-eclampsia usually occurs after the 20th week of gestation, but may develop before this time in the presence of trophoblastic disease. Hypertensive Pregnancy Disorders: a gestational disorder featuring the elevation of maternal blood pressure above 140/90 mm MM Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma Hg, seen in association with proteinuria Proteinuria The presence of proteins in the urine, an indicator of kidney diseases. Nephrotic Syndrome in Children. Preeclampsia Preeclampsia A complication of pregnancy, characterized by a complex of symptoms including maternal hypertension and proteinuria with or without pathological edema. Symptoms may range between mild and severe. Pre-eclampsia usually occurs after the 20th week of gestation, but may develop before this time in the presence of trophoblastic disease. Hypertensive Pregnancy Disorders can lead to early delivery and has a strong association with CP.
  • Stroke: broadly understood as a hemorrhagic or ischemic abnormality in blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure to brain Brain The part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem. Nervous System: Anatomy, Structure, and Classification tissue. A stroke can cause CP if it occurs in the prenatal or postnatal periods.

References

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  2. Johnston, MV. (2020). Encephalopathies. In Kliegman R.M., et al. (Eds.), Nelson textbook of pediatrics. pp. 3168–3186.e1 https://www.clinicalkey.com/#!/content/3-s2.0-B9780323529501006167
  3. Yin, R, Reddihough, D, Ditchfield, M, & Collins, K. (2000). Magnetic resonance imaging findings in cerebral palsy. Journal of Paediatrics and Child Health. 36(2), 139–144. https://doi.org/10.1046/j.1440-1754.2000.00484.x
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  5. Demeši Drljan, Č, et al. (2016). Cerebral palsy in preterm infants. Vojnosanitetski Pregled. 73(4), 343–348. https://doi.org/10.2298/VSP140321019D
  6. Wu, YW, et al. (2015). Risk for cerebral palsy in infants with total serum bilirubin levels at or above the exchange transfusion threshold: A population-based study. JAMA pediatrics. 169(3), 239–246. https://doi.org/10.1001/jamapediatrics.2014.3036
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  9. Hadjipanayis, A, Hadjichristodoulou, C, & Youroukos, S. (1997). Epilepsy in patients with cerebral palsy. Developmental Medicine and Child Neurology. 39(10), 659–663. https://doi.org/10.1111/j.1469-8749.1997.tb07359.x
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