Article Topic:: Cerebral palsy
Name of the Author: Maria A. AlShurman
Keywords: cerebral palsy, overview, epidemiology ,causes, pathogenesis, outcomes, complications

 

An overview and epidemiology:

The definition of Cerebral Palsy has been a dilemma in medical literature since it doesn’t have a specific age limit of the time the brain insult occurred, nor the age of onset of motor disorder (1). However, the international panel agreed on a definition in 2006 : “cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation , that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain ”(2).Although cerebral palsy is the most widespread and costly motor disability of children(3), the incidence and prevalence are not well established because of etiological and geographical variations(4). Nonetheless, the world wide prevalence is 2 per 1000 live births (5).

Pathogenesis and etiology:

The etiology of cerebral palsy is multifactorial. Although, there’s still no complete understanding of the whole picture(2), but it is believed that there is always a cause of CP and every child with CP must undergo an evaluation and a complete investigation in order to establish a proper cause whether it is of prenatal , perinatal or postnatal origin(6).

The type of injuries that could occur to the developing brain include: hypoxia, infection, stroke or hypotension with their subsequent inflammation that occurs after the original insult, can happen in utero, around the time of delivery, post-natal period or later in early childhood. As a result , they divided the causes of developing brain insults according to the time of occurrence: early brain injuries ( usually before 20 weeks gestation), injuries between 24-32 weeks of gestation, injuries around delivery and post-natal injuries(7).

Causes of early brain injuries (often before 20 weeks gestation) that will lead to brain maldevelopment, figure (1), includes:

• Maternal infection—cytomegalovirus—polymicrogyria
• COL4A1 genetic mutations—porencephalic cysts, schizencephaly
• LIS1 genetic mutations—lissencephaly
• GPR56 gene—polymicrogyria

Insults that happen in early/mid-pregnancy (24–32 weeks gestation) include:

• White matter disease of prematurity (periventricular leukomalacia), figure (2)
• Hypoxia, hypotension, sepsis
• Pressure from intraventricular hemorrhage (hemorrhagic parenchymal infarction)

Injuries that occur late in the developed brain include:

• Peripartum asphyxia
• Maternal infection
• Stroke

Lastly, Post-neonatal brain injuries include:

• Meningitis/encephalitis
• Stroke
• Trauma—accidental/non-accidental
• Hypoxia—near drowning

Figure 1: (A) CT scan of a head showing intracranial calcification associated with early cytomegalovirus infection. (B) MRI scan of brain (T2-weighted) showing extensive polymicrogyria typical of a congenital cytomegalovirus infection. (C) MRI scan of brain (T2-weighted) showing lissencephaly, a cerebral maldevelopment often associated with the LIS1 gene mutation. (D) MRI scan of brain (T1-weighted) showing lissencephaly. (E) MRI scan of brain (T1-weighted) showing bilateral open-lipped schizencephaly, which can be associated with COL4a1 genetic mutation predisposing to early infarcts.

Figure 2: MRI scan of brain (T2-weighted) showing periventricular leukomalacia associated with preterm (24– 32 weeks gestation) injury to the brain.

Clinical Presentation:

Unusual restless movements or abnormalities of movement and tone, including hypotonia (floppiness),

spasticity (stiffness) or dystonia (fluctuating tone) are some of the motor features that appear early. There’s also abnormal motor development, including late head control, rolling and crawling and feeding difficulties can also be signs(8).

Early signs and symptoms:

1. a) Neurobehavioral signs: Neurobehavioral signs unclear for CP are excessive irritability, tiredness, sleep disturbances, frequent vomiting, difficult to handle and cuddle, and has poor visual attention.
2. b) Developmental reflexes: procrastinate in disappearance or exaggeration of a developmental reflex may be an early indicator of motor disability.
3. c) Motor tone and posture: Tone in extremities/ trunk might be normal, increased or decreased. Poor head control, persistent or asymmetric hand fisting, and abnormal oral motor patterns (tongue thrusting/ grimacing) may be the early motor signs.
4. d) Motor milestones: a follow-up examination of motor milestones is an effective screening tool for detecting CP early. The American Academy of Pediatrics recommends complete developmental screening at 9, 18, 30, and 48 months visit(5)

Classification:

It is important to classify cerebral palsy in categories, as it has a very diverse spectrum of presentations, and the most appropriate classification is based on motor type, topography of motor impairment and the degree of functional impairment(2)(5)(7).

According to the motor type, it is subdivided into: spastic, dyskinetic, hypotonic and ataxic. For the distribution, it is classified into: hemiplegia, diplegia and quadriplegia. These two classifications do not give a clear idea about activity and participation. That’s where the importance of the third classification (degree of functional impairment) was raised. The gross motor classification system GMCS is used to classify the patient’s level of mobility according to age into 5 levels. Starting from level 1 where the patient has a slight disability to level 5 in which the patient is severely disabled where he can’t maintain posture without the aid of a career or equipments, figure (3)(7).

Spastic CP:

This type of cerebral palsy has signs consistent with upper motor neuron lesion, like: spasticity, hyperreflexia (± clonus), extensor plantar response, mass movements (instead of fine and individual movements), and slow and effortful voluntary movements. Spastic type divided furtherly according to the distribution:

• Diplegic: the problem here is particularly in lower limbs, with usually preserved fine motor functions in upper limbs. It is commonly associated with periventricular leukomalacia (PVL) and periventricular hemorrhagic infarction (PVHI). This type mainly affects preterm babies and the presentation in infants less than 6 months appears differently as the lower limbs are hypotonic. Infants older than 6 months have signs of Commando crawl, W-Sitting, and Tendoachilles tightening. For older children when the spasticity starts to appear more after walking, toe walking could be a very suspicious sign.
• Hemiplegic: In spastic hemiplegia, upper limbs is typically affected more than lower limbs, accompanied with sensory deficit reflected as poor muscle mass on the affected side. They may also have cognitive, visual, and behavioral problems. Including anxiety, oppositional defiance, and specific phobias. The most common cause of hemiplegic CP is stroke ,either prenatal or neonatal, in full term infants and appears as Asymmetry while natural hand movements/ bringing hands in midline or during neonatal reflexes like Moro’s, Early hand dominance (before 12 months), and In sitting position, affected leg tends to extend.
• Quadriplegic: this is the most severe type as all 4 limbs are affected nearly equally, and majority have very little speech and language development, visual impairment, epilepsy, feeding difficulty, and chronic respiratory insufficiency. MRI in these children may show multicystic encephalomalacia. It affects both preterm and full term infants and they have Severe motor and cognitive delay and Poor head control. Initially they may appear hypotonic in the first 4months which then turns to spasticity by 9–12 months, and by 12 months, infants when pulled to sitting are unable to flex their legs and have poor truncal balance(5)

Figure 3: Gross motor function classification system to classify how a child with cerebral palsy mobilizes and are classified according to age.

Dyskinetic CP:

This is characterized by having more than one form of involuntary movements. The limbs usually become stiff during attempted movement or when emotional. Tendon reflexes may be normal or may be difficult to evoke. Athetoid movements of toes especially the great toe (striatal toe) is a significant clue of extrapyramidal dysfunction. Dyskinesia can be seen in some spastic syndromes, but inpurely dyskinetic syndromes contractures are less common.We can additionally sub-classify dyskinetic CP into:

1. Choreo-athetoid CP: Which is defined as rapid disorganized unpredictable contractions of individual muscles/ muscle groups involving face, bulbar muscles, proximal extremities and digits? They also have slow writhing movements involving distal muscles. Oropharyngeal difficulties may result from facial grimacing, and Primitive reflexes are persistent. Hyperbilirubinemia is a chief cause for this type as it occurs in infants who are born with (Rh/ ABO incompatibility/G6PD deficiency).

2. Dystonic CP: It is characterized by co-contraction of agonist and antagonist muscles. Simultaneously, they may have coexistent pyramidal signs and dysarthria. This mainly occurs as a consequence of birth asphyxia(5)

Ataxic CP: Ataxic CP is uncommon and should be differentiated from progressive neurodegenerative disorders. These patients have delayed development in motor and language milestones manifested asataxia and slow, jerky, explosive speech. Ataxia usually improves with time and have no apparent cause.(5)

Diagnosis:

The diagnosis of CP depends on multidisciplinary approach starting from recognition of clinical risk factors to neurological examination and ends up with neuroimaging. And because of its complexity, the diagnosis is often delayed and occurs between 1 to 2 years of age. Nowadays, early and precise diagnosis of CP is being achieved, as it’s more favorable to initiate therapy as early as possible in order to improve long-term outcomes during the period of rapid brain growth and neuroplasticity.

For children who have identifiable risk factors, the best ways to detect CP before 5 months of age include: MRI, GMA and HINE which are a highly sensitive (90%) for CP. On the contrary, for those children that lack risk factors, the diagnosis is often delayed until they develop warning signs which indicate inability to achieve their expected milestones, such as: include the inability to sit independently by 9 months of age, the presence of asymmetric movements or limb preference, or the inability of an infant to bear weight on the plantar surface of the feet.

Neuroimaging:

MRI has been identified to have the highest sensitivity for the diagnosis of CP. There are some findings that increase the suspicion index for CP including: white matter injury (eg: cystic periventricular leukomalacia [CPVL], periventricular hemorrhagic infarction, progressive ventriculomegaly, neonatal stroke), injury to deep gray matter structures including basal ganglia, cerebellar hemorrhage, and developmental brain malformations (eg: lissencephaly, microgyria, cortical dysplasia).

Neuromotor assessment:

In assessing the neuromotor function, 2 tools are considered the most sensitive and used ones. Them being:

• GMA: a tool which uses the pattern of spontaneous movement of infants that are altered in predictable way during normal development. There are 2 specific movement patterns that are highly sensitive and specific for the diagnosis of CP: first, cramped synchronous general movements (70% sensitivity, 90% specificity), characterized by rigid contraction and relaxation of the infant’s trunk and extremities; second, absent fidgety movements (97% sensitivity, 89% specificity) characterized by a lack of the normal small-amplitude fidgety movements that should be present in newborns 10 to 20 weeks of corrected age.
• HINE: according to this tool, it is a standardized neurological examination for age ranges from 2 to 24 months, which contain 26 scored items that assess an infant’s cranial nerve function, posture, movements, tone, and reflexes.

Complications:

As cerebral palsy is mainly a motor disease resulting from insult to the developing brain, skeletal muscles are the most affected organs, and one of the most common complications is muscle contracture- limited joint movement that result from high passive muscle force- which leads to inability to walk and pain. In addition to this, there are non-motor complications which include: epilepsy, which happens in nearly 40% of patients with cerebral palsy, growth retardation and failure to thrive due to oro-motor difficulties, gastroesophageal reflux disease, chronic lung disease, bladder control problems, hip displacement, sleep disturbances, intellectual disability, deafness, blindness, and osteopenia. It’s worth mentioning that respiratory complications, which are mainly due to aspiration and GERD, are responsible for the major morbidity and mortality rates(9).

Management:

The mainstay in the management of CP is the early diagnosis and intervention as it improves the outcomes later on. Beside improving the motor function, enhancing the quality of life for these patients should be always a priority in management(2).

There is no definitive cure for CP, and its management requires multidisciplinary approach concerned mainly with the symptoms(7).

Management of motor impairment:

Physiotherapy and occupational therapy play an essential part in the management of CP, and the most evidence based approaches include:

1- Bimanual training for hemiplegic CP in which the child is trained to use both hands at the same time to undergo repetitive tasks.

2. Constraint induced movement therapy includes restraint of the unaffected limb resulting in usage of affected limb during therapeutic tasks. Restraint may be achieved by the use of a cast or physical restraining by holding the normal limb.
3. Context focused therapy involves changing the environment rather than the child’s approach.
4. Goal directed functional training lays emphasis on activities based on goals set by the child, using motor learning approach.(5)

Management of spasticity:

This includes oral medications like: benzodiazepines, baclofen, and tizanidine; botulinum toxin-A; intrathecal baclofen; alcohol and phenol in addition to surgical and orthopedic procedures(5)

Feeding and nutrition:

If there is oro-motor problems interfering with growth and gaining weight other feeding techniques should be considered like gastrostomy feeding.(5)

Drooling:

Drooling (sialorrhea) is the uncontrolled spontaneous loss of saliva and oral content that occurs normally in infants until 24 months of age. After that the child should have the ability to do most activities without loss of saliva. It’s considered pathological if it carries on after the age of 4 years. It’s often seen with neurological disorders , including neuromuscular incoordination of swallowing and intellectual disabilities.(10)

Improving drooling is considered important as it interferes with social life, and different approaches could be advised starting from behavioral therapy to pharmacotherapy (anticholinergic drugs (trihexphenidyl or glycopyrrolate and BTX-A injection into salivary glands) and surgery(5).

Prevention:
Magnesium sulfate:

Various studies showed increase in the risk of CP in infants who were born before 32 weeksof gestational age and received MgSO4 during labor, as it is found to be a neuroprotective agent.(3)

 

Therapeutic hypothermia:

Neonates born after the 35th week of gestation that were exposed to a perinatal episode of hypoxia which may lead to perinatal hypoxic-ischemic encephalopathy, and hence causing CP, benefited from therapeutic hypothermia. Therapeutic hypothermia was found to improve the survival rate with normal neurological development and decrease the risk of death and major neurological disorders at the age of 18 months.

Therapeutic hypothermia must be implemented in the first 6 hours postpartum.

Indications for hypothermia [35]:

• pH ≤ 7.0 and/or BE base deficiency of at least 16 mmol/l in a cord blood sample or a blood sample taken within 1 hour of life;
• 1 of the following:
  • Apgar score ≤ 5 in 1st, 3rd, 5th, 10th minute after birth;
  • Continuous resuscitation within 10 minutes after birth
• neurological symptoms indicative of moderate/severe encephalopathy on clinical examination (the Sarnat scale is used for qualification)(11)

Caffeine:

Caffeine is one of the most commonly used therapies in neonatal intensive care units for apnea. There are some studies which are concerned in the use of caffeine in premature infants with apneic episodes resulted in a reduction in the risk of motor and cognitive impairment along with the treatment. The finding was statistically significant at 18 months but not at 5 years.(3)

 

Antenatal steroids:

Studies are still debating about the relation between the use of antenatal steroids and the reduction of cerebral palsy risk. In spite of that, some observational studies indicate the clinical benefits of a single course of antenatal steroid therapy in reducing the risk of CP diagnosed in the first year of life or later, the percentage of CP diagnoses was 32% lower in patients whose mothers received a course of steroid therapy during pregnancy that was terminated prematurely.(11)

Prognosis:

Most of the children diagnosed with CP survive to adulthood, and many factors play a role in the upcoming consequences like: severity of impairment, birth weight, and socioeconomic status.

For children who cannot achieve their milestones (mainly head balance by 20 months of age, persistent primitive reflexes, or do not crawl by age of 5), the probability of being able to walk is very low. Whereas children who can sit by age of 2 years or crawl by age of 5, the prognosis for walking is good.

Besides the motor outcome, there is the functional outcome in CP which relies on multiple factors these include: intelligence, physical function, ability to communicate, and personality attributes. Social (e.g., family support) and environmental factors and the availability of specialist medical care have an essential impact.(5)

 

 

 

References:

1. Robaina-Castellanos GR, Riesgo-Rodriguez S, Robaina-Castellanos MS. [Definition and classification of cerebral palsy: a problem that has already been solved?]. Rev Neurol. 2007 Jul;45(2):110–7.
2. Michael-Asalu A, Taylor G, Campbell H, Lelea LL, Kirby RS. Cerebral Palsy: Diagnosis, Epidemiology, Genetics, and Clinical Update. Adv Pediatr [Internet]. 2019;66:189–208. Available from: https://doi.org/10.1016/j.yapd.2019.04.002
3. Korzeniewski SJ, Slaughter J, Lenski M, Haak P, Paneth N. The complex aetiology of cerebral palsy. Nat Rev Neurol. 2018 Sep;14(9):528–43.
4. McIntyre S. The continually changing epidemiology of cerebral palsy. Acta Paediatr Int J Paediatr. 2018;107(3):374–5.
5. Gulati S, Sondhi V. Cerebral Palsy: An Overview. Indian J Pediatr. 2018;85(11):1006–16.
6. Appleton RE, Gupta R. Cerebral palsy: Not always what it seems. Arch Dis Child. 2019;104(8):809–14.
7. Wimalasundera N, Stevenson VL. Cerebral palsy. Pract Neurol. 2016;16(3):184–94.
8. Dean BE. news / clinical update. 2017;29(3):2017.
9. Graham HK, Rosenbaum P, Paneth N, Dan B, Lin JP, Damiano DiL, et al. Cerebral palsy. Nat Rev Dis Prim. 2016;2.
10. Dias BLS, Fernandes AR, Maia Filho H de S. Sialorreia em crianças com paralisia cerebral. J Pediatr (Rio J) [Internet]. 2016;92(6):549–58. Available from: http://dx.doi.org/10.1016/j.jped.2016.03.006
11. Mlodawski J, Mlodawska M, Pazera G, Michalski W, Domanski T, Dolecka-Slusarczyk M, et al. Cerebral palsy and obstetric-neonatological interventions. Ginekol Pol. 2019;90(12):722–7.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Article: Cerebral palsy

Author: Maria A. AlShurman

Keywords: cerebral palsy, overview, epidemiology, causes, pathogenesis, outcomes, complications

 

An overview and epidemiology:

The definition of Cerebral Palsy has been a dilemma in medical literature since it doesn’t have a specific age limit of the time the brain insult occurred, nor the age of onset of motor disorder (1). However, the international panel agreed on a definition in 2006 : “cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation , that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain ”(2).Although cerebral palsy is the most widespread and costly motor disability of children(3), the incidence and prevalence are not well established because of etiological and geographical variations(4). Nonetheless, the world wide prevalence is 2 per 1000 live births (5).

 

Pathogenesis and etiology:

The etiology of cerebral palsy is multifactorial. Although, there’s stillno complete understanding ofthe whole picture(2), but it is believed that there is always a cause of CP and every child with CP must undergo an evaluation and a complete investigation in order to establish a proper cause whether it is of prenatal , perinatal or postnatal origin(6).

The type of injuries that could occur to the developing brain include: hypoxia, infection, stroke or hypotension with their subsequent inflammation that occurs after the original insult, can happen in utero, around the time of delivery, post-natal period or later in early childhood. As a result , they divided the causes of developing brain insults according to the time of occurrence: early brain injuries ( usually before 20 weeks gestation), injuries between 24-32 weeks of gestation, injuries around delivery and post-natal injuries(7).

Causes of early brain injuries (often before 20 weeks gestation) that will lead to brain maldevelopment, figure (1), includes:

• Maternal infection—cytomegalovirus—polymicrogyria
• COL4A1 genetic mutations—porencephalic cysts, schizencephaly
• LIS1 genetic mutations—lissencephaly
• GPR56 gene—polymicrogyria

Insults that happen in early/mid-pregnancy (24–32 weeks gestation) include:

• White matter disease of prematurity (periventricularleukomalacia).figure (2)
• Hypoxia, hypotension, sepsis
• Pressure from intraventricular hemorrhage (hemorrhagic parenchymal infarction)

Injuries that occur late in the developed brain include:

• Peripartum asphyxia
• Maternal infection
• Stroke

Lastly, Post-neonatal brain injuries include:

• Meningitis/encephalitis
• Stroke
• Trauma—accidental/non-accidental
• Hypoxia—near drowning

 

 

 

 

 

 

 

 

 

 

 

 

Figure 1: (A) CT scan of a head showing intracranial calcification associated with early cytomegalovirus infection. (B) MRI scan of brain (T2-weighted) showing extensive polymicrogyria typical of a congenital cytomegalovirus infection. (C) MRI scan of brain (T2-weighted) showing lissencephaly, a cerebral maldevelopment often associated with the LIS1 gene mutation. (D) MRI scan of brain (T1-weighted) showing lissencephaly. (E) MRI scan of brain (T1-weighted) showing bilateral open-lipped schizencephaly, which can be associated with COL4a1 genetic mutation predisposing to early infarcts.

 

 

 

 

 

Figure 2: MRI scan of brain (T2-weighted) showing periventricular leukomalacia associated with preterm (24– 32 weeks gestation) injury to the brain.

 

 

 

 

 

 

 

 

 

 

Clinical Presentation:

Unusual restlessmovements or abnormalities of movement and tone, including hypotonia (floppiness),

spasticity (stiffness) or dystonia (fluctuating tone) are some of the motor features that appear early. There’s also abnormal motor development, including late head control, rolling and crawling and feeding difficulties can also be signs(8).

 

– Early signs and symptoms:

1. a) Neurobehavioral signs: Neurobehavioral signs unclear for CP are excessive irritability, tiredness, sleep disturbances, frequent vomiting, difficult to handle and cuddle, and has poor visual attention.
2. b) Developmental reflexes: procrastinate in disappearance or exaggeration of a developmental reflex may be an early indicator of motor disability.
3. c) Motor tone and posture: Tone in extremities/ trunk might be normal, increased or decreased. Poor head control, persistent or asymmetric hand fisting, and abnormal oral motor patterns (tongue thrusting/ grimacing) may be the early motor signs.
4. d) Motor milestones: a follow-up examination of motor milestones is an effective screening tool for detecting CP early. The American Academy of Pediatrics recommends complete developmental screening at 9, 18, 30, and 48 months visit(5)

 

Classification:

It is important to classify cerebral palsy in categories, as it has a very diverse spectrum of presentations, and the most appropriate classification is based on motor type, topography of motor impairment and the degree of functional impairment(2)(5)(7).

According to the motor type, it is subdivided into: spastic, dyskinetic, hypotonic and ataxic. For the distribution, it is classified into: hemiplegia, diplegia and quadriplegia. These two classifications do not give a clear idea about activity and participation. That’s where the importance of the third classification (degree of functional impairment) was raised. The gross motor classification system GMCS is used to classify the patient’s level of mobility according to age into 5 levels. Starting from level 1 where the patient has a slight disability to level 5 in which the patient is severely disabled where he can’t maintain posture without the aid of a carer or equipments(figure 3)(7).

 

Spastic CP:

This type of cerebral palsy has signs consistent with upper motor neuron lesion, like: spasticity, hyperreflexia (± clonus), extensor plantar response, mass movements (instead of fine and individual movements), and slow and effortful voluntary movements. Spastic type divided furtherly according to the distribution:

• Diplegic: the problem here is particularly in lower limbs, with usually preserved fine motor functions in upper limbs. It is commonly associated with periventricular leukomalacia (PVL) and periventricular hemorrhagic infarction (PVHI). This type mainly affects preterm babies and the presentation in infants less than 6 months appears differently as the lower limbs are hypotonic. Infants older than 6 months have signs of Commando crawl, W-Sitting, and Tendoachilles tightening. For older children when the spasticity starts to appear more after walking, toe walking could be a very suspicious sign.
• Hemiplegic: In spastic hemiplegia, upper limbs is typically affected more than lower limbs, accompanied with sensory deficit reflected as poor muscle mass on the affected side. They may also have cognitive, visual, and behavioral problems. Including anxiety, oppositional defiance, and specific phobias. The most common cause of hemiplegic CP is stroke ,either prenatal or neonatal, in full term infants and appears as Asymmetry while natural hand movements/ bringing hands in midline or during neonatal reflexes like Moro’s, Early hand dominance (before 12 months), and In sitting position, affected leg tends to extend.
• Quadriplegic: this is the most severe type as all 4 limbs are affected nearly equally, and majority have very little speech and language development, visual impairment, epilepsy, feeding difficulty, and chronic respiratory insufficiency. MRI in these children may show multicystic encephalomalacia. It affects both preterm and full term infants and they have Severe motor and cognitive delay and Poor head control. Initially they may appear hypotonic in the first 4months which then turns to spasticity by 9–12 months, and by 12 months, infants when pulled to sitting are unable to flex their legs and have poor truncal balance(5)

 

 

 

 

 

Figure 3: Gross motor function classification system to classify how a child with cerebral palsy mobilizes and are classified according to age.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Dyskinetic CP:

This is characterized by having more than one form of involuntary movements. The limbs usually become stiff during attempted movement or when emotional. Tendon reflexes may be normal or may be difficult to evoke. Athetoid movements of toes especially the great toe (striatal toe) is a significant clue of extrapyramidal dysfunction. Dyskinesia can be seen in some spastic syndromes, but inpurely dyskinetic syndromes contractures are less common.We can additionally sub-classify dyskinetic CP into:

 

1. Choreo-athetoid CP: Which is defined as rapid disorganized unpredictable contractions of individual muscles/ muscle groups involving face, bulbar muscles, proximal extremities and digits? They also have slow writhing movements involving distal muscles. Oropharyngeal difficulties may result from facial grimacing, and Primitive reflexes are persistent. Hyperbilirubinemia is a chief cause for this type as it occurs in infants who are born with (Rh/ ABO incompatibility/G6PD deficiency).

ii.Dystonic CP: It is characterized by co-contraction of agonist and antagonist muscles. Simultaneously, they may have coexistent pyramidal signs and dysarthria. This mainly occurs as a consequence of birth asphyxia(5)

 

Ataxic CP: Ataxic CP is uncommon and should be differentiated from progressive neurodegenerative disorders. These patients have delayed development in motor and language milestones manifested asataxia and slow, jerky, explosive speech. Ataxia usually improves with time and have no apparent cause.(5)

 

Diagnosis:

The diagnosis of CP depends on multidisciplinary approach starting from recognition of clinical risk factors to neurological examination and ends up with neuroimaging. And because of its complexity, the diagnosis is often delayed and occurs between 1 to 2 years of age. Nowadays, early and precise diagnosis of CP is being achieved, as it’s more favorable to initiate therapy as early as possible in order to improve long-term outcomes during the period of rapid brain growth and neuroplasticity.

For children who have identifiable risk factors, the best ways to detect CP before 5 months of age include: MRI, GMA and HINE which are a highly sensitive (90%) for CP. On the contrary, for those children that lack risk factors, the diagnosis is often delayed until they develop warning signs which indicate inability to achieve their expected milestones, such as: include the inability to sit independently by 9 months of age, the presence of asymmetric movements or limb preference, or the inability of an infant to bear weight on the plantar surface of the feet.

 

Neuroimaging:

MRI has been identified to have the highest sensitivity for the diagnosis of CP. There are some findings that increase the suspicion index for CP including: white matter injury (eg: cystic periventricular leukomalacia [CPVL], periventricular hemorrhagic infarction, progressive ventriculomegaly, neonatal stroke), injury to deep gray matter structures including basal ganglia, cerebellar hemorrhage, and developmental brain malformations (eg: lissencephaly, microgyria, cortical dysplasia).

 

Neuromotor assessment:

In assessing the neuromotor function, 2 tools are considered the most sensitive and used ones. Them being:

• GMA: a tool which uses the pattern of spontaneous movement of infants that are altered in predictable way during normal development. There are 2 specific movement patterns that are highly sensitive and specific for the diagnosis of CP: first, cramped synchronous general movements (70% sensitivity, 90% specificity), characterized by rigid contraction and relaxation of the infant’s trunk and extremities; second, absent fidgety movements (97% sensitivity, 89% specificity) characterized by a lack of the normal small-amplitude fidgety movements that should be present in newborns 10 to 20 weeks of corrected age.
• HINE: according to this tool, it is a standardized neurological examination for age ranges from 2 to 24 months, which contain 26 scored items that assess an infant’s cranial nerve function, posture, movements, tone, and reflexes.

 

 

 

Complications:

As cerebral palsy is mainly a motor disease resulting from insult to the developing brain, skeletal muscles are the most affected organs, and one of the most common complications is muscle contracture- limited joint movement that result from high passive muscle force- which leads to inability to walk and pain. In addition to this, there are non-motor complications which include: epilepsy, which happens in nearly 40% of patients with cerebral palsy, growth retardation and failure to thrive due to oro-motor difficulties, gastroesophageal reflux disease, chronic lung disease, bladder control problems, hip displacement, sleep disturbances, intellectual disability, deafness, blindness, and osteopenia. It’s worth mentioning that respiratory complications, which are mainly due to aspiration and GERD, are responsible for the major morbidity and mortality rates(9).

 

Management:

The mainstay in the management of CP is the early diagnosis and intervention as it improves the outcomes later on. Beside improving the motor function, enhancing the quality of life for these patients should be always a priority in management(2).

There is no definitive cure for CP, and its management requires multidisciplinary approach concerned mainly with the symptoms(7).

Management of motor impairment:

Physiotherapy and occupational therapy play an essential part in the management of CP, and the most evidence based approaches include:

1- Bimanual training for hemiplegic CP in which the child is trained to use both hands at the same time to undergo repetitive tasks.

2. Constraint induced movement therapy includes restraint of the unaffected limb resulting in usage of affected limb during therapeutic tasks. Restraint may be achieved by the use of a cast or physical restraining by holding the normal limb.
3. Context focused therapy involves changing the environment rather than the child’s approach.
4. Goal directed functional training lays emphasis on activities based on goals set by the child, using motor learning approach.(5)

 

Management of spasticity:

This includes oral medications like: benzodiazepines, baclofen, and tizanidine; botulinum toxin-A; intrathecal baclofen; alcohol and phenol in addition to surgical and orthopedic procedures(5)

 

 

 

Feeding and nutrition:

If there is oro-motor problems interfering with growth and gaining weight other feeding techniques should be considered like gastrostomy feeding.(5)

 

Drooling:

Drooling (sialorrhea) is the uncontrolled spontaneous loss of saliva and oral content that occurs normally in infants until 24 months of age. After that the child should have the ability to do most activities without loss of saliva. It’s considered pathological if it carries on after the age of 4 years. It’s often seen with neurological disorders , including neuromuscular incoordination of swallowing and intellectual disabilities.(10)

Improving drooling is considered important as it interferes with social life, and different approaches could be advised starting from behavioral therapy to pharmacotherapy (anticholinergic drugs (trihexphenidyl or glycopyrrolate and BTX-A injection into salivary glands) and surgery(5).

 

 

Prevention:

 

Magnesium sulfate:

Various studies showed increase in the risk of CP in infants who were born before 32 weeksof gestational age and received MgSO4 during labor, as it is found to be a neuroprotective agent.(3)

 

Therapeutic hypothermia:

Neonates born after the 35th week of gestation that were exposed to a perinatal episode of hypoxia which may lead to perinatal hypoxic-ischemic encephalopathy, and hence causing CP, benefited from therapeutic hypothermia. Therapeutic hypothermia was found to improve the survival rate with normal neurological development and decrease the risk of death and major neurological disorders at the age of 18 months.

Therapeutic hypothermia must be implemented in the first 6 hours postpartum.

Indications for hypothermia [35]:

• pH ≤ 7.0 and/or BE base deficiency of at least 16 mmol/l in a cord blood sample or a blood sample taken within 1 hour of life;
• 1 of the following:
• Apgar score ≤ 5 in 1st, 3rd, 5th, 10th minute after birth;
• Continuous resuscitation within 10 minutes after birth

 

• neurological symptoms indicative of moderate/severe

encephalopathy on clinical examination (the Sarnat scale is used for qualification)(11)

 

Caffeine:

Caffeine is one of the most commonly used therapies in neonatal intensive care units for apnea. There are some studies which are concerned in the use of caffeine in premature infants with apneic episodes resulted in a reduction in the risk of motor and cognitive impairment along with the treatment. The finding was statistically significant at 18 months but not at 5 years.(3)

 

Antenatal steroids:

Studies are still debating about the relation between the use of antenatal steroids and the reduction of cerebral palsy risk. In spite of that, some observational studies indicate the clinical benefits of a single course of antenatal steroid therapy in reducing the risk of CP diagnosed in the first year of life or later, the percentage of CP diagnoses was 32% lower in patients whose mothers received a course of steroid therapy during pregnancy that was terminated prematurely.(11)

 

 

Prognosis:

Most of the children diagnosed with CP survive to adulthood, and many factors play a role in the upcoming consequences like: severity of impairment, birth weight, and socioeconomic status.

For children who cannot achieve their milestones (mainly head balance by 20 months of age, persistent primitive reflexes, or do not crawl by age of 5), the probability of being able to walk is very low. Whereas children who can sit by age of 2 years or crawl by age of 5, the prognosis for walking is good.

Besides the motor outcome, there is the functional outcome in CP which relies on multiple factors these include: intelligence, physical function, ability to communicate, and personality attributes. Social (e.g., family support) and environmental factors and the availability of specialist medical care have an essential impact.(5)

 

 

 

 

 

 

 

 

 

 

 

References:

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3. Korzeniewski SJ, Slaughter J, Lenski M, Haak P, Paneth N. The complex aetiology of cerebral palsy. Nat Rev Neurol. 2018 Sep;14(9):528–43.
4. McIntyre S. The continually changing epidemiology of cerebral palsy. Acta Paediatr Int J Paediatr. 2018;107(3):374–5.
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8. Dean BE. news / clinical update. 2017;29(3):2017.
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11. Mlodawski J, Mlodawska M, Pazera G, Michalski W, Domanski T, Dolecka-Slusarczyk M, et al. Cerebral palsy and obstetric-neonatological interventions. Ginekol Pol. 2019;90(12):722–7.