Spastic cerebral palsy

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Spastic cerebral palsy
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A child with spastic cerebral palsy
Specialty Neurology

Spastic cerebral palsy is the type of cerebral palsy characterized by spasticity or high muscle tone often resulting in stiff, jerky movements. [1] Cases of spastic CP are further classified according to the part or parts of the body that are most affected. [2] Such classifications include spastic diplegia, spastic hemiplegia, spastic quadriplegia, and in cases of single limb involvement, spastic monoplegia. [3]

Contents

Spastic cerebral palsy affects the motor cortex [1] of the brain, a specific portion of the cerebral cortex responsible for the planning and completion of voluntary movement. [4] Spastic CP is the most common type of overall cerebral palsy, representing roughly 80% of cases. [2] Spastic CP is a permanent condition and will affect an individual across the lifespan. [5] The brain injury that causes spastic CP remains stable over time, but the way spasticity affects a person can change. [1] For example, with age they may develop bone deformities from the pull of spastic muscles, muscular deterioration, and loss of range of motion in a joint. [1] Thus, individuals with spastic CP often have different support needs with time. [6]

Signs and symptoms

People with the spastic type of CP typically have muscles that are "tight" or stiff due to high muscle tone. [2] Symptoms of spastic cerebral palsy vary as the disability can affect individuals differently. [2] However, they typically appear in infancy and early childhood and most children are diagnosed in the first two years of life. [7] The main indicator of spastic cerebral palsy is a delay in reaching motor milestones. [2] The following are some common early signs, though the presence of a listed symptom does not definitively mean that a child has spastic CP: [2]

Prior to 6 months

6–10 months

Older than 12 months of age

Spastic CP is distinguished from other forms of cerebral palsy by its prominent symptom of spasticity or stiff, tight movements and gait patterns such as the scissor gait. [1] However, spasticity as a symptom is also seen in other conditions such as stroke and multiple sclerosis. [1] Thus, the presence of spasticity alone does not warrant a conclusive diagnosis of spastic cerebral palsy.

Changes in spasticity and corresponding postures may also occur with other brain activity, such as excitement, fear or anxiety, or even pain, which increase muscle tension.[ citation needed ]

A person with spastic CP will commonly show, in addition to higher muscle tone, persistent primitive reflexes, greater stretch reflexes, plantar reflex, and ankle clonus. [10]

A third of people with cerebral palsy have seizures - this is most common in spastic CP. [11] Audiovisual, cognitive compromise and behavioral disorders can occur.

Cause

Spastic cerebral palsy is caused by malformation of or damage to the parts of the brain that control movement. [12] What exactly makes some children susceptible to such brain damage is often unknown but it is believed that cerebral palsy may be the result of causal pathways, or chains of events that cause or increase the likelihood of brain injury. [13] Most of the time, children are born with the brain damage resulting in spastic cerebral palsy, but a small percentage experience the damage shortly after birth following a stroke, head injury, or infection. [12]

The following are types of brain damage that can result in spastic cerebral palsy:

Risk factors

The following medical conditions are associated with increased likelihood of spastic cerebral palsy, as well as with other types of cerebral palsy:

Diagnosis

There is no single test to diagnose spastic cerebral palsy. It is typically diagnosed by age 2, though milder cases may go undetected for a longer time. [12] A doctor will typically use a series of tests to assess developmental progress in growth, muscle control, coordination, vision, hearing, and posture. [12] Continuous loss of motor skills likely indicates a condition other than spastic CP such as a genetic muscle disease [12] Some metabolic disorders mimic spastic cerebral palsy and can be ruled out using a magnetic resonance imaging test (MRI). [12]

Some tests to assess a child suspected to have spastic cerebral palsy are:

These tests are imaging techniques used to produce pictures of the brain and to examine them for areas of damage that indicate a diagnosis of spastic cerebral palsy. [12] A child with a spastic CP diagnosis will likely be referred to screening for other conditions associated with spastic cerebral palsy such as epilepsy (seizure disorder), intellectual disability, and visual impairment. [14]

Types

Scientific classifications

The types of spastic cerebral palsy are generally distinguished by the primary areas of the body that are affected. [2]

  • Spastic hemiplegia Hemiplegia is a type of cerebral palsy affecting one vertical half of the body (such as one arm and one leg). [15] [16] The affected side of the body is opposite the affected area of the brain in hemiplegia. [15] [16] For example, if the right side of the brain is damaged, the person will have weakness or paralysis on the left side of the body. [15] [16] People with hemiplegia typically favor an arm or hand and may keep the weaker hand in a fist. [15] [16] Typically, people that have spastic hemiplegia are the most ambulatory of all the forms, although they generally have dynamic equinus (a limping instability) on the affected side and are primarily prescribed ankle-foot orthoses to prevent said equinus. [17]
  • Spastic diplegia describes spastic CP in which muscle tightness predominantly occurs in the legs. [2] The arms may be somewhat affected or not affected at all. [2]
  • Spastic monoplegia is one single limb being affected. [3]
  • Spastic triplegia is three limbs being affected. [18]
  • Spastic quadriplegia is all four limbs more or less equally affected. [19] People with spastic quadriplegia are rarely able to ambulate without assistance and may not stand at all. [8] Among the forms of spastic CP, quad CP is more likely to co-occur with seizures and mild to moderate intellectual disability. [8]

In spastic cerebral palsy in children with low birth weights, 25% of children had hemiplegia, 37.5% had quadriplegia, and 37.5% had diplegia. [20] No one person with a particular type of spastic CP presents in exactly the same way.

Treatment

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Baclofen

There is no cure for spastic cerebral palsy and no way to reverse the initial brain injury. [21] However, various treatments and assistive devices can help mitigate pain and allow those affected to have a higher quality of life. Different combinations of treatments are right for each individual and are determined by individuals and their care team.[ citation needed ]

Some common interventions are:

Prognosis

Every case of spastic cerebral palsy is different. Some people with this type of CP can accomplish activities of daily living independently, while some need the assistance of a personal care aide for certain tasks, and others need 24-hour support. [22] About 1/3 of people with cerebral palsy cannot walk, about 1/2 have intellectual disabilities, and 3/4 experience some level of chronic pain. [22] There is limited research on adults with spastic CP. [23] The brain injury causing CP does not progress or change, but the functional impact and thus, one's needs may change with age. [7]

Some issues specific to adults with spastic and other forms of cerebral palsy are:

Regardless of their particular expression of spastic cerebral palsy, affected individuals can lead fulfilling lives. Improved physical accessibility in society and increased social acceptance can increase community participation. [24] Workforce participation of people with CP has almost doubled in the last four decades. [25] Social inclusion and the presence or absence of policy to address discrimination, in addition to medical intervention, influences the outlook for people living with spastic cerebral palsy. [24]

See also

Related Research Articles

Hemiparesis, or unilateral paresis, is weakness of one entire side of the body. Hemiplegia is, in its most severe form, complete paralysis of half of the body. Hemiparesis and hemiplegia can be caused by different medical conditions, including congenital causes, trauma, tumors, or stroke.

<span class="mw-page-title-main">Cerebral palsy</span> Group of movement disorders that appear in early childhood

Cerebral palsy (CP) is a group of movement disorders that appear in early childhood. Signs and symptoms vary among people and over time, but include poor coordination, stiff muscles, weak muscles, and tremors. There may be problems with sensation, vision, hearing, and speaking. Often, babies with cerebral palsy do not roll over, sit, crawl or walk as early as other children of their age. Other symptoms include seizures and problems with thinking or reasoning, each of which occur in about one-third of people with CP. While symptoms may get more noticeable over the first few years of life, underlying problems do not worsen over time.

Spasticity is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles.

<span class="mw-page-title-main">Tetraplegia</span> Paralysis of all four limbs and torso

Tetraplegia, also known as quadriplegia, is defined as the dysfunction or loss of motor and/or sensory function in the cervical area of the spinal cord. A loss of motor function can present as either weakness or paralysis leading to partial or total loss of function in the arms, legs, trunk, and pelvis; paraplegia is similar but affects the thoracic, lumbar, and sacral segments of the spinal cord and arm function is spared. The paralysis may be flaccid or spastic. A loss of sensory function can present as an impairment or complete inability to sense light touch, pressure, heat, pinprick/pain, and proprioception. In these types of spinal cord injury, it is common to have a loss of both sensation and motor control.

Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spastic cerebral palsy are sometimes used to treat HSP symptoms.

Monoplegia is paralysis of a single limb, usually an arm. Common symptoms associated with monoplegic patients are weakness, numbness, and pain in the affected limb. Monoplegia is a type of paralysis that falls under hemiplegia. While hemiplegia is paralysis of half of the body, monoplegia is localized to a single limb or to a specific region of the body. Monoplegia of the upper limb is sometimes referred to as brachial monoplegia, and that of the lower limb is called crural monoplegia. Monoplegia in the lower extremities is not as common of an occurrence as in the upper extremities. Monoparesis is a similar, but less severe, condition because one limb is very weak, not paralyzed. For more information, see paresis.

Pachygyria is a congenital malformation of the cerebral hemisphere. It results in unusually thick convolutions of the cerebral cortex. Typically, children have developmental delay and seizures, the onset and severity depending on the severity of the cortical malformation. Infantile spasms are common in affected children, as is intractable epilepsy.

Hypertonia is a term sometimes used synonymously with spasticity and rigidity in the literature surrounding damage to the central nervous system, namely upper motor neuron lesions. Impaired ability of damaged motor neurons to regulate descending pathways gives rise to disordered spinal reflexes, increased excitability of muscle spindles, and decreased synaptic inhibition. These consequences result in abnormally increased muscle tone of symptomatic muscles. Some authors suggest that the current definition for spasticity, the velocity-dependent over-activity of the stretch reflex, is not sufficient as it fails to take into account patients exhibiting increased muscle tone in the absence of stretch reflex over-activity. They instead suggest that "reversible hypertonia" is more appropriate and represents a treatable condition that is responsive to various therapy modalities like drug or physical therapy.

Spastic diplegia is a form of cerebral palsy (CP) that is a chronic neuromuscular condition of hypertonia and spasticity—manifested as an especially high and constant "tightness" or "stiffness"—in the muscles of the lower extremities of the human body, usually those of the legs, hips and pelvis. Doctor William John Little's first recorded encounter with cerebral palsy is reported to have been among children who displayed signs of spastic diplegia.

Diplegia, when used singularly, refers to paralysis affecting symmetrical parts of the body. This is different from hemiplegia which refers to spasticity restricted to one side of the body, paraplegia which refers to paralysis restricted to the legs and hip, and quadriplegia which requires the involvement of all four limbs but not necessarily symmetrical. Diplegia is the most common cause of crippling in children, specifically in children with cerebral palsy. Other causes may be due to injury of the spinal cord. There is no set course of progression for people with diplegia. Symptoms may get worse but the neurological part does not change. The primary parts of the brain that are affected by diplegia are the ventricles, fluid filled compartments in the brain, and the wiring from the center of the brain to the cerebral cortex. There is also usually some degeneration of the cerebral neurons, as well as problems in the upper motor neuron system. The term diplegia can refer to any bodily area, such as the face, arms, or legs.

A selective dorsal rhizotomy (SDR), also known as a rhizotomy, dorsal rhizotomy, or a selective posterior rhizotomy, is a neurosurgical procedure that selectively cut problematic nerve roots of the spinal cord. This procedure has been well-established in the literature as a surgical intervention and is used to relieve negative symptoms of neuromuscular conditions such as spastic diplegia and other forms of spastic cerebral palsy. The specific sensory nerves inducing spasticity are identified using electromyographic (EMG) stimulation and graded on a scale of 1 (mild) to 4. Abnormal nerve responses are isolated and cut, thereby reducing symptoms of spasticity.

Spastic quadriplegia, also known as spastic tetraplegia, is a subset of spastic cerebral palsy that affects all four limbs.

Scissor gait is a form of gait abnormality primarily associated with spastic cerebral palsy. That condition and others like it are associated with an upper motor neuron lesion.

<span class="mw-page-title-main">Management of cerebral palsy</span>

Over time, the approach to cerebral palsy management has shifted away from narrow attempts to fix individual physical problems – such as spasticity in a particular limb – to making such treatments part of a larger goal of maximizing the person's independence and community engagement. Much of childhood therapy is aimed at improving gait and walking. Approximately 60% of people with CP are able to walk independently or with aids at adulthood. However, the evidence base for the effectiveness of intervention programs reflecting the philosophy of independence has not yet caught up: effective interventions for body structures and functions have a strong evidence base, but evidence is lacking for effective interventions targeted toward participation, environment, or personal factors. There is also no good evidence to show that an intervention that is effective at the body-specific level will result in an improvement at the activity level, or vice versa. Although such cross-over benefit might happen, not enough high-quality studies have been done to demonstrate it.

<span class="mw-page-title-main">Athetoid cerebral palsy</span> Type of cerebral palsy associated with basal ganglia damage

Athetoid cerebral palsy, or dyskinetic cerebral palsy, is a type of cerebral palsy primarily associated with damage, like other forms of CP, to the basal ganglia in the form of lesions that occur during brain development due to bilirubin encephalopathy and hypoxic–ischemic brain injury. Unlike spastic or ataxic cerebral palsies, ADCP is characterized by both hypertonia and hypotonia, due to the affected individual's inability to control muscle tone. Clinical diagnosis of ADCP typically occurs within 18 months of birth and is primarily based upon motor function and neuroimaging techniques. While there are no cures for ADCP, some drug therapies as well as speech, occupational therapy, and physical therapy have shown capacity for treating the symptoms.

<span class="mw-page-title-main">Ataxic cerebral palsy</span> Medical condition

Ataxic cerebral palsy is clinically in approximately 5–10% of all cases of cerebral palsy, making it the least frequent form of cerebral palsy diagnosed. Ataxic cerebral palsy is caused by damage to cerebellar structures, differentiating it from the other two forms of cerebral palsy, which are spastic cerebral palsy and dyskinetic cerebral palsy.

<span class="mw-page-title-main">Spastic hemiplegia</span> Medical condition

Spastic hemiplegia is a neuromuscular condition of spasticity that results in the muscles on one side of the body being in a constant state of contraction. It is the "one-sided version" of spastic diplegia. It falls under the mobility impairment umbrella of cerebral palsy. About 20–30% of people with cerebral palsy have spastic hemiplegia. Due to brain or nerve damage, the brain is constantly sending action potentials to the neuromuscular junctions on the affected side of the body. Similar to strokes, damage on the left side of the brain affects the right side of the body and damage on the right side of the brain affects the left side of the body. Other side can be effected for lesser extent. The affected side of the body is rigid, weak and has low functional abilities. In most cases, the upper extremity is much more affected than the lower extremity. This could be due to preference of hand usage during early development. If both arms are affected, the condition is referred to as double hemiplegia. Some patients with spastic hemiplegia only experience minor impairments, where in severe cases one side of the body could be completely paralyzed. The severity of spastic hemiplegia is dependent upon the degree of the brain or nerve damage.

Cerebral palsy sport classification is a classification system used by sports that include people with cerebral palsy (CP) with different degrees of severity to compete fairly against each other and against others with different types of disabilities. In general, Cerebral Palsy-International Sports and Recreation Association (CP-ISRA) serves as the body in charge of classification for cerebral palsy sport, though some sports have their own classification systems which apply to CP sportspeople.

Spastic paraplegia 15 (SPG15) is a form of hereditary spastic paraplegia that commonly becomes apparent during childhood or adolescence. The disease is caused by mutations within the ZFYVE26 gene - also known as the SPG15 gene - and is passed down in an autosomal recessive manner.

References

  1. 1 2 3 4 5 6 "Spastic cerebral palsy". Cerebral Palsy Alliance. 2015-11-18. Retrieved 2020-03-10.{{cite web}}: CS1 maint: url-status (link)
  2. 1 2 3 4 5 6 7 8 9 "What is Cerebral Palsy?". Centers for Disease Control and Prevention. 2019-04-30. Retrieved 2020-03-04.
  3. 1 2 "Cerebral palsy spastic monoplegic". Genetic and Rare Diseases Information Center (GARD). Retrieved 2020-03-10.
  4. ↑ Knierim, James (2020). "Chapter 3: The motor cortex". Neuroscience online: An electronic textbook of the neurosciences from the University of Texas at Houston. Retrieved 4 March 2020.{{cite web}}: CS1 maint: url-status (link)
  5. ↑ "Spastic diplegia cerebral palsy". Genetic and Rare Diseases Information Center (GARD). 7 August 2018. Retrieved 2020-03-10.
  6. ↑ Haak, Peterson; Lenski, Madeleine; Hidecker, Mary Jo Cooley; Li, Min; Paneth, Nigel (2009). "Cerebral palsy and aging". Developmental Medicine and Child Neurology. 51 (4): 16–23. doi:10.1111/j.1469-8749.2009.03428.x. ISSN   0012-1622. PMC   4183123 . PMID   19740206.
  7. 1 2 Shrader, MD, M. Wade; Salzbrenner, APRN, Margaret. "Spastic cerebral palsy (for parents)". Kidshealth. Retrieved 16 March 2020.{{cite web}}: CS1 maint: url-status (link)
  8. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 "Cerebral Palsy: Hope Through Research | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2020-03-16.
  9. ↑ "Cerebral palsy - Symptoms and causes". Mayo Clinic. Retrieved 2020-03-20.
  10. ↑ Sobralske, Mary C. (2013). "Common Physical or Sensory Disabilities". In Eddy, Linda L. (ed.). Caring for children with special healthcare needs and their families a handbook for healthcare professionals. Ames, Iowa: Wiley-Blackwell. p. 13. ISBN   9781118783290.
  11. ↑ Shaikh, SafiyaImtiaz; Hegade, Ganapati (2017). "Role of anesthesiologist in the management of a child with cerebral palsy". Anesthesia: Essays and Researches. 11 (3): 544–549. doi:10.4103/0259-1162.194569. PMC   5594763 . PMID   28928544.
  12. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 "Cerebral Palsy: Hope Through Research | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 2020-03-23.
  13. ↑ "What Causes Cerebral Palsy? | Cerebral Palsy Alliance". Cerebral Palsy Alliance. Retrieved 2020-03-24.{{cite web}}: CS1 maint: url-status (link)
  14. ↑ "How does cerebral palsy affect people? | Cerebral Palsy Alliance". Cerebral Palsy Alliance. Retrieved 2020-03-23.{{cite web}}: CS1 maint: url-status (link)
  15. 1 2 3 4 5 6 7 Farag SM, Mohammed MO, El-Sobky TA, ElKadery NA, ElZohiery AK (March 2020). "Botulinum Toxin A Injection in Treatment of Upper Limb Spasticity in Children with Cerebral Palsy: A Systematic Review of Randomized Controlled Trials". JBJS Reviews. 8 (3): e0119. doi: 10.2106/JBJS.RVW.19.00119 . PMC   7161716 . PMID   32224633.
  16. 1 2 3 4 "Hemiplegia". CHASA. Retrieved 2020-03-31.
  17. ↑ Birol Balaban, Evren Yasar, Ugur Dal, Kamil Yazicioglu, Haydar Mohur & Tunc Alp Kalyon "The effect of hinged ankle-foot orthosis on gait and energy expenditure in spastic hemiplegic cerebral palsy" Disability and Rehabilitation, January 2007; 29(2): 139–144
  18. ↑ Panteliadis, Christos P.; Strassburg, Hans-Michael (2004). Cerebral Palsy: Principles and Management. Thieme. p. 19. ISBN   9781588902955 . Retrieved 15 January 2017.
  19. ↑ "Cerebral palsy spastic quadriplegic | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 15 January 2017.
  20. ↑ O’Shea, TM (2008). "Diagnosis, Treatment, and Prevention of Cerebral Palsy". Clin Obstet Gynecol. 51 (4): 816–28. doi:10.1097/GRF.0b013e3181870ba7. PMC   3051278 . PMID   18981805.
  21. 1 2 3 4 5 "What are common treatments for cerebral palsy?". National Institute of Child Health and Human Development. Retrieved 2020-04-05.{{cite web}}: CS1 maint: url-status (link)
  22. 1 2 "How does cerebral palsy affect people? | Cerebral Palsy Alliance". Cerebral Palsy Alliance. Retrieved 2020-04-05.{{cite web}}: CS1 maint: url-status (link)
  23. ↑ Gyoung Yi, MD, You; Jung, MD, Se Hee; Bang, MD, Moon Suk (28 June 2019). "Emerging Issues in Cerebral Palsy Associated With Aging: A Physiatrist Perspective". Annals of Rehabilitation Medicine. 43 (3): 241–249. doi:10.5535/arm.2019.43.3.241. PMC   6637058 . PMID   31311245.
  24. 1 2 CDC (2019-09-04). "Disability and Health Inclusion Strategies | CDC". Centers for Disease Control and Prevention. Retrieved 2020-04-05.
  25. ↑ "Cerebral Palsy Outlook / Prognosis". Cleveland Clinic. Retrieved 2020-04-05.
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