Spastic hemiplegia

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.^[1] 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. The affected side of the body is rigid, weak and has low functional abilities.^[2] 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 suffer 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.^[1]

Causes

There are many different brain disfunctions that can account for the cause for spastic hemiplegia. Spastic hemiplegia occurs either at birth or in the womb. The cause can be all types of strokes, head injuries, hereditary diseases, brain injuries and infections.^[3] Malformations of the veins or arteries in any part of the body can lead to spastic hemiplegia. The artery most commonly affected is the middle cerebral artery. Unborn and newborn babies are susceptible to strokes.^[1] Leukodystrophies are a group of hereditary diseases that are known to cause spastic hemiplegia. Brain infections that cause spastic hemiplegia are meningitis, multiple sclerosis, and encephalitis.^[4] The spasticity occurs when the afferent pathways in the brain are compromised and the communication between the brain to the motor fibers is lost. When the inhibitory signals to deactivate the stretch reflex is lost the muscle remains in a constant contracted state. With spastic hemiplegia, one upper extremity and one lower extremity is affected, so cervical, lumbar and sacral segments of the spinal column can be affected.^[1]

Complications

The muscle spasticity can cause gait patterns to be awkward and jerky. The constant spastic state of the muscle can lead to bone and tendon deformation, further complicating the patient's mobility. Many patients with spastic hemiplegia are subjected to canes, walkers and even wheelchairs. Due to the decrease in weight bearing, patients are at a higher risk of developing osteoporosis.^[5] An unhealthy weight can further complicate mobility. Patients with spastic hemiplegia are a high risk for experiencing seizures.^[6] Oromotor dysfunction puts patients at risk for aspiration pneumonia. Visual field deficits can cause impaired two-point discrimination. Many patients experience the loss of sensation in the arms and legs on the affected side of the body.^[5] Nutrition is essential for the proper growth and development for a child with spastic hemiplegia.

Epidemiology

Spastic Hand

The incidence of cerebral palsy has increased in the past 40 years. It has been estimated that in the United States cerebral palsy occurs in 4 out every 1000 births.^[7] Of these births about 20–30% of them have spastic hemiplegia. Spasticity overall, is the more common type of cerebral palsy, whereas as non-spastic cerebral palsy is less common. Studies show that spastic type cerebral palsy is on the rise, and the occurrence of diplegia type is decreasing. The prevalence of cerebral palsy is higher in areas of low socioeconomic status. This could potentially be because cerebral palsy incidence increases as birth weight decreases.^[8]

Treatment and physical therapy

There is no known cure for cerebral palsy, however there is a large array of treatments proven effective at improving quality of life and relieving some of the symptoms associated with CP, especially SHCP. Some treatments are aimed at improving mobility, strengthening muscle and improving coordination. Although CP is due to permanent damage and is not progressive in nature, without treatment the symptoms can become worse, intensifying in pain and severity, and create complications that were not initially present. Some treatments are preventative measures to help prevent further complications, such as complete paralysis of the arm due to non-use and subsequent worsening hypertonia and joint contracture. Others forms of treatment are corrective in nature. Many treatments target symptoms that are indirectly related to or caused by the SHCP. Many of these treatments are common for other forms of CP as well.^[9] Treatment is individualized based on each case and the specific needs of the patient. Treatments are often combined with other forms of treatment and a long term treatment plan is created and continuously evaluated. Treatment can include the following:

• Physical therapy – Physical therapy is the most common form of treatment. It may include sensory stimulation, stretching, strengthening and positioning. Constraint-induced movement therapy is a newer form of physical therapy for SHCP that involves casting or splinting the unaffected arm to promote use of the affected arm (Taub). The theory behind constraint-induced movement therapy is that new neural pathways are created. Alternative forms of physical therapy include yoga and dance. Physical therapy may also include the use of braces while not actively involved with the therapist.
• Occupational therapy – Usually performed in conjunction with physical therapy, occupational therapy evaluates and treats patients through selected activities in order to enable people to function as effectively and independently as possible in daily life. Occupational therapy is geared toward the individual to achieve optimal results and performance while learning to cope with their disability.
• Speech therapy – Due to difficulties in speech, speech therapy is often necessary. Aside from helping with understanding language and increasing communication skills, speech therapists can also assist children that have difficulty eating and drinking.
• Behavioral therapy — Psychotherapy and counseling are heavily used in treatment of individuals with SHPD to help them cope emotionally with their needs and frustrations. Counseling through social work can be very beneficial for social issues and adjustments to society. Psychotherapy becomes a more important aspect of therapy when more serious issues such as depression become problematic. Play therapy is a common treatment for all young children with or without disabilities, but can be very useful helping children with SHCP. This therapy again is individualized geared to improve emotional and social development; reduce aggression; improve cooperation with others; assist a child in processing a traumatic event or prepare for an upcoming event such as surgery.
• Surgery – Although surgery may become necessary in some cases, physical therapy and the consistent use of braces can help mitigate the need for surgery. Surgical procedures are painful with long and difficult recoveries and do not cure the condition. Most common, is surgery that effectively lengthens the muscle. This type of surgery is usually performed on the legs, but can be performed on the arms as well. Surgeries also may be necessary to realign joins. Other, less popular surgical techniques try to reduce spasticity by severing selected overactive nerves that control muscles. This procedure, known as selective dorsal root rhizotomy, is still somewhat controversial, and is generally used only on the lower extremities of severe cases. Other experimental surgical techniques are also being investigated. The benefits of surgery can also be negated or reversed if the patient does not participate in physical therapy and braces (or casts) are not worn regularly.
• Medicinal – Medication targeting symptoms associated with spasticity is also a relatively new treatment that is utilized, but is still in the early stages of development. Drugs such as baclofen, benzodiazepines (e.g.,diazepam), tizanidin, and sometimes dantrolene have shown promise in the effort to diminish spasticity.^[10] Botulinum toxin ("Botox") type A may reduce spasticity a few months at a time and has frequently been considered a beneficial treatment for children with SHCP and other forms of CP. Botox has been shown to be especially beneficial to reducing spasticity in the gastrocnemius (calf) muscle. This therapy can improve range of motion, reduce deformity, improve response to occupational and physical therapy, and delay the need for surgery. Botox injections have also shown advantages for upper extremities. There is still some doubt for the effectiveness, and some side effects to the relaxed muscles have been a loss of strength for patients with some muscle control. Casting, in conjunction with Botox injections may be an additional option for better results.^[10] Research is constantly investing in new improvements and more experimental therapy and treatment.

References

1. Cerebral palsy at eMedicine
2. Brashear, Allison (2010). Spasticity: Diagnosis and Management. New York: Demos Medical.
3. Tardieu C, Huet de la Tour E, Bret MD, Tardieu G (March 1982). "Muscle hypoextensibility in children with cerebral palsy: I. Clinical and experimental observations". Arch Phys Med Rehabil. 63 (3): 97–102. PMID 7073456.
   Tardieu G, Tardieu C, Colbeau-Justin P, Lespargot A (March 1982). "Muscle hypoextensibility in children with cerebral palsy: II. Therapeutic implications". Arch Phys Med Rehabil. 63 (3): 103–7. PMID 7073451.
4. Green LB, Hurvitz EA (November 2007). "Cerebral palsy". Phys Med Rehabil Clin N Am. 18 (4): 859–82, vii. doi:10.1016/j.pmr.2007.07.005. PMID 17967366.
5. Cerebral Palsy~clinical at eMedicine
6. el-Abd MA, Ibrahim IK (March 1994). "Impaired afferent control in patients with spastic hemiplegia at different recovery stages: contribution to gait disorder". Arch Phys Med Rehabil. 75 (3): 312–7. doi:10.1016/0003-9993(94)90035-3. PMID 8129585.
7. http://www.cdc.gov/ncbddd/cp/data.html
8. Odding E, Roebroeck ME, Stam HJ (February 2006). "The epidemiology of cerebral palsy: incidence, impairments and risk factors". Disabil Rehabil. 28 (4): 183–91. doi:10.1080/09638280500158422. PMID 16467053.
9. Miller, Freeman; Steven J. Bachrach (eds.) (2006). Cerebral Palsy: The Complete Guide for Caregiving 2nd Edition. Baltimore: Johns Hopkins University Press. ISBN 0-8018-8354-7. Cite uses deprecated parameter |coauthors= (help)
10. Taub E, Ramey SL, DeLuca S, Echols K (February 2004). "Efficacy of constraint-induced movement therapy for children with cerebral palsy with asymmetric motor impairment". Pediatrics. 113 (2): 305–12. doi:10.1542/peds.113.2.305. PMID 14754942.