congenital spastic paraplegia


con·gen·i·tal spas·tic par·a·ple·gi·a

a type of cerebral palsy characterized by spastic paralysis of the lower extremities. Synonym(s): infantile spastic paraplegia, Little disease

paraplegia

(par-a-ple'j(e-)a) [ para- + -plegia] Paralysis of the lower portion of the body and of both legs. It is caused by a lesion involving the spinal cord that may be due to maldevelopment, epidural abscess, hematomyelia, acute transverse myelitis, spinal neoplasms, multiple sclerosis, syringomyelia, or trauma.

Patient care

Patient care during the acute period, immediately following traumatic injury, aims at stabilizing the patient and preventing further injury or deterioration. Initial and ongoing neurological assessment by nurses, the neurologist, and the neurosurgeon helps to determine the level and degree of paralysis and the patient's potential for recovery. Supportive medical therapy, based on assessment results, is provided. Specific medical, neurological, and neurosurgical interventions depend on the etiology of the paraplegia. Prescribed therapies are administered, and desired and adverse effects assessed for.

The patient should have early consultations with physical and occupational therapy staff, because correct body alignment, positioning, and exercise can prevent complications, encouraging the patient to think about rehabilitation from the beginning. The respiratory therapist also is involved early on to monitor ventilatory activity and help prevent respiratory complications. If intensive care is required, the health care provider recognizes the need to limit sensory overload by controlling and moderating environmental stimuli and to avoid sleep deprivation by planning an uninterrupted sleep time. Because immobility affects all body systems, they must each be monitored for expected and complicating changes. Medical consultations (as with a pulmonologist or a urologist) are made as necessary, and treatment regimens are developed based on each patient's needs.

The patient experiences paraplegia as a profound loss, affecting not only independent mobility but also self-image and self-esteem. Although the loss may be sudden or gradual, predictable or unexpected, and temporary or permanent, depending on the cause of the patient's paraplegia, it is present nevertheless. Because family members also are affected, the health care provider includes them when helping the patient with grief work and mourning, recognizing that anger and despondency are expected responses. Referral to a mental health care provider can help patients cope with their loss.

Patients with paraplegia are usually transferred to a rehabilitation facility once the acute period has passed. This move often engenders transfer anxiety, as the patient and family fear a lesser level of care as a threat to security and well-being. Behavioral and psychosomatic manifestations may occur. A liaison nurse from the new facility can help the patient bridge the transition by providing information about the facility and the vigorous program the patient will encounter. The family should be encouraged to visit the facility and to bring any questions or concerns to their liaison, while giving the patient positive input.

Rehabilitation requires the patient's active participation to achieve his or her highest potential, and this participation begins with planning. The patient's individualized plan of care should be developed by the entire rehabilitation team, which includes the patient and significant others who make up the support system, as well as the primary physician, nurse, physiatrist, physical therapist, occupational therapist, vocational counselor, dietitian, social worker, psychologist, and neuropsychologist. The goals of the plan include learning to manage neuromuscular deficits and being able to perform activities of daily living (ADLs) with enough independence to function successfully in the home, workplace, and social situations. Activities include proper positioning, range-of-motion exercises, balancing and sitting, transfer activities, ambulation, and use of equipment to aid ambulation (if the patient will be able to walk with the aid of braces, canes, or crutches) or adjustment to being in a wheelchair. Skin care is of great importance, as persons with paraplegia are at risk for pressure sore development because of their motor, sensory, and vasomotor deficits. Poor nutrition, infection, debilitation, edema, and prolonged immobility are contributing factors. Assessment and prevention of breakdown, as well as treatment of most areas of broken skin, fall within the purview of nursing, although severe pressure sores may require surgical débridement and plastic surgery.

Cystometric studies help to assess bladder function and determine the patient's ability to participate in a bladder-retraining program, as opposed to requiring catheter or condom-catheter drainage methods. Bowel incontinence also demands assessment of cause and contributing factors (autonomic dysfunction, sacral injury, immobility, decreased food intake, esp. roughage). Incontinence is managed matter-of-factly, getting the patient involved, observing behavioral cues related to the need for defecation, noting defecation habits and using them for appropriate toileting, and supporting the patient's self-esteem. Bowel retraining involves establishing and maintaining a defecation routine. All members of the rehabilitation team, but esp. mental health care providers, are involved in helping the patient and family cope with the lifestyle changes necessitated by the illness or injury. Psychosocial care begins with hearing the patient's and family's perceptions of the impact of the disability and their expectations for the future, and learning about their personalities, previous coping abilities, and previous adjustment patterns.

The adjustment to discharge to home or group living adds its own set of transfer anxieties. The team teaches the patient and family any special procedures they will need and determines home and vehicle modifications needed to provide access for wheelchair or other necessary equipment. Group sessions with others who have faced similar situations often help both the patient and family. Initiating the move with a “weekend pass, ” followed by a return to process feelings and activities, can also help. It is important to note that rehabilitation, instead of ending with discharge, is an ongoing process central to living a worthwhile life. Involvement in paraplegic group activities including a variety of sports (wheelchair basketball, swimming) helps patients to focus on what they can learn, enjoy, and accomplish.

alcoholic paraplegia

Alcoholic paralysis.

ataxic paraplegia

Lateral and posterior sclerosis of the spinal cord characterized by slowly progressing ataxia and paresis.

cerebral paraplegia

Paraplegia from a bilateral cerebral lesion.

congenital spastic paraplegia

Infantile spastic paraplegia.

paraplegia dolorosa

Paraplegia due to pressure of a neoplasm on the posterior spinal cord and nerve roots; extremely painful despite paralysis.

hereditary spastic paraplegia

Any of a group of inherited diseases of the central nervous system characterized by muscle spasticity, esp. of the lower extremities. Synonym: Troyer syndrome

infantile spastic paraplegia

Spastic paraplegia that occurs in infants, usually due to birth injury. Synonym: congenital spastic paraplegia

peripheral paraplegia

Paraplegia due to pressure on, injury to, or disease of peripheral nerves.

Pott paraplegia

See: Pott, John Percivall

primary spastic paraplegia

Paraplegia from degeneration in corticospinal tracts.

senile paraplegia

Paraplegia resulting from sclerosis of arteries supplying the spinal cord.

spastic paraplegia

Spastic paralysis.

superior paraplegia

Paralysis of both arms.

tetanoid paraplegia

Spastic paraplegia.