Spinal cord injury and traumatic brain injury

For people with SCI and TBI, a trend is seen toward increasing awareness on the effects of aging on the functional status of this group. Owing to medical advances, patients are now living 20 to 50 years past their time of injury. Numerous studies have been done describing quality-of-life issues for people with TBI; however, those studies seldom look specifically at changes in functional levels or what can be done to ameliorate the declines.

Signs of “premature aging” in multiple organ systems in people with SCI have been supported in literature. In a review of 92 studies by Jensen and colleagues, the findings indicated several salient points about the aging adult with SCI: many secondary health conditions occur with aging, many at higher rate compared to the typical population; pain, bowel and bladder impairments, muscle spasms, fatigue, esophageal symptoms, and osteoporosis are the most common conditions reported; and several chronic health conditions, including cardiovascular disease, diabetes, bone mineral density loss, fatigue, and respiratory complications or infections, occur with higher frequency in older individuals or those with longer SCI duration compared with younger individuals or those with shorter SCI duration.

McColl and colleagues, studying the impact of aging on people after SCI, echo the major categories of problems related to aging, such as musculoskeletal problems and joint, sensory, and connective tissue changes; chronic urinary tract infections; heart, respiratory, and other chronic diseases; secondary complications of the initial lesions, such as syringomyelia; and problems related to social and cultural acceptance and access or barriers. Evidence on specific age-related changes is presented in the section on examination.

Decreases in perceived health status and in functional abilities with increases in additional assistance for ADLs have been documented for aging individuals with SCI. In a study of 150 people aging with an SCI, nearly 25% reported decreases in the ability to perform functional activities that they had been able to handle after the acute rehabilitation phase. The subjects who reported decreases in functional ability were generally older (45 years compared with 36 years) and had longer postinjury periods (18 vs. 11 years). The most common symptoms reported by the individuals with decreases in functional status were related to fatigue, pain, and muscle weakness. The ADLs reported to be more difficult were transfers, bathing, and dressing. To maintain their functional levels, those with declines in functional ability reported needing additional equipment.

The need for further assistance with ADLs was echoed in a study by Gerhart and colleagues of individuals with SCI 20 years after the initial injury. The study showed that 22% of the subjects reported an increased need for support with ADLs. Compared with a general group of nondisabled men and women aged 75 to 84 years in which 78% of the men and 64% of women did not need help with ADLs, the population of people with SCI needed increasing support to remain independent, and the need for help occurred at a younger age. On average, those with quadriplegia required more help with ADLs around the age of 49 years, whereas those with paraplegia were able to maintain their functional level until age 54 years. The groups showing functional decreases reported greater fatigue, increased muscle weakness, pain, stiffness, and weight gain. Other studies support the findings that 5, 10, and 15 years after the initial SCI, an association with the need for additional help with increasing age existed.

Liem and colleagues, referencing an international data set of people with SCI, investigated a subset of 352 people at least 20 years after their injury. Increased help with transfers and housework were needed by 32% of the subjects, compared with their functional abilities at acute hospital discharge. With increasing age, women had a higher incidence of reported musculoskeletal impairments, which may have been related to biomechanical differences between men and women (e.g., 40% lower upper body strength).

In addition to the need for ADL support, neurogenic bowel and bladder symptoms also worsen with age in some people with SCIs. With changes in general health status, polypharmacy, decreased activity, and poor nutritional patterns, bowel problems (particularly constipation) become more of an issue. Although constipation seems like a minor issue relative to paralysis, those with SCIs report significant abdominal distention and pain, an increased incidence of perineal and sacral skin breakdown, and in some cases autonomic dysreflexia. In addition to the discomfort of chronic bowel dysfunctions, the required bowel care programs may take more time, which can lead to increased psychosocial issues associated with anxiety about bowel accidents in social and work situations. Additionally, these programs may take time away from social activities for both the person with an SCI and the caregiver. For those with continuing bowel problems that interfere with life and work activities, a colostomy may be an option that increases independence from caregiver support during the bowel program. Another complicating factor related to bowel dysfunction is the typical treatment for pain complaints. Because the origin of pain is often illusive, the most common treatment involves oral medications rather than referrals for therapeutic interventions. Pain medications, especially opioids, increase gastrointestinal difficulties in nondisabled populations, and the problem for patients with SCI is compounded.

Charlifue and colleagues, drawing on the National Spinal Cord Injury Database of 7981 individuals with SCIs that occurred from 1973 to 1998, found a slight decrease in perceived health status the longer one lived after SCI. Evidence was also found that those injured later in life had a higher number of rehospitalizations after injury than those injured at a younger age. Despite palpable problems with the statistical issues within the sample, the study clearly indicated that the best predictor of a complication is a previous history of that complication. On the basis of their results, Charlifue and colleagues suggest that prevention of complications is the best approach to improve quality of health and quality of life for people aging with SCI.

In addition to many multisystem impairments that occur in this population, a recent study confirmed a high occurrence of cardiovascular risk factors among older adults with long-term SCI. In 123 older adults (mean age: 63 years) with SCI with a mean time of 24 years since injury, the median number of risk factors was 3. The presence of these risk factors in individuals with existing disabilities can pose additional consequences to their health and must be addressed.

Across the literature, a call for further research and a need for longitudinal studies is a consistent recommendation. Research is needed to help understand when problems are most likely to emerge in the aging process, to identify modifiable factors can be targeted in this population, what prevention strategies may be efficacious, and to develop and test the efficacy of interventions to address and potentially prevent secondary health conditions.

Individuals with TBI are often neglected by professional health care and insurance providers after the acute rehabilitation period. According to Levin, before 1980, individuals with brain injuries were considered “dead on arrival.” People who would have died from the TBI several decades ago are now living into old age and are coping with the changes caused by the aging process superimposed on their physical limitations and cognitive problems. Of great concern is the possible relationship between a history of brain injury and increased cognitive changes along the dementia continuum. Cognitive decline in the nondisabled population is a problem, but the additive effect for a person with a previous TBI may seriously impair the person’s coping mechanisms when dealing with his or her own health and self-care needs. TBI was found to be an independent significant risk factor in developing dementia even in mild TBI. A meta-analysis in 2017 of 32 studies showed that head injury was associated with increased risks of dementia and Alzheimer’s disease. Further, recent TBI with loss of consciousness acquired in older adulthood has been associated with an increased risk for mortality.

Extensive work has been done on rehabilitation programs and quality-of-life issues related to head injury and, to a lesser extent, cerebral vascular accidents. Unfortunately, the focus of rehabilitation after a TBI has been on the acute and rehabilitation stages of treatment and not the chronic problems that follow this group of patients into their older years. As with other chronic conditions, few patients see a therapist after they are discharged from rehabilitation unless they have new acute events such as musculoskeletal problems or a medical condition that causes a change in functional status.

Although TBI is a lifetime disability, little attention has been paid to the needs of aging people with a TBI who may have recurring needs for physical and cognitive rehabilitation and retraining over the life-span. Individuals who sustain a TBI later in life years will likely have different needs from those injured at a younger age. In a study on the effect of age on functional outcome in mild TBI, Mosenthal and colleagues found that those injured later in life (after age 60) had longer inpatient rehabilitation periods and lagged behind younger patients in functional status at the point of discharge; however, similar to the younger individuals who sustained a head injury, the over-60 individuals showed measurable improvement during the 6-month study period. Therefore aggressive management of older patients with TBI is recommended, and older patients may require continuing management owing to the overlying issues of the aging process. This is echoed in the National Institutes of Health (NIH) consensus document on the treatment of people with TBI, which suggests that specialized interdisciplinary treatment programs need to be put in place to deal with the medical, rehabilitation, family, and social needs of people with TBIs who are over the age of 65 years. The document also concludes that access to and funding for long-term rehabilitation is necessary to meet long-term needs; however, it recognizes that changes in payment methods by private insurance and public programs may jeopardize the recommendations.

Although the authors of the NIH document recognize the need to deal with the aging processes associated with TBI, there continues to be a lack of services and trained professionals available, especially at the community level. As with the SCI population, work is now being done to investigate the relationships among TBI, aging, and health. Breed and colleagues found that older individuals with TBI were more likely than their age-matched nondisabled peers to report metabolic, endocrine, sleep, pain, muscular, or neurological and psychiatric problems. Their findings support those of Hibbard and colleagues and Beetar and colleagues, which suggest that medical personnel need to be prepared to treat a broad range of health issues in the aging TBI population. Fewer of the studies on long-term outcomes and issues in TBI extend to the 10- and 15-year postinjury periods that have been examined for patients with SCIs. In studies 5 years after the initial TBI, improvements in physical and social functions were noted in most areas for at least the first 2 years after injury, with the exception that those with a history of alcohol or drug abuse did less well. One could assume that continued abuse of alcohol or drugs would bode ill for individuals aging with a TBI. In one study of 946 children and adolescents who sustained a TBI, Strauss and colleagues found that patients with severe and permanent mobility and feeding deficits had higher mortality rates, with a 66% chance of surviving to age 50 years. In contrast, survivors with fair or good mobility had a life expectancy only 3 years shorter than that of the general population. However, because both severely and mildly injured individuals with a TBI can live well into and beyond their 50s, the impact of aging on the physical and cognitive deficits must be dealt with assertively to prevent superimposed disability.

Because aging individuals with an SCI or a TBI may have health problems similar to those of any aging population, primary impairments in muscle strength and endurance may be magnified. Therefore individuals with these chronic conditions who make repeat visits regarding medical problems such as musculoskeletal, cardiac, respiratory, and renal diseases should be referred for a comprehensive therapy examination and program that focuses on education, lifestyle changes, and health and wellness promotion interventions.

Fatigue

Fatigue is one of the most common symptoms reported by individuals with chronic conditions such as PPS and MS. Movement and performance of daily activities are more energy-consuming for individuals with disabilities and may cause greater fatigue. Evidence also points to individuals with disabilities aging faster than those without disabilities; cardiovascular data from individuals with SCI indicate that people with SCI may age faster than those without SCI. ^, Cook and colleagues identified the lack of age-specific general population norms as an obstacle in the understanding and estimation of the influence of aging on the fatigue of individuals with neuromuscular conditions.

It is likely that an earlier increase in fatigue with age might be observed in other chronic neuromuscular conditions; however, the effects of chronological age on fatigue in individuals with disabilities are not entirely clear. In a recent study, Cook and colleagues assessed fatigue and age in four clinical populations of individuals with PPS, SCI, MS, and muscular dystrophy (MD), comparing self-reported fatigue experience in different age cohorts with age-matched, US population norms. A total of 1836 surveys were used in data analysis.

The authors concluded that individuals with disabilities reported higher levels of fatigue than the general US population, regardless of age or disability type. Interestingly, the authors noted that the causes of fatigue likely vary by disability type—that is, MS, PPS, and MD are more likely to cause fatigue through a combination of central neurological processes, the effect of sleep disorders such as periodic limb movements (PLMs), or increased physical effort, whereas in SCI fatigue may be a side effect of medications or result from sleep disorders.

Results revealed not only that individuals with disabilities have a higher risk of experiencing fatigue than those without disabilities, but also that the risk for increased fatigue, compared with normative values, increases with age. The reported mean fatigue levels were the highest observed in older PPS age cohorts among the disability samples. In the MS group, fatigue was higher than in any other clinical group except PPS. The highest fatigue reported in the MS sample was in the 35- to 44-year-old age cohort, with lower fatigue in older cohorts except for those 75 years of age and older, but the older group had a small sample size. In the SCI group, peak reported fatigue was in the 55- to 64-year-old age cohort. The results for the SCI group younger than 55 years old were very similar to those for the general population.

In the general population, very little change in fatigue levels for most adults was found moving from young (65 years) to middle (75 years) old age, whereas in the disability samples the authors saw a slight but consistent increase toward greater fatigue at this point in the life-span. This increase in fatigue could be associated with current physical decline associated with disease progression. The authors concluded that more research is needed to determine the specific effects of fatigue on the functioning of people aging with disabilities and to further explore interventions that may shield against or reduce any negative effects that occur.

Bruno and colleagues state that fatigue has been identified as the most commonly reported, most debilitating, and least studied symptom in the postpolio sequelae. Generalized fatigue is typically described as overwhelming exhaustion or flulike aching accompanied by marked changes in level of energy, endurance, and mental alertness. The lack of energy with minimal activity is often described as “hitting a wall,” thus the term “polio wall.” Polio survivors differentiated between the fatigue associated with weakness and a “central fatigue” that leads to attention and cognitive problems. Severe fatigue affects not only physical function but mental function as well—hence the controversial suggestion that the fatigue associated with postpolio is caused by impaired brain function rather than the diffuse degeneration of motor units and motor junctions.

Descriptors for fatigue associated with PPS are significantly different. The fatigue of PPS may not appear at the time of the activity, and recovery does not occur with typical rest periods. It has also been described as a sudden and total wipeout. In a few instances headaches and sweating appear, suggestive of autonomic nervous system overload. Fatigue commonly occurs in the late afternoon or early evening. Fatigue that tends to last all day is atypical in PPS and should alert the therapist to consider other possible diagnoses.

Similar to the fatigue of PPS, MS fatigue profoundly disrupts multiple aspects of general well-being. Krupp reported that 67% of individuals with MS reported fatigue as a major limiting factor in their social and occupational responsibilities compared with no reports in healthy adults. The fatigue of MS is unique in that it is exacerbated by heat, as are many MS symptoms. Fatigue may be acute, chronic, or intermittent or persistent, whether related to a specific diagnosis such as MS, CP, or polio or having no relation to the initial medical diagnosis. It is necessary to consider the role of other symptoms related to the specific medical diagnosis during the examination of fatigue.

The evaluation of fatigue includes the various factors that can induce or worsen fatigue. The history and examination also are used to assess for new medical diagnoses or the possibility of infection, an impending relapse, heat exposure (in the case of MS), and side effects from medications.

Differential diagnoses for fatigue are extensive and may include disorders of several systems, including psychological, cardiopulmonary, neurological, and endocrine disorders, and medication use. Specific conditions that may cause fatigue include depression, myasthenia gravis, hyperparathyroidism, congestive heart failure, sleep apnea, cancer, and infections. Numerous medications commonly used in symptom management can cause fatigue, including antispasticity agents, tricyclic antidepressants, and β-blockers. The challenge is to differentiate the fatigue caused by typical activities from the fatigue of chronic conditions.

Pain

Individuals with chronic diseases often have pain as a common symptom, most likely from long-term atypical biomechanical forces on joints and muscles or from long-standing disease processes. Many developmental disabilities have a component of disordered movement, from hypomobility in the case of DS to hypermobility in the case of spastic CP. These abnormal joint stresses and strains cause long-term damage to the musculoskeletal system. ^{, ,} The natural degradation of joint structures with aging coupled with weakness and atypical ground reaction forces leads to higher incidence of musculoskeletal pain.

Pain that occurs with chronic conditions may be muscular, joint-related, or both. The source of pain needs to be considered because pain may limit the individual’s functional abilities and lead to further decline. Muscle pain is often described as a deep, aching pain similar to the pain experienced during the initial infection. The pain is frequently aggravated by physical activity, stress, and cold temperature. Pain is unusual in that it does not occur at the time of activity but rather 1 to 2 days after a precipitating event.

Joint pain in itself usually results primarily from long-term microtrauma from abnormal biomechanical forces. An example might be the overuse of the shoulder girdle muscles and joint from a lifetime of use of Lofstrand crutches to compensate for lower-extremity impairments after polio. Joint pain is frequently associated with physical activity but is rarely associated with inflammation. Interventions are complicated by the presence of osteoporosis, lack of compensatory substitutions to rest the injured part, and, often, poor response to exercise. Failing joint fusions, uneven limb size, progressive scoliosis, poor posture, and abnormal mechanics may also contribute to pain.

Although pain is not a hallmark of many chronic conditions such as DS and MS, it may occur in some individuals with long-term impairments and thus should be part of a comprehensive examination. Pain frequently associated with MS is related to postural problems and inefficient use of muscles and joints to compensate for loss of function or spasticity.

More than 65% of individuals with PPS have reported neck, shoulder, and back pain radiating to the hip and leg. This pain is expected because the incidence of major postural abnormalities and gait deviations is also high, as shown in Table 35.1 and Box 35.4 .

Differential diagnosis for acute muscle and joint pain includes consideration of chronic musculoskeletal conditions leading to wear and tear and disorders with significant muscle or joint manifestations. The list is extensive and may include osteoarthritis, tendinitis, bursitis, fibromyalgia, rheumatoid arthritis, and polymyalgia rheumatica. The challenge is determining whether muscle or joint pain occurs from long-term wear and tear or from an acute or exacerbating occurrence.

Strength

Examination of strength is another critical component of the examination of individuals with chronic impairments. Long-standing weakness is an expected occurrence from decreased physical activity common in individuals with disabilities and chronic diseases. Strength deficits may result from several causes other than disuse, such as upper motor neuron weakness, fatigue, compensatory movements, pain, or spasticity. It is likely, however, that aging individuals with chronic conditions have adapted to the initial weakness associated with their diagnoses, as in the case of people with SCI or PPS who have led productive and functional lives since the initial diagnosis or acute onset.

Typical aging involves the losses of muscle strength, loss of power, and sarcopenia—processes that may intensify or build on the initial losses of strength. Delineation of weakness from age-related processes versus weakness as part of the disease process or that from lack of physical activity common in people with chronic impairments is difficult if not impractical from both research and clinical perspectives. Too many variables need to be controlled and accounted for unless specific muscle groups were examined initially and the findings well documented, which may have been the case for people with PPS. Many individuals with PPS have extreme difficulty dealing with these new problems because they were taught that hard work was the only way to correct physical problems that followed acute polio. These new problems, which also limit motor function, require that these individuals not work hard. These two concepts are contradictory and can leave an individual frustrated and confused over therapeutic recommendations.

Manual muscle testing of the entire body, although time-consuming, may be necessary to determine the muscular involvement. Muscle testing, however, may not always reveal the full extent of muscle involvement, and functional assessment may provide a better picture of individuals’ potential and true difficulties. Performance and participation in daily activities will provide a better picture of a person’s strengths and impairments. Therefore current functional activities are critical in determining whether current mobility difficulties are related to the aging process, represent an exacerbation of initial disease processes such as in PPS, or are signs and symptoms of new medical concerns.

Range of motion and muscle length

Limitations in joint motion from muscle contractures and from shortening of ligamentous joint structures are common in individuals with chronic disabilities. Likewise, hypermobile joints may also result from compensatory techniques forcing more mobility. An evaluation of the individual’s activity levels and goals is vital before intervening with muscle and joint deficits. In some instances, during the convalescent stage of the initial diagnoses, as in polio or SCI, selective tightness was allowed to give some stability to joints with paralyzed muscles. In addition, the body may develop useful contractures to maintain or regain function. The presence of spasticity, particularly in severe cases, may have also contributed to the development of contractures in older adults with CP, SCI, MS, or TBI. Before making attempts to stretch contractures, therapists should carefully evaluate the functions that may be lost if gains in range of motion (ROM) are achieved. Equally important is to evaluate what functions would be gained and what the cost would be. Consider that after 20 to 40 years these contractures resist any significant elongation, and aggressive intervention may cause more harm and loss of function.

Box 35.5 lists the secondary conditions that may develop in an individual with CP. These impairments not only cause pain but also limit mobility and interfere with performance of ADLs and leisure activities. Thus a thorough evaluation of musculoskeletal status and periodic monitoring are imperative to maintaining quality of life and social participation for these individuals.

Tone

Examination of tone is a focal component of the examination of individuals with chronic neuromuscular conditions. This group of individuals may demonstrate a wide range of tone deficits from hypotonia seen with DS, to hypertonicity seen in people with TBI, SCI, MS, or CP, to a mixed type with episodes of high and low tone.

Spasticity can interfere with mobility and may coexist with weakness. It may cause pain and atypical postures, predispose the individual to contractures (particularly those with severe spasticity), and interfere with hygiene and self-care. On the opposite end of the tone spectrum, people with DS have hypotonia that may or may not limit mobility. Tone deficits in DS are not a primary limiting factor to mobility and physical activity, as the characteristic finding of marked hypotonia tends to gradually diminish with age.

Cognitive function

Cognitive deficits may be a major disabling feature in genetic and developmental conditions such as DS and CP. Similarly, cognitive dysfunction may be a key symptom in chronic conditions such as MS, TBI, and SCI. The degree of cognitive deficits affects not only health management, but also the long-term planning for aging processes in people with chronic disabilities and conditions. The need for lifelong external support is a considerable issue for those who are unable to independently care for themselves as they age. The degree of cognitive deficits in this population may range from memory deficits to mental retardation; the degree of cognitive and perceptual deficits determines participation in functional activities and self-care, mobility, compliance, and even health status.

Standardized outcome measures exist to measure cognitive impairments; the examiner is cautioned to consider the validity and reliability of outcome measures for specific populations before use. The reader is referred to chapters discussing specific diagnoses for detailed outcome measures. Because cognition is multidimensional, examination for deficits will need to be directed at different components of cognition. Screening may be as simple as a three-item recall or short questions determining the ability to follow commands, attention, level of consciousness, orientation, judgment, construction, and higher memory function.

Mobility and posture

In individuals with chronic impairments, addressing inefficient alignments and postures is of critical importance, particularly if this area was not addressed early in their care. The dire effects of such malalignments and compensations described in the following paragraphs eventually affect functional abilities.

Asymmetrical or abnormal gait patterns, crutch walking, and propelling manual wheelchairs for several decades are frequently the major sources of the pain, weakness, and fatigue in people with chronic movement dysfunctions after a medical problem such as TBI, SCI, CP, PPS, or cerebrovascular accident (CVA). The incidence of pain in a group of 114 patients with confirmed PPS increased from 84% in those who were ambulatory without orthotics to 100% in those who used crutches or wheelchairs for locomotion. A high prevalence of osteoarthritis in patients with PPS was documented in the hand and wrist by radiography. More than twice the number of subjects with PPS had osteoarthritis of the wrist or hand than would be expected in a healthy population of the same age. The risk factor was significantly increased with lower-extremity muscle paralysis and use of assistive devices.

In an electromyographic study of walking in patients with PPS, Perry demonstrated overuse and substitution activity of the vastus lateralis, biceps femoris, and gluteus maximus muscles when the soleus is nonfunctional. Such substitution and overcompensation in the long term, however, lead to microtrauma of ligaments and joint structures and exhaustion of neuromuscular units.

In addition to sitting in poorly supporting chairs, sofas, auto seats, and wheelchairs, the individual with chronic impairments may have trunk muscle paresis or asymmetries of the pelvic base and may spend up to 16 h/day in the seated position. The typical posture is slumped, hanging on posterior vertebral ligaments with loss of lumbar and cervical curves. Neck, shoulder, and back pain are therefore commonly reported.

Levels and types of mobility will be diverse in individuals with chronic neuromuscular impairments. Mobility may range from independent ambulation to gait with an assistive device all the way to wheelchair dependency, depending on existing impairments and cognitive function. Thorough examination should determine the current mobility levels and possible modification needs depending on new impairments or functional limitations. It may be necessary to include a seating examination in people who are wheelchair dependent or sit for the majority of the awake hours.

Balance and coordination

Deficits in balance and coordination in individuals with chronic conditions predispose these people to falls and limit safe mobility. As individuals with these conditions age, typical age-related changes in balance may add to the existing deficits and magnify functional limitations and impairments. Coordination and balance deficits need to be examined under functional conditions and in the context of the individual’s daily activities. Standardized outcome measures are also available to objectively measure impairments; however, the reliability and validity of such measures in specific populations may be deficient. The use of outcome measures may provide a multidimensional look into deficits and provide objective baseline measures to track progress with intervention as well as progression of the movement dysfunction.

Environmental temperature intolerance

Consideration for the effects of heat and/or cold may be necessary when working with individuals with chronic diseases, and the examination must include a component of temperature intolerance. Two specific diagnoses embody this phenomenon: PPS for cold intolerance and MS for the negative effects of environmental heat.

Regulation of body temperature is often a problem for individuals with MS (see Chapter 17 ). Earlier, it was noted that a feature of fatigue unique to MS is that the fatigue is exacerbated by heat. Other MS symptoms may also be aggravated by heat. The Uthoff phenomenon is an adverse response to external heat, causing fatigue or deterioration of symptoms; it often occurs with exercise. Specific recommendations for cooling during exercise intervention may be necessary to counteract the deleterious effects of heat. It is important that the examiner meticulously determine during the health interview the symptomatic effects, if any, of increased temperature in the aging individual with MS.

Sensory deficits per se are not hallmark features of polio; however, cold intolerance is a commonly reported late-onset symptom. Involved extremities in individuals with PPS are frequently abnormally cold as a result of sympathetic nerve cell involvement leading to decreased vasoconstriction and venoconstriction with heat loss to the environment. The impairment may become worse with PPS. Environmental adaptation can create an easy solution to this problem as long as the individual with PPS is aware first of the problem and second of the adaptations necessary to avoid thermoinstability within the extremities. Preventing versus responding to the inadequate vasoresponse to cold empowers the individual to develop environmental control.

Sleep disturbances

It is common knowledge that older individuals report and manifest sleep disorders. ^, Thus this impairment certainly can be associated with aging individuals with chronic movement dysfunction. Disturbances in sleep are a common symptom in people with chronic neuromuscular diseases. Sleep deprivation is a contributing factor to fatigue in individuals with MS. Similarly, more than 50% of individuals with PPS have been found to have sleep disturbances. These disturbances may be caused by pain, stress, hypoventilation, or obstructive apnea. Bruno proposed a high incidence of abnormal movements in sleep in nearly two thirds of polio survivors, with 52% reporting sleep disturbance caused by these movements. All seven of subjects with PPS in the study demonstrated abnormal movements in sleep. The author points to the importance of eliminating sleep disorders as a cause of fatigue before the diagnosis of postpolio sequelae is made, particularly because it remains a diagnosis of exclusion.

In addition to the typical symptoms of muscular weakness, pain, and fatigue associated with PPS, some individuals also develop sleep disorders such as PLMs. PLMs in sleep are repetitive episodes of muscular contractions with durations of 0.5 to 5 seconds; frequencies of five or more per sleep hour are deemed pathological. The association of PLMs with insomnia or daily sleepiness suggests the diagnosis of PLM disorder. Whereas PLM has been related to the pathophysiology of PPS, the occurrence of PLM in PPS is not well known. In a recent retrospective study of 99 patients with PPS, researchers assessed the frequency of PLMs during sleep, including other sleep-quality variables such as total sleep time, efficiency of sleep, apnea-hypopnea index, and awaking index. Sixteen patients showed a PLM index that was considered pathological. The authors concluded that a close relationship between PLM and PPS exists; however, the prevalence of PPS with or without PLM and its combination with apnea-hypopnea is not clearly established, a finding in agreement with the conclusions of Jubelt and colleagues. ^,

A thorough history and review of systems including a sleep history may reveal the potential causes and guide the therapist in the clinical decision making process for referral and consultation. Sleep apnea, which occurs frequently in polio survivors, and other sleep disorders, including abnormal movements in sleep, may be revealed with specific questioning, triggering the referral process. The therapist plays a role when the sleep disorder is associated with the area of pain management.

Life-threatening conditions

Limited knowledge exists of adult health issues for older individuals with developmental disabilities. Secondary medical conditions warrant further examination, particularly in aging adults with chronic conditions. Acute onset of new symptoms may suggest exacerbation of disease or decreased functional reserves or may arise from long-standing effects of impairments from the initial disease process.

In the case of PPS, bulbar muscle dysfunction may also result from the new weakness. Life-threatening conditions such as hypoventilation, dysphagia, sleep apnea, and cardiopulmonary insufficiency require management by medical specialists. ^{, ,} These problems occur in people with previous bulbar poliomyelitis who may or may not be using ventilatory assistance and in those with severe kyphosis or scoliosis. Respiratory failure may occur primarily in individuals with residual respiratory insufficiency and minimal reserves.