Post-polio syndrome

Clinical manifestations
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By Tarakad S Ramachandran MD and Arun Ramachandran

Post-polio syndrome is manifested by neurologic, musculoskeletal, and systemic symptoms and signs. The most prominent neurologic manifestation is new progressive weakness, sometimes accompanied by atrophy. When this occurs in an extremity, it has been referred to as Post-Polio Progressive Muscular Atrophy (PPMA). However, new weakness can also affect respiratory and bulbar muscles (Dalakas et al 1984; Jubelt and Cashman 1987). The diagnostic criteria for PPMA have been derived from the criteria first promulgated by Mulder and colleagues (Mulder et al 1972), which were updated by the Post-Polio Task Force (Jubelt and Drucker 1999). This includes four criteria: (1) A prior episode of paralytic poliomyelitis with residual motor neuron loss (which can be confirmed through a typical patient history, a neurologic examination, and, if needed, an electrodiagnostic exam); (2) A period of neurologic recovery followed by an interval (usually 15 years or more) of neurologic and functional stability; (3) A gradual or abrupt onset of new weakness or abnormal muscle fatigue, decreased endurance, muscle atrophy, or generalized fatigue; and (4) Exclusion of medical, orthopedic, and neurologic conditions that may be causing the symptoms mentioned in number 3.

Neurologic manifestations. New slowly progressive muscle weakness is the most important neurologic problem (Table 1), occurring in most post-polio syndrome patients (Codd et al 1985; Halstead and Rossi 1987; Jubelt and Drucker 1993). It appears to be related to a disintegration of the lower motor neuron unit (motor unit) (Cashman et al 1987a; Dalakas and Illa 1991) and can occur in muscles previously affected and partially or fully recovered or in muscles previously unaffected (Halstead and Rossi 1987; Jubelt and Drucker 1993). Electromyography (EMG) (Hayward and Seaton 1979) and animal studies (Bodian and Howe 1941; Sabin and Ward 1941) indicate that some clinically unaffected muscles were involved subclinically during the acute poliomyelitis. However, previously affected muscles are more likely than unaffected muscles to later become weak (Halstead and Rossi 1987; Jubelt and Drucker 1993). The distribution of the new weakness, appears to correlate with the severity of paralysis at the time of the acute poliomyelitis and, thus, with the number of surviving motor neurons (Bodian 1949; Cashman et al 1987a).

Table 1. Most Common New Late Manifestations of Poliomyelitis in Patients Referred to Postpolio Clinics*

 

Symptom

Houston***

(n=32)

Madison****

(n=79)

Syracuse †

(n=100)

1991-1993

Syracuse †

(n=100)

1993-1997

 

Fatigue

 

89%

86%

83%

86%

Joint pain

 

71%

77%

72%

73%

Muscle pain

 

71%

86%

74%

73%

Weakness

 

 

 

 

 

Previously affected muscles

 

69%

80%

88%

88%

Previously unaffected muscles

 

50%

53%

61%

59%

Total ‡

 

-----

87%

95%

90%

Atrophy

 

28%

39%

59%

52%

Cold intolerance

 

29%

56%

49%

53%

Respiratory insufficiency

 

-----

39%

42%

36%

Dysphagia

-----

30%

27%

36%

*Modified from (Jubelt and Drucker 1999).

***Adapted from (Halstead and Rossi 1987). All patients met criteria for post-polio syndrome.

****Adapted from (Agre et al 1989). All patients had histories and examinations compatible with diagnosis of previous poliomyelitis.

† First 200 patients with histories and examinations compatible with diagnosis of previous poliomyelitis.

‡ Total percent of patients with new weakness.

New atrophy does not occur as an isolated manifestation and is seen in fewer than half of the patients with new weakness (Cashman et al 1987b; Halstead and Rossi 1987). In addition to the weakness and atrophy caused by the disintegration of the lower motor neuron unit, rarely upper motor neuron signs can occur (Jubelt and Cashman 1987). They include hyperreflexia, Babinski signs, and occasionally, spasticity. Of 180 post-polio syndrome patients, upper motor neuron signs were found in 15 (8.3%) (Jubelt and Cashman 1987). This percentage is similar to the frequency of motor neuron signs found during acute poliomyelitis (Jubelt and Cashman 1987). Muscle pain (myalgias), which occurs in most patients (Codd et al 1985; Halstead and Rossi 1987; Jubelt and Drucker 1993), appears to be due to overuse of weak muscles. Similar symptoms occur in overused weakened muscles in other neuromuscular diseases (Jubelt and Cashman 1987). The pain is a soreness or aching feeling that occurs with minimal exercise. Some patients have muscle tenderness on palpation.

New muscle weakness may also involve specific muscles groups, causing bulbar muscle weakness, respiratory insufficiency, and sleep apnea. Bulbar muscle weakness is most frequently manifested by dysphagia, which has been reported in 10 to 36% of post-polio patients (Buchholz and Jones 1991; Sonies and Dalakas 1995). It is primarily due to pharyngeal and laryngeal muscle weakness; however, local pharyngeal or esophageal problems should also be excluded. Patients may complain of food sticking, making swallowing slow and difficult, often with coughing and choking (Buchholz and Jones 1991). Videofluoroscopic studies may reveal impaired tongue movements, delayed pharyngeal constriction, pooling in the valleculae or pyriform sinuses, and rarely aspiration, which is usually mild (Sonies and Dalakas 1995). Less frequently, other bulbar muscles, such as the facial muscles and vocal cords, may become weaker in post-polio syndrome (Cannon and Ritter 1987; Abaza et al 2001); dysarthria has also been reported (Jubelt and Cashman 1987).

Respiratory insufficiency primarily occurs in patients with severe residual respiratory impairment and minimal reserve from their acute polio illness (Dean et al 1991; Blomstrand and Bake 1992; Soliman et al 2005). Similar to limb weakness, respiratory failure is more likely to occur in patients who required respiratory support during the acute disease and, hence, had more severe disease and in those who contracted polio at an age of 10 years or older (Dean et al 1991; Soliman et al 2005). Patients with post-polio syndrome chronic respiratory failure lose an average of 1.9% of their vital capacity per year (Bach et al 1987a). It usually is due to respiratory muscle weakness but can also be due to central hypoventilation because of the residual damage from acute bulbar poliomyelitis (Plum and Swanson 1959). Other factors such as cardiac or pulmonary disease or scoliosis may contribute to the problem. Initially, respiratory failure begins with nocturnal hypoventilation, and patients may require only nighttime respiratory support (Howard et al 1988). If already on nighttime respiratory support, patients may eventually require total ventilator support (Bach et al 1987a).

Sleep apnea is not an uncommon problem in post-polio patients. It may be central, obstructive, or mixed (Guilleminault and Motta 1978; Steljes et al 1990). Most patients with central sleep apnea had bulbar polio, and some required ventilatory support (Guilleminault and Motta 1978; Siegel et al 1999). Probably residual damage to the brainstem reticular formation predisposes to central sleep apnea. Obstructive sleep apnea appears to be related to pharyngeal muscle weakness, obesity, and musculoskeletal deformities (Steljes et al 1990). Respiratory muscle weakness may also contribute to sleep apnea (Bach and Alba 1991).

Fasciculation and cramps without weakness, muscle pseudohypertrophy, and tingling paresthesias are other neuromuscular problems that occur infrequently in post-polio patients, with or without new weakness (Jubelt and Cashman 1987).

Musculoskeletal manifestations. Pain from joint instability is the major musculoskeletal problem and can occur without new weakness. The long-term overstress of joints because of residual weakness eventually results in joint deterioration and pain (Klein et al 2000a; Vasiliadis et al 2002). Progressive scoliosis, poor posture, unusual mechanics because of weak muscles, uneven limb size, overstressed tendons, failing tendon transfers, and failing joint fusions can all contribute to joint instability and pain. These joint problems frequently lead to loss of mobility and a return to using old assistive devices (Anderson et al 1972). Progressive scoliosis may also contribute to pulmonary failure (Lin et al 2001). Compressive radiculopathies and mononeuropathies may occur as secondary musculoskeletal complications (Jubelt and Cashman 1987; Werner et al 1989).

Systemic manifestations. Fatigue is the most prominent systemic manifestations occurring in up to 80% of patients (Dalakas et al 1984; Halstead and Rossi 1987; Jubelt and Cashman 1987; Agre et al 1989). It is generally described as a disabling generalized exhaustion that follows even minimal physical activity (Halstead et al 1985). Patients frequently refer to hitting the “polio wall.” Their fatigue has also been described as “increasing physical weakness,” “tiredness,” “lack of energy,” and “increasing loss of strength during exercise” (Berlly et al 1991); thus, it can either be perceived as generalized or muscular in origin (Halstead et al 1985; Agre and Rodriquez 1991). The fatigue can also affect mental as well as physical functioning. When generalized fatigue is severe, patients find it difficult to concentrate or collect their thoughts and may appear confused (Berlly et al 1991). It may be improved by decreasing physical activity, pacing daily activities, and taking frequent rest periods and naps (Agre and Rodriquez 1991; Packer et al 1991). Post-polio syndrome fatigue appears to respond better to sleep than that of the chronic fatigue syndrome, although frequent rest periods are also helpful. In their study of fatigued and nonfatigued patients with post-polio syndrome, Östlund and colleagues report that fatigued post-polio patients can be considered a distinct subgroup across the fatigue continuum (Östlund et al 2011).

Other systemic manifestations less frequently reported include increased sleep requirements, cold intolerance, and psychologic stresses (Halstead and Rossi 1987; Jubelt and Cashman 1987). Increased sleep requirements probably relate to severe fatigue. Many post-polio syndrome patients complain of cold intolerance (Halstead and Rossi 1987). They report worsening symptoms, including increasing generalized fatigue and weakness when exposed to cold temperatures (Bruno et al 1985). Patients may also develop coolness and color changes such as cyanosis and blanching of the affected extremity that probably relate to sympathetic intermediolateral column damage from the acute poliomyelitis (Smith et al 1949). Psychological symptoms appear to relate to the reemergence of a supposedly old resolved problem and to the stresses of new disabilities and the accompanying major life style changes (Kohl 1987; Bruno and Frick 1991; Burger and Marincek 2000).

De Grandis and colleagues report a patient who developed restless legs syndrome concurrent with the development of classic post-polio syndrome 40 years after recovery from an episode of paralytic poliomyelitis (De Grandis et al 2009). Marin and colleagues describe the occurrence of restless legs syndrome in a series of 10 patients with post-polio syndrome (Marin et al 2011); however, further studies are needed to establish the relationship and incidence of restless legs syndrome in post-polio syndrome.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Anesthesia
References cited
Contributors