Neuropathic Arthropathy

Many conditions predispose to neuropathic arthropathy (see table Conditions Underlying Neuropathic Arthropathy). Impaired deep pain sensation or proprioception affects the joint’s normal protective reflexes, often allowing trauma (especially repeated minor episodes) and small periarticular fractures to go unrecognized. Increased blood flow to bone from reflex vasodilation results in active bone resorption, which can contribute to bone and joint damage.

Each new injury sustained by the joint causes more distortion as it heals. Hemorrhagic joint effusions and multiple small fractures can occur, accelerating disease progression. Ligamentous laxity, muscular hypotonia, and rapid destruction of joint cartilage are common, predisposing to joint dislocations, which also accelerate disease progression. Advanced neuropathic arthropathy can cause hypertrophic changes, destructive changes, or both.

Arthropathy does not usually develop until years after onset of the neurologic condition but can then progress rapidly and lead to complete joint disorganization in a few months. Pain is a common early symptom. However, because the ability to sense pain is commonly impaired, the degree of pain is often unexpectedly mild for the degree of joint damage. A prominent, often hemorrhagic, effusion and subluxation and instability of the joint are usually present during early stages. Acute joint dislocation sometimes also occurs.

During later stages, pain may be more severe if the disease has caused rapid joint destruction (eg, periarticular fractures or tense hematomas). During advanced stages, the joint is swollen from bony overgrowth and massive synovial effusion. Deformity results from dislocations and displaced fractures. Fractures and bony healing may produce loose pieces of cartilage or bone that can slough into the joint. A coarse, grating, often audible crepitus, usually more unpleasant for the observer than for the patient, may develop.

Although many joints can be involved, the knee and the ankle are most often affected. Distribution depends largely on the underlying disease. Thus, tabes dorsalis affects the knee and hip, and diabetes mellitus affects the foot and ankle. Syringomyelia commonly affects the spine and upper limb joints, especially the elbow and shoulder. Frequently, only 1 joint is affected and usually no more than 2 or 3 (except for the small joints of the feet), in an asymmetric distribution.

Infectious arthritis may develop with or without systemic symptoms (eg, fever, malaise), particularly with diabetes, and requires a high index of suspicion.

• Radiographs

The diagnosis of neuropathic arthropathy should be considered in a patient with a predisposing neurologic disorder who develops a destructive but relatively painless arthropathy, usually several years after the onset of the underlying neurologic condition. Diagnosis is established by characteristic abnormalities on a radiograph in a patient with a predisposing condition and typical symptoms and signs.

Abnormalities on a radiograph in early-stage neuropathic arthropathy are often similar to those in osteoarthritis (see diagnosis of osteoarthritis). The cardinal signs later in disease progression are

• Bone fragmentation

• Bone destruction

• New bone growth

• Loss of joint space

There may also be synovial effusion and joint subluxation. The bones may become deformed, and new bone forms adjacent to the cortex, starting within the joint capsule and often extending up the shaft, particularly in long bones. Calcification and ossification may occur in the soft tissues. Large, bizarrely shaped osteophytes may be present at the joint margins or within joints. Large curved (parrot’s beak) osteophytes frequently develop in the spine in the absence of clinical spinal disease.

Neuropathic arthropathy progresses more rapidly than osteoarthritis.

Neuropathic Arthropathy of the Foot

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SCIENCE PHOTO LIBRARY

• Treatment of cause

• Sometimes surgery

Treatment of the underlying neurologic condition may slow progression of the arthropathy and, if joint destruction is still in the early stages, partially reverse the process.

Early diagnosis of asymptomatic or minimally symptomatic fractures facilitates early treatment; stabilization (with splints, special boots, or calipers) protects the joint from further injury, possibly stopping disease evolution. Stabilization may even prevent neuropathic arthropathy in a patient at risk.

For a grossly disorganized joint, arthrodesis using internal fixation, compression, and an adequate bone graft may be successful. For grossly disorganized hip and knee joints, if neuropathic arthropathy is not expected to be progressive, successful outcomes can be obtained with total hip and knee replacements. However, patients are at higher risk for postoperative complications such as loosening and dislocation of the prosthesis (1).