Polyarteritis Nodosa-Induced Quadriplegia

TO THE EDITOR:

An abrupt onset of quadriplegia without central nervous system or sensory impairment signifies a severe disturbance of lower motor neurons, peripheral nerves, neuromuscular junctures, or muscle fibers. If diffusely tender muscles are present in this setting, a drug-induced, toxic, metabolic, infectious, or inflammatory myopathy is suspected. Rhabdomyolysis occurs when skeletal-muscle cell integrity is substantially disrupted, dispersing cellular contents into vascular channels [1]. Marked elevation of serum creatine kinase levels and evidence of urinary myoglobin are hallmark features of rhabdomyolysis. Surgical factors associated with rhabdomyolysis are diverse; for rheumatic diseases, only forms of polymyositis are reported to cause myopathy associated with rhabdomyolysis [2]. We describe a patient who developed an abrupt onset of quadriplegia associated with rhabdomyolysis. Examination of a muscle biopsy specimen showed polyarteritis nodosa and secondary muscle cell damage.

A 61-year-old man presented with quadriplegia. He was receiving 10 mg of prednisone per day and had been seronegative for symmetrical polyarthritis for 9 months. Three days before admission, the patient had rapidly progressive pain and weakness in his arms and legs after “running out” of prednisone. The day before admission, he could not rise from a supine position. Pertinent data, collected during 1 day at a community hospital, were quadriplegia, diffusely tender muscles, “red urine,” and creatine kinase levels of 17 000 IU/L (normal levels, < 200 IU/L). After transfer to our hospital, pertinent findings consisted of mild symmetric synovitis of the hands and large joints, marked generalized muscle tenderness, normal sensation and cranial nerves, diffuse flaccid muscle tone, markedly decreased strength in his arms and legs (0/5), and globally absent deep tendon reflexes. Abnormal laboratory data were creatine kinase levels of 24 000 IU/L and urinalysis with 3+ myoglobin. A thorough investigation for an underlying primary neurologic, endocrine, or infectious cause was negative. Examination of a right quadriceps muscle biopsy specimen showed diffuse medium-sized vessel polyarteritis nodosa associated with severe, diffuse muscle cell ischemia and necrosis. Despite appropriate treatment (1 g of intravenous methylprednisolone for 3 days), muscle strength did not improve. On day 8, he developed a surgical abdomen; multiple areas of bowel perforations were found. The patient remained hypotensive after surgery and died. At autopsy, multiple organ systems showed evidence of polyarteritis nodosa.

Profound polyarteritis nodosa-induced myopathy manifesting only as quadriplegia is rare. We reviewed the medical literature of the last 100 years and found only one reported patient with similar features [3]. More commonly, polyarteritis nodosa produces less severe muscle symptoms. Moreover, as shown in our patient, clinical markers of muscle involvement, muscle pain, tenderness, and weakness enhance the diagnostic yield muscle site for biopsy. Rhabdomyolysis of immunologic origin has previously only been caused by forms of primary muscle inflammation. In essence, our patient had an unusual form of generalized vascular occlusive disease [4] caused by an underlying immunologic process (polyarteritis nodosa), which resulted in widespread secondary muscle necrosis that manifested as quadriplegia with rhabdomyolysis.

• Copyright ©2004 by the American College of Physicians