Convergence Paralysis as a Manifestation of Polyarteritis Nodosa

Abstract

Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis of medium-sized arteries. The American College of Rheumatology criteria for the diagnosis of PAN includes at least three of: 1. weight loss > 4 kg, 2. livedo reticularis, 3. testicular pain or tenderness, 4. myalgias, weakness or leg tenderness, 5. mono-or polyneuropathy, 6. diastolic hypertension, 7. elevated blood creatinine or urea, 8. hepatitis B antigen or antibody in the serum, 9. aneurysms or occlusions of visceral arteries or 10. granulocytes on small or medium sized artery biopsy.1 Common sites of involvement include skin, joints, kidneys, gastrointestinal tract and peripheral nerves. Central nervous system involvement has been reported in up to 40% of cases; the usual manifestations are encephalopathy, focal deficits and seizures.2 Descriptions of PAN in the pediatric literature has been reported in fewer than 250 children. 3A previously well 15 year old female presented with a two week history of intermittent headache associated with one week of episodic vomiting and vertigo. She also complained of diplopia (horizontal and diagonal), falling to the right side while walking and a fullness in her right ear that made her unable to hear. Further history revealed that she had a "pins and needles" feeling in her left hand and right foot that had been present for three months. She complained of bilateral leg pain. As well, she described a lacy rash on her hands and legs that appeared when exposed to the cold and changed colour from red to purple. The patient also described a six week history of intermittent "stabbing" epigastric pain and a 25 pound weight loss. Her only medication was minocycline 250 mg once daily, which she had been taking for two years to treat acne. Family history revealed that her maternal grandmother had both systemic erythematous lupus and rheumatoid arthritis.On physical examination, she was afebrile. Her blood pressure was elevated at 138/94, the remainder of her vital signs were normal. Her neurologic exam revealed bilateral exotropia; however she was able to fix with both eyes. Extraocular movements were full when each eye was tested independently. She had convergence failure with marked limitation of adduction in the right eye. Her left pupil was mildly dilated but both pupils were reactive to light. Fundoscopy was normal. She did not have nystagmus. There was hearing loss in the right ear. Her Romberg test was positive and her gait also demonstrated falling to the right side. She had muscle wasting, fasciculations, and a sensory deficit of the hypothenar eminence, fifth digit and ulnar side of the fourth digit of the left hand and an isolated sensory deficit to light touch on the medial plantar aspect of her right foot. The remainder of both her motor and sensory exams were normal. THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 423 Convergence Paralysis as a Manifestation of Polyarteritis Nodosa PEER REVIEWED LETTERLivedo reticularis was present on her hands and legs, no bruits were heard. The rest of her gener...