A 59-year-old, right handed, Caucasian engineer presented to the emergency department 12 hours after the sudden onset of left sided weakness and numbness. The patient had been in good health until the year prior to admission. He had an episode of bilateral ankle swelling with cough, pleuritic chest pain and night sweats 12 months prior to admission. Six months later he had left ankle swelling and left foot drop, followed by right knee soreness and swelling a month later, and swelling of the left calf and right foot two months after that. He gave an eleven month history of migrating arthralgias, myalgias and periodic lower back pain. The episodes of myalgia and arthralgia typically lasted three to ten days. The patient reported some symptomatic relief with ibuprofen. He was fatigued throughout this period. There was no history of shoulder or hip pain, conjunctivitis, rash, photosensitivity, gastrointestinal or genitourinary complaints. X-rays of the sacro-iliac joints were normal. He had developed moderate hypertension in the year prior to admission. There was no history of dental work, or dental carries. His family history was unremarkable.Abdominal ultrasounds documented splenomegaly increasing from 13 cm to 18 cm in the year prior to admission. ESR was consistently elevated-ranging between 45 and 85 mm/hr. He had a normocytic anemia. Genetic testing revealed an HLA B27 genotype. Subsequently the patient was diagnosed with HLA B27 associated reactive arthritis.He developed a new cardiac murmur. A transthoracic echocardiogram performed a month before admission revealed 3+ aortic regurgitation, a mildly dilated left ventricle with normal left ventricular ejection fraction, and 1+ mitral regurgitation with a normal valve. A TTE performed four years earlier had been normal. The aortic insufficiency was deemed consistent with HLA B27 associated aortitis and a TEE was scheduled to rule out further valvular disease. The patient presented with acute stroke prior to his scheduled outpatient TEE.On presentation he was alert and oriented. His blood pressure was 164/84. He had a low grade fever. His respiratory rate was 16 and pulse was regular at 88. He had a water hammer pulse with a JVP 5cm above the sternal angle with a bifid contour. The apex was 15 mm in diameter and hyperdynamic. A systolic ejection click was audible at the left upper sternal border. A grade II/VI systolic crescendo-decrescendo murmur was audible at the apex and an Austin Flint rumble was present. The spleen was palpable. There was no lymphadenopathy. Skin, nails and joints were unremarkable. There was no peripheral edema. He had mild dysarthria and a left upper motor neuron facial paresis, left hemiparesis and dense left sensory loss. The left plantar response was extensor.
THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 385From Admission bloodwork revealed a normal WBC with a Hb of 116. Electrolytes, urea, creatinine and blood glucose were normal. Urinalysis was normal. ESR was 64 and CRP was 40. An EKG met criteria for LVH. Chest x-ray was normal. ...