A 39-year-old male* with scleroderma awoke with severe headache, difficulty speaking, and right-sided weakness. When he didn’t improve after several hours, his wife brought him to the emergency department.
Past medical history included hypertension and depression. The patient had not been prescribed immunotherapy for scleroderma. He was generally adherent to his medications (escitalopram, lisinopril, and nifedipine), but ran out last month. He quit smoking 10 years ago when he was diagnosed with scleroderma. He is married, doesn’t drink alcohol, and has no children. He is on disability for peripheral vascular disease. Family history is remarkable for rheumatoid arthritis in his mother and hypothyroidism in his maternal aunt. He acknowledged episodes of Raynaud’s phenomenon in winter.
On examination, vital signs were blood pressure 234/171, pulse 87, respirations 16, and temperature 99 degrees Fahrenheit. Medical exam revealed hard and tight skin around the nose and mouth (microstomia). On neurological examination, he had normal mental status; right-sided facial droop; dysarthria; 3/5 weakness in the right hand, arm, and leg; increased reflexes on the right with a Babinski sign; normal sensation; and slow finger to nose on the right without dysmetria. Initial National Institutes of Health (NIH) stroke score was 8 (2 for facial paralysis, 2 for right arm weakness, 2 for right leg weakness, 2 for severe dysarthria).
Complete blood count, chemistry panel, liver function tests, prothrombin time, and partial thromboplastin time were normal. Chest x-ray revealed an enlarged heart and mild to moderate pulmonary edema. A transthoracic cardiac echo demonstrated concentric left ventricular hypertrophy without thrombus.
Computed tomographic (CT) brain scan revealed an acute left basal ganglia hemorrhage (Figure 1). A large hypodensity in the right basal ganglia, suggestive of a subacute or remote ischemic stroke is also present (Figure 2). Computed tomographic angiogram (CTA) of the head demonstrated normal blood vessels. CTA of the neck revealed “atheromatous plaque with mild aneurysmal dilatation of the mid-cervical left internal carotid artery without high grade stenosis or obstruction.”
The patient’s hypertension was lowered with intravenous nicardipine. Blood pressure was stabilized with oral amlodipine, carvedilol, and lisinopril. Headache disappeared once blood pressure normalized.
A brain MRI was cancelled because the patient had a femoral artery stent incompatible with the MRI magnet. The stent had been placed 2 years prior for peripheral vascular disease.
The patient’s atheromatous plaque and aneurysmal dilatation of the left internal carotid artery were most likely related to chronic hypertension. Vascular surgery was consulted to consider stenting but recommended medical management.
Dysarthria and right hemiplegia improved by the second hospital day with an NIH score of 4 (1 for facial paralysis, 1 for right arm weakness, 1 for right leg weakness and 1 for mild dysarthria). Pulmonary edema resolved. He participated in physical and occupational therapy and returned home.
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2. Amaral TN, Peres FA, Lapa AT et al. Neurologic involvement in scleroderma: A systemic review. Semin Arthritis Rheum. 2013;43:335-347.
3. Chiang CH, Liu CJ, Huang CC et al. Systemic sclerosis and risk of ischaemic stroke: a nationwide cohort study. Rheumatology. 2013;52:161-165.
4. Seshadri S, Wolf PA. Modifiable risk factors and determinants of stroke. In: Grotta JC, Albers GW, Kasner E, et al. Stroke, Pathophysiology, Diagnosis, and Management, 6th Edition. 2016; Elsevier, Inc.