A 32-year-old woman is brought to the hospital due to sudden-onset left-sided weakness. According to the family, she has had fatigue, malaise, and decreased appetite over the last several weeks. She attributed these symptoms to a "viral infection" and treated herself with over-the-counter acetaminophen. Her temperature is 37.4 C (99.4 F), blood pressure is 140/86 mm Hg, and pulse is 82/min. The patient appears confused and agitated. Lungs are clear to auscultation. A 2/6 holosystolic murmur is heard at the cardiac apex. Extremity examination reveals left-sided muscle strength is 1/5 with hyperactive deep tendon reflexes. Babinski sign is present on the left side. Laboratory results are as follows: Cerebrospinal fluid cell counts are normal, and cultures are negative. Which of the following is the most likely diagnosis in this patient?

Question

A 32-year-old woman is brought to the hospital due to sudden-onset left-sided weakness.  According to the family, she has had fatigue, malaise, and decreased appetite over the last several weeks.  She attributed these symptoms to a "viral infection" and treated herself with over-the-counter acetaminophen.  Her temperature is 37.4 C (99.4 F), blood pressure is 140/86 mm Hg, and pulse is 82/min.  The patient appears confused and agitated.  Lungs are clear to auscultation.  A 2/6 holosystolic murmur is heard at the cardiac apex.  Extremity examination reveals left-sided muscle strength is 1/5 with hyperactive deep tendon reflexes.  Babinski sign is present on the left side.  Laboratory results are as follows:  Cerebrospinal fluid cell counts are normal, and cultures are negative.  Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

11ec25c8_d56a_1a07_bc81_bda6ce5c67af_MD0003_00 This young woman with constitutional symptoms (fatigue), thrombocytopenia, probable mitral regurgitation (holosystolic murmur to the apex), and a positive antinuclear antibody (ANA), suggestive of systemic lupus erythematosus (SLE), now likely has a cerebrovascular accident (CVA) (sudden-onset unilateral weakness, negative cerebrospinal fluid [CSF] findings). Given these features, antiphospholipid syndrome (APS) in the setting of SLE is likely. APS presents with a thrombotic event (deep venous thrombus or arterial thrombus) or pregnancy morbidity (fetal loss, severe preeclampsia, placental insufficiency) plus a positive serology for 1 of 3 antiphospholipid antibodies: anticardiolipin antibody, anti-beta2-glycoprotein-I antibody, or lupus anticoagulant. The biggest risk factor for APS is SLE; APS occurs in up to 40% of these patients. A minority of healthy individuals may develop transient antiphospholipid antibodies, so all positive serology for APS should be repeated at 12 weeks to confirm diagnosis. Patients with APS often require anticoagulation for life (heparin product or warfarin) as the risk of recurrent thrombus is high. For those with concomitant SLE, hydroxychloroquine is also added. (Choice B) Multiple sclerosis (MS) presents with neurologic symptoms, but transverse myelitis causing a paraplegia or paraparesis is much more common than hemiparesis. As MS is inflammatory, symptoms tend to develop over hours or days, not suddenly. MS also would not explain this patient's hematologic abnormalities, positive ANA/rapid plasma reagin (RPR), or murmur. (Choice C) Neurosyphilis can occasionally present with an arteritis causing a cerebrovascular event in a young patient, but it is typically accompanied by abnormal CSF cell counts (lymphocytic pleocytosis, 10-100/mm³). In patients with APS, a false positive RPR test is not uncommon as the syphilis antigen used in RPR testing contains cardiolipin. (Choice D) Polyarteritis nodosa can cause a CVA in a minority of patients, but mononeuritis and polyneuropathy are far more common. In addition, other findings such as arthralgias, myalgias, and cutaneous, renal, and/or gastrointestinal disease are often also present. (Choice E) Rheumatic fever causes chorea-like movements (Sydenham chorea). It commonly presents with carditis and migratory arthritis. Educational objective: Antiphospholipid syndrome is characterized by a thrombotic event or pregnancy morbidity in the setting of a positive autoantibody to 1 of 3 phospholipids - anticardiolipin antibody, anti-beta2-glycoprotein-I antibody, or lupus anticoagulant. It is very common in patients with systemic lupus erythematosus.

A 32-Year-Old Woman Is Brought to the Hospital Due to Sudden-Onset