Neurology: Case of the Month

Test Yourself — Patient 49

Churg-Strauss Syndrome

1. Which of the following would be MOST consistent with a diagnosis of Churg-Strauss syndrome (CSS)?
   • [ A ] Early-onset renal failure
   • [ B ] Cerebral infarction
   • [ C ] Active hepatitis B infection
   • [ D ] Beading pattern on angiography of the superior mesenteric artery
   • [ E ] Polyneuropathy
2. Which of the following features would be MOST suggestive of another disorder mimicking the Guillain-Barre syndrome (GBS)?
   • [ A ] Elevated CSF protein on initial assessment, with occasional lymphocytes present
   • [ B ] Slowed nerve conduction velocities on electrodiagnostic studies
   • [ C ] Normal CSF protein on initial assessment, with elevated CSF protein documented two weeks after the initial study
   • [ D ] Recovery to near-normal strength delayed by more than eight weeks
   • [ E ] Initial clinical improvement followed by relapse within four weeks
3. Which of the following features is MOST suggestive of a diagnosis of Wegener's granulomatosis in a patient with asthma, eosinophilia and pulmonary infiltrates?
   • [ A ] Ulceration of the nasal septum
   • [ B ] Positive serum titers for the perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA)
   • [ C ] Necrotizing granulomas on skin biopsy
   • [ D ] Necrotizing granulomas on muscle biopsy
   • [ E ] Atrial fibrillation
4. Which of the following BEST describes a typical presentation of a patient with the Churg-Strauss syndrome (CSS)?
   • [ A ] A 50 year old man with chronic asthma since childhood, managed with increasing doses of corticosteroids, who has developed mild, painless proximal weakness over the last eight years.
   • [ B ] A 45 year old man with known hepatitis B, who develops rapidly progressive weight loss, sudden onset weakness of right ankle dorsiflexion, and mild dyspnea, and is found to have an elevated and fixed left hemidiaphragm on clinical and radiographic evaluation.
   • [ C ] A 50 year old woman with severe, steroid-dependent asthma of two years' duration, who presents with an eight-week history of numbness and tingling of the lower extremities, accompanied by a mild skin rash.
   • [ D ] A 25 year old man with a history of childhood asthma and recent intravenous drug use, who develops fever and the sudden onset of aphasia and hemiparesis, and is found to have a heart murmur.
5. In patients with the Churg-Strauss syndrome (CSS), which of the following features would be LEAST suggestive of a poor prognosis?
   • [ A ] Renal failure
   • [ B ] Palpable purpuric skin rash
   • [ C ] Congestive heart failure
   • [ D ] Recurrent strokes
   • [ E ] Small bowel obstruction

 

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