Summary

This is a case of pulmonary alveolar proteinosis that presented in an inner city hospital in Birmingham, UK. The patient was a previously well 42-year-old man, who went on to experience unusual cerebral complications of the disease. The presentation, imaging findings and diagnostic histology findings are described. Pulmonary alveolar proteinosis is a rare but important diagnosis. Characteristic high-resolution CT findings include diffuse ground glass density with superimposed interlobular septal thickening, which is described as the “crazy paving” pattern. Diagnosis is made by bronchoalveolar lavage. Pathologically the disease is characterised by alveolar filling with a lipid rich, proteinaceous material (positive to periodic acid–Schiff stain) while the lung interstitium remains relatively normal. Morbidity and mortality can be improved by treatment with whole lung lavage.