We describe an exceptional clinical picture, namely, cognitive impairment of the Alzheimer disease type in a man who later developed manifestations typical of amyotrophic lateral sclerosis and who was subsequently found to have adult polyglucosan body disease (APGBD) upon postmortem neuropathologic explorations. The combined occurrence of amyotrophic lateral sclerosis and cognitive impairment of the Alzheimer disease type in APGBD has not been reported before. This case also underlines the diverse clinical presentation of this rare clinicopathologic entity (namely APGBD) and highlights the importance of recognizing the unusual association of clinical features in making the diagnosis.

Malakoplakia is a protean systemic chronic granulomatous disease that usually targets the genito‐urinary system and most particularly the bladder. Only in rare occasions do the kidneys get involved. Myelodysplastic syndrome is a heterogeneous clonal haematopoietic disorder characterised by either a hyper‐ or hypocellular bone marrow dysmyelopoiesis and peripheral blood pancytopenia. The synchronous association of these two unrelated disorders has never been reported. We report a 62‐year‐old woman who initially developed vague, relapsing gastrointestinal symptoms synchronous with renal malakoplakia and myelodysplastic syndrome.

No abstract