Abstract Polyglucosan inclusion bodies have been de- scribed in smooth muscle of the gastrointestinal tract of aged dogs, and rarely in association with enteric dysmotil- ity in humans. We have systematically examined the hu- man small and large bowel for the presence of such inclu- sions in health and motility disorders. Systematic, blinded, dual observer analysis of colonic and ileal tissue from pa- tients (n=80, age 20–92 years) undergoing large bowel re- sections for non-dysmotile conditions, principally neopla- sia was performed, as well as retrospective review of all intestinal tissues referred for specialist histochemistry from patients undergoing surgery for motility disorders. All sections were stained with haematoxylin and eosin and periodic acid-Schiff stains. No polyglucosan bodies were identified in any specimen without dysmotility, regardless of age, but were a feature of 4/104 patients with diverse severe gastrointestinal motility disorders. In contrast to dogs, polyglucosan bodies are not a feature of normal ageing in human gastrointestinal smooth muscle but, in accord with previous suggestions, are seen in rare cases of human gut dysmotility. The significance of this difference is unclear. Keywords Ageing · Inclusion bodies · Myopathy · Polyglucosan bodies Introduction Kamiya et al. [8, 9] reported the finding of polyglucosan inclusion bodies in the digestive tract of aged dogs, with- out associated muscle dysfunction [9]. These were clearly evident on light microscopy with periodic acid-Schiff staining (PAS), were 5–20 μm in diameter, were variably stained with other methods and shown to be immunoreac- tive with polyglucosan antibody [8]. Ultrastructural study revealed a filamentous composition, which was identical to previously reported Lafora bodies found in the central nervous system [6]. The number of inclusions increased with age, and were found throughout the intestinal tract, although predominantly in the ileum and large intestine, as well as in the brain and spinal cord [9]. Polyglucosan bodies may be seen in smooth muscle in some traditional hereditary muscle glycogenoses and in disorders such as Lafora’s disease [6] and adult polyglu- cosan disease [5, 15]. The latter disorder, while systemic, includes symptoms of bladder and bowel dysfunction [15]. Specific to visceral smooth muscle, PAS-positive material has been discovered in detrusor muscle in associ- ation with bladder dysfunction [1], and in the gastroin- testinal tract, where we have described a familial myopa- thy affecting the smooth muscle of the internal anal sphincter in a number of women who presented with proc- talgia fugax and hypertrophy of the internal sphincter [10, 13] (Fig. 1a). The characteristic pathological features of this disorder were highlighted by PAS staining, with ovoid inclusion bodies, 2–30 μm in length, demonstrated. These inclusion bodies resisted diastase predigestion, and had a similar staining profile [2] and ultrastructural appearance [19] to the polyglucosan structures of corpora amylacea, seen as a usual feature in brains from the older population [7]. Similar or perhaps identical inclusion bodies have been reported in three patients with intestinal pseudo-ob- struction and visceral myopathy, which was either idio- pathic (two patients: colonic inclusions) [4] or found (a single case) in association with a probable diagnosis of scleroderma (ileal inclusions) [20, 21]. Charles H. Knowles · Carole D. Nickols · Roger Feakins · Joanne E. Martin A systematic analysis of polyglucosan bodies in the human gastrointestinal tract in health and disease Acta Neuropathol (2003) 105 : 410–413 DOI 10.1007/s00401-002-0662-1 Received: 3 June 2002 / Revised: 11 November 2002 / Accepted: 11 November 2002 / Published online: 18 January 2003 SHORT ORIGINAL COMMUNICATION C. H. Knowles Academic Department of Surgery, Barts and the London, Queen Mary’s School of Medicine, London C. D. Nickols · R. Feakins Department of Histopathology, Barts and the London, Queen Mary’s School of Medicine, London, UK J. E. Martin (✉) Department of Histopathology, Barts and the London, Queen Mary’s School of Medicine, Institute of Pathology, Royal London Hospital, Stepney Way, Whitechapel, London, E1 1BB, UK Tel.: +44-207-3777349, Fax: +44-207-3770949, e-mail: j.e.martin@qmul.ac.uk © Springer-Verlag 2003