Lafora body-like inclusions
Synopsis
Lafora-like ground glass inclusions
- eosinophilic, PAS-diastase positive intracytoplasmic inclusions
- negative for Hepatitis B surface antigen
Immunohistochemistry
positive staining with KM279, a monoclonal antibody against polyglucosan derived from Lafora inclusions (17378668)
Electron microscopy
intracytoplasmic inclusions composed of degenerated organelles, glycogen and irregular fibrillar structures (17378668)
vesicular structures containing granular material (17378668)
(Ultrastructural changes in both cases differed from classical Lafora inclusions and ruled out hepatitis B surface antigen, glycogenosis type IV and fibrinogen storage disease.)
Etiology - Associations
cyanamide treatment
progressive myoclonic ataxia associated with coeliac disease (11598336)
adult-onset rapidly progressive spinal muscular atrophy of shoulder girdle with gammopathy (6627777)
alpha-thalassaemia major and iron chelation therapy (17378668)
trisomy 21 with bone marrow allograft for acute myeloid leukemia (17378668)
Differential diagnosis
hepatocytic ground glass inclusions
- classical Lafora inclusions (Lafora bodies) in Lafora disease
- hepatitis B
- glycogenosis type 4 (GSD4)
- fibrinogen storage disease
See also
References
O?shea AM, Wilson G, Ling S, Minassian B, Turnbull J, Cutz E. Lafora-like ground glass inclusions in hepatocytes of pediatric patients. A report of two case. Pediatr Dev Pathol. 2007 Mar 22;:1. PMID: 17378668
Ng IO, Sturgess RP, Williams R, Portmann B. Ground-glass hepatocytes with Lafora body like inclusions?histochemical, immunohistochemical and electronmicroscopic characterization. Histopathology. 1990 Aug;17(2):109-15. PMID: 2172143
Tuzun E, Gurses C, Baykan B, Buyukbabani N, Ozturk AS, Gokyigit A. Lafora body-like inclusions in a case of progressive myoclonic ataxia associated with coeliac disease. Eur Neurol. 2001;46(3):157-8. PMID: 11598336