Neuronal ceroid lipofuscinosis in a 31-year-old woman presenting as biventricular heart failure with restrictive features

Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology
Michael E FealeyThomas A Orszulak

Abstract

A 31-year-old woman presented with dyspnea and left-sided chest discomfort and was found to have biventricular heart failure with impaired ventricular filling. Clinically, she was thought to have restrictive cardiomyopathy or constrictive pericarditis. Transmission electron microscopy of myocardial tissue unexpectedly revealed crosshatched, curvilinear, and fingerprint depositions, which were characteristic for neuronal ceroid lipofuscinosis. Cardiac involvement by this inherited disorder is discussed in light of the findings in this patient and in 15 other reported cases.

References

Jan 1, 1986·Journal of Molecular and Cellular Cardiology·D ArmstrongA Ellis
Apr 12, 2001·Ultrastructural Pathology·R BoldriniC Bosman
Aug 8, 2001·Journal of the Neurological Sciences·S A JosephsonJ C Morris
May 4, 2004·Seizure : the Journal of the British Epilepsy Association·Sanjib SinhaS K Shankar

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