An Uncommon Cause of Recurrent Presyncope, Dizziness, and Tachycardia: A Case Report of Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS)
Martin Philipp Dieterle,
Ayman Husari,
Sophie Nicole Prozmann,
Hendrik Wiethoff,
Albrecht Stenzinger,
Manuel Röhrich,
Uwe Pfeiffer,
Wolfgang Rüdiger Kießling,
Helena Engel,
Harald Sourij,
Thorsten Steinberg,
Pascal Tomakidi,
Stefan Kopf,
Julia Szendroedi
Affiliations
Martin Philipp Dieterle
Center for Dental Medicine, Division of Oral Biotechnology, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Ayman Husari
Center for Dental Medicine, Department of Orthodontics, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Sophie Nicole Prozmann
Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Hendrik Wiethoff
Institute of Pathology, Heidelberg University Hospital, 69120 Heidelberg, Germany
Albrecht Stenzinger
Institute of Pathology, Heidelberg University Hospital, 69120 Heidelberg, Germany
Manuel Röhrich
Department of Nuclear Medicine, University Hospital Heidelberg, 69120 Heidelberg, Germany
Uwe Pfeiffer
Pfalzklinikum for Psychiatry and Neurology AdÖR, Weinstr. 100, 76889 Klingenmünster, Germany
Cancer Immune Regulation Group, German Cancer Research Center, Im Neuenheimer Feld 280, 69120 Heidelberg, Germany
Harald Sourij
Division of Endocrinology and Diabetology, Department of Internal Medicine, Medical University of Graz, 8010 Graz, Austria
Thorsten Steinberg
Center for Dental Medicine, Division of Oral Biotechnology, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Pascal Tomakidi
Center for Dental Medicine, Division of Oral Biotechnology, Medical Center—University of Freiburg, Faculty of Medicine, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany
Stefan Kopf
Department of Internal Medicine I and Clinical Chemistry, University of Heidelberg, 69120 Heidelberg, Germany
Julia Szendroedi
Department of Internal Medicine I and Clinical Chemistry, University of Heidelberg, 69120 Heidelberg, Germany
Neurovegetative and autonomic symptoms are common presentations of various diseases, ranging from psychosomatic to severe organic disorders. A 23-year-old man presented with a history of recurrent presyncope, dizziness, and tachycardia. Repeated diagnostic work-up in various clinical settings could not identify any definite cause for approximately eight years. However, the incidental detection of postprandial and exercise-induced hypoglycemia was suggestive of an insulin-related disorder. A 72 h plasma glucose fasting test revealed endogenous hyperinsulinism. Upon imaging studies, no tumor mass potentially indicating insulinoma could be detected. 68Ga-DOTA-Exendin-4 PET/CT showed diffuse tracer enrichment throughout the whole pancreas. A subtotal pancreatectomy was performed, and the diagnosis of diffuse, adult-onset nesidioblastosis was established histopathologically. This corresponds to the clinical findings of a functional β-cell disorder, also known as non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). After nine months, the symptoms recurred, making complete pancreatectomy necessary. Postoperative laboratory evaluation exhibited no residual endogenous C-peptide production. This case illustrates the diagnostic challenges in patients presenting with unspecific, neurovegetative and autonomic symptoms with a severe and rare underlying cause.
