Postmortem Diagnosis of Diabetic Ketoacidosis Presenting as the Dead-in-Bed Syndrome

Objective:To report a case of a young male with type 1 diabetes mellitus found dead in his bed, initially assumed to have died from hypoglycemia (i.e., the “dead in bed” syndrome). However, his autopsy findings revealed that diabetic ketoacidosis (DKA) was the cause of death.MethodsWe present the laboratory and autopsy findings of the patient, highlighting the importance of laboratory analyses of the vitreous humor and microscopy of kidney tissue when investigating the cause of sudden death in patients with type 1 diabetes.ResultsA 25-year-old healthy male with type 1 diabetes on continuous subcutaneous insulin infusion therapy was found dead in his undisturbed bed. An autopsy included vitreous humor analyses. His results were as follows: glucose of 755 mg/dL (reference range 70-105 mg/dL), anion gap > 36 mEq (reference range 4-12 mEq/L), elevated acetone at 66 mg/dL (reference range negative), which were consistent with DKA. Renal microscopy demonstrated subnuclear vacuoles in the proximal tubules, 1 of 2 lesions were described as an Armanni-Ebstein lesion, which is a postmortem finding in patients who die from diabetic coma.ConclusionThe most likely cause of death at home in young patients with type 1 diabetes is severe hypoglycemia. However, an autopsy of the present case confirmed DKA based on vitreous humor biochemistry and microscopic examination of the kidneys, which demonstrated the Armanni-Ebstein phenomenon. Analysis of the vitreous fluid and microscopic examination of the kidneys for the presence of Armanni-Ebstein lesion can be used to help determine the cause of death in patients with type 1 diabetes mellitus. (Endocr Pract. 2014;20:e123-e125)     

Hypothalamic Germinoma Masquerading as Superior Mesenteric Artery (Sma) Syndrome

ObjectiveTo report a case of superior mesenteric artery (SMA) syndrome secondary to hypothalamic germinoma.MethodsWe describe the clinical presentation, diagnostic work-up, management, and clinical course of a patient admitted with SMA syndrome who was subsequently found to have a hypothalamic germinoma.ResultsAn adolescent boy was admitted to the surgical ward with progressive weight loss over a 2 year period and postprandial vomiting. He was diagnosed with SMA syndrome based on evidence of proximal duodenal dilatation, extrinsic compression of the distal duodenum, and a narrowed aortomesenteric angle (16°). Investigations performed to exclude thyrotoxicosis unexpectedly revealed secondary hypothyroidism and further evaluation demonstrated evidence of pan-hypopituitarism. Psychiatric evaluation excluded anorexia nervosa and bulimia. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously enhancing hypothalamic lesion, but a normal pituitary gland. Hormone replacement with hydrocortisone, desmopressin, testosterone, and thyroxine resulted in weight gain and resolution of gastrointestinal symptoms. A transventricular endoscopic biopsy subsequently confirmed a hypothalamic germinoma and he was referred to an oncologist.ConclusionSMA syndrome secondary to severe weight loss is an uncommon cause of upper gastrointestinal obstruction. While there have been reports of poorly controlled diabetes mellitus and thyrotoxicosis manifesting as SMA syndrome, there are no published reports to date of SMA syndrome secondary to hypothalamic/pituitary disease. Management of SMA syndrome is conservative, as symptoms of intestinal obstruction resolve with weight gain following treatment of the underlying cause. Awareness of this uncommon presentation of endocrine cachexia/hypothalamic disease will prevent unnecessary laparotomies and a misdiagnosis of an eating disorder. (Endocr Pract. 2013;19:e29-e34)     

Sparganosis Presenting as Cauda Equina Syndrome with Molecular Identification of the Parasite in Tissue Sections

A 52-year-old woman presented with lower back pain, progressive symmetrical paraparesis with sensory impairment, and sphincter disturbance. Magnetic resonance imaging (MRI) of the whole spine revealed multiple intradural extramedullary serpiginous-mass lesions in the subarachnoid space continuously from the prepontine to the anterior part of the medulla oblongata levels, C7, T2-T8, and T12 vertebral levels distally until the end of the theca sac and filling-in the right S1 neural foramen. Sparganosis was diagnosed by demonstration of the sparganum in histopathological sections of surgically resected tissues and also by the presence of serum IgG antibodies by ELISA. DNA was extracted from unstained tissue sections, and a partial fragment of mitochondrial cytochrome c oxidase subunit 1 (cox1) gene was amplified using a primer set specific for Spirometra spp. cox1. After sequencing of the PCR-amplicon and alignment of the nucleotide sequence data, the causative agent was identified as the larva of Spirometra erinaceieuropaei.     

Sheehan Syndrome Presenting as Central Diabetes Insipidus: A Rare Presentation of an Uncommon Disorder

ObjectiveTo add to the current scant literature on rare clinical presentations of Sheehan syndrome.MethodsWe describe the study patient’s clinical, laboratory, and imaging findings and review the literature for publications regarding varied clinical presentations of Sheehan syndrome.ResultsA 36-year-old multigravida woman developed severe postpartum hemorrhage and disseminated intravascular coagulation followed by Sheehan syndrome, with central diabetes insipidus as the primary presenting feature. This was diagnosed when, postoperatively, she developed polyuria with a urine output of 11 L in 24 hours with an accompanying rise in creatinine. She had laboratory evidence of diabetes insipidus, with serum osmolality greater than urine osmolality. Her clinical status improved significantly with intranasal desmopressin supplementation, thus confirming the diagnosis of Sheehan syndrome. Although Sheehan syndrome is a known complication of postpartum hemorrhage, central diabetes insipidus is seldom considered or suspected. Hypovolemia is usually presumed to be secondary to blood loss and polyuria resulting from a diuretic phase of acute renal failure.ConclusionsIt is important to consider posterior pituitary ischemia resulting from Sheehan syndrome presenting as central diabetes insipidus as a cause of polyuria because appropriate hormonal replacement initiated early can possibly improve clinical status and patient outcomes. (Endocr Pract. 2011;17:108-114)     

Incidental Radiologic Finding of an Anterior Superior Mediastinal Mass Masquerading as Metastatic Thyroid Cancer in Patients with Treated Thyroid Cancer

ObjectiveTo demonstrate that an anterior superior mediastinal mass on radiologic imaging may represent an incidental finding and not metastases in patients with a history of treated well-differentiated thyroid cancer.MethodsWe report the clinical presentation and outcomes of 4 patients with a history of thyroid cancer who were incidentally found to have anterior superior mediastinal masses on imaging. We also review the relevant literature.ResultsFour young adults with a history of stage I papillary thyroid cancer treated with total thyroidectomy and radioiodine were incidentally found to have thymic enlargement on imaging studies within a 3-year posttreatment window. In each case, this enlargement was believed to be secondary to thymic hyperplasia and not metastatic disease, and each patient has exhibited a benign clinical course. Review of the literature revealed few reports of an association between thymic hyperplasia and thyroid cancer.ConclusionsThymic hyperplasia may be discovered on posttreatment imaging studies in patients with a history of well-differentiated thyroid cancer, particularly in young adults who have received radioiodine therapy. Recognition of the possible coexistence of this incidental finding in patients with thyroid cancer may help to avoid unnecessary invasive procedures and treatments. (Endocr Pract. 2011;17:65-69)     

Ischemic Ventricular Tachycardia Presenting as a Narrow Complex Tachycardia

This report describes a patient presenting with a narrow complex tachycardia in the context of prior myocardial infarction and impaired ventricular function. Electrophysiological studies confirmed ventricular tachycardia and activation and entrainment mapping demonstrated a critical isthmus within an area of scar involving the His-Purkinje system accounting for the narrow QRS morphology. This very rare case shares some similarities with upper septal ventricular tachycardia seen in patients with structurally normal hearts, but to our knowledge has not been seen previously in patients with ischemic heart disease.     

Pulmonary Thromboembolism Presenting as Asthma

When a patient presents with wheezing, pulmonary embolism is not usually considered as a possible cause. However, undoubtedly bronchoconstriction can be caused by pulmonary emboli and occasionally wheezing may be so obvious as to suggest a diagnosis of bronchial asthma. Eleven cases are reported in which wheezing was attributable to recurrent pulmonary emboli and one in which it was a clamant feature after a single embolic incident.