Marchiafava-Bignami Syndrome in a Diabetic Patient

Document Type

Conference Proceeding

Publication Date

10-2024

Publication Title

Journal of the Endocrine Society

Abstract

Introduction: Marchiafava-Bignami Syndrome (MBS), described as demyelination and necrosis of the corpus callosum, is a rare and fatal neurological disorder commonly caused by chronic alcoholism and malnutrition. However, there have been isolated cases of MBS reported in nonalcoholic patients, including those with uncontrolled diabetes mellitus. We report a diabetic patient who presented with anoxic hypoglycemic encephalopathy, subsequently diagnosed with MBS without a preceding history of chronic alcoholism or malnutrition. Clinical Presentation: A 56-year-old male with a past medical history of longstanding, uncontrolled type 2 diabetes mellitus, hypertension, pulmonary hypertension, congestive heart failure, chronic kidney disease(CKD), and class 3 obesity with a BMI of 43 kg/m2 was hospitalized for unresponsiveness. On arrival to the ED, his blood glucose was 37 mg/dl, and he received 1 amp of dextrose. On examination, Glasgow Coma Scale was 6 and the patient was intubated and transferred to intensive care. Labs were remarkable for hemoglobin A1c 7.0% in the setting of anemia with hemoglobin 10.4 g/dL (12.0-15.5), C-peptide 14.3 ng/ml (1.1-4.4), and insulin 4.0 uIU/mL (< 25), with a corresponding blood glucose of 141 mg/dl. MRI brain revealed necrosis and demyelination of the splenium of the corpus callosum, consistent with MBS. The patient was found to be persistently hypoglycemic, requiring IV dextrose infusion and octreotide for sulfonylurea-induced hypoglycemia, in the setting of renal dysfunction. The patient’s family corroborated noncompliance with diabetes medications. Extensive tests including CSF analysis for various infections and malignancies yielded no results. The patient was eventually transferred to hospice care and expired, after prolonged hospitalization. Conclusion: The etiology of MBS is most commonly alcoholism and malnutrition; however, there are rare case reports of MBS in patients with poorly controlled diabetes who are not alcoholic or malnourished. Wide glycemic fluctuations and subsequent osmotic changes are thought to contribute to the pathophysiology of MBS in diabetic patients. Hypoglycemia can induce osmotic stress in oligodendroglial cells, leading to the structural and functional abnormalities seen in MBS. In this patient with longstanding uncontrolled diabetes and medication noncompliance, in the setting of CKD and sulfonylurea use, wide glycemic fluctuations likely contributed to the development of this atypical case of MBS. Reference: Marchiafava-Bignami disease triggered by poorly controlled diabetes mellitus A.I. Pérez Álvarez, C. Ramón Carbajo, G. Morís de la Tassa, J. Pascual Gómez https://www.elsevier.es/ en-revista-neurologia-english-edition–495-articulo-marchiafava-bignami-disease-triggered-by-poorly-S2173580816300633

Volume

8

Issue

Suppl 1

First Page

A438

Last Page

A439

Comments

ENDO 2024 Endocrine Society Annual Meeting, June 1-4, 2024, Boston, MA

DOI

10.1210/jendso/bvae163.838

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