Anti-Ma-1 and Anti-Ma-2 antibodies in isolated fatal hypothalamitis

Mario Bustos, Hara Berger, Zeina Carolina Hannoush, Alejandro Ayala, Rochelle Freire, Atil Yilmaz Kargi

Research output: Contribution to journalArticle

2 Scopus citations

Abstract

Lymphocytic hypothalamitis (LHT) is a rare disease characterized by pituitary dysfunction, autonomic instability, metabolic disturbances, and neuropsychiatric disorders. We report the case of a 30-year-old man found to have LHT that progressed despite treatment with methylprednisolone and intravenous immunoglobulin (IVIG). A literature review was conducted to identify prior studies pertaining to LHT. Our patient presented with several weeks of fatigue, cold intolerance, weight loss, confusion, and headache. Laboratory tests were consistent with panhypopituitarism. Brain magnetic resonance imaging revealed an infiltrative enhancing mass in the area of the hypothalamus, and stereotactic biopsy findings showed reactive inflammatory changes. A course of hormone replacement (levothyroxine), methylprednisolone, and IVIG was initiated. The patient's course was complicated by a fatal tonsillar herniation. Autopsy revealed LHT. The diagnosis and management of autoimmune LHT are challenging. The entity should be considered in the setting of panhypopituitarism with a hypothalamic mass. Differentiating paraneoplastic and nonparaneoplastic hypothalamitis should be established for management and prognosis. Definitive treatment remains unclear; treatment with corticosteroids should be attempted, followed by consideration of other immunosuppressive agents, such as rituximab. If a paraneoplastic syndrome is suspected, management should also be directed toward the primary tumor.

Original languageEnglish (US)
Pages (from-to)106-111
Number of pages6
JournalJournal of the Endocrine Society
Volume2
Issue number2
DOIs
StatePublished - Feb 2018

Keywords

  • Encephalitis
  • Hypothalamitis
  • Management
  • PLE

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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