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Lethal acute demyelinization with encephalo-myelitis as a complication of cured Cushing's disease

N Chevalier1, S Hieronimus, F Vandenbos

  • 1Hôpital de l'Archet, CHU de Nice, France. chevalier.n@chu-nice.fr

Annales D'Endocrinologie
|September 21, 2010
PubMed

Insights

Cushing's disease patients may develop autoimmune disorders post-cure. This case highlights a rare instance of acute disseminated encephalomyelitis (ADEM) presenting as psychiatric illness after Cushing's disease treatment, leading to a fatal outcome.

Area of Science:

  • Neuroimmunology
  • Endocrinology
  • Autoimmune Diseases

Background:

  • Cushing's disease is linked to increased mortality, particularly cardiovascular.
  • Post-cure hypercortisolism, autoimmune, or inflammatory diseases may develop.
  • Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory neurological disorder.

Observation:

  • A 34-year-old woman with a history of psychiatric illness developed ADEM four months after surgical cure of Cushing's disease.
  • The initial presentation of ADEM mimicked a psychiatric illness, leading to a diagnostic delay.
  • The patient's death occurred following the delayed diagnosis and treatment of ADEM.

Findings:

  • This case represents the first reported instance of ADEM developing after surgical cure of Cushing's disease.
  • The study hypothesizes that the correction of hypercortisolism may have triggered the onset of ADEM.
  • Atypical, psychiatric-like presentation of ADEM contributed to delayed diagnosis and management.

Implications:

  • This case underscores the potential for autoimmune complications following the treatment of Cushing's disease.
  • Highlights the importance of considering neuroinflammatory conditions in patients with a history of hypercortisolism, especially with atypical neurological or psychiatric symptoms.
  • Emphasizes the critical role of early diagnosis and prompt treatment in managing ADEM to prevent severe outcomes.

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