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Pituitary Stalk Interruption Syndrome: A Case and Literature Review
1Department of Endocrinology, Diabetes & Metabolism, University of Arizona College of Medicine, Phoenix, Arizona.
AACE Clinical Case Reports
|February 3, 2025
Summary
Pituitary stalk interruption syndrome (PSIS) is a rare disorder often missed in childhood. This case highlights the importance of brain imaging for diagnosing PSIS in adults with hypopituitarism.
Area of Science:
- Endocrinology
- Neuroscience
- Radiology
Background:
- Pituitary stalk interruption syndrome (PSIS) is a rare congenital disorder characterized by a triad of pituitary abnormalities.
- PSIS typically presents in childhood with multiple anterior pituitary hormone deficiencies.
Observation:
- A 42-year-old female presented with symptoms of hypopituitarism including premature menopause, weight loss, and dizziness.
- Physical examination revealed short stature and absent secondary sexual features.
- Brain MRI demonstrated an ectopic posterior pituitary, absent pituitary stalk, and small anterior pituitary.
Findings:
- Laboratory tests confirmed panhypopituitarism, including deficiencies in FSH, LH, cortisol, free T4, and IGF-1.
- Secondary adrenal insufficiency was confirmed via ACTH stimulation test.
- Hormone replacement therapy led to symptomatic improvement.
Implications:
- This case underscores the potential for delayed diagnosis of PSIS into adulthood.
- Early diagnosis through dedicated brain imaging is crucial for timely management of PSIS.
- Recognizing PSIS is essential for patients presenting with unexplained hypopituitarism.
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