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Cushing Syndrome I: Introduction

Cushing Syndrome I: Introduction

Cushing syndrome refers to the collection of clinical manifestations that arise when tissues are exposed to excessive amounts of cortisol or cortisol-like medications over an extended period. Cortisol, a glucocorticoid produced by the adrenal cortex, regulates metabolism, immune responses, and the body’s adaptation to stress. When its concentration remains chronically elevated, these physiological pathways become dysregulated, resulting in the characteristic features of the syndrome.Exogenous...

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Intracranial hypertension is a sustained elevation of intracranial pressure (ICP) above 22 mm Hg. In supine adults, normal ICP is ~7–15 mm Hg.The rigid, nonexpandable cranium contains three components—brain tissue, blood, and cerebrospinal fluid (CSF)—that total ~1,700 mL in a typical adult: 1,400 mL brain (~80%), 150 mL blood (~10%), and 150 mL CSF (~10%). According to the Monro–Kellie doctrine, total intracranial volume is effectively fixed. When one component expands, CSF and venous blood...

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Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...

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Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas

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Published on: January 17, 2018

Pituitary Stalk Interruption Syndrome: A Case Series.

Binod Prusty¹, Devadarshini Sahoo¹, Sambit Das¹

¹Endocrinology, Diabetes and Metabolism, Kalinga Institute of Medical Sciences, Bhubaneswar, IND.

|June 20, 2025

Summary

Pituitary stalk interruption syndrome (PSIS) is a rare congenital endocrine disorder. Early detection and hormone replacement are crucial for managing this condition and preventing long-term health issues.

Keywords:

growth hormone deficiency hypogonadotropic hypogonadism hypothyroidism pituitary stalk interruption syndrome short stature

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Area of Science:

Pediatric Endocrinology
Neuroendocrinology
Genetics

Background:

Pituitary stalk interruption syndrome (PSIS) is a rare congenital endocrine disorder.
It results from developmental anomalies of the pituitary gland, specifically the pituitary stalk and anterior pituitary.
This leads to deficiencies in anterior pituitary hormones.

Observation:

The classic triad of PSIS includes an ectopic posterior pituitary, anterior pituitary hypoplasia or aplasia, and an abnormal pituitary stalk.
PSIS presents with diverse clinical manifestations and can be identified at various life stages.
Cases may manifest as isolated growth hormone deficiency or multiple pituitary hormone deficiencies.

Findings:

The exact etiology of PSIS is unknown, but genetic mutations are suspected as a causative factor.
Hormone replacement therapy is a key treatment modality.
Early diagnosis is essential for effective management.

Implications:

Prompt diagnosis and intervention in PSIS are vital for mitigating long-term health consequences.
Understanding the genetic basis may lead to improved diagnostic and therapeutic strategies.
This case series highlights the varied clinical presentations of PSIS across different age groups.