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Cushing’s disease

Overview of hypothalamic-pituitary axis and
hormones.
Assessment of anterior pituitary function (basic
biochemical tests).
MRI and visual field assessment in patients with
HPA disease
Pituitary gland – Anatomy & Function
The pituitary gland is a pea-sized gland at the base of the brain,
divided into anterior/posterior lobes, and secretes various
hormones.
                                              Hypothala
                                              mic
                                    Target              Pituitary Feedback
                                              Regulator
                                    Gland               Hormone Hormone
                                              y
                                              Hormone
                                    Thyroid
                                              TRH      TSH       T4 T3
                                    gland
                                    Gonad     LHRH     LH        E2, T
                                                                 Inhibin,
                                    Gonad     LHRH     FSH
                                                                 E2, T
                                    Many      GHRH,
                                                       GH        IGF-1
                                    organs    SMS
                                    Breast    PIF      Prolactin ?
                                              CRH,
                                    Adrenal            ACTH      Cortisol
                                              ADH
Cushing’s disease - Introduction

Cushing’s disease vs. syndrome
Cushing’s syndrome = group of symptoms that
occur during ↑↑↑excess cortisol production
due to:
exogenous e.g. steroid medication
endogenous causes
pituitary gland adenoma – Cushing’s disease
Adrenal gland adenoma/carcinoma
ectopic ACTH tumours e.g. small cell lung cancer
Cushing’s disease = most
common form
of endogenous Cushing’s
syndrome.
It is caused by a pituitary
gland tumour that
secretes excessive
amounts of ACTH. This
type of tumor is
Pituitary Tumors:
hormone-secreting or non-secreting; most are benign;
may cause visual disturbances and headaches as they
grow and compress surrounding tissues; often results in
excessive amounts of one pituitary hormone and
decreases in others.
Signs and symptoms: Pituitary tumors may manifest
with signs and symptoms related to pituitary
hypofunction, specific hormone(s) hypersecretion,
and/or mass effect. Impingement on the chiasm or its
branches by a pituitary tumor may result in visual field
defects; the most common is bitemporalhemianopsia
Lateral extension of the pituitary mass to the cavernous
sinuses may result in diplopia, ptosis, or altered facial
sensation.Among the cranial nerves, the third nerve is
the most commonly affected.
Epidemiology

Incidence of endogenous Cushing syndrome is
about 13 cases per million individuals/yr. Of
these cases, approximately:
70% are due to Cushing disease, i.e. a pituitary ACTH-
producing tumor
15% to ectopic ACTH
15% to a primary adrenal tumor.
"normal" diurnal variation =
cortisol peaks 8 a.m. and
halves around 4 p.m. By
midnight cortisol should be
about zero or close to it with
blood and salivary levels.

Cushing's = circadian rhythm
is lost, "flat" diurnal levels of
cortisol, or even higher levels
at night instead of in the
morning.
Signs & Symptoms
depression and mood swings
moon face
weight gain around the trunk (central obesity)
proximal myopathy
Easy bruising, thin skin
Striae (purple)
Hypertension and mild diabetes
Oligo-/Amenorrhoea females
impotence - males
decrease in sex drive
Increased hair growth (hirsutism)
Cushing disease : headaches, polyuria, nocturia,
visual problems, or galactorrhea.
If sufficient mass effect from the tumor is
present on the anterior pituitary,
hyposomatotropism, hypothyroidism,
hyperprolactinemia or hypoprolactinemia, and
hypogonadism may develop.
1) Diagnose Cushing’s syndrome
2) Investigate causes of Cushing’s Syndrome
3) Treatment
se without definitive lesions on MRI should undergo inferior petrosal sinus sampling (IPSS). IPSS is th
Cushing's disease neuro case presentation open office

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Cushing's disease neuro case presentation open office

  • 1. Cushing’s disease Overview of hypothalamic-pituitary axis and hormones. Assessment of anterior pituitary function (basic biochemical tests). MRI and visual field assessment in patients with HPA disease
  • 2. Pituitary gland – Anatomy & Function The pituitary gland is a pea-sized gland at the base of the brain, divided into anterior/posterior lobes, and secretes various hormones. Hypothala mic Target Pituitary Feedback Regulator Gland Hormone Hormone y Hormone Thyroid TRH TSH T4 T3 gland Gonad LHRH LH E2, T Inhibin, Gonad LHRH FSH E2, T Many GHRH, GH IGF-1 organs SMS Breast PIF Prolactin ? CRH, Adrenal ACTH Cortisol ADH
  • 3. Cushing’s disease - Introduction Cushing’s disease vs. syndrome Cushing’s syndrome = group of symptoms that occur during ↑↑↑excess cortisol production due to: exogenous e.g. steroid medication endogenous causes pituitary gland adenoma – Cushing’s disease Adrenal gland adenoma/carcinoma ectopic ACTH tumours e.g. small cell lung cancer
  • 4. Cushing’s disease = most common form of endogenous Cushing’s syndrome. It is caused by a pituitary gland tumour that secretes excessive amounts of ACTH. This type of tumor is
  • 5. Pituitary Tumors: hormone-secreting or non-secreting; most are benign; may cause visual disturbances and headaches as they grow and compress surrounding tissues; often results in excessive amounts of one pituitary hormone and decreases in others. Signs and symptoms: Pituitary tumors may manifest with signs and symptoms related to pituitary hypofunction, specific hormone(s) hypersecretion, and/or mass effect. Impingement on the chiasm or its branches by a pituitary tumor may result in visual field defects; the most common is bitemporalhemianopsia Lateral extension of the pituitary mass to the cavernous sinuses may result in diplopia, ptosis, or altered facial sensation.Among the cranial nerves, the third nerve is the most commonly affected.
  • 6. Epidemiology Incidence of endogenous Cushing syndrome is about 13 cases per million individuals/yr. Of these cases, approximately: 70% are due to Cushing disease, i.e. a pituitary ACTH- producing tumor 15% to ectopic ACTH 15% to a primary adrenal tumor.
  • 7. "normal" diurnal variation = cortisol peaks 8 a.m. and halves around 4 p.m. By midnight cortisol should be about zero or close to it with blood and salivary levels. Cushing's = circadian rhythm is lost, "flat" diurnal levels of cortisol, or even higher levels at night instead of in the morning.
  • 8. Signs & Symptoms depression and mood swings moon face weight gain around the trunk (central obesity) proximal myopathy Easy bruising, thin skin Striae (purple) Hypertension and mild diabetes Oligo-/Amenorrhoea females impotence - males decrease in sex drive Increased hair growth (hirsutism)
  • 9. Cushing disease : headaches, polyuria, nocturia, visual problems, or galactorrhea. If sufficient mass effect from the tumor is present on the anterior pituitary, hyposomatotropism, hypothyroidism, hyperprolactinemia or hypoprolactinemia, and hypogonadism may develop.
  • 10. 1) Diagnose Cushing’s syndrome 2) Investigate causes of Cushing’s Syndrome 3) Treatment
  • 11. se without definitive lesions on MRI should undergo inferior petrosal sinus sampling (IPSS). IPSS is th