Cushing's disease neuro case presentation open office

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Neuro case presentation

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  • 1. Cushings diseaseOverview of hypothalamic-pituitary axis andhormones.Assessment of anterior pituitary function (basicbiochemical tests).MRI and visual field assessment in patients withHPA disease

2. Pituitary gland Anatomy & FunctionThe pituitary gland is a pea-sized gland at the base of the brain,divided into anterior/posterior lobes, and secretes varioushormones.HypothalamicTargetPituitary FeedbackRegulatorGland Hormone HormoneyHormoneThyroidTRHTSH T4 T3glandGonad LHRH LHE2, T Inhibin,Gonad LHRH FSH E2, TManyGHRH, GHIGF-1organsSMSBreastPIFProlactin ?CRH,AdrenalACTHCortisolADH 3. Cushings disease - IntroductionCushings disease vs. syndromeCushings syndrome = group of symptoms thatoccur during excess cortisol productiondue to:exogenous e.g. steroid medicationendogenous causespituitary gland adenoma Cushings diseaseAdrenal gland adenoma/carcinomaectopic ACTH tumours e.g. small cell lung cancer 4. Cushings disease = mostcommon formof endogenous Cushingssyndrome.It is caused by a pituitarygland tumour thatsecretes excessiveamounts of ACTH. Thistype of tumor is 5. Pituitary Tumors:hormone-secreting or non-secreting; most are benign;may cause visual disturbances and headaches as theygrow and compress surrounding tissues; often results inexcessive amounts of one pituitary hormone anddecreases in others.Signs and symptoms: Pituitary tumors may manifestwith signs and symptoms related to pituitaryhypofunction, specific hormone(s) hypersecretion,and/or mass effect. Impingement on the chiasm or itsbranches by a pituitary tumor may result in visual fielddefects; the most common is bitemporalhemianopsiaLateral extension of the pituitary mass to the cavernoussinuses may result in diplopia, ptosis, or altered facialsensation.Among the cranial nerves, the third nerve isthe most commonly affected. 6. EpidemiologyIncidence of endogenous Cushing syndrome isabout 13 cases per million individuals/yr. Ofthese cases, approximately:70% are due to Cushing disease, i.e. a pituitary ACTH-producing tumor15% to ectopic ACTH15% to a primary adrenal tumor. 7. "normal" diurnal variation =cortisol peaks 8 a.m. andhalves around 4 p.m. Bymidnight cortisol should beabout zero or close to it withblood and salivary levels.Cushings = circadian rhythmis lost, "flat" diurnal levels ofcortisol, or even higher levelsat night instead of in themorning. 8. Signs & Symptomsdepression and mood swingsmoon faceweight gain around the trunk (central obesity)proximal myopathyEasy bruising, thin skinStriae (purple)Hypertension and mild diabetesOligo-/Amenorrhoea femalesimpotence - malesdecrease in sex driveIncreased hair growth (hirsutism) 9. Cushing disease : headaches, polyuria, nocturia,visual problems, or galactorrhea.If sufficient mass effect from the tumor ispresent on the anterior pituitary,hyposomatotropism, hypothyroidism,hyperprolactinemia or hypoprolactinemia, andhypogonadism may develop. 10. 1) Diagnose Cushings syndrome2) Investigate causes of Cushings Syndrome3) Treatment 11. se without definitive lesions on MRI should undergo inferior petrosal sinus sampling (IPSS). IPSS is th