3rd Year LPUSC School of Health Sciences Copyrights apply Copyrights apply Copyrights apply HPAxis
• A complex set of direct influences and
feedback interactions among three components: the hypothalamus, the pituitary gland and the effector glands Copyrights apply Raised Intracranial Pressure MJ 15 Male • CC: headaches • Noticed to be gaining weight at age 14 yrs old with occasional fatigue and cold intolerance • Dull, continuous headaches of 4/5 relieved with paracetamol. Headaches are worse on lying down and on turning to sides. • Upon getting out of bed, had feeling of blacking out. He is also noticed to be moving his tablet horizontally when reading. • He claims to be urinating frequently • Because of the progressive headaches becoming 7-8/10 the past week with episodes of vomiting. Complete the above case • Is there a neurologic problem? • If yes, what level is the neurologic problem? • Localize • What is the temporal profile? • Etiologic DDX Differentials • Pituitary Gland Tumor ( Macroadenoma) • Brainstem glioma/tumor • Giant carotid aneurysm • Cavernous sinus syndrome • Migraine Work up • IMAGING • Brain MRI with and without contrast is the gold standard. • ENDOCRINE EVALUATION • Baseline serum electrolytes, serum and urine osmolality, thyroid studies, morning and evening cortisol levels, growth hormone levels, and luteinizing and follicle-stimulating hormone levels, in pediatric as well as adult patients. • In emergent cases, hormonal testing should be limited to diagnosing diabetes insipidus, hypoadrenalism, and hypothyroidism, as these hormones require the initiation of treatment prior to surgery. • HISTOLOGIC • Three main types: adamantinomas, papillary craniopharyngiomas, and mixed tumors. • The MIB-1 labeling index is a measure of the disease’s proliferative activity. It is determined by using an immunohistochemical method with monoclonal antibody MIB-1 and may be useful for the planning of adjuvant therapy. One study reported that an MIB-1 labeling index of greater than 7% predicted regrowth/recurrence. Diabetes Insipidus versus SIADH T1 Weighted Images Adamantinomatous craniopharyngioma Papillary craniopharyngiomas Craniopharyngioma • Slow-growing, extra-axial, epithelial-squamous, calcified, and cystic tumor arising from remnants of the craniopharyngeal duct and/or Rathke cleft and occupying the sellar/suprasellar region Craniopharyngioma • Rare malformational tumours of low histological malignancy arising along the craniopharyngeal duct. • Benign but locally invasive tumours of the sellar region that arise from ectopic embryonic remnants of Rathke's pouch, • The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP), differ in genesis and age distribution. • ACPs are diagnosed with a bimodal peak of incidence (5–15 years and 45–60 years), whereas PCPs are restricted to adults mainly in the fifth and sixth decades of life. • ACPs are driven by somatic mutations in CTNNB1 (encoding β-catenin) that affect β-catenin stability and are predominantly cystic in appearance. • PCPs frequently harbour somatic BRAFV600E mutations and are typically solid tumours. • Clinical manifestations due to increased intracranial pressure, visual impairment and endocrine deficiencies should prompt imaging investigations, preferentially MRI. Craniopharyngioma • Most frequently arise in the pituitary stalk and project into the hypothalamus. They extend horizontally along the path of least resistance in various directions, as follows: • Anteriorly - Into the prechiasmatic cistern and subfrontal spaces • Posteriorly - Into the prepontine and interpeduncular cisterns, cerebellopontine angle, third ventricle, [3] posterior fossa, and foramen magnum • Laterally - Toward the subtemporal spaces • Clinical behavior and the choice of surgical approach are dictated by the primary location of the tumor and its extension pattern. • Prechiasmatic craniopharyngiomas (extending into the subfrontal spaces) • Retrochiasmatic craniopharyngiomas (expanding into the posterior fossa) may become large before being diagnosed. Treatment Approach 1. attempt a gross total resection 2. perform a planned subtotal resection followed by radiotherapy or some other adjuvant therapy.
Successful management is determined by the ability to preserve independent social functioning,
prevent symptomatic recurrence, and increase survival rate. There has been significant debate in recent years regarding the outcomes of GTR (Gross total removal) in the pediatric population given the high risk for hypothalamic injury and deficits, which can be life- altering in children (i.e., extreme obesity, deterioration in educational abilities). Prognosis • Neuropsychological deficits represent the major limiting factor for independent social functioning because • (1) patients often can overcome minor neurologic deficits and • (2) hormone replacement therapies are widely available. • The degree of psychosocial impairment correlates directly with the degree of hypothalamic injury sustained at the time of surgery. • Atypical antidepressants. These medications don't fit neatly into any of the other antidepressant categories. More commonly prescribed antidepressants in this category include trazodone, mirtazapine (Remeron), vortioxetine (Trintellix), vilazodone (Viibryd) and bupropion (Wellbutrin SR, Wellbutrin XL, others). Bupropion is one (antidepressants) of the few antidepressants not frequently associated with sexual side effects. • Tricyclic antidepressants. Tricyclic antidepressants — such as • Selective serotonin reuptake inhibitors (SSRIs). imipramine (Tofranil), nortriptyline (Pamelor), amitriptyline, doxepin Doctors often start by prescribing an SSRI. These and desipramine (Norpramin) — tend to cause more side effects than medications generally cause fewer bothersome side newer antidepressants. So tricyclic antidepressants generally aren't effects and are less likely to cause problems at higher prescribed unless you've tried other antidepressants first without therapeutic doses than other types of antidepressants improvement. are. SSRIs include fluoxetine (Prozac), paroxetine • Monoamine oxidase inhibitors (MAOIs). MAOIs — such as (Paxil, Pexeva), sertraline (Zoloft), citalopram tranylcypromine (Parnate), phenelzine (Nardil) and isocarboxazid (Celexa) and escitalopram (Lexapro). (Marplan) — may be prescribed, often when other medications haven't • Serotonin and norepinephrine reuptake worked, because they can have serious side effects. Using an MAOI requires a strict diet because of dangerous (or even deadly) inhibitors (SNRIs). Examples of SNRI medications interactions with foods — such as certain cheeses, pickles and wines include duloxetine (Cymbalta), venlafaxine (Effexor — and some medications, including pain medications, decongestants XR), desvenlafaxine (Pristiq) and levomilnacipran and certain herbal supplements. Selegiline (Emsam), an MAOI that (Fetzima). you stick on your skin as a patch, may cause fewer side effects than other MAOIs. These medications can't be combined with SSRIs. (anxiolytics) • Benzodiazepines • Ca channel inhibitors • Antihistamine