The stiffness leads to a change in posture, and patients develop a rigid gait. Persistent lumbar hyperlordosis often occurs as it progresses. The muscle stiffness initially fluctuates, sometimes for days or weeks, but eventually begins to consistently impair mobility. As the disease progresses, patients sometimes become unable to walk or bend. Chronic pain is common and worsens over time but sometimes acute pain occurs as well. Stress, cold weather, and infections lead to an increase in symptoms, and sleep decreases them.SPS patients experience superimposed spasms and extreme sensitivity to touch and sound. These spasms primarily occur in the proximal limb and axial muscles. There are co-contractions of agonist and antagonist muscles. Spasms usually last for minutes and can recur over hours. Attacks of spasms are unpredictable and are often caused by fast movements, emotional distress, or sudden sounds or touches. In rare cases, facial muscles, hands, feet, and the chest can be affected and unusual eye movements and vertigo occur. There are brisk stretch reflexes and clonus occurs in patients. Late in the diseases progression, hypnagogic myoclonus can occur. Tachycardia and hypertension are sometimes also present.Because of the spasms, patients may become increasingly fearful, require assistance, and lose the ability to work, leading to depression, anxiety, and phobias, including agoraphobia and dromophobia. Most patients are psychologically normal and respond reasonably to their situations.Paraneoplastic SPS tends to affect the neck and arms more than other variations. It progresses very quickly, is more painful, and is more likely to include distal pain than classic SPS.
Macimorelin (INN) – or Macrilen (trade name) – is a drug that was developed by Æterna Zentaris for use in the diagnosis of adult growth hormone deficiency. Macimorelin acetate, the salt formulation, is a synthetic growth hormone secretagogue receptor agonist. It is a growth hormone secretagogue receptor (ghrelin receptor) agonist causing release of growth hormone from the pituitary gland.Macimorelin acetate is described chemically as D-Tryptophanamide, 2-methylalanyl-N-[(1R)-1-(formylamino)-2-(1H-indol-3-yl)ethyl]-acetate. Macimorelin (Macrilen™) was invented and first synthesized by the research group of Professor Martinez at University of Montpellier, Centre National de la Recherche Scientitifique (CNRS), France. This transpired from a long-lasting research collaboration with Aeterna Zentaris. Aeterna Zentaris later in-licensed macimorelin as a development candidate from the CNRS and proceeded with the pre-clinical and clinical development of the compound. As of January 2014, it was in Phase III clinical trials. The phase III trial for growth hormone deficiency is expected to be complete in December 2016.As of December 2017, it became FDA-approved as a method to diagnose growth hormone deficiency. Traditionally, growth hormone deficiency was diagnosed via means of insulin tolerance test (IST) or glucagon stimulation test (GST). These two means are done parenterally, whereas Macrilen boasts an oral formulation for ease of administration for patients and providers. The U.S. Food and Drug Administration (FDA) considers it to be a first-in-class medication. See also
List of growth hormone secretagogues

References
External links
"Macimorelin". Drug Information Portal. U.S. National Library of Medicine. "Macimorelin acetate". Drug Information Portal. U.S. National Library of Medicine.