What is a
Hypothalamic
Hamartoma?


The tiny hypothalamus serves as the Health Maintenance Organization of the body, regulating its homeostasis, or stable state of equilibrium. The hypothalamus also generates behaviors involved in eating, drinking, general arousal, rage, aggression, embarrassment, escape from danger, pleasure and copulation. It does an amazing number of housekeeping chores for such a small piece of tissue. Its lateral and anterior parts seem to support activation of the parasympathetic nervous system: drop in blood pressure; slowing of pulse; and regulation of digestion, defecation, assimilation, and reproduction in such a way as to contribute on the whole to rest and recovery. The medial and posterior hypothalamus regulate activation: acceleration of pulse and breathing rates, high blood pressure, arousal, fear and anger. Stimulation of specific groups of cells in these areas can elicit pure behaviors. For example, rats placed in an experimental situation where they can press a lever to stimulate a pleasure center will do so to the exclusion of eating and drinking. Stimulation of another area can produce rage.


1. Hypothalamus = Homeostasis

The main function of the hypothalamus is homeostasis, or maintaining the body's status quo. Factors such as blood pressure, body temperature, fluid and electrolyte balance, and body weight are held to a precise value called the set-point. Although this set-point can migrate over time, from day to day it is remarkably fixed.

To achieve this task, the hypothalamus must receive inputs about the state of the body, and must be able to initiate compensatory changes if anything drifts out of whack. The inputs include:

nucleus of the solitary tract - this nucleus collects all of the visceral sensory information from the vagus and relays it to the hypothalamus and other targets. Information includes blood pressure and gut distension.
reticular formation - this catchall nucleus in the brainstem receives a variety of inputs from the spinal cord. Among them is information about skin temperature, which is relayed to the hypothalamus.
retina - some fibers from the optic nerve go directly to a small nucleus within the hypothalamus called the suprachiasmatic nucleus. This nucleus regulates circadian rhythms, and couples the rhythms to the light/dark cycles.
circumventricular organs - these nuclei are located along the ventricles, and are unique in the brain in that they lack a blood-brain barrier. This allows them to monitor substances in the blood that would normally be shielded from neural tissue. Examples are the OVLT, which is sensitive to changes in osmolarity, and the area postrema, which is sensitive to toxins in the blood and can induce vomiting. Both of these project to the hypothalamus.
limbic and olfactory systems - structures such as the amygdala, the hippocampus, and the olfactory cortex project to the hypothalamus, and probably help to regulate behaviors such as eating and reproduction.
The hypothalamus also has some intrinsic receptors, including thermoreceptors and osmoreceptors to monitor temperature and ionic balance, respectively.

Once the hypothalamus is aware of a problem, how does it fix it? Essentially, there are two main outputs:

neural signals to the autonomic system - the (lateral) hypothalamus projects to the (lateral) medulla, where the cells that drive the autonomic systems are located. These include the parasympathetic vagal nuclei and a group of cells that descend to the sympathetic system in the spinal cord. With access to these systems, the hypothalamus can control heart rate, vasoconstriction, digestion, sweating, etc.
endocrine signals to/through the pituitary - recall that an endocrine signal is a chemical signal sent via the bloodstream. Large hypothalamic cells around the third ventricle send their axons directly to the posterior pituitary, where the axon terminals release oxytocin and vasopressin into the bloodstream. Smaller cells in the same area send their axons only as far as the base of the pituitary, where they empty releasing factors into the capillary system of the anterior pituitary. These releasing factors induce the anterior pituitary to secrete any one of at least six hormones, including ACTH and thyroid-stimulating hormone (TSH).
Ultimately the hypothalamus can control every endocrine gland in the body, and alter blood pressure (through vasopressin and vasoconstriction), body temperature, metabolism (through TSH), and adrenaline levels (through ACTH).

In the news lately: The hypothalamus controls body weight and appetite, but it is not entirely clear how. Sensory inputs, including taste, smell, and gut distension, all tell the hypothalamus if we are hungry, full, or smelling a steak. Yet it is mysterious how we are able to vary our eating habits day to day and yet maintain about the same weight (sometimes despite all efforts to the contrary!) . The "set-point" theory is an old one in diet science, but until recently the mechanics of maintaining that set point were unknown. It appears that there is an endocrine component to the appetite system. Recent studies in mice have shown that the fat cells of normal overfed mice will release a protein called leptin (or OB, after the gene name), which reduces appetite and perks up metabolism. Leptin is presumably acting on the hypothalamus. Underfed mice, on the other hand, produce little or no leptin, and they experience an increase in appetite and a decrease in metabolism. In both of these mice, the result is a return to normal weight. But what would happen if a mouse (or human) had a defective OB gene? Weight gain would never trigger fat cells to release leptin, the hypothalamus would never slow the appetite or increase metabolism, and the mouse would slowly but surely become obese (how the gene got its name). Sure enough, shortly after these experiments hit the news, the human OB gene was discovered and a few obese patients were found to have the mutation. Many more obese patients had normal OB genes, however, indicating that there is much more to the story yet to be discovered.