A new diagnosis....

We met with another Neurologist Monday morning and after a long appointment they believe they know what is causing RJ's seizures. They believe that he might have P.O.T.S . This stands for Postural Orthostatic Tachycardia Syndrome. This is also linked to NMS or Neurally Mediated Syncope. The general definition of this syndrome is:

Upright posture is a fundamental human activity requiring rapid and effective circulatory and neurologic compensations in order to maintain blood pressure and consciousness. The orthostatic tachycardia syndrome is a disabling disease state described at least since 1940 and is the most common reason for referral for chronic orthostatic intolerance. Patients are often unable to hold jobs or attend schools. Yet, our understanding of its pathophysiology remains incomplete. An operational definition of the syndrome (often denoted by the acronym POTS for postural orthostatic tachycardia syndrome) includes symptoms of orthostatic intolerance associated with an increase in heart rate from the supine to upright position of more than 30 beats per minute or to a heart rate greater than 120 beats per minute within 10 minutes of head-up tilt (HUT). An exaggerated increase in heart rate, often accompanied by hypotension in association with dizziness, nausea, palpitations, heat and fatigue in the upright position has been described under other names including the hyperadrenergic syndrome of Streeten, idiopathic hypovolemia of Fouad et al, and most recently the postural orthostatic syndrome of Low et al. POTS is common, affecting an undisclosed number of patients mostly in the age range of 12 to 50 years, mostly female (approximately 80%), often with onset following a viral infection or other inflammatory condition. There is an as yet undetermined but increasing apparent prevalence in children and adolescents. Resting tachycardia is common. Symptoms of anxiety may proliferate and appear to be autonomically mediated in many cases; there is also overlap with anxiety/panic disorder.

Is there any treatment?
Therapies for POTS are targeted at relieving low blood volume or regulating circulatory problems that could be causing the disorder. No single treatment has been found to be effect for all. A number of drugs seem to be effective in the short term. Whether they help in long term is uncertain. Simple interventions such as adding extra salt to the diet and attention to adequate fluid intake are often effective. The drugs fludrocortisone (for those on a high salt diet) and midodrine in low doses are often used to increase blood volume and narrow blood vessels. Drinking 16 ounces of water (2 glassfuls) before getting up can also help raise blood pressure. Some individuals are helped by beta receptor blocking agents. There is some evidence that an exercise program can gradually improve orthostatic tolerance.
What is the prognosis?
POTS may follow a relapsing-remitting course, in which symptoms come and go, for years. In most cases (approximately 80 percent), an individual with POTS improves and becomes functional, although some residual symptoms are common.

The way to determine for sure that this is what RJ has is to do a Tilt Table Test.
What is a tilt table test?
The tilt table is done to see if standing up provokes a sudden fall in blood pressure (neurally mediated hypotension), an excessive increase in pulse rate (Postural tachycardia syndrome) or fainting (neurally mediated syncope). The patient lies on a stretcher-like support. Straps like seatbelts are attached around the abdomen and legs and the patient is tilted upright at an angle. The exact angle varies and may be from 60 to 90 degrees. The tilting goes on for up to 45 minutes. The patient is gradually tilted to an upright position until systolic blood pressure drops to 70 mm Hg or the appearance of orthostatic symptoms such as dizziness, lightheadedness or faintness. The purpose is to hopefully reproduce the patient’s problem in a controlled laboratory setting. It may not be performed on all patients, such as patients with a persistent fall in blood pressure each time they stand up (orthostatic hypotension) because the blood pressure will fall progressively beginning as soon as the tilting starts.

We are meeting with the neurologist right after the test this morning. We will have the results right away. I am nervous. Right now I am waiting in the waiting room while they are administering the test. Wish us luck.....