Periodic Paralysis

Recently a patient went to see a Dr. Wang at the University of Washington Medical Center. She presented with muscle weakness, and a potassium level of 3.5. After a great deal of testing, Dr. Wang was certain she had HKPP, until he saw her potassium level. He diagnosed her as having "Post Traumatic Stress Disorder". The doctor reported that they had a seminar recently on HKPP. The problem is that neurologists are being given inaccurate information. In genetic HKPP or Hypokalemic Periodic Paralysis, it is the SHIFT of potassium out of the blood, and into muscle cell, that causes depolarization (nonresponsive muscle). It does not require actual hypokalemia (or a blood level of 3.5 or less). Having a very low potassium level is related to Thyrotoxic Periodic Paralysis. Many patients will have a small downward shift in their potassium level, but it is difficult to draw blood at the exact time of the shift. Generally a dip from normal levels (for the patient) is enough to cause weakness. That is why patients must avoid any activity, food or drug that can lower (or shift) potassium. When I went to PUBMED Health to read up on Hypokalemic Periodic Paralysis. I saw that misinformation was posted there by David C. Dugdale III, M.D. He wrote: "Unlike other forms of periodic paralysis, persons with congenital hypokalemic periodic paralysis have normal thyroid function and very low blood levels of potassium during episodes of weakness. This results from potassium moving from the blood into muscle cells in an abnormal way. He writes that the mutation is: CNA4A."

David C. Dugdale III, M.D. current research interests include: **Panic disorder in primary care Effective use of insulin and other diabetes treatments in primary care Patient-doctor communication in primary care Colon cancer screening

He is not a neurologist and obviously has not read current research

available about hypokalemic periodic paralysis. He even has the mutation wrong! This type of misinformation is constantly being repeated through "respected" medical websites and journals. It is very difficult to stop doctors from repeating medical facts that have been disproved by recent research and the work of patient groups. One problem with getting new information into the hands of doctors is that it takes years to reverse inaccurate information. It doesn't help that there is not a definitive study that has been done on the actual variations in serum levels of potassium in all forms of Hypokalemic Periodic Paralysis. It is currently unknown at which serum K+ level, paralysis or depolarization, occurs. The use of the term "normokalemic" appears more frequently in literature due to this huge oversight in research. It is the shift of ions (sodium, calcium, and potassium) through the mutated, or malformed

gate, that determines the physiological response. It is not the level of potassium, as measured by a lab, taken from a patient's arm, at different times during the attacks of paralysis that determines if the patient's muscles are non-responsive and cannot move. Even the use of a tourniquet on the patient's arm can change the level of potassium in the sample due to red blood cells bursting. "Hemoconcentration and possible hematoma due to infiltration of plasma and/or blood into tissue. Affects water balance of cells. Red cells and platelets rupture and release potassium" BD Troubleshooting erroneous potassiums poster. This type of misinformation drives me crazy! Here is some recent information about HKPP. Even this information has continued to evolve as researchers in Germany continue to ask more pertinent questions Free article at Skeletal Muscle Channelopathies: New insights into the periodic paralyses and nondystrophic myotonias Daniel Platt, B.S. and Robert Griggs, M.D. Hypokalemic periodic paralysis HypoPP is caused by mutations in both the -subunit of the Nav1.4 channel and the homologous 1-subunit of the skeletal muscle calcium channel, Cav1.1. Missense mutations were first identified in the calcium channel gene, CACNA1S (HypoPP1), which accounts for approximately 60% of cases (29-31). Later, it was found that about 10% of cases were due to mutations in the sodium channel gene, SCN4A (HypoPP2) (32-34). In general, HypoPP is characterized by reversible attacks of muscle weakness concomitant with decreased blood potassium concentrations. The attacks may be triggered by rest after strenuous exercise, by a meal rich in carbohydrates, or by exposure to cold. Patients typically wake up paralyzed, and attacks usually last several hours to days. Some older HypoPP patients develop progressive, persistent weakness that takes the form of a proximal myopathy. Phenotypic differences between HypoPP1 (calcium channel) and HypoPP2 (sodium channel) include: (1) earlier onset of disease in HypoPP1; (2) myalgias in HypoPP2; (3) on muscle biopsy, predominance of tubular aggregates in HypoPP2 and vacuoles in HypoPP1; and (4) aggravation of HypoPP2 by acetazolamide (31).