THE HEREDITARY SYNDROME OF THROMBOCYTOPATHIA, BLEEDING TENDENCY, EXTREME MIOSIS, MUSCULAR FATIGUE, ASPLENIA, HEADACHE, ETC (STORMORKENS SYNDROME) - .1. THE HEADACHE

A new, autosomal dominantly inherited syndrome with a bleeding disorde r was described in 1985 by Stormorken and his co-workers.1,2 In this m ultifaceted syndrome, there were the following integral components: th rombocytopathia, extreme miosis with Argyll Robertson-like traits, mus cular fatigue, a tendency to spasms, asplenia, ichthyosis, dyslexia, e tc. Headache with migraine traits was also present in the family in al l 4 generations in which this syndrome had been observed. Nasal and co njunctival bleeding were part of the headache picture in some of the i ndividuals exhibiting the hemorrhagic syndrome. While the attack-relat ed bleeding disturbances only involved family members who also suffere d from the hemorrhagic syndrome, the headache per se may seem to behav e differently: The affected son's headache seems to have developed int o a headache with tension headache traits, whereas the other, unaffect ed, son's headache has common migraine traits. The familial headache w hich in earlier generations clearly had migraine traits, therefore, ma y be inherited independently from the hemorrhagic disorder. In other w ords, a migraine or migraine-like headache is most probably not an obl igatory integral part of this syndrome. The thrombocytopathis in this disorder comprises abnormal serotonin storage, uptake, and release (St ormorken and co-workers, to be published). The admittedly somewhat far fetched possibility also exists that the headache, although being simi lar to migraine, differs essentially from it and may be an expression of the serotonin aberration.