Abstract
Familial Dysautonomia (FD) is an autosomal recessive genetic disease where autonomic and sensory functions are defective affecting many body systems including the vascular. Plasma level of the neurotransmitter Calcitonin Gene Related Peptide (CGRP) is decreased in FD patients. This compound has been implicated to take part in the pathogenesis of migraine. We aimed to evaluate the symptoms of headaches in FD patients and to test the hypothesis that these patients will have a low incidence of migrainous headache. Sixty-five FD patients were evaluated by a medical headache questionnaire. Mean age was 23.73 + 10.82 years (mean 21 years) and there were 37 males (57 %).Thirty-eight patients (58.5 %) described having episodic headache conforming to criteria of tension headache, and in 17 of those 38 (44.7 %) headache were dependent on changes in blood pressure, except from one patient who had complaints that matched diagnosis of acephalic migraine. None of the patients had symptoms compatible with migraine or cluster headache. Results show that the headache is a very common complaint in FD, there is lack of migraine symptoms in this group. This might be attributed to defective sensory innervation and deficiency of CGRP. FD could be regarded as a human model for CGRP deficiency when studying the pathogenesis of migraine.
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Acknowledgments
Dr. Steiner and Dr. Maayan conceived the project, designed the study and wrote the manuscript. Dr. Shihman interviewed the patients and analyzed the data. Dr.Yovchev collected data from the clinical charts.
Prof. Steiner serves on the editorial boards of the Journal of Neurovirology, the Journal of Neurological Sciences, and Medicine Neurology (Hebrew); serves on a data safety monitoring board for Actelion Pharmaceuticals Ltd. And Hoffmann-La Roche Ltd; and has received research support from the Israel Science Foundation and the Israeli Ministry of Health Chief Scientist.
Dr. Shihman, Dr. Maayan and Dr. Yovchev have nothing to disclose.
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Shihman, B., Steiner, I., Yovchev, I. et al. Lack of migraine in headaches of familial dysautonomia patients. J Neural Transm 120, 399–402 (2013). https://doi.org/10.1007/s00702-012-0903-y
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DOI: https://doi.org/10.1007/s00702-012-0903-y