Patients with mastocytosis can display various disabling general and neuropsychological symptoms among one third of them, including general signs such as fatigue and musculoskeletal pain, which can have a major impact on quality of life. Neurological symptoms are less frequent and mainly consist of acute or chronic headache (35%), rarely syncopes (5%), acute onset back pain (4%), and in a few cases, clinical and radiological symptoms resembling or allowing the diagnosis of multiple sclerosis (1.3%). Headaches are associated with symptoms related to mast cell activation syndrome (flushes, prurit, and so forth) and more frequently present as migraine (37.5%), with often aura (66%). Depression-anxiety like symptoms can occur in 40% to 60% of the patients and cognitive impairment is not rare (38.6%). The pathophysiology of these symptoms could be linked to tissular mast cell infiltration or to mast cell mediators release or both. The tryptophan metabolism could be involved in mast cell-induced neuroinflammation through indoleamine-2,3-dioxygenase activation. Treatments targeting mast cell may be useful to target neuropsychological features associated with mastocytosis, including tyrosine kinase inhibitors.
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