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.
Abbreviations:
a7nAChR (alpha-7 nicotinic receptor), DCSD (cognitive impairment without dementia), DSM (Diagnostic and Statistical Manual of Mental Disorders), EAE (experimental autoimmune encephalitis), IDO (indoleamine-2,3-dioxygenase), NMDA (N-methyl-D-aspartate), MC (mast cell), SCF (stem cell factor), SSRI (selective serotonin reuptake inhibitors), TNF (tumor necrosis factor)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: March 22, 2016
Accepted:
March 15,
2016
Received in revised form:
March 4,
2016
Received:
January 30,
2016
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© 2016 Elsevier Inc. All rights reserved.
