Mixed organic brain syndrome as a manifestation of systemic mastocytosis.

Abstract

Systemic mastocytosis is a disease characterized by an excessive accumulation of mast cells, and associated with skin lesions, flushing, diarrhea, tachycardia, and psychiatric manifestations. In order to define more clearly the psychiatric manifestations, ten patients with this disorder underwent unstructured psychiatric interviews and a battery of psychologic testing. Both revealed a pattern of cognitive and affective changes in the majority of these patients, best categorized as an atypical or mixed organic brain syndrome. The cognitive changes consisted of diminished attention and memory, and the affective changes of anger, irritability, and, to a lesser extent, depression. These manifestations fluctuated with the level of disease activity, and appeared in some cases to respond to histamine antagonists and disodium cromoglycate, medications used to control the excessive mast cell activity. It is important for psychiatrists to be aware that mental status changes can represent psychiatric manifestations of mastocytosis, a readily treatable medical disorder.

Current Options in the Treatment of Mast Cell Mediator-Related Symptoms in Mastocytosis

“…Those patients are often misdiagnosed due to the nonspecific nature of the symptoms and the lack of studies of MC activity in the human brain in patients with SM. Symptoms include decreased attention span, difficulty in concentration, forgetfulness, irritability, depression, poor motivation, confusion, anger, anxiety, lethargy and sleepiness [49]. Headache is a frequent complaint with heterogeneous presentation.…”

“…Oral sodium cromolyn has been successfully used in the control of several mast cell-mediator related symptoms such as abdominal pain, diarrhea [29,52,[147][148][149][150][151][152][153][154][155], mixed organic brain syndrome [49], to restore cognitive abilities [14], and in a lesser extent in other manifestation such as pruritus and flushing. Furthermore, improvement of bone pain has been described in a case of mastocytosis with bone sclerosis [156].…”

Mastocytosis: Mediator-Related Signs and Symptoms

“…Chronic symptoms include decreased attention span, difficulty in concentration, forgetfulness, irritability or depression and are described as mixed organic brain syndrome [31]. Poor motivation, confusion, anger, anxiety, lethargy and sleepiness are also reported.…”

General Anesthesia in a Patient With Urticaria Pigmentosa Referred for Electroconvulsive Therapy

“…7 There are 3 other studies which specifically describe depression in mastocytosis, and report rates of depression ranging from 40% to 70%. [7][8][9] In one of the early investigations examining the association between mastocytosis and psychiatric illness, 4 of 10 patients with systemic mastocytosis (most of whom had been diagnosed with urticarial pigmentosa), were found to have depression. 9 These patients reported that their depression was triggered by the stress of coping with symptoms of mastocytosis, and seemed more of a reaction to the disorder than an endogenous aspect of it.…”