A patient with familial dysautonomia may periodically suffer a dysautonomic crisis. This can be associated with a number of serious complications and can impact the patient psychologically.


Clinical features of a crisis:

(1) erythematous and blotchy skin rash

(2) severe and persistent vomiting

(3) marked sweating

(4) hypertension, which may be severe

(5) personality change (withdrawn, negative, irritable)

(6) distal vasoconstriction with cold hands and feet yet having a reddish color

(7) tachycardia

(8) difficulty in swallowing and/or speaking



(1) aspiration pneumonia

(2) dehydration

(3) consequences of severe hypertension


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