Paroxysmal Autonomic Instability with Dystonia (PAID) is a cause of fever and a systemic response. It occurs when there is a disturbance in the autonomic centers in the diencephalons (thalamus, hypothalamus) or their neural connections.


Synonyms: midbrain dysregulatory syndrome, paroxysmal sympathetic storm, diencephalic seizure


Precipitating factors:

(1) traumatic brain injury

(2) midbrain glioma

(3) hydrocephalus


Clinical features:

(1) episodes lasting minutes to hours with:

(1a) hyperthermia

(1b) agitation

(1c) diaphoresis

(1d) tachycardia

(1e) tachypnea

(1f) hypertension

(1g) hypertonia

(1h) extensor posturing

(2) exclusion of infection, drug reaction malignant hyperthermia and neuroleptic malignant syndrome


Laboratory findings:

(1) elevated blood catecholamines (epinephrine, norepinephrine) during an episode

(2) negative cultures


Therapy may include:

(1) morphine sulfate

(2) lorazepam

(3) beta-blocking agent (if catecholamines elevated)

(4) clonidine

(5) intrathecal baclofen

(6) dantrolene


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