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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