(Kiosk 3) Autonomic Storm in Demyelinating Disease: A Case Report

Case Diagnosis: Acute disseminated encephalomyelitis (ADEM)

Case Description or Program Description: 24-year-old male presented with one week duration of right-sided weakness, diplopia, and cognitive decline. Magnetic resonance imaging showed enhancing infiltrative lesions of the internal capsule, midbrain, and pons. Cerebrospinal fluid showed absence of oligoclonal bands. Testing for N-methyl-D-aspartate, West Nile virus, toxoplasmosis, and human immunodeficiency were negative. The patient was diagnosed with ADEM necessitating treatment with steroids, intravenous immunoglobulin, and plasmapheresis. Hospital course was complicated by episodes of fevers, tachycardia, and hypertension accompanied by right arm dystonic posturing. Workup was negative for infection, seizures, medication side effects, or substance withdrawal. This autonomic disarray suggested paroxysmal sympathetic hyperactivity (PSH) so he was transferred to neuro-intensive care unit (neuro-ICU) where he was managed with sedatives, pain control, and beta-blockers.

Setting: Inpatient rehabilitation facility

Assessment/Results: Following resolution of his autonomic dysfunction, the patient was downgraded from neuro-ICU and admitted to acute inpatient rehabilitation for rehab of severe oral dysphagia and cognitive and motor deficits. Propranolol and muscle relaxants were continued for management of PSH.

Discussion (relevance): To our knowledge, there are few cases of PSH in a patient with ADEM. PSH is most frequently encountered in traumatic brain injury. It is attributed to dysfunction of the autonomic centers in the diencephalon leading to cortically instigated release of adrenomedullary catecholamines. This results in hypertension, tachycardia, and tachypnea. Episodes of PSH are treated with a combination of abortive and preventive medications including beta-blockers, narcotics, benzodiazepines, sympatholytics, gabapentin, and muscle relaxants to decrease the frequency and severity of episodes. If untreated, increased sympathetic activity could increase risk of end-organ damage with increased risk for secondary injury to the brain.

Conclusions: Besides motor and cognitive decline, it is important to recognize PSH as a potential complication of ADEM that may impact the patient’s medical condition and functional status. Prompt treatment is paramount to prevent further impairment and disability.