Abstracts

Rationale: Paroxysmal sympathetic hyperactivity (PSH) formerly known as diencephalic autonomic seizure/epilepsy is a rare syndrome characterized by hyperhidrosis, episodic hypertension, tachypnea, tachycardia, and an abnormal posturing. This syndrome has been reported to occur after severe brain injuries such as head trauma, diencephalic tumors, intracerebral hemorrhages, or leptomeningeal diseases; however, little is known about this disorder in the setting of acute leukemia. Here we describe an unusual case of PSH in a patient with acute myeloid leukemia (AML) together with a review of the literature regarding the prevalence of this disorder in patients with cancer.Methods: Clinical data including continuous video Electroencephalography (cVEEG) recordings were extracted from the patient s file. We searched PubMed for reports published from 1948-2013 using the following terms: diencephalic epilepsy/seizure/storm , or paroxysmal sympathetic hyperactivity/storm . Results: A 53 year old female patient with history of therapy-related acute myeloid leukemia after treatment for ovarian cancer presented to MD Anderson Cancer Center with acute recurrent episodes of staring spells and jerky movements of the upper extremities accompanied by elevations in blood pressure, hyperthermia, tachycardia, and tachypnea. Symptoms lasted between 5-30 minutes and occurred 2-4 times per day. Initial neurologic examination revealed altered mental status. Blood and urine cultures, serum chemistries, and paraneoplastic panel were normal. Lumbar puncture revealed normal opening pressure. CSF cytology and infectious work up was negative. Transcranial Doppler and brain MRI and MRV were unremarkable. Continuous video electroencephalography (cVEEG) revealed brief staring spells, head bobbing, and bilateral upper extremity jerking activity with no significant EEG abnormalities other than generalized slowing and high amplitude EMG artifact (VEEG will be shown). Use of high frequency filter and review of the central regions failed to demonstrate any obvious epileptiform activity. Despite therapeutic doses of intravenous (IV) levetiracetam, phenytoin, and diazepam, the episodic abnormal posturing didn t abate. The lack of response to multiple anti-epileptic drugs (AEDs) and cVEEG analysis led to the diagnosis of PSH. IV Morphine sulfate (2mg every 6 hours) successfully restored the patient s vital signs to normal with cessation of the abnormal movements.Conclusions: To our knowledge there is no prior report of PSH in the context of liquid tumors. This report highlights the key role of cVEEG in the diagnosis and management of PSH. A timely diagnosis of this syndrome will preclude unnecessary administration of potentially toxic AEDs, especially in cancer patients receiving chemotherapy. Moreover, prompt diagnosis and treatment will reduce morbidity and mortality from the autonomic dysfunction in PSH. Further studies using subdural and/or depth electrodes are needed to disregard the possibility of seizures arising from deep brain areas such as diencephalon in PSH.