Abstracts

Rationale: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a progressive neurodegenerative disorder associated with mitochondrial DNA mutations. MELAS has multi-systems presentation including neurologic, muscular, endocrine, auditory, cardiac, psychiatric and even gastrointestinal/urinary symptoms. Clinical course and prognosis are variable often leading to disability and premature death. Nonconvulsive status epilepticus (NCSE) with hallucinations has been reported with MELAS. This case report documents the first instance of MELAS with NCSE, confusion, aggressive behaviors, hallucinations and paranoid delusions. Methods: Case report with literature review. Results: 39-year-old married Caucasian male with 10+ year history of MELAS (myopathy, lactic acidosis, seizures, strokes, sensory neural hearing loss, and diabetes), confirmed by genetic testing, presented with altered mental status, new onset psychotic features (auditory hallucinations and paranoid delusions), and physically violent behaviors toward wife for one month. Outpatient olanzapine 5mg qd did not control his psychosis with violent behaviors and was discontinued upon admission. Seizures had previously been controlled with phenytoin 100mg tid and gabapentin 400mg tid. Significant admission laboratories included: HgbA1c 8.0%, lactate 3.5mmol/L, phenytoin level = 25.1mcg/mL (free phenytoin level = 2.0mcg/mL). Head CT showed no acute changes compared to prior outpatient studies. Video-EEG revealed predominantly left temporal epileptiform activity, multiple complex partial seizures, and NCSE lasting from 5-60 minutes with T5 phase reversal. Adjunctive intravenous valproate sodium (VPA level = 75.8mcg/mL) resulted in total seizure control, normalization of mental status, and elimination of all hallucinations/delusions and aggressive behaviors without further psychotropics. With combined divalproex sodium, phenytoin, and gabapentin, the patient remained symptom free for > 12 months after discharge and was considered by his wife to be at baseline. Conclusions: MELAS with NCSE can present with psychosis, aggression, and confusion. Rapid video-EEG confirmation of diagnosis and aggressive AED intervention are required.