Dramatic Cataplexy Improvement Following Right Parietal Surgery

Abstract

This is the case of a 34-year-old woman with severe narcolepsy with cataplexy who experienced a dramatic reduction in cataplexy symptoms after resection of a right parietal astrocytoma. The patient underwent detailed neurological exam, neuropsychological testing, polysomnography and multiple sleep latency testing following surgery. Keywords: narcolepsy, cataplexy, astrocytoma, parietal lobe Citation: Fam DJ, Shammi P, Mainprize TG, Murray BJ. Dramatic cataplexy improvement following right parietal surgery. J Clin Sleep Med 2015;11(7):829-830.pii: jc-00471-14 http://dx.doi.org/10.5664/jcsm.4860 N arcolepsy is a disorder resulting in disrupted transition between sleep states from an orexin/hypocretin signaling impairment. Cataplexy is the emotionally triggered loss of motor tone, which is pathognomonic. Here we describe the fi rst reported case of dramatic improvement in cataplexy symptoms following resection of a right parietal astrocytoma. REPORT OF CASEA 34-year-old right-handed woman had 18 years of narcolepsy with cataplexy. Her symptoms began with multiple daily episodes of cataplexy triggered by virtually any emotional stimulus including the thought of humor. She had sleepiness, daily hypnogogic hallucinations, and sleep paralysis on awakening several times weekly.Investigations included a multiple sleep latency test (MSLT) showing a short sleep latency and multiple sleep onset REM periods (SOREMPS). She was positive for HLA DQB1*0602. She underwent several medication trials at an outside institution including methylphenidate, dextroamphetamine, modafi nil, and fl uoxetine. The last of these provided modest improvement in cataplexy. She discontinued all narcolepsy treatment in 2005. She found her symptoms greatly impaired social interactions.In July 2013, she had worsening occipital headaches. MRI brain showed a right parietal neoplasm. Surgical resection was performed in January 2014 and histology suggested a lowgrade astrocytoma (Figure 1).Immediately after surgery, she had a left homonymous inferior quadrantanopia and trouble performing rudimentary calculations. Despite this, the patient spontaneously reported that she was delighted to experience a dramatic reduction in cataplexy symptoms. For the fi rst several weeks there were no cataplexy events. There were no changes in her medications.Approximately four months after surgery, she described her cataplexy as at least 75% improved in both frequency and On initial examination, she remained fully alert. Affective range of expression was normal. There was an element of receptive dysprosody and diffi culty judging the emotional content of speech, including sarcasm. Language examination was normal. She had dyscalculia including diffi culty with serial 7's. She had some diffi culty with right-left discrimination and identifying her own individual fi ngers. Further testing for neglect, apraxia, graphesthesia, and stereognosis was unrevealing. Besides her visual fi eld defi cits, the remainder of her neurologic exam was normal.Repeat polysomnography and MSLT f...