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Narcolepsy- and loss of sphincter control 493 Postgrad Med J: first published as 10.1136/pgmj.72.850.493 on 1 August 1996. Downloaded from -cataplexy and loss of sphincter control

AN Vgontzas, SE Sollenberger, A Kales, EO Bixler, A Vela-Bueno

Summary patient continued to experience blurred vision, We describe the case of a 34-year-old man left-sided and faecal incontinence on who presented intermittent faecal incon- an intermittent basis, but not concurrently, tinence as a manifestation of cataplexy. and the patient was unsure whether these The patient's history was positive for symptoms might be associated with any emo- the full narcoleptic tetrad (sleep attacks, tional condition, he was referred to a neurol- cataplexy, sleep and hypnagogic ogist (SES) for a thorough evaluation. ) while extensive neuropsy- His initial neuroexamination was positive for chiatric work up was negative for any right-beating nystagmus in right gaze and a neurologic or psychiatric illness. Repeat slight increase in deep tendon reflexes in the polysomnograms (including a polysomno- left upper extremity as compared to the right. gram with a full montage) were There was no sensory level in the trunk, and no positive for pathologic sleepiness, but 'saddle' anesthesia was demonstrated. The there was no evidence of a seizure dis- remainder of the neuroexamination was nor- order. The course of the patient's symp- mal. A examination includ- tomatology and the favourable response of ing cultures for acid-fast bacilli and his symptoms to and imipra- cryptococcus and serologic tests for Lyme mine support the theory that his inter- disease and syphilis, magnetic resonance ima- mittent loss of sphincter control is part of ging ofthe brain, brainstem, and cervical spine, his narcolepsy-cataplexy. and nerve conduction studies (both motor and sensory) in the lower extremities and left upper Keywords: narcolepsy-cataplexy, sphincter control extremity were normal. Because of an addi- tional history of excessive daytime sleepiness, the patient was referred to our sleep disorders Narcolepsy is a disorder of excessive sleepiness clinic for further evaluation. characterised by daytime sleep attacks, cata- A thorough sleep history revealed a 10- to plexy, and hypnagogic halluci- 15-year history of excessive sleepiness and nations. 1-3 Cataplexy occurs in 60-70% of all sleep attacks which occurred in the classroom, cases of narcolepsy and is considered a at work and while driving (four years ago he

pathognomonic symptom of the disorder. had wrecked his car). During the last four http://pmj.bmj.com/ Clinical features of the cataplectic attack may years, the patient's sleepiness had become include various degrees of weakness of the worse; he complained of brief periods of entire voluntary musculature or more fre- behavioural automatism ('memory lapses') quently certain muscle groups as manifested and presented brief episodes of loss of muscle by buckling of the knees, 'clumsiness' or head control such as buckling of his knees, dropping droop and sagging of the jaw. Less frequently, of tools from his hands and head drops to the a cataplectic attack involves speech, respiration front which sometimes were associated with on September 27, 2021 by guest. Protected copyright. and extraocular muscles. The sphincter mus- laughter. Also, more recently, he began ex- cles are considered to be uninvolved in a periencing vivid hypnagogic auditory halluci- cataplectic attack'; loss of sphincter control as nations and episodes of sleep paralysis. His a feature of cataplexy is not mentioned in family history was significant for his mother textbooks on sleep disorders medicine.2 and a maternal aunt and uncle with histories of excessive daytime sleepiness. A comprehensive Sleep Research and Treatment Center, Case report psychiatric evaluation including a thorough Department of psychiatric history, mental status examination , A 34-year-old married man was referred to our and psychological testing (Minnesota Multi- Pennsylvania State Sleep Disorders Clinic for evaluation of symp- phasic Personality Inventory) was negative for University College of toms consistent with narcolepsy. Approxi- a psychiatric disorder. Medicine, Hershey, mately six months earlier the patient had an The was PA 17033, USA patient evaluated in the Sleep AN Vgontzas eight-day period in which he experienced fairly Laboratory for one eight-hour night recording A Kales persistent diplopia and weakness of his left arm and two one-hour daytime according to EO Bixler and left leg with intermittent episodes of faecal techniques previously described.46 All records A Vela-Bueno incontinence not associated with urgency. The were scored for sleep according to standard episode resolved without any residual symp- criteria..4 His sleep laboratory testing was Chambersburg toms. A clinical examination by his family Hospital, Chambers- negative for obstructive or central burg, PA, USA physician and several laboratory tests including and nocturnal myoclonus. No sleep-onset SE Sollenberger electroencephalogram (EEG) and computed rapid-eye movement (REM) periods were tomography scan of the brain were negative for noted. However, he demonstrated shortened Accepted 22 November 1995 abnormal findings. However, because the sleep latencies during the two naps (6 and 10 494 Vgontzas, SoUenberger, Kales, Bixler, Vela-Bueno

Clinical features and management of narcolepsy Learningisummary points * sleep attacks are brief, irresistible, episodes of sleep * loss of sphincter control may be a rare * sleep attacks are sometimes associated with dreaming manifestation of cataplexy Postgrad Med J: first published as 10.1136/pgmj.72.850.493 on 1 August 1996. Downloaded from * duration of sleep attacks varies from a few to thirty minutes * cataplexy should be included in the differential * most patients feel refreshed after a sleep attack list of neurologic disorders associated with loss * chronic excessive daytime sleepiness between attacks of sleep of sphincter control * and are the primary and most effective treatment * and have been reported to have moderate and substantial effects, respectively respectively. Evaluation of the sleep EEG and * therapeutic naps may decrease the need for the videotape was negative for paroxysmal events or abnormal movements. Following this , the patient was prescribed imipra- mine 10 mg each morning. He has been free of Clinical features and management of cataplexy episodes offaecal incontinence for the last nine months. * attacks of sudden reduction or loss of muscle tone * attacks are most often triggered by emotional stimuli, ie, surprise, anger, Discussion laughter * may be accompanied by hypnagogic hallucinations Our case demonstrates that loss of * consciousness is almost always maintained sphincter * last from a few seconds to several minutes control may be, though rarely, a cataplectic * tricyclics (, ) in low doses (10-50 mg) are the manifestation. This is supported by the pre- treatment of choice sence of the narcoleptic tetrad (daytime sleep * and gamma-hydroxybutyrate have been reported to have attacks, cataplexy, sleep paralysis and hypna- significant anticataplectic effects gogic hallucinations); positive family history of excessive sleepiness; objective documentation of pathologic sleepiness in the sleep laboratory minutes, respectively) and a moderately shor- and lack of evidence of seizure activity; a tened REM latency during the nocturnal significant response of his faecal incontinence recording (62 minutes). to treatment with methylphenidate and a more The patient was diagnosed with narcolepsy- definite response to imipramine; and lack of cataplexy and was placed on methylphenidate. evidence of any disease of the central or His sleep attacks responded favourably to peripheral nervous system or psychiatric dis- increasing doses of methylphenidate, and he order (during initial and repeat follow-up reported an 80% improvement in his daytime visits), which might have explained the pa- sleepiness on 30 mg daily dose. Also, his tient's symptomatology. The presence of ob- episodes of faecal incontinence became very jective neurological findings (in our case infrequent. transient), particularly oculomotor disorders, Because of the occasional occurrence of is not infrequent in narcoleptics, especially these episodes of faecal incontinence, the when the condition is familial.' patient was re-evaluated clinically by his Thiele and Bernhardt in 1933 described a http://pmj.bmj.com/ neurologist, who reported a normal neurologi- patient with narcolepsy-cataplexy and episodes cal examination with the exception of slightly of enuresis.7 However, they attributed the increased deep tendon reflexes on the left. The enuretic episodes to seizure activity rather than patient was then re-evaluated in our Sleep to cataplexy. In our case, sleep studies, a fully Laboratory, almost a year following his initial preserved consciousness during the episodes, evaluation, for one night and one morning after and a positive response to methylphenidate

he was free of stimulants for about one week. and imipramine, do not support such a on September 27, 2021 by guest. Protected copyright. This time, in addition to the standard poly- possibility. A cataplectic attack can affect any somnographic recordings, he was monitored voluntary muscle including ocular muscles and with 12 additional EEG channels following the diaphragm, and there is no theoretical reason international 10/20 system to assess for possi- that sphincter muscles should be spared. ble paroxysmal EEG activity during sleep. In summary, loss of sphincter control may During the nocturnal recording, the patient occur as a feature, though very rarely, of demonstrated a sleep latency of 11 minutes cataplexy. Also, cataplexy should be included and a REM latency of 2 hours and 31 minutes. in the differential list of neurologic disorders During the first , his sleep latency was three which may result in loss of sphincter control, minutes and his REM latency 52 minutes. and an assessment for the presence of narco- During the second nap, his sleep and REM lepsy-cataplexy in atypical cases may provide latencies were six minutes and 37 minutes, useful diagnostic clues.

1 Roth B. Narcolepsy and . Basel: S Karger, 1980. 5 Kales A, Bixler EO, Soldatos RJ, Cadieux RJ, Manfredi R, 2 Guilleminault C. Narcolepsy . In: Kryger MH, Vela-Bueno A. Narcolepsy/cataplexy. IV. Diagnostic value Roth T, Dement WC, eds. 1tinciples and practice of sleep of daytime nap recordings. Acta Neurol Scand medicine. Philadelphia: WB Saunders Company, 1994; 1987; 75: 223-30. pp 549-61. 6 Roth B, Nevsimalova, Sonka K, Docekal P. A quantitative 3 Kales A, Cadieux RJ, Soldatos CR, et al. Narcolepsy- polygraphic study of daytime and sleep in cataplexy. I. Clinical and electrophysiologic characteristics. patients with excessive diurnal sleepiness. Schweiz Arch Arch Neurol 1982; 39: 164-8. Neurol Psychiatr 1984; 135: 265-72. 4 Rechtschaffen A, Kales A. A manual of standardized 7 Thiele R, Bernhardt H. Beitrczge zur Kenntniss der Narko- terminology, techniques and scoring system for the sleep stages lepsie. Berlin: S Karger, 1933. of human subjects. Washington, DC: Public Health Service, US Government Printing Office, 1968.