Psychosomatics 42:356-358, August 2001
© 2001 The Academy of Psychosomatic Medicine
Pseudocataplexy
Lois E. Krahn, M.D.,
Mark R. Hansen, M.D., and
John W. Shepard, M.D.
Received February 15, 2001; accepted March 15, 2001. From the Sleep Disorders Center, Department of Psychiatry & Psychology, Mayo Clinic, Rochester, MN. Address reprint requests to Dr. Krahn, Department of Psychiatry & Psychology, Mayo Building—West 11A, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905.
Key Words: Epilepsy • Pseudoseizures • Pseudocataplexy
The clinical challenges inherent in assessing epileptic patients with pseudoseizures coexisting with seizures are well recognized.1 The nonepileptic nature of these events must be identified so that anticonvulsant medications are not prescribed inappropriately. Instead, the factors underlying the spells should be addressed, which potentially include somatization, dependency needs, sexual abuse, and unconscious conflicts.
Despite narcolepsy being a fairly common disorder with a prevalence similar to multiple sclerosis (0.56/1,000),2 pseudocataplexy, a phenomenon analogous to pseudoseizures, has never been reported in the literature. Narcolepsy is a neuropsychiatric disorder characterized by excessive daytime sleepiness (EDS), cataplexy, sleep paralysis, hypnagogic hallucinations, and disrupted nocturnal sleep. These symptoms are linked to abnormally regulated and dissociated rapid eye movement (REM) sleep, which inappropriately intrudes into wakefulness. Recent research points to hypocretin deficiency in the brain as the underlying neurochemical abnormality.3
Case Report
At age 17, Ms. G. developed EDS that significantly interfered with her academic performance at school. Thirty-two years later, while undergoing a divorce at age 49, she reported sleep paralysis and spells lasting up to 10 min triggered by anger. These consisted of leg weakness and dysarthric speech. At age 53, Ms. G. underwent polysomnography followed by a multiple sleep latency test that revealed an extremely brief initial sleep latency (mean<0.5 min) and REM sleep present for three of the four daytime naps. She had many spells witnessed by multiple sleep specialists who were convinced these represented cataplexy (even though deep tendon reflexes were not tested). The diagnosis of narcolepsy was established and treatment included scheduled daytime naps, methylphenidate (20 mg tid for EDS), and imipramine (50 mg for cataplexy). The EDS was adequately controlled but the cataplexy persisted despite imipramine (75 mg qid; serum imipramine level=511 ng/ml; normal range=125–275 ng/ml). Ms G. was hospitalized on a psychiatric unit for an imipramine taper and trial of tranylcypromine (Parnate; 20 mg bid), a monoamine oxidase inhibitor selected for its REM suppressing properties. Methylphenidate was also discontinued because of potential drug-drug interactions. During her hospitalization, no axis I psychiatric disorders were identified. On tranylcypromine, Ms G. had decreased cataplexy, never when alone but frequent episodes in social settings and when driving. Her driver's license was revoked. The tranylcypromine effectively controlled EDS by suppressing REM sleep, and a benzodiazepine was added to optimize her nocturnal sleep.
For the next 12 years, Ms. G. remained on these medications with cataplexy occurring on a monthly basis. She was increasingly overwhelmed by situations and often demonstrated dramatic behavior, numerous somatic complaints, anxious mood, and tangential thought form. She was angry that her seven children never visited her. The family expressed suspicion that many of her spells occurred at times that made her the center of attention. They questioned if all of her spells were true cataplectic events.
At age 65, Ms. G. returned for follow-up of her cataplexy. In the clinic hallway she experienced a spell that caused her to abruptly sit down while walking. She had flaccid upper extremities but was able to speak clearly. On examination, her biceps deep tendon reflexes were brisk. Subsequent attempts to trigger cataplexy with surprise and a humorous video were unsuccessful. A maintenance of wakefulness test on medication confirmed the absence of REM sleep during four daytime sessions and an improved initial sleep latency (mean=14.9 min). The family requested a psychiatric consultation, which did not find any specific psychiatric disorder but did find dependent and narcissistic personality traits. Narcolepsy with cataplexy coexisting with pseudocataplexy was diagnosed. The treatment plan included minimizing circumstances that positively reinforced these spells and use of an additional dose of tranylcypromine (10 mg prn).
Cataplexy, present in 75% of patients with narcolepsy,2 is rarely witnessed by physicians and is most often identified by means of subjective patient report. Cataplexy is characterized by preserved consciousness and bilateral muscle weakness involving the face, neck or extremities, sometimes associated with slurred speech, and most typically provoked by laughter, surprise, or anger.4 Checking deep tendon reflexes is a simple definitive test that confirms transient areflexia and atonia of voluntary muscles. The mechanisms implicated in cataplexy are identical to those that control the onset of REM sleep. Excitatory neurons from the pedunculopontine nuclei project to the ventromedial medullary neurons. These neurons have inhibitory synapses as they terminate on the anterior horn cells of the spinal cord. Depending on the duration of the cataplectic episode, the areflexia may last from several seconds to over 10 min, and subsequently reflexes are normal.5,6
On the basis of the objective sleep data, Ms. G. had definite narcolepsy. The description of the many observed spells is convincing for cataplexy, although areflexia noted during deep tendon reflex testing would have made this conclusive. This practical bedside test is not widely known or practiced in the assessment of suspicious spells. In no other disease state do patients have complete but transient bilateral areflexia.
When the normal deep tendon reflexes coexist with a probable cataplectic spell, several explanations exist other than pseudocataplexy. Unsatisfactory technique may conceivably explain the findings, although the simple nature of the reflex testing procedure makes this unlikely. Additionally, the spell may be concluding at the time of the examination but, given the prolonged nature of this patient's events, this is also improbable.
Several features of Ms. G.'s history are unusual. Her cataplexy emerged 32 years after EDS developed. More typically cataplexy and EDS develop within months of each other, although there is a previous report of EDS preceding cataplexy by 60 years.7 Unlike EDS, where the onset of symptoms can be subtle and difficult to pinpoint, cataplexy is generally a startling experience allowing patients to recall clearly the context of specific episodes. The association of cataplexy and Ms. G.'s divorce may not be coincidental. Severe stress has been linked to the development of narcolepsy, most clearly in a pair of monozygotic twins with concordant narcolepsy but a differing age of onset.8 Furthermore, the emotional turmoil of divorce may have served to trigger the cataplexy. Patients experiencing acute grief have been reported to have increased frequency of cataplexy.9 Last, most patients with narcolepsy are treated with a psychostimulant, sometimes combined with a tricyclic or selective serotonergic antidepressant to suppress REM sleep and therefore cataplexy.10 The decision to select tranylcypromine over more conventional agents demonstrates the severity of this patient's disease.
Why Ms. G. developed pseudocataplexy is unknown. Her perception that her adult children were neglecting her is likely a factor. Her family observed that the events only occurred in public, and they interpreted this as a maladaptive attempt to get attention. Distinctly different than pseudoseizures, where having spells exclusively in an observed setting is suggestive of a nonepileptic process,1 emotions stirred up by social interactions may be provocative in the case of cataplexy. Furthermore, Ms. G. was facing significant psychosocial issues that she appeared ill-equipped to handle. For example, her medical disorder rendered her unable to drive or work. The lifestyle disruption caused by narcolepsy is viewed as similar to that caused by epilepsy.11 Whether these spells represent involuntary and unconscious behavior also remains unclear. Unlike many female patients with pseudoseizures, there was no history of abuse.1 No diagnosis was established of a somatoform or factitious disorder.
Whether pseudocataplexy occurs in the absence of true cataplexy is unknown. In the case of pseudoseizures, patients without a seizure disorder are known to experience these events.1 They are suspected to have witnessed seizures in a family member or on television. Because cataplexy is rarely witnessed or depicted in the media, fewer models exist for suggestible patients.
Unlike EDS, which has many causes, cataplexy is essentially pathognomonic of narcolepsy. Therefore, concluding that a patient has a history of cataplexy carries tremendous diagnostic significance. The observation, in this case, where spells occur that mimic cataplexy complicates the recognition and treatment of narcolepsy. Because pseudocataplexy has never been previously described, the overall prevalence of this condition and the implications for the diagnosis of narcolepsy are unknown.
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Krahn L, Black J, Silber M: Narcolepsy: new understanding of irresistible sleep. Mayo Clin Proc 2001; 76:185-194[Abstract]
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