Repeated Self-induced Syncope and Subsequent Seizures A Case Report Chi-Wan Lai, MD, Dewey K. Ziegler, MD \s=b\ A patient with an unusual "compulsion" to induce syncope over a period of years by bilateral compression of the carotid arteries subsequently had recurrent seizures. The EEG showed patterns typical of cerebral ischemia during the syncope and epileptogenic foci in both temporal lobes after sleep deprivation. It is difficult to distinguish between seizure and syncope associated with involuntary movements when making a differential diagnosis. We hypothesize that the frequent self-induced ischemic insult to the brain caused a cicatrix to develop, which in turn caused the frequent seizure disorder; and that because this ischemia functioned as a stimulus to the reward site in the limbic system, the patient repeatedly induced it. (Arch Neurol 1983;40:820-823) TTabitual self-induced syncope by bilateral compression of carotid arteries has never before been re¬ ported to our knowledge. We describe a patient who indulged in this practice at extremely frequent intervals over a period of six years. Spontaneous, recurrent typical complex partial sei¬ zures and generalized convulsions then developed. The EEG disclosed epileptiform abnormalities in the temporal lobes. ment of the University of Kansas Medical Center, Kansas City. The patient had an unusual history. At age 14 years, she discovered that if she pressed both sides of her neck, she could produce an altered state of consciousness that was pleasurable to her. She had become so "addicted" to this habit that she performed the act several times a day, sometimes up to once every five to ten minutes while she was home. On several occasions she noted that the act would cause kicking movements of her legs or jerking movements of her arms or body, followed by confusion, or rarely, by total blackout. There was no urinary inconti¬ nence, focal weakness, or numbness; but after the spell, there was generalized fatigue and sleepiness. The act was usually performed while the patient was sitting. Occasionally, after successive spells occur¬ ring within a short period, the woman became extremely confused and then com¬ pressed her neck while standing. This sequence ended with her falling to the floor and sustaining physical injury. She had a long history of abuse of alcohol and multi¬ ple illegal drugs. A simultaneous EEG/ECG recording performed with the patient's consent. Unilateral light massage or compression of was the carotid sinus was performed on either side, but failed to change the EEG or ECG recording. The patient then performed her habitual maneuver. While sitting, she brought her right hand to the anterior neck area, thumb firmly pressing the left side, and the other four fingers simulta¬ neously pressing the right side of the neck. The patient also appeared to be holding her breath and performing Valsalva's maneu¬ ver. Within 14.5 s, she began to exhibit twitching facial muscles, blinking eyes, and myoclonic jerks of the arms and a rhythmic falling forward of the head, accompanied by a momentary lapse of con¬ sciousness. Her hand dropped and she appeared dazed with a vacant stare, sug¬ gesting pained perplexity. Within a few seconds, she regained full consciousness. The EEG showed generalized, high-volt¬ age, 2.5- to 3.0-Hz delta activities within 14.5 s of the compression, which were coincidental with the clinical manifesta¬ tion of muscle jerking and lapses of con¬ sciousness. The EEG slowing lasted for 24 s, with a rapid return to the normal base¬ line. No ECG change or epileptiform abnormality was noted during or preceding these spells (Fig 2). On two occasions dur¬ ing the recording, the patient clearly per¬ formed Valsalva's maneuver for more than REPORT OF A CASE A 21-year-old woman was first examined at the age of 20 years because she had experienced a generalized tonic-clonic con¬ vulsion with urinary incontinence. Results of physical and neurologic examinations at that time were normal, as were results of blood chemistry tests, VDRL, ECG, CSF examination, and a computed tomographic scan of the head. Routine EEG taken on waking and sleeping was normal, but an EEG with nasopharyngeal leads taken after sleep deprivation revealed frequent paroxysmal focal spikes in the temporal lobes, predominantly on the right side (Fig 1). Phénobarbital sodium (90 mg at bed¬ time) was prescribed. The patient had more episodes of nocturnal grand mal, probably because of noncompliance with the use of her medication; she was subse¬ quently referred to the Neurology DepartAccepted for publication Dec 28, 1982. From the Neurology Department, University of Kansas Medical Center, Kansas City. Reprint requests to Neurology Department, University of Kansas Medical Center, 39th at Rainbow Boulevard, Kansas City, KS 66103 (Dr Lai). / ^^^ ^ ^ ^- *«-**^ / /^ ^ ^ ^ ^/ ^^ EEG recording after sleep deprivation shows focal paroxysmal spikes at T4. Fig 1. — Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/01/2015 Fig 2.—Continuous ECG/EEG tracings, each lasting for 10 s. Top left, EEG immediately after compression (arrow) shows artifacts of electromyogram and eye blinking without any significant ECG or EEG change. Top right, At arrow, 14.5 s after compression, EEG reveals generalized high-voltage 2.5- to 3.0-Hz delta activities, coincidental with clinical manifestation of muscle jerking and lapse of consciousness. Bottom left, Arrow indicates when patient's hand dropped and she appeared dazed with vacant stare, suggesting pained perplexity. Bottom right, EEG spontaneously recovers to normal alpha rhythm. Throughout tracing, no ECG change was found. Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/01/2015 20 s without carotid compression. This act failed to produce the subjective "pleasur¬ able" sensation, the motor phenomenon, and the EEG changes seen during carotid compression. The risk of this habit was fully explained to the patient, and treatment with a psy¬ chotherapist was arranged. The patient initially was reluctant to take an anticonvulsant regularly for seizure prophylaxis. During a follow-up period, she reported episodes of déjà vu, followed by confusion, features typical of complex partial sei¬ zures. She also reported experiencing occasional nocturnal grand mal seizures. Finally, the patient consented to take phé¬ nobarbital sodium (100 mg at bedtime) regularly, and she has had no seizures in the last six months. She also has stopped indulging in the habit for the last nine months and has had no further syncopal episodes. COMMENT Syncope is defined as a transient loss of consciousness of abrupt onset, resulting from an impairment in cere¬ bral metabolism. Such an impair¬ ment, in turn, results from a brief interruption of the cerebral circula¬ tion or from the shortage of required energy substrates, ie, glucose and oxy¬ gen, which are constituents of the blood delivered to the brain.1 In healthy young subjects, syncope can be induced by a variety of mecha¬ nisms: (1) by mechanical stimulation of the vagai receptors, using ocular compression2 or massage of the carot¬ id sinus,34 with resultant reflex cardi¬ ac arrest or hypotension; (2) by an increase in the intrathoracic pressure (eg, by performing Valsalva's maneu¬ ver), with a secondary decrease in cardiac output56; (3) by stimulation of the autonomie nervous system by emotional or painful stimuli, with resultant hypotension or bradycardia7; (4) by acute hypoxia in a lowpressure chamber8; or (5) by direct compression of the carotid arteries from the surface of the neck.9 Interestingly, the experiments by Rosen et al9 are closely analogous to our patient's habit. They used an inflatable cuff on the lower third of the neck and produced a pressure of 600 mm Hg within 0.125. After acute arrest of the carotid circulation for 5 to 10 s, they observed fixation of the eyeballs, blurring of vision, constric¬ tion of the visual field, and within 6 s, loss of consciousness, and after 8 s, anoxic convulsion. As the subject became unconscious, the pressure cuff was deflated; the subject would then spontaneously recover. The fact that our patient experienced convulsive syncope with the presumed occlusion of only the carotid arteries supports the proposition of Gastaut and Fischer-Williams: that such syncope is due to the liberation of fonogenie centers of the pontobulbar reticular formation from telencephalic and diencephalic centers.2 The striking involuntary move¬ ments that accompanied syncope in our patient are an uncommon, but well-known, manifestation of tran¬ sient global cerebral ischemia. Duvoisin noted that brief duration, rapidity of recovery, and absence of postictal phenomena are reliable clues for dis¬ tinguishing syncope from epileptic seizures5; yet some epileptic seizures, for example, absence, can be very brief without postictal phenomena. Myoclonic jerks or tonic spasms occurring in this setting occasionally make it difficult to differentiate between syncope and seizure. Gastaut and Fischer-Williams found that gen¬ eralized clonic convulsions of the typi¬ cal grand mal type were not seen in syncopal attacks.2 In a study of 35 cases of convulsive syncope among blood donors, however, two patients were observed to have only clonic movements for three to five beats, and another two were reported to have tonic spasms followed by clonic con¬ vulsions.10 When an EEG can be per¬ formed during clinical symptoms, dif¬ ferentiation is usually easy because of the typical slow waves of diffuse dis¬ tribution in cerebral ischemia, which were noted as early as 1942 by Foster et al.3 Whether there is a relationship between true seizure disorders and the susceptibility to syncope is highly debated. Whether some or all patients subject to recurrent syncope have oth¬ er EEG abnormalities as well is also debated. In 1949, Kershman found that 65% of 114 patients with syncope had diffuse cerebral dysrhythmia in the interictal period.11 O'Doherty found, in 1953, paroxysmal slow, sharp wave activity in five cases of tussive syncope, which he thought was an epileptic phenomenon.12 In 1958, Lloyd-Smith and Tatlow reported a case of syncope that was induced by venipuncture in a patient whose EEG changes and clinical phenomena were strongly suggestive of a seizure.13 When pentylenetetrazol activation was used to study seizure threshold, the lowest threshold to the drug was found in epileptic subjects, the high¬ est threshold in control subjects, and a threshold close to those with seizure disorders in patients with syncope.14 The coexistence of syncope and sei¬ zure is generally rare. Gastaut15 found in his three-year study of patients with loss of consciousness, with or without convulsion, 417 cases of syn¬ cope, 377 cases of epilepsy, and only three with both syncope and epilepsy. Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/01/2015 In these three cases, ocular compres¬ sion produced syncope and pentylenetetrazol did induce seizure, but seizure and syncope never occurred in sequence.15 Our patient clearly suffered recur¬ rent seizures of complex partial and generalized convulsive type, with interictal epileptiform discharge in the temporal lobes and a favorable response to anticonvulsant therapy. There was no family history of epilep¬ sy, no history of birth injury or febrile convulsion, and no other possible cause of seizure. Although the patient had a history of abuse of alcohol and other drugs, the seizures were never temporally related to the withdrawl or use of any of these agents. We hypothesize that the genesis of her recurrent seizures with temporal-lobe foci is the extremely frequent self-induced cere¬ bral ischemia over a period of six years. Because the hippocampus is one of the most sensitive areas to anoxic damage, the repeated cerebral ische¬ mia may have resulted in scar forma¬ tion there. The mechanism of seizure genesis may be comparable to the recurrent seizures that occur second¬ ary to cerebral infarct.16 If the causeeffect relationship between syncope and seizure is valid, we believe this case demonstrates an important pos¬ sible complication of syncope and strong indication for careful evalua¬ tion and treatment. The "addiction" this patient had to the habit of carotid compression and subsequent syncope is a striking phe¬ nomenon. It is reminiscent of the com¬ pulsive induction of minor seizures that are self-induced in some children by moving their hands in front of their eyes to produce a flickering light.17 The patient admitted that a pleasurable sensation with definite sexual overtones was associated with the habit. We postulate that this sen¬ sation resulted from ischemie stimu¬ lation of the limbic system, analogous to hallucinatory visual sensations resulting from ischemie stimulation of the occipital cortex that occurs in posterior cerebral artery insufficiency and probably in migraine. Repetitive self-strangulation as a maneuver to induce or enhance sexual excitement is a well-known phenomenon in men and is usually associated with an overt history of a variety of sexual deviations.1819 This phenomenon is probably best known to medicolegal personnel because the practice is occa¬ sionally fatal and is usually reported when the bodies (usually nude) are the subject of investigation by coroners. It has been estimated that 50 deaths per year result from this practice, which interestingly has been reported only rarely in women.20 Whether these per¬ sons who abuse themselves in this way primarily induce cerebral ische¬ mia by compression of the carotid arteries or asphyxia by compression of the trachea is of course not known, although Resnik19 favors the former mechanism. No case of repetitive strangulation, however, has been reported to have a compulsive fre¬ quency similar to that of our patient. It is interesting to speculate on why this patient repeatedly indulged in the production of cerebral ischemia. The existence of a reward center in the limbic system is well documented,21 and ischemia could have served as a stimulus to this area, thereby produc¬ ing repetitive behavior such as has been observed in animals. References 1. Noble RJ: Syncope. Cardiovasc Clin 1981; 12:119-130. 2. Gastaut H, Fischer-Williams M: Electroen- cephalographic study of syncope: Its differentiation from epilepsy. Lancet 1957;2:1018-1025. 3. Foster FM, Roseman E, Gibbs FA: Electroencephalogram accompanying hyperactive carot- id sinus reflex and orthostatic syncopy. Arch Neurol Psychiatry 1942;48:957-967. 4. Engel GL, Romano J, McLin TR: Vasodepressor and carotid sinus syncope. Arch Intern Med 1944;74:100-119. 5. Duvoisin RC: Convulsive syncope induced by the Weber maneuver. Arch Neurol 1962;7:219\x=req-\ 226. 6. Klein LJ, Saltzman HA, Heyman A, et al: Syncope induced by the Valsalva maneuver: A study of the effects of arterial blood gas tensions, glucose concentration and blood pressure. Am J Med 1964;37:263-268. 7. Braham J, Hertzeanu H, Yahini JH, et al: Reflex cardiac arrest presenting as epilepsy. Ann Neurol 1981;10:277-278. 8. Luft UC, Noell WK: Manifestations of brief instantaneous anoxia in man. J Appl Physiol 1956;8:444-454. 9. Rosen LR, Kabat H, Anderson JP: Acute arrest of cerebral circulation in man. Arch Neu- rol Psychiatry 1943;50:510-528. 10. Lin JT-Y, Ziegler DK, Lai CW, et al: Convulsive syncope in blood donor. Ann Neurol 1982;11:525-528. 11. Kershman J: Syncope and seizures. J Neurol Neurosurg Psychiatry 1949;12:25-33. 12. O'Doherty DS: Tussive syncope and its relation to epilepsy. Neurology 1953;3:16-21. 13. Lloyd-Smith D, Tatlow WFT: Syncope and seizure. Electroencephalogr Clin Neurophysiol 1958;10:153-157. 14. Fuglsang-Frederiksen V: Activation of EEG disturbances with Metrazol (Pentazol) in epileptics, normals and patients with syncopal attacks. Electroencephalogr Clin Neurophysiol 1952;4:471-480. 15. Gastaut H: Syncope and seizure. Electroencephalogr Clin Neurophysiol 1958;10:571-572. 16. Louis S, McDowell F: Epileptic seizures in nonembolic cerebral infarction. Arch Neurol 1967;17:414-418. 17. Andermann K, Berman S, Cooke PM, et al: Self-induced epilepsy: A collection of self\x=req-\ induced epilepsy cases compared with some other photoconvulsive cases. Arch Neurol 1962;6:49\x=req-\ 65. 18. Litman RE, Swearingen C: Bondage and suicide. Arch Gen Psychiatry 1972;27:80-85. 19. Resnik HLP: Erotized repetitive hanging: A form of self-destructive behavior. Am J Psy- chother 1972;26:4-21. 20. Sass FA: Sexual asphyxia in the female. J Forensic Sci 1975;20:181-185. 21. Milner PM: Self-stimulation and mechanisms of motivation, in Physiological Psychology. New York, Holt Rinehart & Winston Inc, 1970, pp 378-412. Gouty Neuropathy Peyton Delaney, MD \s=b\ A patient with gout had a peripheral neuropathy that improved with the lowering of the serum uric acid level. He had a relapse when hyperuricemia recurred, suggesting that the hyperuricemia of gout may produce a treatable form of peripheral neuropathy. (Arch Neurol 1983;40:823-824) 'T'he neurologie complications of gout of either consisting peripheral nerve entrapment16 or spi¬ nal cord compression.712 When a gen¬ eralized peripheral neuropathy devel¬ ops in a gouty patient, other causes of neuropathy are sought, since hyper¬ uricemia has not been reported as a causal agent.1314 The following case demonstrates that the hyperuricemia of gout may produce a treatable form of peripheral neuropathy. are rare, REPORT OF A CASE A 53-year-old man with chronic, tophaceous gout and nephrolithiasis had pro¬ gressive numbness of his feet and difficul- Accepted for publication March 7, 1983. From the Department of Medicine, Division of Neurology, University of Texas Health Science Center and Medical Service, Neurology Section, Audie L. Murphy VA Hospital, San Antonio, Tex. Reprint requests to 4499 Medical Dr, Suite 255, San Antonio, TX 78229 (Dr Delaney). ty walking for six months. He denied alco¬ hol abuse, diabetes mellitus, exposure to toxins, or a family history of gout or neuropathy. He took colchicine and allopurinol erratically. Multiple tophi were present over the extremities. His gait was wide based and Romberg's sign was present. Myotatic reflexes were hypoactive in the arms and absent in the legs. Plantar responses were neutral. There was slightly diminished strength of the anterior tibialis and exten¬ sor digitorum brevis muscles. No atrophy was seen. Vibratory and position sense and light touch were markedly reduced distally in all limbs. Signs of a right carpal tunnel syndrome (CTS) were present. The serum uric acid level was 10.1 mg/ dL (normal, 3.5 to 8.5 mg/dL). A complete blood cell count and ESR, the serum cal¬ cium level, and results of hepatic and renal function studies were normal. Vitamin B12 and whole blood folate levels, and results of thyroid function tests, a urinary screen for heavy metals, lead, and porphobilinogen, a five-hour glucose tolerance test, serum protein electrophoresis, and antinuclear antibody and rheumatoid factor tests were unremarkable. The CSF was normal. Results of an occult neoplasm workup were negative. Results of nerve conduction studies (NCS) were consistent with an axonal neuropathy and a right carpal tun¬ nel syndrome (Table). The patient refused a nerve biopsy and carpal tunnel release procedure. The dose of allopurinol was increased to 200 mg twice daily. Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/01/2015 Six months later, the patient claimed that his walking had improved and that his feet were no longer numb. Many of the tophi had regressed, knee reflexes had returned but were still hypoactive, strength and gait were normal, only mini¬ mal distal sensory loss was present, and previous signs of CTS were absent. The serum uric acid level was 6.9 mg/dL. Results of repeated NCS showed much improvement (Table). Three years later the patient had acute renal colic resulting in a nephrolithotomy. His uric acid level was 9.3 mg/dL. His gait was wide based, myotactic reflexes were absent in the legs, there was diminished strength of the anterior tibialis and exten¬ sor digitorum brevis muscles, and vibrato¬ ry and position sense and light touch were moderately reduced distally in all limbs. Results of a repeat workup for peripheral neuropathy were negative, and included normal phosphoribosyltransferase activi¬ ty.15 The NCS had worsened (Table). He had taken no nonprescription drugs, did not abuse alcohol, and had no exposure to toxin. He was given 200 mg of allopurinal daily and follow-up examination revealed a serum uric acid level of 7.5 mg/dL with subjective improvement. Knee reflexes had returned, his strength and gait were nor¬ mal, and mild distal sensory loss was present. Results of repeat NCS again were improved (Table). COMMENT Since the earliest description of