Transient loss of consciousness with muscle jerks: syncope or epilepsy ?

W. Wieling *, CA Remme §, J.G van Dijk +
Department of Medicine* and Experimental Cardiology § , Academic Medical Centre, University of Amsterdam, Amsterdam (The Netherlands)
Department of Neurology +, Leids University Medical Centre, Leiden (The Netherlands)

A 35 year old otherwise healthy engineer was referred to the syncope unit for analysis of an episode of loss of consciousness, which occurred during a flight on return from a week-long holiday in Turkey. Prior to boarding the airplane at midnight, the patient felt tired and was continuously yawning without any other specific complaints. Once seated in the aircraft, he fell asleep almost immediately. After approximately 30 minutes of sleep he retrieved an item from the overhead compartment without problems, after which he quickly fell asleep again. About one hour later, he woke up feeling weak and extremely thirsty, and decided to have a softdrink. The patients’ partner, a cardiology resident, was subsequently woken up by the sound of the softdrink can falling on the floor and she noticed that the patient was unconscious with muscle spasms and an upward turning of the eyes. Shortly after this, the muscle spasms were replaced by jerking of (most noticeably) the arm muscles, after which he became completely flaccid. His pulse was very slow and weak and he was breathing superficially. Before he could be transferred from his chair to the aisle, he regained consciousness. The duration of the period of unconsciousness was short (< 3 min). On regaining consciousness he was well orientated but complained of tiredness, weakness and slowness in thinking and speech. There was no sign of urine incontinence or tongue biting. When he tried to stand up and walk a few steps he collapsed again without losing consciousness. After a few minutes he was able to stand up and walk by himself. On examination, his pulse was approximately 40-45 beats/min (regular) with a blood pressure of 100/60. He complained of muscle pain in his arms and shoulders, blurred vision, tiredness, and cold sweats. These symptoms gradually disappeared during the following 2 hours, after drinking 3 to 4 glasses of water with sugar. On arrival at the airport, he was checked by ambulance personnel and found to have a normal heart rate, blood pressure and blood glucose level. Physical and neurological examinations and EKG analysis performed at the Emergency Department of the Academic Medical Centre showed no abnormalities, after which he was referred to our syncope unit.

The patient denied earlier episodes of loss of consciousness. However, during visits to hospitals and while watching TV programs with surgical scenes he sometimes had a tendency to feel weak. The patient used no medications. Based on the premonitiory symptoms of feeling weak, prolonged motionless sitting, the documentation of a very slow and weak pulse during the episode, the tendency to faint again after regaining consciousness and the previous sensations of nearfaints during hospital encounters a clinical diagnosis of vasovagal syncope was made. The patient was reassured. Specific instructions for future travel by airplane were given. Since the episode occurred, the patient has traveled by plane on several occasions without any problems.

Editor's comments

The differential diagnosis of a patient with a transient loss of consciousness accompanied by muscle jerks includes an epileptic seizure and an episode of convulsive syncope. In the present case the short duration of unconsciousness and rapid reorientation after regaining consciousness makes an epileptic seizure highly unlikely. An underlying cardiac abnormality is also unlikely, since the patient has no cardiac history and both the physical exam and the EKG were normal. Thus, the current case report points in the direction of convulsive vasovagal syncope. Vasovagal episodes are the most common in-flight medical events [1]. In addition to prolonged motionless sitting, mild hypoxia occurring during air travel (the cabin pressure on commercial aircraft is usually adjusted to be equivalent to the barometric pressure found at an altitude of 1500 to 2500 m above sea level) may be involved. Even mild hypoxia predisposes to vasovagal faints. It appears that hypoxic syncope results from the superimposed vasodilator effects of hypoxia on the cardiovascular system[2].

Jerky movements mimicking an epileptic seizure may occur during syncope. The circumstances determining whether they do or do not appear are not well known. In clinical practice there is a clear tendency for jerks to appear following prolonged asystole of at least 10-14 seconds or a prolonged period of very low blood pressure. In the fainting lark, an experimentally induced syncope with a very abrupt cessation of cerebral perfusion (see case 4 in this section) the jerky movements appeared almost as soon as the subject hit the ground. The 'anoxic threshold' for myoclonic jerks is lower in children than in adults, and it is lowest in early childhood[3][4]. In contrast to clonic movements in epilepsy, jerks in syncope are usually not rhythmic, not synchronous in the extremities, and less coarse as compared to those observed in epilepsy. Furthermore, in syncope the jerky movements never occur before falling, whereas in epilepsy they may occur before falling[5][6]. In a typical tonic-clonic epileptic seizure, however, the fall is due to the stiff tonic phase and the clonic movements occur afterwards. The myoclonic activity is presumably due to a lack of inhibition from higher centers, but the site of origin remains unknown[5].

The prevalence of myoclonic jerks in patients with syncope is not well known. In a prospective study Newman et al. documented tetany, clonic movements, and twitching in 46% of 178 blood donors having a vasovagal reaction[7]. In the fainting lark myoclonic jerks occurred in 90%. These vastly differing percentages suggest that the occurrence of jerky movements depends on how hypoperfusion.affects cerebral perfusion, but which factors are involved is unknown.

Urinary incontinence is uncommon in reflex syncope, but does occur and so does fecal incontinence. Incontinence cannot be used as discriminating factors between epilepsy and syncope[8][9].

Typical symptoms and signs of epilepsy are tongue-biting, a cyanotic facial color and an aura. Benbadis et al. compared 34 patients with epileptic seizures to 45 syncopal patients. Eight patients with documented epileptic seizures suffered a lateral tongue bite. The tongue was lacerated in only one of 45 patients with syncope and this was at the tip, suggesting that lateral tongue biting is highly specific (99%) for the diagnosis of epileptic seizures[10]. Consistent turning of the head to one side is also reported as a specific sign for epilepsy[9], but lateral deviation of the head was also observed in experimental syncope induced by ocular compression[11]. Emotional stress has been reported as a precipitating factor both for reflex syncope and epilepsy[12][13], but in syncope emotional factors may provoke and thus occur immediately prior to fainting, whereas stress in epilepsy appears to have a less immediate effect in the sense that seizures may occur during a longer period of stress. Important symptoms that distinguish between reflex syncope and epilepsy are the duration of the loss of consciousness and postictal confusion. In the recovery phase of an episode of syncope there is usually little to no confusion[8][9]. Finally, circumstances such as prolonged standing and autonomic symptoms such as cold sweat and nausea make epilepsy unlikely as the cause for the event[7]. Epileptic seizures usually do not have a clear trigger. They occur more randomly i.e. in standing, sitting or supine position.

Sensitivity and specificity of data from the medical history are compared in Table 1.

Table 1. Diagnostic accuracy of specific items from history taking.

Factors strongly suggesting epilepsy

	Hoefnagels			Sheldon
	Sens	Spec	LR+	Sens	Spec	LR+
Tongue biting	0.41	0.94	7.3	0.45	0.97	16.5
Turning of the head	NR	NR	NR	0.43	0.97	13.5
Muscle pain	0.39	0.85	2.6	0.16	0.95	3.4
Duration loss of cons. (>5 min)	0.68	0.55	1.5	NR	NR	NR
Cyanosis	0.29	0.98	16.9	0.33	0.94	5.8
Postictal confusion	0.85	0.83	5	0.94	0.69	3.0

Factors strongly suggesting syncope

	Hoefnagels			Sheldon
	Sens	Spec	LR+	Sens	Spec	LR+
Prolonged sitting or standing	NR	NR	NR	0.40	0.98	20.4
Sweating prior to loss of cons.	0.36	0.98	18	0.35	0.94	5.9
Nausea	0.28	0.98	14	0.28	0.94	4.7
History of pre-syncope	NR	NR	NR	0.73	0.73	2.6
Paleness	0.81	0.66	2.8	NR	NR	NR

sens= sensitivity; spec=specificity; LR+: likelihood ratio of a positive test result; NR=not reported

References

<biblio>

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2. Halliwill pmid=15531565
3. Stephenson Stephenson JBP Fits and faints. Blackwell Scientific Publications Ltd, Oxford, 1990
4. Stephenson2 Stephenson JBP, McLeod KA Reflex Anoxic Seizures. In: David TJ (ed) Recent Advances in Pediatrics 18. Churchill Livingstone, Edinburgh, 2000
5. Gastaut Gastaut H (1974) Syncopes: generalized anoxic cerebral seizures. In: Magnus O, Haas AM (eds) Handbook of clinical neurology. Amsterdam, pp 815-836
6. Lempert pmid=8053660
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10. Benbadis pmid=7487261
11. Gastaut2 pmid=13482270
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13. Lieshout pmid=1661644

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