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Hair-braiding and combing-induced syncope: A paroxysmal nonepileptic event
J Epilepsy 1993;6:115-117 © 1993 Butterworth-Heinemann
Hair-Braiding and Combing-Induced Syncope: A Paroxysmal Nonepileptic Event James J. Riviello, Jr., and Stephen D. Rioux
Epilepsy is considered in the differential diagnosis of any paroxysmal event, especially if an aura is associated with altered awareness or a loss of consciousness. We have evaluated three female children, referred for possible epilepsy, who developed premonitory symptoms and lost consciousness during hairbraiding or combing. The patients all had migraine. This situation may resemble stretch syncope. Increased recognition that hairdressing may lead to a syncopal event may avoid a misdiagnosis of epilepsy. Key Words: Hair-braiding--Haircombing--Syncope.
Syncope, a transient loss of consciousness, results from acute cerebral hypoxia (1). The differential diagnosis of syncope includes epilepsy, and a misdiagnosis of epilepsy is frequently made because convulsive movements may result from a syncopal event, a reflex anoxic seizure (1). An accurate history is necessary to distinguish these. Specific information regarding the setting of the event and determining if a specific stimulus precipitated the event is critical. For example, stretching with neck extension has recently been described as a cause of syncope in the adolescent (2). We have evaluated three patients referred for possible epilepsy who lost consciousness while their hair was being braided or
Received December 17, 1992; accepted December 28, 1992. From the Section of Pediatric Neurology, Department of Pediatrics, Maine Medical Center, Portland, ME, and the Department of Pediatrics, University of Vermont School of Medicine, Burlington, VT, U.S.A. Address correspondence and reprint requests to Dr. J. J. Riviello, Jr., at his new address, Epilepsy Program, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, U.S.A.
combed. All had headaches and a family history of migraines.
Case Histories Case 1
This 7-year-old girl was referred for evaluation of a seizure. While her mother was removing a braid from her hair, she complained of stomach pain, suddenly arched her back, and then had a 2-3-min generalized tonic-clonic seizure. She had postictal lethargy, with blurring of vision in the right eye; no bowel or bladder incontinence occurred. There were no prior overt seizures, but she had rare staring spells, and recently developed headaches, and had recurrent episodes of paresthesias in the right hand that were not associated with headaches or altered awareness. The family history was positive for migraines. Physical examination, cranial computed tomography (CT), initial EEG, and 24-h ambulatory EEG (AEEG) were normal. No treatment was initiated. A second episode occurred 2 weeks later, while her hair was being combed. She complained of stomach pain, screamed, and then lost consciousness for 45 s. j EPILEPSY, VOL. 6, NO. 2, 1993
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]. J. RIVIELLO, JR., AND S. D. RIOUX
Afterward she appeared lethargic and complained of paresthesias in the right foot. She was started on phenobarbital. No further episodes occurred until she was taken off phenobarbital approximately 6 months later. This event occurred in the morning, again while her hair was being combed. She complained of stomach pain, lost consciousness, and had ocular supraversion with clonic movements of the right arm for about 20-30 s. She was tired afterward but had no headache. Her vision had become dim prior to the event. The phenobarbital was not restarted.
Case 2
This 5-year-old girl with a past history of febrile seizures treated with phenobarbital was referred for evaluation of possible seizure recurrence. She had suddenly complained that she was not able to see, became frightened, had generalized pallor with cyanosis of the lips, and then lost consciousness for 45-50 s. She was incontinent for urine. There was no prior history of headaches, although the family history was strongly positive for migraines. The examination and EEG were normal, and the tentative diagnosis was basilar artery migraine. She subsequently developed bitemporal headaches. She returned 11/2years later following another episode of loss of consciousness. He mother had been combing her hair while she was standing with the neck extended when she developed spots in front of her eyes, had fading vision, became pale, and then lost consciousness for approximately 1 min. Following this, she had a bitemporal headache and vomited. AEEG, cranial CT, cardiac examination, electrocardiogram, and echocardiogram were normal. Several subsequent episodes occurred when she either stretched or extended her neck.
Case 3
This 10-year-old girl was referred for syncope and headaches. The headaches had been present for 2-3 months, occurred in the evenings, were throbbing, and readily responded to acetaminophen. The episode of syncope occurred in the morning. She had taken a shower, and w h e n standing with her neck extended while her mother was braiding her hair, she saw spots in front of her eyes and then lost consciousness for approximately 1-11h rain. There was no incontinence. By the time she reached the emergency department, she had returned to normal. The family history was strongly positive for migraines, and the 116 J EPILEPSY, VOL. 6, NO. 2, 1993
patient had both motion sickness and somnambulism. Her examination was normal, and because of the experience with the above patients, no further testing was done.
Discussion
These three girls were all referred for evaluation of possible epilepsy. All clearly had premonitory symptoms followed by a syncopal episode during hairbraiding or combing, all have migraines, and, on one occasion, the first girl had a presumed seizure precipitated by the same stimuli. Primary epilepsy is unlikely, since all events were precipitated by either hair-braiding or combing, the premonitory symptoms followed the stimulus, and epileptiform features were absent on the EEGs in two. Stephenson has also reported five cases of syncope occurring with various hairdressing procedures: brushing, combing, cutting, damping, and blow-drying, with a presumed vagal event as the mechanism (1). This situation is similar to stretch syncope reported by Pelekanos et al. (2). The age of onset was later in his patients, five of six were males, and four of six also had a family history of fainting. They documented that stretching induced cardiovascular changes similar to those induced by the Valsalva maneuver, which leads to bradycardia. The postulated mechanism of stretch syncope is the Valsalva maneuver in combination with hyperextension of the neck superimposed on a familial tendency to faint. No mention of migraine was made. Of interest, our second child subsequently had similar episodes while stretching. Our children all had both headaches and a strong family history of migraine, which predisposes to syncope. Symptoms related to orthostatic hypotension, including syncope, occur with a greater frequency in patients with migraine, presumably secondary to autonomic instability. These may occur as part of a migraine syndrome, such as with basilar artery migraine (3), or they may occur independently. The presenting episode in our Case 2 was, in fact, basilar artery migraine. The incidence of syncope has varied widely in childhood migraine patients. Prensky and Sommer reported that only 1.2% of their children with migraine had syncope (4). In Barlow's series of juvenile migraine patients, 10/300 had syncope unrelated to a migraine attack (5). Bille reported an incidence of syncope in 12.3% of the patients with migraine, but 28.8% had orthostatic symptoms (6). The presence of migraine may therefore provide the predisposition for fainting, postulated as important in the mechanism of stretch syncope (2).
HAIR-BRAIDING AND COMBING-INDUCED SYNCOPE
The generalized seizure that occurred in our Case 1 is confusing. Reflex epilepsy may occur following various stimuli, a reflex anoxic seizure may follow a syncopal event, and an actual epileptic seizure, classified as an anoxic-epileptic seizure, may follow an anoxic stimulus. However, an anoxic-epileptic seizure is rare, occurring in only 1/5,000 in a series of patients with syncope (1). Epileptic seizures induced by syncopal attacks have also been reported by Battaglia et al. (7), and Emery has reported status epilepticus secondary to breath-holding and pallid syncope (8). Increased recognition that hairdressing may lead to a syncopal event is needed. These cases emphasize the importance of an accurate history of any paroxysmal episode in order to avoid a misdiagnosis of epilepsy.
References 1. Stephenson JBP. Fits and faints. Oxford: Blackwell Scientific Publications, 1990. 2. Pelekanos JT, Dooley JM, Camfield PR, Finley J. Stretch syncope in adolescence. Neurology 1990;40:705-7. 3. Bickerstaff ER. Basilar artery migraine. Lancet 1961; 2:15-7. 4. Prensky AL, Sommer D. Diagnosis and treatment of migraine in children. Neurology 1979;29:506-10. 5. Barlow CF. Headaches and migraine in childhood. Oxford: Blackwell Scientific Publications, 1984:151-4. 6. Bille B. Migraine in school children. Acta Paediatrica 1962;51(Suppl 136):1-151. 7. Battaglia A, Guerrini R, Gastaut H. Epileptic seizures induced by syncopal attacks. J Epilepsy 1989;2:13746. 8. Emery ES. Status epilepticus secondary to breathholding and pallid syncopal spells. Neurology 1990; 40:859.
j EPILEPSY, VOL. 6, NO. 2, 1993
117
Hair-Braiding and Combing-Induced Syncope: A Paroxysmal Nonepileptic Event James J. Riviello, Jr., and Stephen D. Rioux
Epilepsy is considered in the differential diagnosis of any paroxysmal event, especially if an aura is associated with altered awareness or a loss of consciousness. We have evaluated three female children, referred for possible epilepsy, who developed premonitory symptoms and lost consciousness during hairbraiding or combing. The patients all had migraine. This situation may resemble stretch syncope. Increased recognition that hairdressing may lead to a syncopal event may avoid a misdiagnosis of epilepsy. Key Words: Hair-braiding--Haircombing--Syncope.
Syncope, a transient loss of consciousness, results from acute cerebral hypoxia (1). The differential diagnosis of syncope includes epilepsy, and a misdiagnosis of epilepsy is frequently made because convulsive movements may result from a syncopal event, a reflex anoxic seizure (1). An accurate history is necessary to distinguish these. Specific information regarding the setting of the event and determining if a specific stimulus precipitated the event is critical. For example, stretching with neck extension has recently been described as a cause of syncope in the adolescent (2). We have evaluated three patients referred for possible epilepsy who lost consciousness while their hair was being braided or
Received December 17, 1992; accepted December 28, 1992. From the Section of Pediatric Neurology, Department of Pediatrics, Maine Medical Center, Portland, ME, and the Department of Pediatrics, University of Vermont School of Medicine, Burlington, VT, U.S.A. Address correspondence and reprint requests to Dr. J. J. Riviello, Jr., at his new address, Epilepsy Program, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, U.S.A.
combed. All had headaches and a family history of migraines.
Case Histories Case 1
This 7-year-old girl was referred for evaluation of a seizure. While her mother was removing a braid from her hair, she complained of stomach pain, suddenly arched her back, and then had a 2-3-min generalized tonic-clonic seizure. She had postictal lethargy, with blurring of vision in the right eye; no bowel or bladder incontinence occurred. There were no prior overt seizures, but she had rare staring spells, and recently developed headaches, and had recurrent episodes of paresthesias in the right hand that were not associated with headaches or altered awareness. The family history was positive for migraines. Physical examination, cranial computed tomography (CT), initial EEG, and 24-h ambulatory EEG (AEEG) were normal. No treatment was initiated. A second episode occurred 2 weeks later, while her hair was being combed. She complained of stomach pain, screamed, and then lost consciousness for 45 s. j EPILEPSY, VOL. 6, NO. 2, 1993
115
]. J. RIVIELLO, JR., AND S. D. RIOUX
Afterward she appeared lethargic and complained of paresthesias in the right foot. She was started on phenobarbital. No further episodes occurred until she was taken off phenobarbital approximately 6 months later. This event occurred in the morning, again while her hair was being combed. She complained of stomach pain, lost consciousness, and had ocular supraversion with clonic movements of the right arm for about 20-30 s. She was tired afterward but had no headache. Her vision had become dim prior to the event. The phenobarbital was not restarted.
Case 2
This 5-year-old girl with a past history of febrile seizures treated with phenobarbital was referred for evaluation of possible seizure recurrence. She had suddenly complained that she was not able to see, became frightened, had generalized pallor with cyanosis of the lips, and then lost consciousness for 45-50 s. She was incontinent for urine. There was no prior history of headaches, although the family history was strongly positive for migraines. The examination and EEG were normal, and the tentative diagnosis was basilar artery migraine. She subsequently developed bitemporal headaches. She returned 11/2years later following another episode of loss of consciousness. He mother had been combing her hair while she was standing with the neck extended when she developed spots in front of her eyes, had fading vision, became pale, and then lost consciousness for approximately 1 min. Following this, she had a bitemporal headache and vomited. AEEG, cranial CT, cardiac examination, electrocardiogram, and echocardiogram were normal. Several subsequent episodes occurred when she either stretched or extended her neck.
Case 3
This 10-year-old girl was referred for syncope and headaches. The headaches had been present for 2-3 months, occurred in the evenings, were throbbing, and readily responded to acetaminophen. The episode of syncope occurred in the morning. She had taken a shower, and w h e n standing with her neck extended while her mother was braiding her hair, she saw spots in front of her eyes and then lost consciousness for approximately 1-11h rain. There was no incontinence. By the time she reached the emergency department, she had returned to normal. The family history was strongly positive for migraines, and the 116 J EPILEPSY, VOL. 6, NO. 2, 1993
patient had both motion sickness and somnambulism. Her examination was normal, and because of the experience with the above patients, no further testing was done.
Discussion
These three girls were all referred for evaluation of possible epilepsy. All clearly had premonitory symptoms followed by a syncopal episode during hairbraiding or combing, all have migraines, and, on one occasion, the first girl had a presumed seizure precipitated by the same stimuli. Primary epilepsy is unlikely, since all events were precipitated by either hair-braiding or combing, the premonitory symptoms followed the stimulus, and epileptiform features were absent on the EEGs in two. Stephenson has also reported five cases of syncope occurring with various hairdressing procedures: brushing, combing, cutting, damping, and blow-drying, with a presumed vagal event as the mechanism (1). This situation is similar to stretch syncope reported by Pelekanos et al. (2). The age of onset was later in his patients, five of six were males, and four of six also had a family history of fainting. They documented that stretching induced cardiovascular changes similar to those induced by the Valsalva maneuver, which leads to bradycardia. The postulated mechanism of stretch syncope is the Valsalva maneuver in combination with hyperextension of the neck superimposed on a familial tendency to faint. No mention of migraine was made. Of interest, our second child subsequently had similar episodes while stretching. Our children all had both headaches and a strong family history of migraine, which predisposes to syncope. Symptoms related to orthostatic hypotension, including syncope, occur with a greater frequency in patients with migraine, presumably secondary to autonomic instability. These may occur as part of a migraine syndrome, such as with basilar artery migraine (3), or they may occur independently. The presenting episode in our Case 2 was, in fact, basilar artery migraine. The incidence of syncope has varied widely in childhood migraine patients. Prensky and Sommer reported that only 1.2% of their children with migraine had syncope (4). In Barlow's series of juvenile migraine patients, 10/300 had syncope unrelated to a migraine attack (5). Bille reported an incidence of syncope in 12.3% of the patients with migraine, but 28.8% had orthostatic symptoms (6). The presence of migraine may therefore provide the predisposition for fainting, postulated as important in the mechanism of stretch syncope (2).
HAIR-BRAIDING AND COMBING-INDUCED SYNCOPE
The generalized seizure that occurred in our Case 1 is confusing. Reflex epilepsy may occur following various stimuli, a reflex anoxic seizure may follow a syncopal event, and an actual epileptic seizure, classified as an anoxic-epileptic seizure, may follow an anoxic stimulus. However, an anoxic-epileptic seizure is rare, occurring in only 1/5,000 in a series of patients with syncope (1). Epileptic seizures induced by syncopal attacks have also been reported by Battaglia et al. (7), and Emery has reported status epilepticus secondary to breath-holding and pallid syncope (8). Increased recognition that hairdressing may lead to a syncopal event is needed. These cases emphasize the importance of an accurate history of any paroxysmal episode in order to avoid a misdiagnosis of epilepsy.
References 1. Stephenson JBP. Fits and faints. Oxford: Blackwell Scientific Publications, 1990. 2. Pelekanos JT, Dooley JM, Camfield PR, Finley J. Stretch syncope in adolescence. Neurology 1990;40:705-7. 3. Bickerstaff ER. Basilar artery migraine. Lancet 1961; 2:15-7. 4. Prensky AL, Sommer D. Diagnosis and treatment of migraine in children. Neurology 1979;29:506-10. 5. Barlow CF. Headaches and migraine in childhood. Oxford: Blackwell Scientific Publications, 1984:151-4. 6. Bille B. Migraine in school children. Acta Paediatrica 1962;51(Suppl 136):1-151. 7. Battaglia A, Guerrini R, Gastaut H. Epileptic seizures induced by syncopal attacks. J Epilepsy 1989;2:13746. 8. Emery ES. Status epilepticus secondary to breathholding and pallid syncopal spells. Neurology 1990; 40:859.
j EPILEPSY, VOL. 6, NO. 2, 1993
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