Some people with mental retardation may experience seizures, but seizures do not necessarily mean the person has or will develop mental impairment. Many people with epilepsy have normal or above-average intelligence. Famous people who are known or rumored to have had epilepsy include the Russian writer Dostoyevsky, the philosopher Socrates, the military general Napoleon, and the inventor of dynamite, Alfred Nobel, who established the Nobel prize.
Several Olympic medalists and other athletes also have had epilepsy. Seizures sometimes do cause brain damage, particularly if they are severe. However, most seizures do not seem to have a detrimental effect on the brain. Any changes that do occur are usually subtle, and it is often unclear whether these changes are caused by the seizures themselves or by the underlying problem that caused the seizures. While epilepsy cannot currently be cured, for some people it does eventually go away.
One study found that children with idiopathic epilepsy , or epilepsy with an unknown cause, had a 68 to 92 percent chance of becoming seizure-free by 20 years after their diagnosis. The odds of becoming seizure-free are not as good for adults, or for children with severe epilepsy syndromes, but it is nonetheless possible that seizures may decrease or even stop over time.
This is more likely if the epilepsy has been well-controlled by medication or if the person has had epilepsy surgery. What causes epilepsy? Epilepsy is a disorder with many possible causes. Anything that disturbs the normal pattern of neuron activity—from illness to brain damage to abnormal brain development—can lead to seizures. Epilepsy may develop because of an abnormality in brain wiring, an imbalance of nerve signaling chemicals called neurotransmitters, or some combination of these factors.
Researchers believe that some people with epilepsy have an abnormally high level of excitatory neurotransmitters that increase neuronal activity, while others have an abnormally low level of inhibitory neurotransmitters that decrease neuronal activity in the brain. Either situation can result in too much neuronal activity and cause epilepsy.
One of the most-studied neurotransmitters that plays a role in epilepsy is GABA, or gamma-aminobutyric acid, which is an inhibitory neurotransmitter. Research on GABA has led to drugs that alter the amount of this neurotransmitter in the brain or change how the brain responds to it. Researchers also are studying excitatory neurotransmitters such as glutamate.
Abnormalities in brain wiring that occur during brain development also may disturb neuronal activity and lead to epilepsy. Research has shown that the cell membrane that surrounds each neuron plays an important role in epilepsy. Cell membranes are crucial for neurons to generate electrical impulses. For this reason, researchers are studying details of the membrane structure, how molecules move in and out of membranes, and how the cell nourishes and repairs the membrane.
A disruption in any of these processes may lead to epilepsy. Studies in animals have shown that, because the brain continually adapts to changes in stimuli, a small change in neuronal activity, if repeated, may eventually lead to full-blown epilepsy. Researchers are investigating whether this phenomenon, called kindling , may also occur in humans. In some cases, epilepsy may result from changes in non-neuronal brain cells called glia. These cells regulate concentrations of chemicals in the brain that can affect neuronal signaling.
About half of all seizures have no known cause. However, in other cases, the seizures are clearly linked to infection, trauma, or other identifiable problems. Genetic factors. Research suggests that genetic abnormalities may be some of the most important factors contributing to epilepsy. Some types of epilepsy have been traced to an abnormality in a specific gene.
Many other types of epilepsy tend to run in families, which suggests that genes influence epilepsy. Some researchers estimate that more than genes could play a role in this disorder. Several types of epilepsy have now been linked to defective genes for ion channels , the "gates" that control the flow of ions in and out of cells and regulate neuron signaling. Another gene, which is missing in people with progressive myoclonus epilepsy , codes for a protein called cystatin B. This protein regulates enzymes that break down other proteins.
Another gene, which is altered in a severe form of epilepsy called LaFora disease , has been linked to a gene that helps to break down carbohydrates. While abnormal genes sometimes cause epilepsy, they also may influence the disorder in subtler ways. For example, one study showed that many people with epilepsy have an abnormally active version of a gene that increases resistance to drugs. This may help explain why anticonvulsant drugs do not work for some people. Abnormalities in the genes that control neuronal migration—a critical step in brain development— can lead to areas of misplaced or abnormally formed neurons, or dysplasia , in the brain that can cause epilepsy.
In some cases, genes may contribute to development of epilepsy even in people with no family history of the disorder. These people may have a newly developed abnormality, or mutation , in an epilepsy-related gene. Other disorders. In many cases, epilepsy develops as a result of brain damage from other disorders. Strokes, heart attacks, and other conditions that deprive the brain of oxygen also can cause epilepsy in some cases. About 32 percent of all newly developed epilepsy in elderly people appears to be due to cerebrovascular disease, which reduces the supply of oxygen to brain cells.
Meningitis, AIDS, viral encephalitis, and other infectious diseases can lead to epilepsy, as can hydrocephalus—a condition in which excess fluid builds up in the brain. Epilepsy also can result from intolerance to wheat gluten known as celiac disease , or from a parasitic infection of the brain called neurocysticercosis. Seizures may stop once these disorders are treated successfully.
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However, the odds of becoming seizure-free after the primary disorder is treated are uncertain and vary depending on the type of disorder, the brain region that is affected, and how much brain damage occurred prior to treatment. Epilepsy is associated with a variety of developmental and metabolic disorders, including cerebral palsy, neurofibromatosis, pyruvate deficiency, tuberous sclerosis, Landau-Kleffner syndrome, and autism. Epilepsy is just one of a set of symptoms commonly found in people with these disorders.
Head injury. In some cases, head injury can lead to seizures or epilepsy. Safety measures such as wearing seat belts in cars and using helmets when riding a motorcycle or playing competitive sports can protect people from epilepsy and other problems that result from head injury. Prenatal injury and developmental problems.
The developing brain is susceptible to many kinds of injury. Maternal infections, poor nutrition, and oxygen deficiencies are just some of the conditions that may take a toll on the brain of a developing baby. These conditions may lead to cerebral palsy, which often is associated with epilepsy, or they may cause epilepsy that is unrelated to any other disorders. About 20 percent of seizures in children are due to cerebral palsy or other neurological abnormalities.
Abnormalities in genes that control development also may contribute to epilepsy. Advanced brain imaging has revealed that some cases of epilepsy that occur with no obvious cause may be associated with areas of dysplasia in the brain that probably develop before birth. Seizures can result from exposure to lead, carbon monoxide, and many other poisons. They also can result from exposure to street drugs and from overdoses of antidepressants and other medications. Seizures are often triggered by factors such as lack of sleep, alcohol consumption, stress, or hormonal changes associated with the menstrual cycle.
These seizure triggers do not cause epilepsy but can provoke first seizures or cause breakthrough seizures in people who otherwise experience good seizure control with their medication. Sleep deprivation in particular is a universal and powerful trigger of seizures. For this reason, people with epilepsy should make sure to get enough sleep and should try to stay on a regular sleep schedule as much as possible.
For some people, light flashing at a certain speed or the flicker of a computer monitor can trigger a seizure; this problem is called photosensitive epilepsy. Smoking cigarettes also can trigger seizures. The nicotine in cigarettes acts on receptors for the excitatory neurotransmitter acetylcholine in the brain, which increases neuronal firing. Seizures are not triggered by sexual activity except in very rare instances.
What are the different kinds of seizures? Doctors have described more than 30 different types of seizures. Seizures are divided into two major categories— focal seizures and generalized seizures. However, there are many different types of seizures in each of these categories.
Focal seizures. Focal seizures, also called partial seizures, occur in just one part of the brain. About 60 percent of people with epilepsy have focal seizures. These seizures are frequently described by the area of the brain in which they originate.
For example, someone might be diagnosed with partial frontal lobe seizures. In a simple focal seizure , the person will remain conscious but may experience unusual feelings or sensations that can take many forms. The person may experience sudden and unexplainable feelings of joy, anger, sadness, or nausea.
He or she also may hear, smell, taste, see, or feel things that are not real. In a complex focal seizure , the person has a change in or loss of consciousness. His or her consciousness may be altered, producing a dreamlike experience. People having a complex partial seizure may display strange, repetitious behaviors such as blinks, twitches, mouth movements, or even walking in a circle. These repetitious movements are called automatisms. They also may fling objects across the room or strike out at walls or furniture as though they are angry or afraid.
These seizures usually last just a few seconds. Some people with focal seizures, especially complex focal seizures, may experience auras —unusual sensations that warn of an impending seizure. These auras are actually simple partial seizures in which the person maintains consciousness. The symptoms an individual person has, and the progression of those symptoms, tends to be stereotyped , or similar every time. The symptoms of focal seizures can easily be confused with other disorders. For instance, the dreamlike perceptions associated with a complex partial seizure may be misdiagnosed as migraine headaches, which also can cause a dreamlike state.
The strange behavior and sensations caused by partial seizures also can be mistaken for symptoms of narcolepsy, fainting, or even mental illness. It may take many tests and careful monitoring by a knowledgeable physician to tell the difference between epilepsy and other disorders. Generalized seizures. Generalized seizures are a result of abnormal neuronal activity in many parts of the brain.
These seizures may cause loss of consciousness, falls, or massive muscle spasms. There are many kinds of generalized seizures. These seizures are sometimes referred to as petit mal seizures , which is an older term. Tonic seizures cause stiffening of muscles of the body, generally those in the back, legs, and arms. Clonic seizures cause repeated jerking movements of muscles on both sides of the body. Myoclonic seizures cause jerks or twitches of the upper body, arms, or legs. Atonic seizures cause a loss of normal muscle tone.
The affected person will fall down or may nod his or her head involuntarily. Tonic-clonic seizures are sometimes referred to by an older term: grand mal seizures. Not all seizures can be easily defined as either partial or generalized. Some people have seizures that begin as partial seizures but then spread to the entire brain. Other people may have both types of seizures but with no clear pattern.
In some cases, this has led to the affected person being arrested or admitted to a psychiatric hospital. To combat these problems, people everywhere need to understand the many different types of seizures and how they may appear. What are the different kinds of epilepsy? Just as there are many different kinds of seizures, there are many different kinds of epilepsy. Doctors have identified hundreds of different epilepsy syndromes —disorders characterized by a specific set of symptoms that include epilepsy. Some of these syndromes appear to be hereditary. For other syndromes, the cause is unknown.
Epilepsy syndromes are frequently described by their symptoms or by where in the brain they originate. People should discuss the implications of their type of epilepsy with their doctors to understand the full range of symptoms, the possible treatments, and the prognosis. People with absence epilepsy have repeated absence seizures that cause momentary lapses of consciousness. These seizures almost always begin in childhood or adolescence, and they tend to run in families, suggesting that they may be at least partially due to a defective gene or genes.
Some people with absence seizures have purposeless movements during their seizures, such as a jerking arm or rapidly blinking eyes. Others have no noticeable symptoms except for brief times when they are "out of it. However, these seizures may occur so frequently that the person cannot concentrate in school or other situations. Childhood absence epilepsy usually stops when the child reaches puberty.
Absence seizures usually have no lasting effect on intelligence or other brain functions. Temporal lobe epilepsy. Temporal lobe epilepsy, or TLE, is the most common epilepsy syndrome with partial seizures. These seizures are often associated with auras. TLE often begins in childhood. Research has shown that repeated temporal lobe seizures can cause a brain structure called the hippocampus to shrink over time.
The hippocampus is important for memory and learning. While it may take years of temporal lobe seizures for measurable hippocampal damage to occur, this finding underlines the need to treat TLE early and as effectively as possible. Neocortical epilepsy is characterized by seizures that originate from the brain's cortex, or outer layer. The seizures can be either focal or generalized. They may include strange sensations, visual hallucinations, emotional changes, muscle spasms, convulsions, and a variety of other symptoms, depending on where in the brain the seizures originate.
There are many other types of epilepsy, each with its own characteristic set of symptoms. Many of these, including Lennox-Gastaut syndrome and Rasmussen's encephalitis , begin in childhood. Children with Lennox-Gastaut syndrome have severe epilepsy with several different types of seizures, including atonic seizures, which cause sudden falls and are also called drop attacks. This severe form of epilepsy can be very difficult to treat effectively.
Rasmussen's encephalitis is a progressive type of epilepsy in which half of the brain shows continual inflammation. It sometimes is treated with a radical surgical procedure called hemispherectomy see the section on Surgery. Some childhood epilepsy syndromes, such as childhood absence epilepsy, tend to go into remission or stop entirely during adolescence, whereas other syndromes such as juvenile myoclonic epilepsy and Lennox-Gastaut syndrome are usually present for life once they develop.
Seizure syndromes do not always appear in childhood, however. Epilepsy syndromes that are easily treated, do not seem to impair cognitive functions or development, and usually stop spontaneously are often described as benign. Benign epilepsy syndromes include benign infantile encephalopathy and benign neonatal convulsions.
Other syndromes, such as early myoclonic encephalopathy , include neurological and developmental problems. However, these problems may be caused by underlying neurodegenerative processes rather than by the seizures. Benign rolandic epilepsy, now formally known as benign childhood epilepsy with centrotemporal spikes , is a seizure syndrome that is so named because it usually stops before age 16, and the seizure discharge is localized on EEG studies to a limited area of the brain around the Rolandic or central sulcus.
The seizures begin between ages 3 and 13, are infrequent, and usually occur during sleep, especially the early morning hours, so anti-epileptic drugs are often not prescribed. The seizures usually consist of muscular twitching confined to one side of the face or body or tingling of one side of the tongue or mouth and may be accompanied by gurgling noises. Some children may have daytime seizures, which may be one sided or bilateral and behavioral or learning problems, in which case anti-epileptic drugs may be prescribed.
Several types of epilepsy begin in infancy. The most common type of infantile epilepsy is infantile spasms , clusters of seizures that usually begin before the age of 6 months. During these seizures the infant may bend and cry out. Anticonvulsant drugs often do not work for infantile spasms, but the seizures can be treated with ACTH adrenocorticotropic hormone or prednisone. When are seizures not epilepsy? While any seizure is cause for concern, having a seizure does not by itself mean a person has epilepsy. First seizures, febrile seizures, nonepileptic events, and eclampsia are examples of seizures that may not be associated with epilepsy.
First seizures. Many people have a single seizure at some point in their lives. Often these seizures occur in reaction to anesthesia or a strong drug, but they also may be unprovoked, meaning that they occur without any obvious triggering factor. Unless the person has suffered brain damage or there is a family history of epilepsy or other neurological abnormalities, these single seizures usually are not followed by additional seizures.
One recent study that followed patients for an average of 8 years found that only 33 percent of people have a second seizure within 4 years after an initial seizure. People who did not have a second seizure within that time remained seizure-free for the rest of the study. For people who did have a second seizure, the risk of a third seizure was about 73 percent on average by the end of 4 years.
The doctor also may order brain scans to identify abnormalities that may be visible in the brain. These tests may help the doctor decide whether or not to treat the person with antiepileptic drugs. In some cases, drug treatment after the first seizure may help prevent future seizures and epilepsy. However, the drugs also can cause detrimental side effects, so doctors prescribe them only when they feel the benefits outweigh the risks. Evidence suggests that it may be beneficial to begin anticonvulsant medication once a person has had a second seizure, as the chance of future seizures increases significantly after this occurs.
Febrile seizures. Sometimes a child will have a seizure during the course of an illness with a high fever. Please log in first to purchase, by clicking here. Featured Resources:. South Carolina Evidence Handbook Annotated, 14t South Carolina Rules Annotated Similar Products:. Pattern Jury Instructions for Federal Criminal Pattern jury instructions for federal criminal cases. We screened all videopolysomnographic recordings for motor episodes, and, after selecting any motor event which had occurred during the recordings, we assembled those which could be defined as pathological.
We finally compared seizure semiology with the other clinical and demographic data. Diagnosis of NFLE was straightforward when patients displayed one or more episodes associated with clear-cut ictal epileptic frontal activity during polysomnography. When ictal EEG was uninformative, we required the recording of more than one seizure with a stereotypic motor pattern, during one or more polysomnography recording.
On the basis of the different intensity, duration and features of the motor pattern, we classified the epileptic seizures into three groups according to Montagna Montagna, NPD had a longer duration 20 s—2 min and a more complex behaviour characterized by wide, often violent, sometimes ballistic movements, with dystonic posturing of the head, trunk and limbs such as head rotation, torsion of the trunk and choreo-athetoid movements of the arms and legs with vocalization.
ENW were the longest episodes 1—3 min in which the characteristic feature was stereotypic paroxysmal ambulation during sleep, often agitated and accompanied by screaming and bizarre, dystonic movements. One hundred and ninety-eight polysomnography recordings were screened and contained at least one paroxysmal episode. The other 26 had uneventful recordings and were excluded from the analysis. Patients were thereafter followed up by us for from 1 to 23 years mean 3.
The diagnoses, multiple in 17 patients, were made on clinical grounds without polysomnography, and included sleep-talking in 24 patients, sleep-walking in 21, primary enuresis in seven, sleep-terror in five, bruxism in four, head banging in one and REM-sleep behaviour disorder in another. Twenty-five patients had a family history of epilepsy, eight of whom belonging to six families had NFLE confirmed by polysomnography in three with nocturnal episodes quite similar to those of the proband.
In these cases, a pedigree study disclosed in all a transmission pattern consistent with autosomal dominant inheritance. In fact, five kindreds presented two or three affected members with a vertical transmission in two generations. The other family had four affected individuals in three generations. Six patients had a family history both for epilepsy and otherwise typical parasomnias.
Seven patients only had significant perinatal suffering, three had had febrile convulsions and three a mild head trauma preceding seizure onset by age 16, 15 and 5 years. Eighteen patients complained that psychological stress was a trigger for the nocturnal seizures, while in three cases seizures appeared after sleep deprivation and in one case peri-menstruation.
Patients often complained of nocturnal sleep discontinuity, with sleep disrupted by repeated arousals. These were, in such cases, reported and described by their relatives, who instigated the medical consultation. In 18 cases, patients or relatives reported only episodes of duration and semiology suggesting PA, and in 47 cases they reported more complex behaviours with the characteristics of NPD, with six of them also reporting the association of briefer episodes like PA.
In 11 cases, daytime seizures had begun before the nocturnal episodes, in 15 cases they had appeared simultaneously and in eight cases after nocturnal seizure onset. In six cases, however, daytime seizures were sporadic, present for a few days or years, in two cases, no more than five seizures in all, in one case seizures for a few days in a row only and in another case occasional seizures from age 10 months to 4 years.
We noted remarkable facial asymmetry in three patients; slight hemisomatic asymmetry in one case; slight mental retardation in two patients; and asymmetric tendon jerks in another two patients. We recorded two or more seizures with a stereotypic motor pattern in 93 of patients, and in seven we recorded only a single episode but with clear-cut epileptic EEG activity upon polysomnography recording.
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We recorded PA episodes, lasting from 2 to 20 s mean 9. Patients with PA had a mean of seven episodes per polysomnography recording. As belated movements, the patients moved their backs or chewed and emitted sounds. After the attacks, all patients quickly went back to sleep. In eight patients, seizures were characterized by asymmetric sustained posture of the limbs with choreo-athetoid movements such as vermicular movements of the fingers or of the feet Fig.
We recorded NPD episodes, lasting from 25 to 98 s mean 33 s. There was a mean of three NPD episodes per polysomnography recording. In nine patients, attacks displayed a violent ballistic pattern with flailing of the limbs Fig. Compared with PA, these episodes were characterized by prolonged tonic or dystonic postures sometimes with superimposed clonic jerks of the involved limbs. Intraindividual stereotypy was remarkable in all types of attack and in all patients.
In fact, attacks, whether PA, NPD or ENW, were remarkable for their conserved temporal and motor pattern which remained constant from one attack to another. Such a high degree of phenotypic stereotypy was confirmed by us in those patients who underwent several polysomnography recordings: in one case of PA, up to 52 identical episodes were recorded, and 11 NPD and 14 ENW episodes in other individuals. Whenever possible, patients were interviewed during the seizures. Twenty-seven patients were interviewed during PA episodes. Patients were in contact immediately at the end of the episode and 12 out of the 27 also during the PA episode: when interviewed, the patient answered promptly and relevantly, and in some cases reported an undefined sensation such as falling, sudden arousal, imminent death.
During the ENW, all of the four patients interviewed were not in contact, but they correctly answered questions immediately at the end of the seizure. In 12 patients, a typical NPD seizure developed into a full-blown secondarily generalized seizure. All of these patients had reduced or withdrawn antiepileptic drugs before polysomnography.
In all patients, the periodic movements mimicking those observed during the seizure involved the legs the feet in 15, the legs in five , whereas only five displayed movements of the arms. Only three percent of seizures occurred during REM sleep.
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Aspecific focal EEG theta activity was recorded in 12 patients. Right and left hemispheres were affected equally. In nine of these 45 cases, the focal abnormalities were detected only on sphenoidal leads. Four patients showed focal slow activity. In such cases, EEG was often masked by muscular artefacts or characterized by an abrupt transition to wake activity or light sleep, often preceded by a K-complex. The first EEG modifications were either a diffuse 14 cases or focal five cases flattening of background activity, or focal theta activity 15 cases , or a rhythmic delta activity six cases.
In eight patients only, ictal EEG was characterized by spikes and spike-and-wave activity and in eight cases by a small amplitude fast activity. In 21 cases, the abnormalities affected the right-sided regions, in 21 those on the left. Autonomic modifications were remarkable in most of the cases: tachycardia 88 cases , sustained tachypnoea 40 cases and irregular respiratory rhythm 37 cases appeared synchronously with seizure onset or preceding the movement artefact.
We also recorded the photoplethysmogram in 13 patients: polysomnography recordings displayed abrupt flattening of the photoplethysmogram at the beginning of the seizure, sometimes preceding the motor attack and the other autonomic modifications Fig. The videopolysomnographic data of the different type of seizures are summarized in Tables 1 and 2.
Based on our earlier experience Lugaresi and Cirignotta, ; Lugaresi et al. Only one patient had to stop carbamazepine because of somnolence and ataxia.
In five cases, carbamazepine was withdrawn because it was ineffective, and phenytoin in two or clobazam in three, in one case associated with valproic acid were instituted, without any significant efficacy. One patient did not receive carbamazepine but underwent neurosurgery for a vascular right frontal malformation, with total disappearance of the seizures. Only two patients were not put on carbamazepine. One had received clonazepam and another phenytoin, phenobarbital and valproate in other institutions. Seventeen patients refused any drug because their seizures were not disturbing, or because they had a spontaneous reduction of seizure frequency two cases.
Of the 30 patients investigated, nine sporadic and 21 with positive family history, all tested negative for the CHRNA4 mutations. No patient had ENW alone. In all the patients with at least two seizure types, the attack onset was characterized by the same stereotypic movement. Within the PA group, all of the patients had a normal personal history and normal neurological and neuroradiological examinations.
None of these patients had seizures during the daytime or during wakefulness, or secondarily generalized seizures. Thirteen percent of patients presented seizures also during the daytime.
The clinical data for these different groups are reported in Tables 3 and 4. However, the wide range 1—64 years of age certainly reflects a degree of heterogeneity in the causative mechanisms. We also found a high male prevalence 7 : 3 , a finding not universal for all of the partial epilepsies, and typically encountered instead in some parasomnias such as the REM sleep behaviour disorders, in which the male predominance is even higher 9 : 1. However, detailed pedigree analysis of our cases demonstrated clear genetic, probably autosomal dominant, transmission in relatively few patients only eight cases belonging to six families , a surprisingly low rate compared with that reported by others Oldani et al.
This discrepancy reflects the restrictive criteria we adopted in our study we required at least two generations affected, and not just one affected relative compared with others, and probably also some variation in the selection of the clinical material. We acknowledge, however, that our criteria could be unnecessarily restrictive, especially in the light of the recent demonstration of a dominantly inherited epilepsy with variable foci and, therefore, variable clinical manifestations Scheffer et al.
Even within the cases which conformed to an autosomal model of genetic transmission ADNFLE , we confirmed the genetic heterogeneity of the syndrome, since none of our patients tested positive for the mutations found in previously described families with ADNFLE. These findings can be explained in several ways: they could reflect an ascertainment bias, since we placed special emphasis on the family and personal history of sleep disturbances, and the lack of a comparable normal population in our study; or instead they could be due to errors in the diagnosis of the attacks, which were mistaken for parasomnias and instead were true epileptic seizures.
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In this second case, the familial recurrence in our material would be much higher and would compare well with that reported by other authors Oldani et al. We feel uneasy, however, in attributing all of the paroxysmal events, whether present in the familial or in the personal history of our patients, to epilepsy. We interpreted the anamnestic episodes as parasomnias because of their age-dependent course, rarity of episodes and their being not violent and often not disturbing for the patient.
The episodes fulfilled the diagnostic criteria put forth for parasomnias International Classification of Sleep Disorders, and, moreover, they often ended well before the onset of the clear-cut epileptic seizures. Relatives often could clearly distinguish between the two different types of attack. However, we have no direct proof, since these earlier episodes usually were not monitored electrophysiologically and, therefore, we cannot exclude that they too represented some kind of epileptic seizures in infancy or childhood, with somewhat differing clinical patterns. A third explanation is that they were true parasomniac episodes, and this entails the possibility that NFLE and arousal disorders share some common pathogenic mechanism, present in both the acquired and the genetic cases.
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Such an intriguing possibility was not excluded even in the works which established the genetic linkage and mutations in families with ADNFLE Scheffer et al. The peculiar functional organization of the frontal lobes and the well documented involvement of some mesial orbital frontal areas in sleep regulation Sterman and Wyrwicka, are not against such a possibility. Personal antecedents typically found in the focal lesional epilepsies such as birth anoxia, febrile convulsions, neuropsychological defects indicating encephalopathy, etc.
VideoEEG recordings demonstrated that NFLE comprises a spectrum of distinct phenomena, different in intensity but representing a continuum of the same epileptic condition, since seizures of different intensity could co-exist in the same patient. Moreover, when PA, NPD and ENW were present in the same patient, the semiology at the start of the attack was similar, and brief seizure fragments represented the beginning of the more prolonged episodes.
This feature Sforza et al. This takes us to the reason for distinguishing between PA, NPD and ENW as different clinical aspects of one heterogeneous syndrome, or for just lumping all these differing manifestations together in one broad nosological definition, as other authors have done Oldani et al. We believe that detailed dissection of the features of each patient and group of patients with NFLE, e. Our results show how the different groups of patients seem to have different causations and prognosis and, therefore, we advocate maintaining this semeiologic distinction.
PA are underestimated on clinical grounds alone, since they are reported by patients only if especially frequent and violent. In some patients with PA only, daytime tiredness, fatigue and sleepiness were not rare and were often the main complaint, which seemed to correlate with the frequency of the PA. This has been noted before, daytime somnolence being the only symptom reported by patients with paroxysmal awakenings Peled and Lavie, The most usual motor pattern of the PA was a more or less sudden jerk of the upper limbs and trunk.
The stereotypic repetition of the same pattern, sometimes tens of times for long stretches during sleep, and the presence of choreo-athetoid and dystonic postures of the limbs help to differentiate the PA from the physiological hypnic jerks or sleep starts upon purely visual inspection alone, even when EEG epileptic activity is not recorded. NPD attacks also begin with a paroxysmal arousal, but go on to display the pattern typical of a seizure originating in the supplementary motor areas, with extension of the ipsilateral limb, flexion of the contralateral limbs and rotation of the head; other attacks instead display rhythmic or ballistic movements, or the rocking pelvic and body movements of the mesial frontal orbital seizures.
ENW remain more difficult to record because of their lower frequency. The striking feature of ENW, and one which has been described since their first description Pedley and Guilleminault, ; Plazzi et al. Only in some of our cases did sphenoidal EEG electrodes prove useful, as shown by the fact that 13 patients had ictal epileptic EEG activity detected only by these electrodes.
The diagnosis of NFLE may thus represent a diagnostic dilemma. A diagnosis of parasomnia—pavor nocturnus or somnambulism—is often made prior to or even after the videopolysomnography recordings, especially in children Pedley and Guilleminault,; Maselli et al. The differential features of these parasomnias with NFLE, shown in Table 5 , include the different time course of the parasomnias, somnambulism rarely occurring for the first time in adulthood Hublin et al.
The mean lifetime duration of somnambulism is 7 years Sours et al. Seizure frequency and semiology are also important clinical criteria to distinguish epilepsy from disorders of arousal. Though the latter can show up in clusters, they are mainly sparse, the mean frequency of episodes being once every 1—4 months Sours et al. In fact, it is extremely unusual for pavor nocturnus or somnambulism to reach such a high frequency as the 2—3 episodes per night we recorded as a mean in our patients.