Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
The International League Against Epilepsy (ILAE) define an epileptic seizure as "a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain." Epileptic seizures can occur in someone who does not have epilepsy – as a consequence of head injury, drug overdose, toxins, eclampsia or febrile convulsions, for example.
Medically, when used on its own, the term seizure implies an epileptic seizure. The lay use of this word can also include sudden attacks of illness, loss of control, spasm or stroke. Where the physician is uncertain as to the diagnosis, the medical term paroxysmal event and the lay terms spells, funny turns or attacks may be used.
Possible causes include:
- Syncope (fainting)
- Reflex anoxic seizures
- Breath-holding spells of childhood
- Hypoglycaemia
- Cataplexy
- Hyperekplexia, also called startle syndrome
- Migraine
- Narcolepsy
- Non-epileptic myoclonus
- Opsoclonus
- Parasomnias, including night terrors
- Paroxysmal kinesigenic dyskinesia
- Repetitive or ritualistic behaviours
- Tics
- AADC Deficiency
Seizures are purely occipital and primarily manifest with elementary visual hallucinations, blindness or both.
They are usually frequent and diurnal, develop rapidly within seconds and are brief, lasting from a few seconds to 1–3 min, and, rarely, longer.
Elementary visual hallucinations are the most common and characteristic ictal symptoms, and are most likely to be the first and often the only clinical manifestation. They consist mainly of small multicoloured circular patterns that often appear in the periphery of a visual field, becoming larger and multiplying during the course of the seizure, frequently moving horizontally towards the other side.
Other occipital symptoms, such as sensory illusions of ocular movements and ocular pain, tonic deviation of the eyes, eyelid fluttering or repetitive eye closures, may occur at the onset of the seizures or appear after the elementary visual hallucinations. "Deviation of the eyes", often associated with ipsilateral turning of the head, is the most common (in about 70% of cases) nonvisual ictal symptom. It is often associated with ipsilateral turning of the head and usually starts after visual hallucinations, although it may also occur while the hallucinations still persist. It may be mild, but more often it is severe and progresses to hemiconvulsions and secondarily generalised tonic clonic seizures (GTCS). Some children may have seizures of eye deviation from the start without visual hallucinations.
"Forced eyelid closure and eyelid blinking" occur in about 10% of patients, usually at a stage at which consciousness is impaired. They signal an impending secondarily GTCS.
"Ictal blindness", appearing from the start or, less commonly, after other manifestations of occipital seizures, usually lasts for 3–5 min. It can occur alone and be the only ictal event in patients who could, at other times, have visual hallucinations without blindness.
Complex visual hallucinations, visual illusions and other symptoms resulting from more anterior ictal spreading rarely occur from the start. They may terminate in hemiconvulsions or generalised convulsions.
Ictal headache, or mainly orbital pain, may occur and often precedes visual or other ictal occipital symptoms in a small number of patients.
Consciousness is not impaired during the visual symptoms (simple focal seizures), but may be disturbed or lost in the course of the seizure, usually before eye deviation or convulsions.
Occipital seizures of ICOE-G may rarely progress to extra-occipital manifestations, such as hemiparaesthesia. Spread to produce symptoms of temporal lobe involvement is exceptional and may indicate a symptomatic cause.
Post-ictal headache, mainly diffuse, but also severe, unilateral and pulsating, or indistinguishable from migraine headache, occurs in half the patients, in 10% of whom it may be associated with nausea and vomiting.
Circadian distribution: Visual seizures are predominantly diurnal and can occur at any time of the day. Longer seizures, with or without hemi or generalised convulsions, tend to occur either during sleep, causing the patient to wake up, or after awakening. Thus, some children may have numerous diurnal visual seizures and only a few seizures that are exclusively nocturnal or occur on awakening.
Frequency of seizures: If untreated, patients experience frequent and brief visual seizures (often several every day or weekly). However, propagation to other seizure manifestations, such as focal or generalised convulsions, is much less frequent.
Generalized seizures can be either absence seizures, myoclonic seizures, clonic seizures, tonic-clonic seizures or atonic seizures.
Generalized seizures occur in various seizure syndromes, including myoclonic epilepsy, familial neonatal convulsions, childhood absence epilepsy, absence epilepsy, infantile spasms (West's syndrome), Juvenile Myoclonic Epilepsy and Lennox-Gastaut syndrome.
Panayiotopoulos syndrome occurs exclusively in otherwise normal children and manifests mainly with infrequent autonomic epileptic seizures and autonomic status epilepticus. Onset of seizures is from age 1 to 14 years with 76% starting between 3–6 years. Autonomic seizures consist of episodes of disturbed autonomic function with nausea, retching and vomiting as predominant symptoms. Other autonomic manifestations include pallor (or, less often, flushing or cyanosis), mydriasis (or, less often, miosis), cardiorespiratory and thermoregulatory alterations, incontinence of urine and/or feces, hypersalivation, and modifications of intestinal motility. In approximately one fifth of the seizures the child becomes unresponsive and flaccid (syncope-like epileptic seizures or ictal syncope) before or often without convulsions. Syncope-like epileptic seizures (ictal syncope) with the child becoming "completely unresponsive and flaccid like a rag doll" occur in one fifth of the seizures. More-conventional seizure symptoms often appear after the onset of autonomic manifestations. The child, who was initially fully conscious, becomes confused and unresponsive. Eyes turn to one side or gaze widely open. Only half of the seizures end with brief hemiconvulsions or generalized convulsions. Autonomic symptoms may be the only features of the seizures. None of the above symptoms alone is a prerequisite for diagnosis. Recurrent seizures may not be stereotyped. The same child may have brief or prolonged seizures and autonomic manifestations may be severe or inconspicuous. The full emetic triad (nausea, retching, vomiting) culminates in vomiting in 74% of the seizures; in others only nausea or retching occur, and in a few, none of the emetic symptoms are apparent.
Most of the seizures are prolonged and half of them last more than 30 minutes thus constituting autonomic status epilepticus, which is the more common nonconvulsive status epilepticus in normal children. Characteristically, even after the most severe seizures and autonomic status epilepticus, the child is normal after a few hours of sleep, which is both diagnostic and reassuring. However, it has been recently reported that sometime after status epilepticus in children with Panayiotopoulos syndrome a. growth of the frontal and prefrontal lobes is slightly decreased and b.the scores on the neuropsychological tests is decreased.
Focal onset hemiconvulsions or generalised convulsions occur in nearly half of the seizures. These are usually shorter than the preceding autonomic manifestations but in a few cases a. they may be prolonged constituting convulsive status epilepticus or b. the preceding autonomic manifestations are brief and not apparent
Seizures can occur at any time but they are more common during sleep.
During generalized febrile seizures, the body will become stiff and the arms and legs will begin twitching. The child loses consciousness, although their eyes remain open. Breathing can be irregular. They may become incontinent (wet or soil themselves); they may also vomit or have increased secretions (foam at the mouth). The seizure normally lasts for less than five minutes. The child's temperature is usually greater than .
Panayiotopoulos syndrome is now the formally approved nomenclature for this syndrome in the new International League against Epilepsy report on classification, which abandoned a number of previously used descriptive terms such as early onset benign childhood epilepsy with occipital paroxysms, early onset benign childhood occipital epilepsy, nocturnal childhood occipital epilepsy. The reason for this is that these descriptive terms were criticized as incorrect because in Panayiotopoulos syndrome:
“An autonomic seizure is an epileptic seizure characterized by altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset. The altered autonomic function may be objective or subjective or both.”
“Autonomic status epilepticus is an autonomic seizure which lasts for more than 30 minutes, or a series of such seizures over a 30 minute period without full recovery between seizures.”
The inter-ictal EEG shows occipital paroxysms, often demonstrating fixation-off sensitivity. However, some patients may only have random occipital spikes, whereas others may have occipital spikes only in the sleep EEG, and a few may have a consistently normal EEG. Photoparoxysmal abnormalities occur in patients whose seizures are triggered by lights.
Ictal EEG, preceded by regression of occipital paroxysms, is characterised by the sudden appearance of an occipital discharge that consists of fast rhythms, fast spikes or both. Ictal EEG during blindness show pseudo-periodic slow waves and spikes, which differ from those seen in ictal visual hallucinations. There are usually no postictal abnormalities.
The most common symptom of abdominal epilepsy is abdominal pain followed by uncontrollable vomiting, usually preceded by lethargy. Symptoms also include generalized tonic-clonic seizures followed by sleep, confusion, and unresponsiveness.
People with PSE experience epileptiform seizures upon exposure to certain visual stimuli. The exact nature of the stimulus or stimuli that triggers the seizures varies from one patient to another, as does the nature and severity of the resulting seizures (ranging from brief absence seizures to full tonic–clonic seizures). Many PSE patients experience an “aura” or feel odd sensations before the seizure occurs, and this can serve as a warning to a patient to move away from the trigger stimulus.
The visual trigger for a seizure is generally cyclic, forming a regular pattern in time or space. Flashing lights or rapidly changing or alternating images (as in clubs, around emergency vehicles, in action movies or television programs, etc.) are examples of patterns in time that can trigger seizures, and these are the most common triggers. Static spatial patterns such as stripes and squares may trigger seizures as well, even if they do not move. In some cases, the trigger must be both spatially and temporally cyclic, such as a certain moving pattern of bars.
Also note: Some people may also find overhead fans spinning to be of the same nature.
Several characteristics are common in the trigger stimuli of many PSE patients. The patterns are usually high in luminance contrast (bright flashes of light alternating with darkness, or white bars against a black background). Contrasts in colour alone (without changes in luminance) are rarely triggers for PSE. Some patients are more affected by patterns of certain colours than by patterns of other colours. The exact spacing of a pattern in time or space is important and varies from one individual to another: a patient may readily experience seizures when exposed to lights that flash seven times per second, but may be unaffected by lights that flash twice per second or twenty times per second. Stimuli that fill the entire visual field are more likely to cause seizures than those that appear in only a portion of the visual field. Stimuli perceived with both eyes are usually much more likely to cause seizures than stimuli seen with one eye only (which is why covering one eye may allow patients to avoid seizures when presented with visual challenges). Some patients are more sensitive with their eyes closed; others are more sensitive with their eyes open.
Sensitivity is increased by alcohol consumption, sleep deprivation, illness, and other forms of stress.
Generalized epilepsy, also known as primary generalized epilepsy or idiopathic epilepsy, is a form of epilepsy characterised by generalised seizures with no apparent cause. Generalized seizures, as opposed to focal seizures, are a type of seizure that impairs consciousness and distorts the electrical activity of the whole or a larger portion of the brain (which can be seen, for example, on electroencephalography, EEG).
Generalized epilepsy is "primary" because the epilepsy is the originally diagnosed condition itself, as opposed to "secondary" epilepsy, which occurs as a symptom of a diagnosed condition.
General symptoms of migralepsy are:
- Flashes of light
- Geometric or animate forms
- Visual hallucinations
- Vomiting
- Headache
- Blindness
- Loss of consciousness
- Convulsions
A febrile seizure, also known as a fever fit or febrile convulsion, is a seizure associated with a high body temperature but without any serious underlying health issue. They most commonly occur in children between the ages of 6 months and 5 years. Most seizures are less than five minutes in duration and the child is completely back to normal within sixty minutes of the event.
Febrile seizures may run in families. The diagnosis involves verifying that there is not an infection of the brain, there are no metabolic problems, and there have not been prior seizures that have occurred without a fever. There are two types of febrile seizures: simple febrile seizures and complex febrile seizures. Simple febrile seizures involve an otherwise healthy child who has at most one tonic-clonic seizure lasting less than 15 minutes in a 24-hour period. Blood testing, imaging of the brain or an electroencephalogram (EEG) is typically not needed for the diagnosis. Examination to determine the source of the fever is recommended. In otherwise healthy looking children a lumbar puncture is not necessarily required.
Neither anti-seizure medication nor anti-fever medication are recommended in an effort to prevent further simple febrile seizures. In the few cases that last greater than five minutes a benzodiazepine such as lorazepam or midazolam may be used. Outcomes are generally excellent with similar academic achievements to other children and no change in the risk of death for those with simple seizures. There is tentative evidence that children have a slight increased risk of epilepsy at 2%. Febrile seizures affect two to five percent of children before the age of five. They are more common in boys than girls. After a single febrile seizure there is a 15 to 70% chance of another one.
Diagnosis may be made by noting the correlation between exposure to specific visual stimuli and seizure activity. More precise investigation can be carried out by combining an EEG with a device producing "Intermittent Photic Stimulation" (IPS). The IPS device produces specific types of stimuli that can be controlled and adjusted with precision. The testing physician adjusts the IPS device and looks for characteristic anomalies in the EEG, such as photoparoxysmal response (PPR), that are consistent with PSE and/or may herald the onset of seizure activity. The testing is halted before a seizure actually occurs.
Sometimes diagnostic indicators consistent with PSE can be found through provocative testing with IPS, and yet no seizures may ever occur in real-life situations. Many people will show PSE-like abnormalities in brain activity with sufficiently aggressive stimulation, but they never experience seizures and are not considered to have PSE.
Ictal refers to a physiologic state or event such as a seizure, stroke, or headache. The word originates from the Latin "ictus", meaning a blow or a stroke. In electroencephalography (EEG), the recording during a seizure is said to be "ictal". The following definitions refer to the temporal relation with seizures.
Pre-ictal refers to the state immediately before the actual seizure, stroke, or headache, though it has recently come to light that some characteristics of this stage (such as visual auras) are actually the beginnings of the ictal state.
Post-ictal refers to the state shortly after the event.
Interictal refers to the period between seizures, or convulsions, that are characteristic of an epilepsy disorder. For most people with epilepsy, the interictal state corresponds to more than 99% of their life. The interictal period is often used by neurologists when diagnosing epilepsy since an EEG trace will often show small interictal spiking and other abnormalities known by neurologists as subclinical seizures. Interictal EEG discharges are those abnormal waveforms not associated with seizure symptoms.
Peri-ictal encompasses pre-ictal, ictal and post-ictal.
The connection between migraines and epileptic seizures is currently being researched and not much is known. Patients have been shown to have had migraines long before developing epileptic symptoms, creating the possibility of severe cases of migraines creating epilepsy. However, not every migraine may be accompanied by a seizure and sometimes the seizures happen without any migraine involvement. Due to this, finding the origin of migralepsy is difficult and enveloped somewhere in the overlap between both conditions. Some patients have shown that their relatives suffered from migraines as well and even some from migralepsy, forming the possibility that migralepsy is genetic in origin and forms only rarely as both, generally resulting in only one condition or the other.
It is unknown as to what causes abdominal epilepsy. While a causal relationship between seizure activity and the GI symptoms has not been proven, the GI symptoms cannot be explained by other pathophysiological mechanisms, and are seen to improve upon anticonvulsant treatment. Because the condition is so rare, no high-quality studies exist. There have been too few reported cases to identify risk factors, genetic factors, or other potential causes.
Benign familial infantile epilepsy (BFIE), also known as benign familial infantile seizures (BFIS) or benign familial infantile convulsions (BFIC) is an epilepsy syndrome. Affected children, who have no other health or developmental problems, develop seizures during infancy. These seizures have focal origin within the brain but may then spread to become generalised seizures. The seizures may occur several times a day, often grouped in clusters over one to three days followed by a gap of one to three months. Treatment with anticonvulsant drugs is not necessary but they are often prescribed and are effective at controlling the seizures. This form of epilepsy resolves after one or two years, and appears to be completely benign. The EEG of these children, between seizures, is normal. The brain appears normal on MRI scan.
A family history of epilepsy in infancy distinguishes this syndrome from the non-familial classification (see benign infantile epilepsy), though the latter may be simply sporadic cases of the same genetic mutations. The condition is inherited with an autosomal dominant transmission. There are several genes responsible for this syndrome, on chromosomes 2, 16 and 19. It is generally described as idiopathic, meaning that no other neurological condition is associated with it or causes it. However, there are some forms that are linked to neurological conditions. One variant known as infantile convulsions and choreoathetosis (ICCA) forms an association between BFIE and paroxysmal kinesigenic choreoathetosis and has been linked to the PRRT2 gene on chromosome 16. An association with some forms of familial hemiplegic migraine (FHM) has also been found. Benign familial infantile epilepsy is not genetically related to benign familial neonatal epilepsy (BFNE), which occurs in neonates. However, a variation with seizure onset between two days and seven months called "benign familial neonatal–infantile seizures" (BFNIS) has been described, which is due to a mutation in the SCN2A gene.
During puberty, seizure frequency increases to one to two times per week. Mental function has a rapid decline, as observed by a lack of coordination, failure to complete education, and fine motor activities. In rare cases, some suffered from loss of vision.
The epileptic seizures which can be observed in infants with West syndrome fall into three categories, collectively known as infantile spasms. Typically, the following triad of attack types appears; while the three types usually appear simultaneously, they also can occur independently of each other:
- "Lightning attacks": Sudden, severe myoclonic convulsions of the entire body or several parts of the body in split seconds, and the legs in particular are bent (flexor muscle convulsions here are generally more severe than extensor ones).
- "Nodding attacks": Convulsions of the throat and neck flexor muscles, during which the chin is fitfully jerked towards the breast or the head is drawn inward.
- "Salaam or jackknife attacks": a flexor spasm with rapid bending of the head and torso forward and simultaneous raising and bending of the arms while partially drawing the hands together in front of the chest and/or flailing. If one imagined this act in slow motion, it would appear similar to the Muslim ceremonial greeting (Salaam), from which this type of attack derives its name.
Northern Epilepsy Syndrome causes recurrent seizures between the ages of five to ten. These seizures, that may last up to 15 minutes, can be classified mostly as tonic-clonic, but partial seizures could also occur. The seizures commonly involve muscle rigidity, convulsions, and loss of consciousness. Generally, the recurrence is one to two times per month.
In the years following the onset of seizures, a noticeable decrease in intellectual capacity is observed.
The only sign of BFNE are seizures, generally tonic-clonic, which occur within the first week of life. Seizures often begin as apnea, cyanosis, and hypertonia and last less than 1 minute.
People with BFNE are not more likely to develop epileptic seizures later in life.
Characteristics of paroxysmal sympathetic hyperactivity include:
- fever
- tachycardia
- hypertension
- tachypnea
- hyperhidrosis or diaphoresis
- dystonic posturing
- pupillary dilation
- flushing
In cases where PSH episodes develop post-injury, specifically traumatic brain injury, symptoms typically develop quickly, usually within a week. Symptom onset has been seen to average 5.9 days post-injury. Episodes vary in duration and occurrence. Episodes can last as little as a few minutes or as long as ten hours, and they can occur multiple times a day. Episode duration has been seen to average 30.8 minutes and occur five to six times a day. Episodes can occur naturally or arise from external triggers. Common triggers include pain or stimulation, body turning or movements, and bladder distention. Bladder distention has been observed in patients being treated in intensive care units with the concurrent use of catheters. Symptoms of PSH can last from weeks to years following initial onset. As episodes persist over time, they have been found to become less frequent in occurrence but last for prolonged periods.
Epileptic spasms, also known as infantile spasms, juvenile spasms, or West syndrome is an uncommon-to-rare epileptic disorder in infants, children and adults. It is named after the English physician, William James West (1793–1848), who first described it in an article published in The Lancet in 1841. The original case actually described his own son, James Edwin West (1840–1860). Other names for it are "generalized flexion epilepsy", "infantile epileptic encephalopathy", "infantile myoclonic encephalopathy", "jackknife convulsions", "massive myoclonia" and "Salaam spasms". The term "infantile spasms" can be used to describe the specific seizure manifestation in the syndrome, but is also used as a synonym for the syndrome itself. West syndrome in modern usage is the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia), and developmental regression – although the international definition requires only two out of these three elements.
The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal.
Paroxysmal sympathetic hyperactivity (PSH) is a syndrome that causes episodes of increased activity of the sympathetic nervous system. Hyperactivity of the sympathetic nervous system can manifest as increased heart rate, increased respiration, increased blood pressure, diaphoresis, and hyperthermia.
Previously, this syndrome has been identified as general dysautonomia but now is considered a specific form of it. It has also been referred to as paroxysmal sympathetic instability with dystonia, or PAID, and sympathetic storm. Recently, however, studies have adopted the name paroxysmal sympathetic hyperactivity to ensure specificity. PSH is observed more in younger patients than older ones. It is also seen more commonly in men than women. There is no known reason why this is the case, although it is suspected pathophysiological links may exist. In patients surviving traumatic brain injury, the occurrence of these episodes is one in every three. PSH can also be associated with severe anoxia, subarachnoid and intracerebral hemorrhage, and hydrocephalus.