Temporal lobe epilepsy is considered to be one of the most common types of epilepsy. Structures that constitute the mesial temporal lobe, primarily hippocampus, are hypothesized to be the origin of excitatory signals. Cognition, memory and functional ability are all impaired as a result of seizure activity. Imaging studies and EEG confirm the diagnosis, whereas treatment is primarily surgical, as this form of epilepsy is resistant to antiepileptics.
Presentation
The clinical presentation of patients suffering from TLE starts with partial seizures and the appearance of an aura. Epigastric sensations, fear, deja vu or olfactory hallucinations are forms of aura that are encountered in these patients and are quite common [9]. In neonates and infants, however, these auras may be difficult to attain and recognize [9]. Seizures are characterized by arrest of motor behavior, blank stare, and automatisms [5]. At some point, symptoms that indicate memory loss, executive function, language deficits and various other impairments [11]. Both autobiographical and working memory loss has been observed [11], whereas significant disability may be seen as a result of slowed mental processing speed, mood and personality disorders, as well as extensive changes in emotional responses [5] [11]. Namely, failure to recognize fear, sadness and disgust have been observed, which may be used as indicative signs of temporal lobe involvement [12]. Interictal (the period between seizures) depression is diagnosed in up to one third of patients [5], suggesting that this condition carries a significant psychological burden.
Entire Body System
- Hypersomnia
Subjective and objective hypersomnia highly prevalent in adults with epilepsy. Epilepsy Behav. 106:107023. 10.1016/j.yebeh.2020.107023 [PubMed] [CrossRef] [Google Scholar] Gupta A., Shukla G., Srivastava A. K., Singh M. [ncbi.nlm.nih.gov]
Gastrointestinal
- Nausea
They may experience strange tastes or smells, butterflies in the stomach, nausea or get the feeling of déjà vu. They may also wander off or perform unusual movements such as plucking at clothes or chewing. [youngepilepsy.org.uk]
[…] movements (such as picking at clothing) Other sensory symptoms may include the following sensations that start in one area and spread: Numbness Tingling A feeling that the flesh is crawling Autonomic symptoms may include the following: Abdominal pain or nausea [webmd.com]
Some children may have auras, which are a type of focal onset aware seizure that can include: déjà vu (a feeling of already having been in the present situation) a smell, taste, sound, or vision an emotion (such as fear) nausea or a rising sensation in [kidshealth.org]
Temporal lobe seizures are often accompanied by strange sensations, sweating, flushing, vertigo, epigastric sensations, taste, and hearing of sounds or melodies, nausea, hallucinations or feelings of déjà vu. [epilepsyqueensland.com.au]
- Vomiting
We reviewed other publications of peri-ictal autonomic symptoms considered to have a lateralizing significance, such as peri-ictal vomiting, urinary urge, ictal pilo-erection. [ncbi.nlm.nih.gov]
Ictal speech, spitting, vomiting, drinking, urge to urinate and automatisms with preserved consciousness suggest seizure onset in the non-dominant temporal lobe. Postictal speech disturbance suggests a dominant temporal lobe seizure. [epilepsydiagnosis.org]
[…] perceptual phenomena, as well as autonomic symptoms is possible in this epilepsy syndrome: déjà vu, jamais vu, “stranger behind the back”, feeling of detachment from oneself, sensation of enlightenment, extreme pleasure, fear and dread, anxiety, nausea, vomiting [doctorstrizhak.com]
Episodes of illness were defined by the presence of fever, rhinorrhea, cough, vomiting, diarrhea, or rash. [dx.doi.org]
- Gagging
Some subjects also presented with gagging, retching, rhythmic groaning, head nodding and bilateral tonic extension of the limbs. [scielo.br]
Cardiovascular
- Tachycardia
It may also be administered, with care, to persons with regular wide complex tachycardias if it is likely that the tachycardia is not a ventricular tachycardia. [ecgwaves.com]
Let us now learn about the three forms of atrial tachycardia: Atrial tachycardia with block Multifocal atrial tachycardia Paroxysmal atrial tachycardia 30. [slideshare.net]
AF, atrial fibrillation; AFL, atrial flutter; AT, atrial tachycardia; AV, atrioventricular; AVNRT, atrioventricular nodal reentrant tachycardia; AVRT, atrioventricular reentrant tachycardia; EAT, ectopic atrial tachycardia; MAT, multifocal atrial tachycardia [unboundmedicine.com]
Tachycardia refers to a fast resting heart rate, usually over 100 beats per minute. Tachycardia can be dangerous, depending on its underlying cause and on how hard the heart has to work. [medicalnewstoday.com]
Musculoskeletal
- Muscle Spasm
She complained of feeling ‘funny’ before its onset, and then confusion set in with mild muscle spasms. These spasms lasted for about three to five minutes and she had several, with about five to ten minute intervals between. [netdoctor.co.uk]
This seizure disorder can cause a variety of symptoms such as strange sensations, changes in behavior or emotions, muscle spasms, or convulsions. [neurosurgeonsofnewjersey.com]
Skin
- Sweating
Temporal lobe seizures are often accompanied by strange sensations, sweating, flushing, vertigo, epigastric sensations, taste, and hearing of sounds or melodies, nausea, hallucinations or feelings of déjà vu. [epilepsyqueensland.com.au]
[…] such as picking at clothing) Other sensory symptoms may include the following sensations that start in one area and spread: Numbness Tingling A feeling that the flesh is crawling Autonomic symptoms may include the following: Abdominal pain or nausea Sweating [webmd.com]
Autonomic phenomena: changes in heart rate and sweating. Patients may experience an epigastric fullness sensation or nausea. [patient.info]
Psychiatrical
- Anger
</p> <h2>Key points</h2> <ul><li>Temporal lobe epilepsy involves seizures that can produce fear, joy or anger or a sense of déjà vu or jamais vu. [aboutkidshealth.ca]
They can also be characterised by irritating senses of smell or taste, autonomic symptoms (flushing, sweating, changes of heart rate), emotional changes (rising fear or anxiety, anger, depression, euphoria) or memory symptoms (déjà vu or jamais vu – a [epilepsyqueensland.com.au]
Other symptoms may include visual agnosia (inability to visually recognize objects), loss of normal fear and anger responses, memory loss, distractibility, seizures, and dementia. [web.archive.org]
Signs and symptoms of focal aware seizures include: Déjà vu (a feeling of familiarity), a memory, or jamais vu (a feeling of unfamiliarity) Sudden sense of fear or anxiety, anger, sadness, joy A rising sick feeling in the stomach (the feeling you get [my.clevelandclinic.org]
[…] tachycardia), flushing, gastrorintestinal, pallor, piloerection, respiratory Cognitive/psychic – Déjà vu or jamais vu, dissociation, depersonalization or derealization, forced thinking, aphasia/dysphasia, memory Emotional/affective - agitation, aggression, anger [emedicine.medscape.com]
- Visual Hallucination
In a study published this week in Medical Humanities, the authors feel that temporal lobe epilepsy is a more likely explanation as it can produce complex visual hallucinations, which are usually brief, fragmentary, and stereotyped, just like those Chopin [medicalnewstoday.com]
These visual illusions differ from the visual hallucinations associated with occipital lobe seizure in that there is no formed visual image. Objects may appear smaller or larger than usual. [patient.info]
Less frequent are primitive visual hallucinations (figures, blobs of colors), speech arrest, vertigo, and smells. Seizures may be provoked by sleep. [doctorstrizhak.com]
hallucinations linked to other qualities of perception such as voices, emotions, or time Any form of: Auditory perceptual abnormality; Olfactory hallucinations; Gustatory hallucinations; Rotation or disequilibrium feelings linked to other perceptual [pni.org]
- Delusion
[…] symptomatology, and include: viscerosensory aura typically from lesions of the amygdala or insular cortex psychic (experienced) aura typically from limbic structures variable including dysmnesic phenomena, cognoscitive phenomena, emotional phenomena, delusions [radiopaedia.org]
Arzy and Schurr concluded that the man suffered from “grandiose religious delusion of revelation and missionary zeal in the context of post-ictal psychosis (PIP).” [timesofisrael.com]
At the time of discharge, 28 days after her admission, the patient’s delusions, irritability, and mood lability had resolved, but she continued to have hypergraphia and hyperreligiosity. Mrs. [consultant360.com]
She did not have a religious delusion. Case 3 The patient’s mother of this 44‐year‐old man was a devout Buddhist, who regularly prayed with him that his epilepsy would be cured. [dx.doi.org]
- Euphoria
Some symptoms of a temporal lobe seizure may be related to these functions, including having odd feelings — such as euphoria, deja vu or fear. During a temporal lobe seizure, you may remain aware of what's happening. [web.archive.org]
They can also be characterised by irritating senses of smell or taste, autonomic symptoms (flushing, sweating, changes of heart rate), emotional changes (rising fear or anxiety, anger, depression, euphoria) or memory symptoms (déjà vu or jamais vu – a [epilepsyqueensland.com.au]
Some symptoms of a temporal lobe seizure may be related to these functions, including having odd feelings — such as euphoria, deja vu or fear. Temporal lobe seizures are sometimes called focal seizures with impaired awareness. [mayoclinic.org]
Symptoms Causes Diagnosis Treatment Symptoms MTLE typically causes seizures that last 30 seconds to two minutes and may include: Before the seizure: An aura or far-away look Euphoria Déjà vu Rising sensation in the abdomen Sudden or strange odor or taste [texaschildrens.org]
- Depersonalization
[…] sensations, olfactory, somatosensory, vestibular, visual Autonomic – Heart rate Change (asytole, bradycardia, palpitations, tachycardia), flushing, gastrorintestinal, pallor, piloerection, respiratory Cognitive/psychic – Déjà vu or jamais vu, dissociation, depersonalization [emedicine.medscape.com]
He would appear to have been depersonalized, as it took three days for his body to be reunited with his soul. [dx.doi.org]
Neurologic
- Seizure
Homepage Rare diseases Search Search for a rare disease Familial mesial temporal lobe epilepsy with febrile seizures Disease definition A rare, genetic, familial partial epilepsy disease characterized by simple partial seizures, complex partial seizures [orpha.net]
There are a lot of older names for seizures that occur in TLE, including "psychomotor seizures," "limbic seizures," "temporal lobe seizures," "complex partial," and "simple partial." The modern name for these seizures is "focal onset seizures." [epilepsy.com]
The seizures of temporal-lobe epilepsy often start in childhood. Temporal lobe epilepsy is difficult to diagnose because temporal lobe seizures may not show up on an EEG. [medicinenet.com]
Seizures beginning in the occipital lobe are rare. [hopkinsmedicine.org]
[…] for absence seizures. [medical-dictionary.thefreedictionary.com]
- Aura
Auras are variable in symptomatology, and include: viscerosensory aura typically from lesions of the amygdala or insular cortex psychic (experienced) aura typically from limbic structures variable including dysmnesic phenomena, cognoscitive phenomena, [radiopaedia.org]
We reviewed at least three seizures from each patient and analyzed the following clinical data: presence of aura, duration of aura, ictal and post-ictal period, clinical semiology of aura, ictal and post-ictal period. [ncbi.nlm.nih.gov]
Presentation[1] Aura occurs in the majority of temporal lobe seizures. Most auras and automatisms last a very short period - seconds or 1 to 2 minutes. [patient.info]
- Amnesia
Defining the phase of memory processing most relevant to this form of amnesia, and the roles of physiological and structural pathology, requires further research. [ncbi.nlm.nih.gov]
- Jamais Vu
</p> <h2>Key points</h2> <ul><li>Temporal lobe epilepsy involves seizures that can produce fear, joy or anger or a sense of déjà vu or jamais vu. [aboutkidshealth.ca]
They can also be characterised by irritating senses of smell or taste, autonomic symptoms (flushing, sweating, changes of heart rate), emotional changes (rising fear or anxiety, anger, depression, euphoria) or memory symptoms (déjà vu or jamais vu – a [epilepsyqueensland.com.au]
Psychic phenomena: Feeling of déjà vu (familiarity) or jamais vu (unfamiliarity). Depersonalisation (ie feeling of detachment from oneself) or derealisation (surroundings appear unreal). Fear or anxiety. [patient.info]
Symptoms Hallucinations: hearing of sounds, vertigo, visual hallucinations and illusions, déjà vu, jamais vu Motor seizures: hand automatisms, grimacing, clonic movements in the face, arm posturing, vocalizations, whole body rotation, speech disturbance [doctorstrizhak.com]
- Deja Vu
There could be confusion, mumbling, feeling funny, gastric uprisings, anxiety, deja vu. A deja vu feeling is very common, stomach upsets. [youtube.com]
However, deja vu is a normal phenomenon occurring in 70 percent of the population and unless such phenomenological detail is obtained, patients' symptomatology may be misinterpreted. [pni.org]
Some symptoms of a temporal lobe seizure may be related to these functions, including having odd feelings — such as euphoria, deja vu or fear. Temporal lobe seizures are sometimes called focal seizures with impaired awareness. [mayoclinic.org]
vu/jamais vu, or emotional seizure with fear. [epilepsydiagnosis.org]
Some symptoms of a temporal lobe seizure may be related to these functions, including having odd feelings — such as euphoria, deja vu or fear. During a temporal lobe seizure, you may remain aware of what's happening. [web.archive.org]
Workup
The initial diagnosis of epilepsy can be made based on clinical criteria, whereas a confirmation of TLE can be made through the following methods [2] [13]:
- Imaging studies - MRI is considered as a very good first-line imaging procedure that identifies hippocampal atrophy with great specificity [2]. Additional studies include positron emission tomography (PET) and single photon emission computed tomography (SPECT), methods that are valuable in detecting extra-temporal sources of excitatory signals, especially in patients in whom MRI findings are inconclusive, or in those who already had surgery [3]. The role of SPECT is not as significant unless it is performed during an attack [3]. MRI spectroscopy has also been listed as one of the possible methods, due to its even higher specificity.
- EEG - Evaluation of brain signaling, in combination with imaging studies, is considered as the optimal diagnostic approach in patients suffering from epilepsy of any type. Both invasive and noninvasive EEG studies may be performed, but video EEG is recommended in all patients [5]. Invasive methods include intracranial depth electrodes and subdural grids (also known as iEEG), whereas epidural, scalp and foramen ovale electrodes are non-invasive forms of EEG [3]. In addition to evaluating brain signals at the location where imaging studies have determined the presumable focus, a thorough evaluation of the entire brain should be conducted, as bilateral changes may be observed, which may substantially complicate treatment.
EEG
- Epileptiform Activity
However, little is currently known about the modulation of these networks by epileptiform activity, such as interictal spikes and seizures. [ncbi.nlm.nih.gov]
- Temporal Intermittent Rhythmic Delta Activity
and subsequently to temporal intermittent rhythmic delta activity and temporal lobe-onset seizures. [ncbi.nlm.nih.gov]
Interictal EEG Features of TLE The interictal EEG in TLE is typically characterized by temporal spike or sharp-wave discharges and temporal intermittent rhythmic delta activity (TIRDA). [hindawi.com]
- Temporal Epileptiform Discharges
The electroencephalogram ( EEG ) of mesial TLE contains interictal features often associated with anterior temporal epileptiform discharges with a maximal voltage over the basal temporal electrodes. [journals.lww.com]
Treatment
Temporal lobe epilepsy is not responsive to pharmacotherapy, which shifts the focus of management to surgery. The goal is to completely excise and remove the zone in which the epileptic signal originates [3], and procedures such as anterior temporal lobectomy (ATL) or selective amygdalohippocampectomy (SAH) are indicated [10]. With successfully resected regions, many patients become seizure-free, but a small percentage is left with some form of disability, as neurosurgery carries a minor, but significant risk of brain injury. For resection to be complete and successful, a thorough preoperative assessment guided by EEG and imaging studies is detrimental [13]. In the case of bilateral hippocampal involvement, the risks and benefits of surgery should be discussed.
Prognosis
The prognosis of patients with successful surgical therapy is very good [10], and marked reduction or even disappearance of seizure activity can be achieved. The diagnosis of TLE, however, is often delayed for a considerable amount of time, which correlates with a poorer prognosis. TLE is practically nonresponsive to pharmacological therapy and precious time is often used on attempting to find the appropriate antiepileptic regimen. In fact, studies have identified that it takes up to 9 or even 10 years for surgical therapy to be instated [6], while some reports suggest that more than 20 years elapse prior to confirming TLE [6]. The importance of choosing the optimal surgical approach also has important prognostic factors in long-term management of patients, due to the fact that neurosurgical procedures are quite sensitive and may result in further damage. For these reasons, a careful and detailed pre-operative assessment through imaging modalities and EEG is necessary.
Etiology
Although the exact cause of epilepsy in general remains incompletely understood, the principal source of aberrant signaling in the case of TLE is the hippocampus [7], which undergoes sclerosis, atrophy and neuronal loss, with similar effects seen in the amygdala and parahippocampal gyri. As a result, diffuse pathological changes are observed in the morphology and structure [8]. TLE has shown to be strongly associated with several conditions [3] [9]:
- Cortical dysplasia, a congenital condition that is also a recognized cause of epilepsy, has shown to be present in up to 20% of patients suffering from TLE.
- Various brain tumors, including astrocytomas, gangliomas, oligodendrogliomas and neuroepithelial tumors have been diagnosed in up to 60% of patients undergoing brain surgery.
- Vascular changes, such as cavernous hemangiomas and arteriovenous malformation are also brought into connection with TLE, although the exact mechanism remains unknown.
- Several forms of brain trauma are considered as potential triggers of epilepsy. Namely, trauma that results in loss of consciousness for more than 30 minutes, causes penetrating injuries or linear skull fractures, but also scarring that is visible on MRI are able to provoke excitatory signaling from the temporal lobe [3].
In addition to these conditions, a hypothesis that some previous condition in early childhood triggered the pathological sequence has been suggested as well. Febrile seizures, birth injuries and status epilepticus have shown to be present in a substantial amount of patients who developed TLE, suggesting that other factors may also play a role in etiology [8].
Epidemiology
Global estimations suggest that the prevalence of all forms of epilepsy combined reach up to 1% of the population [6], with significant variations across the world. In developed countries, prevalence rates are approximately 1 per 1000 individuals, whereas developing countries have shown rates of up to 50 per 1000 individuals [1]. In general, epilepsy is most frequently encountered in young children and the elderly [1], but early childhood and adolescence is considered to be the principal age of onset [9]. TLE is the most common form of partial seizures, which comprise almost 60% of all cases of epilepsy, with estimations that of 1,200,000 Americans who suffer from epilepsy, 400,000 are affected by TLE [5]. Exact prevalence and incidence rates of TLE, however, are unknown at this moment [1]. In addition to diseases that are mentioned as potential causes of TLE, such as cortical dysplasia, tumors and vascular malformations [3], febrile seizures during infancy and birth injuries have also been identified as potential risk factors for later development of TLE [8].
Pathophysiology
The most common and most important pathological finding in patients with TLE is atrophy and sclerosis of the hippocampus [2], one of the constitutive parts of the mesial temporal lobe, together with the amygdala, uncus, parahippocampal and dentate gyri. Presumably, hippocampal sclerosis and atrophy are a result of neuronal loss that occurs in the temporal lobe and cause disruption of signaling, causing enhanced excitation and hypersynchronnous discharge of neurons in that area that eventually lead to epilepsy [7]. In addition, various pathophysiological processes have been described - loss of interneurons, impaired neurogenesis and mossy fiber sprouting (branching of additional neurons) [8]. The exact mechanism of epilepsy, however, is still not completely understood.
Prevention
Current prevention strategies do not exist, as the exact pathophysiological mechanism of TLE remains unknown. Given the fact that recent evidence about the involvement of inflammatory changes in seizures have been published, anti-inflammatory drugs, but also growth hormone secretagogues have been proposed as potentially preventing agents [8]. Further studies, however, need to be conducted in order to determine the exact role of inflammation and other pathophysiological events in the development of TLE.
Summary
Partial-onset seizures comprise almost 60% of all epilepsies and temporal lobe epilepsy (TLE) is the most common form of partial seizures worldwide [1]. Of approximately 1,200,000 people suffering from partial seizures in the United States, TLE is the cause in more than 400,000, indicating its importance in clinical practice [2]. Theories regarding its etiology are numerous, but various studies have established that in up to a third of patients, some preexisting condition is thought to be the trigger for abnormal excitatory signaling in the mesial temporal lobe [3] [4]. Tumors, cortical dysplasia, vascular malformations, head trauma and encephalomalacia have all been discovered in a significant amount of patients who developed TLS [3]. Hence, these conditions present as significant risk factors for its appearance, but the exact pathophysiological mechanisms remain to be fully disclosed. The principal pathological findings are hippocampal sclerosis, neuronal damage and atrophy, which leaves a detrimental effect on various processes, including memory, cognition and motor function [2]. Additional structures that constitute the mesial temporal lobe - the amygdala, uncus, dentate gyrus and parahippocampal gyrus, have all shown similar pathological degradation and are also considered as potential sources of epileptic signaling [2]. The clinical presentation somewhat varies across different age groups (partly due to a varying etiology between the pediatric, adolescent and adult population), but universally includes partial seizures and the presence of auras (olfactory, epigastric or gustatory sensations). Memory and attention impairments, as well as reduced speed of mental function, mood changes and emotional abnormalities appear at some point and can be severely debilitating [5]. The initial diagnosis of epilepsy is made based on clinical findings, but imaging and electroencephalographic (EEG) studies are necessary to identify the exact origin of aberrant signals. Magnetic resonance imaging (MRI) is an effective diagnostic method, whereas single photon emission computed tomography (SPECT) and positron emission tomography (PET) may be considered when MRI findings are inconclusive [3]. Both invasive and noninvasive EEG studies, including video EEG are equally necessary as imaging studies and the combination of findings is imperative in determining the exact site of epileptic signals [4]. Because TLE is resistant to antiepileptic drugs (AEDs) in virtually all cases, a surgical approach as early as possible is advocated to assure a good quality of life and reduce functional impairment [6]. With either complete resection or removal of the structures in the mesial temporal lobe, much better outcomes have been encountered [6], which is why a prompt definite diagnosis by using all available tools and surgery can significantly reduce the burden of this condition in daily life.
Patient Information
Epilepsy is considered to be a major issue in clinical practice, as estimates suggest that 1% of the world population suffers from any of the recognized forms. Temporal lobe epilepsy (TLE) one of the most common causes, with more than 400,000 people who live in the United States are presumed to suffer from TLE. This form of epilepsy is established to be one of the most severe, since it does not respond to antiepileptic drugs and requires other therapeutic modalities. The exact cause of TLE remains to be discovered, but in up to 30% of patients, an accompanying structural abnormality such as brain tumors or cortical dysplasia has been identified. Studies have observed loss of neuronal function and atrophy of the hippocampus, one of the most important parts of the brain when it comes to memory, whereas the amygdala (principally responsible for emotions such as fear) may be affected as well. TLE may appear at any age, but it's onset is primarily seen in young children. The first and most important symptom is seizures that involve only one brain hemisphere (partial seizures) and the arrest of motor activity with a blank stare during the onset of seizures. They may be followed or preceded by auras (strange sensations in the stomach, deja-vu or visual and auditory hallucinations), together with various neurological complaints. Patients with TLE may suffer from memory loss, inability to perform daily functions, language difficulties and severe cognitive disability. The diagnosis is made through the use of imaging studies such as magnetic resonance imaging (MRI) and positron emission tomography (PET scan), together with eletroencephalography (EEG). These methods are used to visualize and map the exact location where abnormal signaling occurs, which is necessary for treatment. In the case of TLE, surgery is the recommended therapeutic method. In general, removal of parts of the brain that are determined to be the source of epileptic signals is necessary and early surgery has shown drastic success in reducing or completely abolishing the appearance of seizures. Despite the advantages of surgery, the diagnosis is delayed in many patients due to attempts to handle seizures with antiepileptic drugs, which is why a detailed diagnostic workup is essential.
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