Vertiginous epilepsy

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Vertiginous epilepsy is infrequently the first symptom of a seizure, characterized by a feeling of vertigo. When it occurs, there is a sensation of rotation or movement that lasts for a few seconds before full seizure activity. While the specific causes of this disease are speculative there are several methods for diagnosis, the most important being the patient's recall of episodes. Most times, those diagnosed with vertiginous seizures are left to self-manage their symptoms or are able to use anti-epileptic medication to dampen the severity of their symptoms.[ citation needed ]

Contents

Vertiginous epilepsy has also been referred to as epileptic vertigo, vestibular epilepsy, vestibular seizures, and vestibulogenic seizures in different cases, but vertiginous epilepsy is the preferred term. [1]

Signs and Symptoms

The signs of vertiginous epilepsy often occur without a change in the subject’s consciousness so that they are still aware while experiencing the symptoms. [2] It is often described as a sudden onset of feeling like one is turning in one direction, typically lasting several seconds. [2] Although subjects are aware during an episode, they often cannot remember specific details due to disorientation, discomfort, and/or partial cognitive impairment. [2] This sensation of rotational movement in the visual and auditory planes is also known as a vertiginous aura (symptom), which can precede a seizure or may constitute a seizure itself. [3] Auras are a “portion of the seizure that occur before consciousness is lost and for which memory is retained afterwards.” [4] Auras can be focused in different regions of the brain and can thus affect different functions. Some such symptoms that may accompany vertiginous epilepsy include:

Many people tend to mistake dizziness as vertigo, and although they sound similar, dizziness is not considered a symptom of vertiginous epilepsy. Dizziness is the sensation of imbalance or floating, impending loss of consciousness, and/or confusion. [2] This is different from vertigo which is characterized by the illusion of rotational movement [2] caused by the “conflict between the signals sent to the brain by balance- and position-sensing systems of the body”. [5]

Mechanism

Although a specific cause has not been identified to always induce vertiginous epilepsy there have been a number of supported hypotheses to how these seizures come about, the most common being traumatic injury to the head. Other causes include tumor or cancers in the brain, stroke with loss of blood flow to the brain, and infection. A less tested hypothesis that some believe may play a larger role in determining who is affected by this disease is a genetic mutation that predisposes the subject for vertiginous epilepsy. [4] This hypothesis is supported by occurrences of vertiginous epilepsy in those with a family history of epilepsy. [6]

Labeled lobes of the brain. Observe the spatial relationship between lobes. Because the temporal, occipital, and parietal lobes are so close, electrical activity can easily travel between them. Gray728.svg
Labeled lobes of the brain. Observe the spatial relationship between lobes. Because the temporal, occipital, and parietal lobes are so close, electrical activity can easily travel between them.

Vertiginous epilepsies are included in the category of the partial epilepsy in which abnormal electrical activity in the brain is localized. [3] With current research, it is presumed that the most likely cause to produce vertigo are epilepsies occurring in the lateral temporal lobe. [2] These abnormal electrical activities can either originate from within the temporal lobe or may propagate from an epilepsy in a neighboring region of the brain. Epilepsies in the parietal and occipital lobes commonly propagate into the temporal lobe inducing a vertiginous state. [2] This electrical propagation across the brain explains why so many different symptoms may be associated with the vertiginous seizure. The strength of the electrical signal and its direction of propagation in the brain will also determine which associated symptoms are noticeable.[ citation needed ]

Diagnosis

The most important factor in diagnosing a patient with vertiginous epilepsy is the subject’s detailed description of the episode. [2] However, due to the associated symptoms of the syndrome a subject may have difficulty remembering the specifics of the experience. This makes it difficult for a physician to confirm the diagnosis with absolute certainty. A questionnaire may be used to help patients, especially children, describe their symptoms. Clinicians may also consult family members for assistance in diagnosis, relying on their observations to help understand the episodes. [7] In addition to the description of the event, neurological, physical and hematologic examinations are completed to assist in diagnosis. For proper diagnosis, an otological exam (examination of the ear) should also be completed to rule out disorders of the inner ear, which could also be responsible for manifestations of vertigo. [8] This may include an audiological assessment and vestibular function test. [8] During diagnosis, history-taking is essential in determining possible causes of vertiginous epilepsy as well as tracking the progress of the disorder over time. [8]

Other means used in diagnosis of vertiginous epilepsy include:

The EEG measures electrical activity in the brain, allowing a physician to identify any unusual patterns. While EEGs are good for identifying abnormal brain activity is it not helpful in localizing where the seizure originates because they spread so quickly across the brain. [3] MRIs are used to look for masses or lesions in the temporal lobe of the brain, indicating possible tumors or cancer as the cause of the seizures. [2] When using a PET scan, a physician is looking to detect abnormal blood flow and glucose metabolism in the brain, which is visible between seizures, to indicate the region of origin. [2]

Management

Watson-Crick double helix, genetics DNA orbit animated.gif
Watson-Crick double helix, genetics

There is no real way to prevent against vertiginous episodes out of the means of managing the disease. As head trauma is a major cause for vertiginous epilepsy, protecting the head from injury is an easy way to avoid possible onset of these seizures. With recent advances in science it is also possible for an individual to receive genetic screening, but this only tells if the subject is predisposed to developing the condition and will not aid in preventing the disease. [4]

There are a range of ways to manage vertiginous epilepsy depending on the severity of the seizures. For simple partial seizures medical treatment is not always necessary. [2] To the comfort of the patient, someone ailed with this disease may be able to lead a relatively normal life with vertiginous seizures. If, however, the seizures become too much to handle, antiepileptic medication can be administered as the first line of treatment. [2] There are several different types of medication on the market to deter epileptic episodes but there is no support to show that one medication is more effective than another. [2] In fact, research has shown that simple partial seizures do not usually respond well to medication, leaving the patient to self-manage their symptoms. [2] A third option for treatment, used only in extreme cases when seizure symptoms disrupt daily life, is surgery wherein the surgeon will remove the epileptic region. [2]

Epidemiology

Vertiginous epilepsy onset can happen between the ages of 4–50, although typically symptoms will begin occurring in adolescence or young adulthood. [4] Research studies have shown no inclination for this disease between sexes.[ citation needed ]

Example of Optokinetic Nystagmus Optokinetic nystagmus.gif
Example of Optokinetic Nystagmus

History

Caloric reflex testing was developed and used for testing vestibular function of deaf children and in diagnosis of childhood vertigo. A man named Barany (1902) published the first monograph on vestibular nystagmus that recognized the clinical usefulness of caloric responses. Barany's theory of production of the vestibulo-ocular reflex in caloric testing remains as the accepted explanation along with the description of nystagmus created by rotation in both adults and infants. Barany's work would later influence a researcher named Galebsky that would help him conclude that semicircular canals were functional at birth for both calorization and rotation. His work would also confirm that the eyelid of a newborn is raised in response to a vestibular as well as an auditory stimulus. [9]

In the late 19th century and early 20th century, Hughlings Jackson [10] and W.R. Gowers [11] recognized that episodes of dizziness were a symptom of epilepsy. [6] During this time, when epileptic symptoms were brought to the forefront of study, there was no distinction between dizziness and vertigo. Sir George Frederick (1868–1941), known for his work in pediatric rheumatoid arthritis referred to as Still’s disease, was the first to publish a description of episodic vertigo in children within the broad category of ‘headaches in children’ in 1924. [9] Until recently, it was not widely recognized that episodes of suspected dizziness might be caused by epilepsy. [6]

Research

There have been early and consistent strategies for measurement to better understand vertiginous epilepsy including caloric reflex test, posture and gait, or rotational experimentation. [9]

In Japan, Kaga et al prepared a longitudinal study of rotation tests comparing congenital deafness and children with delayed acquisition of motor system skills. They were able to demonstrate the development of post-rotation nystagmus response from the frequency of beat and duration period from birth to six years to compare to adult values. Overall, the study demonstrated that some infants from the deaf population had impaired vestibular responses related to head control and walking age. A side interpretation included the evaluation of the vestibular system in reference to matching data with age. [9]

Research in this area of medicine is limited due to its lacking need for urgent attention. But, the American Hearing Research Foundation (AHRF) conducts studies in which they hope to make new discoveries to help advance treatment of the disease and possibly one day prevent vertiginous seizures altogether.[ citation needed ]

Related Research Articles

Seizure Period of symptoms due to excessive or synchronous neuronal brain activity

An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much of the body with loss of consciousness, to shaking movements involving only part of the body with variable levels of consciousness, to a subtle momentary loss of awareness. Most of the time these episodes last less than two minutes and it takes some time to return to normal. Loss of bladder control may occur.

Robert Bárány Austria-born otologist, winner of 1914 Nobel Prize in Physiology or Medicine (1876-1936)

Robert Bárány was an ethnic Hungarian otologist from Austria. He received the 1914 Nobel Prize in Physiology or Medicine for his work on the physiology and pathology of the vestibular apparatus.

Dizziness Neurological condition causing impairment in spatial perception and stability

Dizziness is an imprecise term that can refer to a sense of disorientation in space, vertigo, or lightheadedness. It can also refer to disequilibrium or a non-specific feeling, such as giddiness or foolishness.

Labyrinthitis Medical condition

Labyrinthitis is inflammation of the labyrinth – a maze of fluid-filled channels in the inner ear. Some sources might confuse it with vestibular neuritis which means inflammation of the vestibular nerve – the nerve in the inner ear that sends messages related to motion and position to the brain. Both conditions involve inflammation of the inner ear.labyrinths that house the vestibular system, senses changes in the head's position or the head's motion. Inflammation of these inner ear parts results in a sensation of the world spinning and also possible hearing loss or ringing in the ears. It can occur as a single attack, a series of attacks, or a persistent condition that diminishes over three to six weeks. It may be associated with nausea, vomiting, and eye nystagmus.

Benign paroxysmal positional vertigo Medical condition

Benign paroxysmal positional vertigo (BPPV) is a disorder arising from a problem in the inner ear. Symptoms are repeated, brief periods of vertigo with movement, characterized by a spinning sensation upon changes in the position of the head. This can occur with turning in bed or changing position. Each episode of vertigo typically lasts less than one minute. Nausea is commonly associated. BPPV is one of the most common causes of vertigo.

Landau–Kleffner syndrome (LKS)—also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder—is a rare childhood neurological syndrome.

Aura (symptom) Symptom of epilepsy and migraine

An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is a seizure.

Psychogenic non-epileptic seizure Events resembling an epileptic seizure, but without the electrical discharges associated with epilepsy

Psychogenic non-epileptic seizures (PNES) are events resembling an epileptic seizure, but without the characteristic electrical discharges associated with epilepsy. PNES fall under the category of disorders known as functional neurological disorders (FND), also known as conversion disorders. A more recent term to describe these events is dissociative non-epileptic seizures. These are typically treated by psychologists or psychiatrists. PNES has previously been called pseudoseizures, psychogenic seizures, and hysterical seizures, but these terms have fallen out of favor.

Vertigo Type of dizziness where a person has the sensation of moving or surrounding objects moving

Vertigo is a condition where a person has the sensation of movement or of surrounding objects moving when they are not. Often it feels like a spinning or swaying movement. This may be associated with nausea, vomiting, sweating, or difficulties walking. It is typically worse when the head is moved. Vertigo is the most common type of dizziness.

Temporal lobe epilepsy Chronic focal seizure disorder

Temporal lobe epilepsy (TLE) is a chronic disorder of the nervous system which is characterized by recurrent, unprovoked focal seizures that originate in the temporal lobe of the brain and last about one or two minutes. TLE is the most common form of epilepsy with focal seizures. A focal seizure in the temporal lobe may spread to other areas in the brain when it may become a focal to bilateral seizure.

Frontal lobe epilepsy (FLE) is a neurological disorder that is characterized by brief, recurring seizures that arise in the frontal lobes of the brain, often while the patient is sleeping. It is the second most common type of epilepsy after temporal lobe epilepsy (TLE), and is related to the temporal form by the fact that both forms are characterized by the occurrence of partial (focal) seizures. Partial seizures occurring in the frontal lobes can occur in one of two different forms: either simple partial seizures or complex partial seizures. The symptoms and clinical manifestations of frontal lobe epilepsy can differ depending on which specific area of the frontal lobe is affected.

Abdominal epilepsy is a rare condition most frequently found in children, consisting of gastrointestinal disturbances caused by epileptiform seizure activity. Though a few cases of it have been reported in adults too. It has been described as a type of temporal lobe epilepsy. Responsiveness to anticonvulsants can aid in the diagnosis. Distinguishing features of abdominal epilepsy include (1) Abnormal laboratory, radiographic, and endoscopic findings revealing paroxysmal GI manifestations of unknown origin (2) CNS symptoms (3) Abnormal EEG. Most published medical literature dealing with abdominal epilepsy is in the form of individual case reports. A 2005 review article found a total of 36 cases described in the medical literature.

Epilepsy surgery involves a neurosurgical procedure where an area of the brain involved in seizures is either resected, ablated, disconnected or stimulated. The goal is to eliminate seizures or significantly reduce seizure burden. Approximately 60% of all people with epilepsy have focal epilepsy syndromes. In 15% to 20% of these patients, the condition is not adequately controlled with anticonvulsive drugs. Such patients are potential candidates for surgical epilepsy treatment.

Vestibular migraine (VM) is vertigo with migraine, either as a symptom of migraine or as a related neurological disorder.

Migralepsy is a rare condition in which a migraine is followed, within an hour period, by an epileptic seizure. Because of the similarities in signs, symptoms, and treatments of both conditions, such as the neurological basis, the psychological issues, and the autonomic distress that is created from them, they individually increase the likelihood of causing the other. However, also because of the sameness, they are often misdiagnosed for each other, as migralepsy rarely occurs.

Epilepsy is a disorder in which nerve cell activity in the brain is disturbed, causing seizures.During a seizure, a person experiences abnormal behavior, symptoms, and sensations, sometimes including loss of consciousness. There are few symptoms between seizures. A seizure is a single occurrence, whereas epilepsy is a neurological condition characterized by two or more unprovoked seizures. Epilepsy is the most common childhood brain disorder in the United States. Nearly 3 million people have been diagnosed with this disease, while 450,000 of them are under the age of 17. Two thirds of the child population will overcome the side effects, including seizures, through treatment during adolescence. Some treatments include surgery, medication and therapy, surgery however is only done if the child has drug resistant epilepsy.

People with epilepsy may be classified into different syndromes based on specific clinical features. These features include the age at which seizures begin, the seizure types, and EEG findings, among others. Identifying an epilepsy syndrome is useful as it helps determine the underlying causes as well as deciding what anti-seizure medication should be tried. Epilepsy syndromes are more commonly diagnosed in infants and children. Some examples of epilepsy syndromes include benign rolandic epilepsy, childhood absence epilepsy and juvenile myoclonic epilepsy. Severe syndromes with diffuse brain dysfunction caused, at least partly, by some aspect of epilepsy, are also referred to as epileptic encephalopathies. These are associated with frequent seizures that are resistant to treatment and severe cognitive dysfunction, for instance Lennox-Gastaut syndrome and West syndrome.

Occipital epilepsy Medical condition

Occipital epilepsy is a neurological disorder that arises from excessive neural activity in the occipital lobe of the brain that may or may not be symptomatic. Occipital lobe epilepsy is fairly rare, and may sometimes be misdiagnosed as migraine when symptomatic. Epileptic seizures are the result of synchronized neural activity that is excessive, and may stem from a failure of inhibitory neurons to regulate properly.

Vestibular rehabilitation Form of physical therapy for vestibular disorders

Vestibular rehabilitation (VR), also known as vestibular rehabilitation therapy (VRT), is a specialized form of physical therapy used to treat vestibular disorders or symptoms, characterized by dizziness, vertigo, and trouble with balance, posture, and vision. These primary symptoms can result in secondary symptoms such as nausea, fatigue, and lack of concentration. All symptoms of vestibular dysfunction can significantly decrease quality of life, introducing mental-emotional issues such as anxiety and depression, and greatly impair an individual, causing them to become more sedentary. Decreased mobility results in weaker muscles, less flexible joints, and worsened stamina, as well as decreased social and occupational activity. Vestibular rehabilitation therapy can be used in conjunction with cognitive behavioral therapy in order to reduce anxiety and depression resulting from an individual's change in lifestyle.

Musicogenic seizure, also known as music-induced seizure, is a rare type of seizure, with an estimated prevalence of 1 in 10,000,000 individuals, that arises from disorganized or abnormal brain electrical activity when a person hears or is exposed to a specific type of sound or musical stimuli. There are challenges when diagnosing a music-induced seizure due to the broad scope of triggers, and time delay between a stimulus and seizure. In addition, the causes of musicogenic seizures are not well-established as solely limited cases and research have been discovered and conducted respectively. Nevertheless, the current understanding of the mechanism behind musicogenic seizure is that music triggers the part of the brain that is responsible for evoking an emotion associated with that music. Dysfunction in this system leads to an abnormal release of dopamine, eventually inducing seizure.

References

  1. ↑ Nielsen, J. M., MD. "Tornado Epilepsy Simulating Ménière's Syndrome." Neurology 9.11 (1959): 794. Neurology. Web. 23 Mar. 2014. <http://www.neurology.org/content/9/11/794.extract>.
  2. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Stern, John M. "Focal Vertiginous Epilepsy." Atlas of Epilepsies. (2010): 463–465. Print.
  3. 1 2 3 4 5 Benbadis, Selim. Wolters Kluwer Health. (2013): Web. 23 Mar. 2014. <http://www.uptodate.com/contents/localization-related-partial-epilepsy-causes-and-clinical-features>.
  4. 1 2 3 4 Ottman, R. United States. NCBI. Autosomal Dominant Partial Epilepsy with Auditory Features. Seattle: University of Washington, 2007. Web.
  5. ↑ "Dizziness: Lightheadedness and Vertigo Topic Overview." WebMD. N.p., 2 Jan 2013. Web. 23 Mar 2014. <http://www.webmd.com/brain/tc/dizziness-lightheadedness-and-vertigo-topic-overview>.
  6. 1 2 3 Kogeorgos, J., D.F. Scott, and M Swash. "Epileptic Dizziness." British Medical Journal . 282. (1981): n. page.
  7. ↑ Erbek, Seyra H., Selim S. Erbek, Ismail Yilmaz, Ozgal Topal, Nuri Ozgirgin, Levent N. Ozluoglu, and Fusun Alehan. "Vertigo in childhood: A clinical experience ." International Journal of Pediatric Otorhinolaryngology. 70. (2006): 1547–1554.
  8. 1 2 3 Choung, Yun-Hoon, Keehyun Park, Sung-Kyun Moon, Chul-Ho Kim, and Sang Jun RYu. "Various causes and clinical characteristics in vertigo in children with normal eardrums." Journal of Pediatric Otorhinolaryngology. 67. (2003): 889–894.
  9. 1 2 3 4 Snashall S. The history of balance in children: a review. Audiological Medicine [serial online]. October 2009;7(3):132–137. Available from: CINAHL with Full Text, Ipswich, MA.
  10. ↑ Jackson H., Diagnosis of epilepsy. Medical Times and Gazette 1879; 1:29.
  11. ↑ Gowers WR., The borderlands of epilepsy. London: Churchill, 1907: 40–75.
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