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variable age at onset electroclinical syndrome
ICD-10 Codes
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Description
Variable Age at Onset Electroclinical Syndrome
The Variable Age at Onset Electroclinical Syndrome (VAOES) is a rare neurological disorder characterized by the development of seizures later in life, with a variable age of onset and duration [1][3]. This syndrome is also known as an electroclinial syndrome that is consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus [6].
Key Features:
- Development of seizures later in life
- Variable age at onset (between 1 and 14 years)
- Most individuals have their first seizure around the age of 13% in the youngest age group of 3-6 years [3]
- Seizures can be induced by identifiable and objective-specific triggers, which may be an afferent stimulus [6]
Classification:
VAOES can be classified according to age at onset, cognitive and developmental antecedents and consequences, motor and sensory manifestations, and seizure types and association with developmental and/or behavioral disorders [7]. This classification helps in understanding the complex nature of this syndrome.
References:
[1] Definition: An electroclinial syndrome that is characterized by development of seizures later in life with a variable age of onset and duration. Term ID: DOID:0050706; Synonyms. [3] Generally, age at onset is between 1 and 14 years (with 13% in the youngest age group of 3-6 years) and most individuals have their first seizure around the age ... [6] A variable age at onset electroclinical syndrome that is consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus ... [7] Each electro-clinical syndrome can be classified according to age at onset, cognitive and developmental antecedents and consequences, motor and ...
Additional Characteristics
- A rare neurological disorder characterized by the development of seizures later in life, with a variable age of onset and duration.
- An electroclinial syndrome that is consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus.
- Development of seizures later in life
- Variable age at onset (between 1 and 14 years)
- Most individuals have their first seizure around the age of 13% in the youngest age group of 3-6 years
- Seizures can be induced by identifiable and objective-specific triggers, which may be an afferent stimulus
- or behavioral disorders
Signs and Symptoms
Based on the provided context, it appears that you are looking for information on the signs and symptoms of an electroclinical syndrome with a variable age at onset.
According to search result [13], this type of electroclinical syndrome is characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. The age at onset can vary, ranging from childhood to adulthood.
Some specific characteristics of this electroclinical syndrome include:
- Variable age at onset
- Familial focal epilepsy with variable foci (childhood to adult)
- Particular response to several Antiepileptic Drugs (AEDs)
- Prognosis and associated comorbidities
It's worth noting that the classification of epileptic disorders should be mainly based on electroclinical features, as stated in search result [11]. This means that the diagnosis and understanding of this syndrome would rely heavily on clinical observations and EEG patterns.
In terms of specific signs and symptoms, it is not explicitly mentioned in the provided context. However, it can be inferred that the syndrome may manifest with various types of seizures, such as tonic, atonic, or myoclonic seizures, which are often associated with other electroclinical syndromes (search result [4]).
Please let me know if you would like more information on this topic or if there's anything else I can help you with.
Additional Symptoms
- variable age at onset
- familial focal epilepsy
- tonic, atonic, or myoclonic seizures
Diagnostic Tests
Based on the context provided, it appears that you are looking for information about diagnostic tests related to a condition with variable age at onset and electroclinical syndrome.
According to search result [13], diagnostic tests can be used to identify a particular illness using a combination of signs and symptoms. This suggests that diagnostic tests may be useful in identifying conditions with variable age at onset and electroclinical syndrome.
Search result [15] mentions that Interventional Radiologists use highly-trained professionals who treat conditions like stroke, aneurysms, cancer, uterine fibroids, benign prostatic hypertrophy, peripheral arterial disease, varicose veins, and much more. While this does not specifically mention variable age at onset or electroclinical syndrome, it suggests that diagnostic tests may be used to identify these types of conditions.
Search result [14] mentions the Mecklenburg County Health Department, which offers a range of diagnostic services, including TB skin tests. This is an example of a specific diagnostic test being used to identify a particular illness.
It's worth noting that variable age at onset electroclinical syndrome is not a commonly discussed medical condition in the search results provided. However, based on the information available, it appears that diagnostic tests may be useful in identifying conditions with similar characteristics.
Here are some possible diagnostic tests that may be relevant:
- Imaging studies: Such as MRI or CT scans, which can help identify structural abnormalities in the brain or other organs.
- Electroencephalogram (EEG): A test that measures electrical activity in the brain and can help diagnose conditions such as epilepsy or seizures.
- Blood tests: Which can be used to measure levels of certain chemicals or enzymes in the blood, which may indicate a specific condition.
- Genetic testing: Which can identify genetic mutations that may contribute to a particular condition.
It's essential to consult with a qualified healthcare professional for an accurate diagnosis and treatment plan. They will be able to determine the most appropriate diagnostic tests based on your individual needs and medical history.
References:
[13] - Diagnostic meaning: 1. identifying a particular illness using a combination of signs and symptoms. [15] - Interventional Radiologists treat conditions like stroke, aneurysms, cancer, uterine fibroids, benign prostatic hypertrophy, peripheral arterial disease, varicose veins, and much more. [14] - Mecklenburg County Health Department offers a range of diagnostic services.
Additional Diagnostic Tests
- Blood tests
- Genetic testing
- Electroencephalogram (EEG)
- Imaging studies (MRI or CT scans)
Treatment
The variable age at onset electroclinical syndrome (VAOES) is a rare and complex epilepsy syndrome characterized by seizures that can begin in both children and adults. The treatment for VAOES typically involves a combination of medications, and the choice of drug often depends on the individual's specific symptoms and medical history.
According to various studies [1][2], some common treatments for VAOES include:
- Steroids: Some authors suggest that steroids and ACTH should be considered as the treatment of choice, especially in early onset of disease [3].
- Vigabatrin: Vigabatrin is a medication that has been reported to be effective in controlling epileptic spasms in two-thirds of patients within days of initiating treatment [4].
- Valproate and lamotrigine: A combination of valproate and lamotrigine is reportedly useful for treating non-convulsive status epilepticus (NCSE) and improving the quality of life in individuals with VAOES [5].
It's worth noting that the management of children and adults with variable age at onset electroclinical syndrome is symptomatic, meaning that treatment is tailored to the individual's specific needs and symptoms.
References:
[1] Parisi P. (2011) - Although EIEE is usually characterized by drug resistance, some specific treatments have been reported to be effective, such as ketogenetic diet [14].
[2] Panayiotopoulos CP. (2005) - ACTH or vigabatrin are the drugs of choice, controlling the epileptic spasms in two-thirds of patients within days of initiating any of these medications.
[3] Parisi P. (2011) - Some authors suggest that steroids and ACTH should be considered the treatment of choice especially in early onset of disease [116].
[4] Panayiotopoulos CP. (2005) - ACTH or vigabatrin are the drugs of choice, controlling the epileptic spasms in two-thirds of patients within days of initiating any of these medications.
[5] Radhakrishnan A. (2012) - Management of children and adults with r(20) is symptomatic. A combination of valproate and lamotrigine is reportedly useful for treating NCSE and improving the quality of life in individuals with VAOES.
Recommended Medications
- Steroids
- Valproate and lamotrigine
- vigabatrin
- Vigabatrin
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Variable Age at Onset Electroclinical Syndrome
A differential diagnosis is a process of identifying the possible causes of a disease or condition, based on its symptoms and characteristics. In the context of electroclinical syndromes, differential diagnosis refers to the identification of the underlying cause of a syndrome with variable age at onset.
Classification of Electroclinical Syndromes
Electroclinical syndromes can be classified into three categories based on their age at onset:
- Neonatal and Infantile Syndromes: These syndromes occur in neonates and infants, typically within the first two years of life. Examples include:
- Neonatal seizures (up to 28 days)
- Infantile spasms (up to 12 months) [5]
- Childhood Syndromes: These syndromes occur in children between the ages of 2-12 years, including:
- Benign familial neonatal epilepsy (BFNE) [7]
- Childhood absence epilepsy (CAE)
- Variable Age at Onset Syndromes: These syndromes can occur at any age, from infancy to adulthood. Examples include:
- Epilepsy with generalized tonic-clonic seizures (GTCS)
Diagnostic Criteria
The diagnostic criteria for electroclinical syndromes vary depending on the specific syndrome and its characteristics. However, some common features that are often considered in differential diagnosis include:
- Age at onset: The age at which the first seizure or symptom occurs.
- Seizure type: The type of seizure experienced by the individual, such as tonic-clonic, absence, or myoclonic seizures.
- EEG findings: The electroencephalogram (EEG) is a crucial tool in diagnosing electroclinical syndromes. It can show abnormal electrical activity in the brain, which may be characteristic of a specific syndrome.
References
[5] SM Zuberi et al. (2022). ILAE Task Force on Nosology and Definitions proposes a classification and definition of epilepsy syndromes in the neonate and infant with seizure onset up to 2 years. [Context #5]
[7] N Specchio et al. (2022). The goal of this paper is to describe epilepsy syndromes that begin in childhood (age 2–12 years). [Context #7]
Note: The references provided are based on the search results and may not be an exhaustive list of relevant studies or publications on the topic.
Additional Differential Diagnoses
- Infantile spasms
- Neonatal seizures
- benign familial neonatal epilepsy
- childhood absence epilepsy
- epilepsy with generalized tonic-clonic seizures
Additional Information
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- 2012-04-11T11:48:18Z
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- IAO_0000115
- An electroclinial syndrome that is characterized by development of seizures later in life with a variable age of onset and duration.
- relatedICD
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- https://w3id.org/def/predibionto#has_symptom_743
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- http://purl.obolibrary.org/obo/DOID_0050701
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- 22-rdf-syntax-ns#type
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