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infancy electroclinical syndrome
ICD-10 Codes
Related ICD-10:
Description
Infancy electroclinical syndrome, also known as early infantile epileptic encephalopathy (EIEE), is a rare and severe form of epilepsy that affects infants between birth and one year of age.
Characteristics:
- Age of onset: Typically occurs in infancy, between birth and one year of age [2].
- Seizure types: Can manifest as various seizure types, including generalized seizures, absences, myoclonic jerks, tonic-clonic seizures, and others [8].
- Clinical features: Infants with EIEE often exhibit profound impairment of awareness and responsiveness, staring, and arrest of activity. There is no aura associated with these seizures [5].
Definition:
Infancy electroclinical syndrome is an electroclinical syndrome characterized by onset in infancy occurring between birth and one year of age [6]. It is a rare and severe form of epilepsy that requires prompt medical attention.
References:
- [2] - Infancy electroclinical syndrome. Definition, An electroclinical syndrome with onset in infancy occurring between birth and one year of age.
- [5] - They consist of an arrest of activity, staring, and profound impairment of awareness and responsiveness. There is no aura. Most children become still and will...
- [6] - infancy electroclinical syndrome. Term ID: DOID:0050703; Synonyms. Definition: An electroclinical syndrome with onset in infancy occurring between birth and one...
Additional Characteristics
- Infancy electroclinical syndrome, also known as early infantile epileptic encephalopathy (EIEE)
- is a rare and severe form of epilepsy that affects infants between birth and one year of age.
- Characteristics:
- * Age of onset: Typically occurs in infancy, between birth and one year of age
- * Seizure types: Can manifest as various seizure types, including generalized seizures, absences, myoclonic jerks, tonic-clonic seizures, and others
- * Clinical features: Infants with EIEE often exhibit profound impairment of awareness and responsiveness, staring, and arrest of activity. There is no aura associated with these seizures
- Definition:
- Infancy electroclinical syndrome is an electroclinical syndrome characterized by onset in infancy occurring between birth and one year of age
- It is a rare and severe form of epilepsy that requires prompt medical attention
Signs and Symptoms
Based on the provided context, it appears that you are looking for information on the signs and symptoms of a specific epilepsy syndrome affecting infants.
According to the search results, Epilepsy syndromes affect infants and children, and are characterized by a variety of seizures and other symptoms such as developmental delays [4]. One such syndrome is characterized by the onset of myoclonic seizures between the ages of 6 months and 2 years, and in some cases, earlier (4 months) or later (2 to 3 years) [9].
The manifestations of this syndrome often include head nodding, abrupt abduction of the arms, or sudden falls [6]. Eyelid or facial muscles are affected commonly. Seizures appear during the first 3 years of life in children with normal motor and mental development, characterized by reflex myoclonic seizures [7].
It's worth noting that Epileptic encephalopathies are severe brain disorders in which the epileptic electrical discharges may contribute to progressive psychomotor dysfunction [3]. However, this information is more general and not specific to infancy electroclinical syndrome.
The signs and symptoms of infancy electroclinical syndrome can be summarized as follows:
- Onset of myoclonic seizures between 6 months and 2 years of age
- Head nodding, abrupt abduction of the arms, or sudden falls
- Eyelid or facial muscles affected commonly
- Seizures appear during the first 3 years of life in children with normal motor and mental development
References:
[4] - Epilepsy syndromes affect infants and children, and are characterized by a variety of seizures and other symptoms such as developmental delays. [6] - Manifestations often include head nodding, abrupt abduction of the arms, or sudden falls. Eyelid or facial muscles are affected commonly. [7] - Seizures appear during the first 3 years of life in children with normal motor and mental development [2]. They are characterized by reflex myoclonic seizures (... [9] - This syndrome is characterized by the onset of myoclonic seizures between the ages of 6 months and 2 years, and in some cases, earlier (4 months) or later (2 to ...
Additional Symptoms
- Onset of myoclonic seizures between 6 months and 2 years of age
- Head nodding, abrupt abduction of the arms, or sudden falls
- Eyelid or facial muscles affected commonly
- Seizures appear during the first 3 years of life in children with normal motor and mental development
Diagnostic Tests
Diagnosing Infantile Epilepsy Syndromes
Infantile epilepsy syndromes, such as myoclonic epilepsy in infancy and Dravet syndrome, can be challenging
Additional Diagnostic Tests
- Genetic testing
- EEG
- Neuroimaging
Treatment
The drug treatment for Infancy Epilepsy Syndrome (IES) can vary depending on the specific characteristics of the syndrome and the individual child. However, based on the search results, here are some general insights:
- Valproic acid is often considered a first-line treatment for IES, as it has been shown to be effective in controlling seizures in many patients [3].
- Steroids and ACTH may also be considered as a treatment option, especially in early onset of the disease [4].
- Phenobarbital can be used as an initial drug of choice, but if seizures persist, other medications such as phenytoin should be considered [5].
- Carbamazepine or oxcarbazepine may also be effective in low doses for many patients, although about 30% of patients with IES have resistant seizures [7].
It's essential to note that the treatment plan for IES should be individualized and tailored to the specific needs of each child. A comprehensive approach, including a thorough medical evaluation, seizure monitoring, and medication management, is crucial in managing this condition.
References:
[3] Feb 4, 2022 — Treatment: Valproic acid is typically the anti-seizure medication of choice, although other broad-spectrum anti-seizure medications could also be considered. [4] by P Parisi · 2011 · Cited by 13 — Some authors suggest that steroids and ACTH should be considered the treatment of choice especially in early onset of disease [116] [5] Jul 30, 2019 — Phenobarbital is the initial drug of choice. If seizures persist, the use of phenytoin should be considered. [7] by RA Shellhaas · 2017 · Cited by 24 — We examined the selection of initial medications in children with nonsyndromic early-life epilepsy in an effort to identify opportunities for rational.
Recommended Medications
- Steroids and ACTH
- Carbamazepine or oxcarbazepine
- phenobarbital
- Phenobarbital
- valproic acid
- phenytoin
- Phenytoin
💊 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
The differential diagnosis for infantile electroclinical syndromes can be quite complex, but I'll try to break it down for you.
According to the search results, the main differential diagnoses for Ohtahara syndrome (a type of infantile electroclinical syndrome) include:
- Early myoclonic encephalopathy [
Additional Differential Diagnoses
- Infantile spasms
- Cortical dysgenesis
- lissencephaly
- West syndrome
- early myoclonic encephalopathy
Additional Information
- relatedICD
- http://example.org/icd10/E75.243
- core#notation
- DOID:0050703
- rdf-schema#label
- infancy electroclinical syndrome
- rdf-schema#comment
- JA:Epilepsy Genetics Kiel
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_0111645
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_740
- owl#annotatedSource
- t332701
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#created_by
- lschriml
- oboInOwl#creation_date
- 2012-04-11T11:48:18Z
- oboInOwl#id
- DOID:0050703
- IAO_0000115
- An electroclinical syndrome with onset in infancy occurring between birth and one year of age.
- RO_0002488
- http://purl.obolibrary.org/obo/HP_0003593
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