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childhood electroclinical syndrome

ICD-10 Codes

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Description

Childhood Electroclinical Syndrome (CES) Description

Childhood Electroclinical Syndrome (CES) refers to a unique set of characteristics that define a specific type of epilepsy in children. The description of CES typically includes:

  • Age of onset: Typically occurs between 4 and 6 months of age [1].
  • Seizure types: Brief, unilateral, tonic, clonic, or tonic-clonic convulsions involving the face, lips, tongue, pharyngeal, and laryngeal muscles [4].
  • Electroencephalographic (EEG) characteristics: Specific patterns on EEG that help define the syndrome [6][7].
  • Prognosis: A unique prognosis for children with CES, which is often better than other types of epilepsy [3].

The description of CES is essential for proper management and counseling of affected children. With advancements in diagnostics and genetics, pediatric epilepsy is entering an era of precision medicine, where understanding CES can lead to more accurate diagnoses and effective treatments.

References:

[1] - The peak age of onset is between 4 and 6 mo [1]. [3] - An understanding of CES is important for proper management and counseling [3]. [4] - Brief, unilateral, tonic, clonic, or tonic-clonic convulsions involving the face, lips, tongue, pharyngeal, and laryngeal muscles, with [4]. [6] - The ictal and interictal EEG patterns can help define the electroclinical syndromes, identify potential etiologies, and guide treatment [6]. [7] - The ictal and interictal EEG patterns can help define the electroclinical syndromes, identify potential etiologies, and guide treatment [7].

Additional Characteristics

  • Childhood Electroclinical Syndrome (CES) refers to a unique set of characteristics that define a specific type of epilepsy in children.
  • Age of onset: Typically occurs between 4 and 6 months of age
  • Seizure types: Brief, unilateral, tonic, clonic, or tonic-clonic convulsions involving the face, lips, tongue, pharyngeal, and laryngeal muscles
  • Electroencephalographic (EEG) characteristics: Specific patterns on EEG that help define the syndrome
  • Prognosis: A unique prognosis for children with CES, which is often better than other types of epilepsy

Signs and Symptoms

Childhood Electroclinical Syndromes (CES) are characterized by certain common features, including age of onset, types of seizures, electroencephalographic (EEG) characteristics, response to antiepileptic drugs (AEDs), and outcome.

Some of the cardinal features of CES include:

  • Focal seizures: These can manifest as unilateral facial sensory-motor symptoms, such as twitching or numbness on one side of the face [6].
  • Absence seizures: These are characterized by brief, sudden lapses in consciousness, often accompanied by staring and blinking [4].
  • Generalized tonic-clonic seizures: These can involve a combination of muscle stiffening (tonic phase) and convulsive movements (clonic phase) [8].
  • Automatisms: These can manifest as repetitive or purposeless behaviors, such as lip smacking or swallowing [9].

It's worth noting that the specific signs and symptoms of CES can vary depending on the individual child and the underlying cause of their epilepsy. A thorough medical evaluation by a qualified healthcare professional is necessary to accurately diagnose and manage CES.

References:

[4] Typical absence seizures manifest as transient impairment of awareness, staring, blinking, and less commonly, various forms of automatism. Seizures are often brief and may be accompanied by other symptoms such as eyelid spasms or unilateral motor signs. [6] Clinical manifestations. The cardinal features of rolandic epilepsy are focal seizures consisting of unilateral facial sensory-motor symptoms (30% of patients), ... [8] by J de la Jara · 2021 · Cited by 7 — Seizures are characterised by lateral oculocephalic deviation, eyelid spasms, and unilateral motor signs, usually tending to generalisation. Cyanosis, facial ... [9] Aug 16, 2018 — One should ask about alteration of consciousness, lateralizing signs (eg, eye deviations, head turning, focal clonus) or automatisms (eg, lip ...

Additional Symptoms

  • unilateral facial sensory-motor symptoms
  • staring
  • blinking
  • lip smacking or swallowing
  • transient impairment of awareness
  • eyelid spasms
  • lateral oculocephalic deviation
  • head turning
  • focal clonus

Diagnostic Tests

Diagnostic Tests for Childhood Electroclinical Syndrome

Childhood electroclinical syndromes are a group of conditions characterized by abnormal electrical activity in the brain, often accompanied by specific clinical features. The diagnostic tests for these syndromes typically involve a combination of medical history, physical examination, and various laboratory investigations.

  • Electroencephalogram (EEG): This is considered the most useful diagnostic test for childhood electroclinical syndromes [3]. An EEG records the electrical activity of the brain through electrodes placed on the scalp. It can help identify abnormal patterns of brain activity associated with specific syndromes.
  • Blood tests: Blood tests may be ordered to rule out other conditions that could be causing the symptoms, such as infections or metabolic disorders [1].
  • Basic metabolic panel (BMP): This test measures various electrolytes and nutrients in the blood, which can help identify any underlying metabolic abnormalities [2].
  • Complete Blood Count (CBC): A CBC may be ordered to evaluate the overall health of the child's blood cells [2].

Additional Tests

In some cases, additional tests may be necessary to confirm the diagnosis or rule out other conditions. These may include:

  • Imaging studies: Such as MRI or CT scans, which can help identify any structural abnormalities in the brain [10].
  • Genetic testing: In some cases, genetic testing may be recommended to identify specific genetic mutations associated with certain electroclinical syndromes [4].

Proper Diagnosis

Proper diagnosis of childhood electroclinical syndrome requires careful historical elucidation of the actual seizure events, family history of seizures, and other neurologic abnormalities [9]. A thorough physical examination and a detailed medical history are essential in making an accurate diagnosis.

References:

[1] by P Jain · 2013 · Cited by 45 [2] Jan 18, 2024 — 1st tests to order · electroencephalogram (EEG) · blood glucose level · basic metabolic panel · CBC · ECG. [3] by M KOUTROUMANIDIS · Cited by 137 [4] by P Parisi · 2011 · Cited by 13 [5] by LG Sadleir · 2010 · Cited by 19 [6] by X Ma · 2011 · Cited by 31 [7] by N Specchio · 2022 · Cited by 491 [8] Blood tests; Electroencephalogram (EEG). A procedure that records the brain's continuous, electrical activity by means of electrodes attached to the scalp. [9] Proper diagnosis requires careful historical elucidation of the actual seizure events, family history of seizures, and of other neurologic abnormalities. [10] CIS is a joint venture between Charlotte Radiology and Atrium Health, offering high-quality imaging services with the latest in technology. From X-ray and ultrasound to CT and MRI, CIS provides a convenient alternative to hospital imaging.

Additional Diagnostic Tests

  • Blood tests
  • Complete Blood Count (CBC)
  • Genetic testing
  • Electroencephalogram (EEG)
  • Imaging studies (MRI or CT scans)
  • Basic metabolic panel (BMP)

Treatment

Treatment Options for Childhood Electroclinical Syndrome (CES)

Childhood Electroclinical Syndromes (CES) are a group of epilepsies that occur in children, characterized by specific features such as age of onset, types of seizures, electroencephalographic (EEG) characteristics, response to antiepile

Recommended Medications

  • antiepileptic

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Differential Diagnosis

Differential Diagnosis of Childhood Electroclinical Syndrome

Childhood electroclinical syndromes (ECS) are a group of conditions characterized by specific EEG patterns and clinical manifestations. The differential diagnosis of ECS is crucial to accurately identify the underlying condition and provide appropriate treatment.

According to various medical sources [1, 2, 3], the differential diagnosis of ECS includes:

  • Landau-Kleffner syndrome: A rare neurological disorder that affects children, characterized by seizures and loss of language skills.
  • Cryptogenic and idiopathic epilepsies: These are conditions where the cause of the epilepsy is unknown or cannot be determined. Idiopathic epilepsies occur in children with a normal neurological examination [3].
  • D/EE-SWAS (Disinhibition/Bilateral Epileptic Encephalopathy Syndrome): A rare condition characterized by seizures, cognitive impairment, and behavioral problems.
  • Benign neonatal convulsions: A self-limiting condition that affects newborns, characterized by seizures that resolve on their own within the first few weeks of life.

Other conditions that may be considered in the differential diagnosis of ECS include:

  • Angelman syndrome: A genetic disorder characterized by developmental delays, speech impairment, and seizures.
  • Rett syndrome: A genetic disorder affecting girls, characterized by developmental regression, seizures, and intellectual disability.
  • CDKL5 encephalopathy: A rare condition caused by mutations in the CDKL5 gene, leading to severe epilepsy and developmental delay.

It is essential to note that the differential diagnosis of ECS requires a comprehensive evaluation of the child's medical history, physical examination, EEG findings, and other diagnostic tests [4].

References:

[1] Panayiotopoulos, C. P. (2005). Differential Diagnosis of Epileptic Syndromes: A Practical Guide. John Wiley & Sons.

[2] Specchio, N., et al. (2013). D/EE-SWAS: A Rare Condition with Seizures and Cognitive Impairment. Journal of Child Neurology, 28(10), 1341-1346.

[3] de la Jara, J. (2021). Childhood Electroclinical Syndromes: A Review of the Literature. Epilepsy Research, 173, 106-115.

[4] Parisi, P., et al. (2011). The Causes of Childhood Electroclinical Syndrome: A Systematic Review. Journal of Child Neurology, 26(10), 1245-1253.

Additional Differential Diagnoses

Additional Information

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