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neonatal period electroclinical syndrome

ICD-10 Codes

Related ICD-10:

I49.40 P07.01 Q90.9 Q93.52 P91.819 P07.37 G81.9 P24.1 E75.23 G25.83 A33 P22.1 P91 E88 P55.0 E25.0 G40.84 E77.8 Q86.1 G61.0 Q00.2 P09 P77.3 P54.5 A50.06 G80 E75.249 B97.4 E75.11 G11.19 P91.82 P70.3 T50.8X5 E71.5 P61.5 Z3A.24 E87.21 A50.0 G04.3 P09.2 P12 P10.4 P36.19 P94 D81.819 E78.72 L00 O69.5 Q07.03 I49.49 E71.120 E88.4 H90 R94.02 E75.28 P96.2 Q64.12 Z98.89 E72.03 E76.22 A39.1 P07.20 P58.42 P91.823 D81.810 E75.09 G40.814 P91.5 P03.9 T71.111 E80.5 Q93.51 T37.8X5 E75.240 G70.81 O35.11 P04.1A Q28 E67.2 E71.50 G81.1 A50.42 E72.59 O36.8 P04 P74.422 P02.70 I69.292 G40.82 E71.41 P28.40 A88.8 P07.22 E71.518 P19.1 P74.42 P50.1 G40.834 O43.122 P91.811 P36.3 A50.9 E76 G40.822 P50 T71.20 P04.0 Q91.7 E74.820 G40.811 G80.1 O35.12 O36.51 P07.23 E74.810 H35.17 Z05.43 P07.24 G40.901 E71.31 P03.6 Z00.11 Z36.83 Q86.8 E79.89 P92.4 Q04.3 P70 Q25.79 R94.39 P07.0 P07.2 P07.26 O35.14 E71.313 P05.17 I63.6 G73.1 T78.8 G37.8 P23.3 H47.03 D61.02 G83.8 Q99.8 O29.21 P76.0 T42.6X5 Q44.70 T46.905 E70.21 J84.841 A50.43 G71.8 I47.21 P91.4 F82 E71.4 R46.4 P50.3 P05.08 P03.819 P83.0 R06.82 T42 P35.1 E72 G40.833 Q87.89 T71.191 P01.1 E75.0 P07.21 G40.823 I46.9 P28.19 P57.0 E71.311 Q38 Q82.3 E75.241 I45.6 G40.4 T46.995 P07.34 R62.59 R40.1 P07.31 P12.2 T44.905 A50 Q04.4 T41.1X5 P50.9 P07.25 G93 P04.14 O29.213 P72.2 R25 T88.51 S06.1X7 P09.4 G90.1 T36.5X5 P76.2 P07.35 R68.19 Q90 Q80.4 O14.04 G93.7 O43.123 E79.2 Q92.1 G70.80 E71.314 P05.01 P04.13 T42.3X5 P28.39 P52.22 P91.62 T71.231 G71.29 Q60.6 P23.9 Q43.8 E71.541 T74.4 P80.8 I69.813 P04.89 Z05.42 E71.53 P02.1 E75.27 E75.243 O45.093 G90.8 B06.09 B97.11 P74.21 P07.33 P80 Q93.3 P81 E71.44 D81.81 Q74.3 A50.53 P58.1 R40.211 O29.29 E76.02 O45.8 P07.36 Z00.71 A50.09 P61.6 Z33.1 G71.1 Q27 E75.242 Q87.19 E71.511 O29.219 Q91.3 G70.2 G71.22 P72.9 E72.51 Z3A.49 H55 T41.0X5 Z28.81 P28.0 I49.2 G96.8 Q23.4 E76.01 E70.2 Z36.4 O07.33 Q75.05 P71.9 F78.A1 E74.05 P36.30 P84 O43.02 T71.131 G40.842 P09.3 A08.0 P83.3 E71.30 E00 P15.5 H44.533 G40.81 D52 P91.9 P74.0 E71.448 R83.8 P24.81 Z3A.01 Z3A.35 P74.41 E79.81 P24.31 P71.3 Z3A.23 E71.1 Q84.8 G40.83 E75.02 E71.110 Q33.8 P70.2 P07.39 G47.51 P35.8 P05.02 P78.2 G31.82 P52.2 Q43.2 P09.5 P93.0 E72.50 E72.02 P92.1 Q87.86 R68.0 M34.1 P07.17 Q32.2

Description

Neonatal Period Electroclinical Syndrome (NPES)

The Neonatal Period Electroclinical Syndrome (NPES) is a rare and complex condition that affects newborns, typically occurring within the first 44 weeks of gestational age. This syndrome is characterized by a unique set of electroclinical features, which include:

  • Onset in the neonatal period: NPES typically begins during the neonatal period, which is the first four weeks after birth.
  • Electrographic seizures: Infants with NPES may experience electrographic seizures, which are abnormal electrical discharges in the brain that can be detected on an electroencephalogram (EEG).
  • Unilateral focal clonic seizures: Some infants with NPES may also exhibit unilateral focal clonic seizures, which are seizures that affect one side of the body.
  • Neuronal involvement: The condition is associated with neuronal involvement, which means that the brain cells are affected.

Key Features

Some key features of NPES include:

  • Unilateral sensory symptoms: Infants with NPES may experience unilateral sensory symptoms, such as tingling, prickling, or freezing sensations on one side of the body.
  • Less commonly numbness: Numbness is a less common symptom associated with NPES.

References

  1. [3] An electroclinical syndrome with onset in the neonatal period less than 44 weeks of gestational age.
  2. [6] neonatal period electroclinical syndrome. Term ID: DOID:0050702; Synonyms.
  3. [8] Neonatal period electroclinical syndrome is characterized by onset in the neonatal period before 44 weeks of gestational age.

Note: The information provided above is based on the search results and context provided, which includes references to medical literature and definitions from reputable sources.

Additional Characteristics

  • A rare and complex condition that affects newborns
  • Characterized by electrographic seizures, unilateral focal clonic seizures, and neuronal involvement
  • May include unilateral sensory symptoms, such as tingling or freezing sensations on one side of the body
  • Typically begins during the neonatal period (first four weeks after birth)

Signs and Symptoms

Neonatal Period Electroclinical Syndrome: Signs and Symptoms

The neonatal period electroclinical syndrome, also known as neonatal seizures or convulsions, is a condition characterized by epileptic fits occurring from birth to the end of the neonatal period. The signs and symptoms of this syndrome can vary depending on the underlying cause and individual factors.

Common Signs and Symptoms:

  • Sudden, paroxysmal, abnormal alterations of electrographic activity [4][14]
  • Seizures or convulsions that may be short-lived or prolonged [1][2][3]
  • Autonomic symptoms such as changes in heart rate, blood pressure, and breathing patterns [4]
  • Neurodevelopmental and psychiatric comorbidities of varying degree [3]

Specific Symptoms:

  • In some cases, the electroclinical syndrome may present with distinct features such as:
    • Panayiotopoulos syndrome, characterized by seizures with predominantly autonomic symptoms and a specific EEG pattern [4]
    • Benign neonatal familial convulsions, which involve a distinct epileptic syndrome with an autosomal mode of transmission [5]

Important Considerations:

  • Neonatal seizures have unique pathophysiology and electrographic findings due to the developmentally immature brain during this period [14]
  • Early diagnosis and classification are crucial for effective management and treatment [6][7]

References:

[1] Context result 1 [2] Context result 1 [3] Context result 1 [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7 [14] Context result 14

Additional Symptoms

  • Sudden, paroxysmal, abnormal alterations of electrographic activity
  • Seizures or convulsions that may be short-lived or prolonged
  • Autonomic symptoms such as changes in heart rate, blood pressure, and breathing patterns
  • Neurodevelopmental and psychiatric comorbidities of varying degree

Diagnostic Tests

Diagnostic Tests for Neonatal Period Electroclinical Syndrome

The diagnosis of neonatal period electroclinical syndrome (NPES) involves a combination of clinical evaluation, laboratory tests, and imaging studies.

  • Electroencephalography (EEG): EEG is a crucial diagnostic tool for NPES. It helps to identify the type of seizure activity and can be used to monitor the progression of the condition [1][2].
  • Imaging Studies: Imaging studies such as magnetic resonance imaging (MRI) may be performed to rule out underlying structural abnormalities or neuro-metabolic derangements that may be contributing to the NPES [3][4].
  • Laboratory Tests: Laboratory tests including liver function tests, and other end-organ function tests may be ordered if there is a concern for perinatal asphyxia [5].
  • Genetic Testing: Genetic testing may be considered in some cases to help clarify etiologic diagnosis and predict the risk of epilepsy in families with positive epilepsy history [6][7].

It's essential to note that the approach to diagnosis should emphasize the electro-clinical syndrome, physical examination, and imaging studies. A strict electroclinical semiology is also important for diagnosing NPES [8].

Additional Diagnostic Tests

  • Laboratory Tests
  • Imaging Studies
  • Genetic Testing
  • Electroencephalography (EEG)

Treatment

The management of neonatal seizures involves the use of antiepileptic drugs (AEDs) as the mainstay of treatment. In the neonatal age group, AEDs are used to control and prevent seizures.

  • Phenobarbital: Traditionally, phenobarbital has been one of the most commonly used medications for treating neonatal seizures [1]. However, its use may be associated with negative effects on neurodevelopment [7].
  • Phenytoin/Fosphenytoin: These medications have also been widely used in the treatment of neonatal seizures, despite their efficacy being limited to approximately 50% of cases [1].
  • Levetiracetam: This AED has gained popularity over the past decade and is now used less commonly than phenobarbital for initial treatment. Its use has increased due to its favorable side effect profile [6].
  • Midazolam: In some cases, intravenous benzodiazepines like midazolam may be required for persistent seizures [5].

The choice of AED depends on various factors, including the severity and type of seizure, as well as the individual neonate's medical history and response to treatment.

References:

[1] LA Slaughter · 2013 - Phenobarbital and phenytoin/fosphenytoin have traditionally been the most commonly used medications to treat neonatal seizures, despite only approximately 50% ...

[6] JM Ziobro · 2021 - Levetiracetam is used less commonly than phenobarbital for the initial treatment of neonatal seizures. Over the past decade, use of levetiracetam increased from ...

[7] by C Grayson · Cited by 2 — Although phenobarbital has proven highly effective at achieving seizure cessation, its net effect on neurodevelopment could still be negative, due to its ...

💊 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 of neonatal period electroclinical syndrome involves considering various conditions that can present with similar symptoms.

According to the search results, differential diagnosis includes benign familial neonatal-infantile seizures and benign familial infantile epilepsy [6]. These conditions are characterized by a genetic predisposition to seizures in infants and young children.

Additionally, neuroimaging, together with pre- and perinatal history, electroclinical features, and neurological examination, can play an important role in the differential diagnosis of neonatal seizures [5]. This suggests that a comprehensive evaluation is necessary to rule out other conditions that may mimic neonatal seizures.

Furthermore, strict electroclinical semiology is the backbone for diagnosing neonatal seizures, which implies that a detailed analysis of the electroencephalogram (EEG) and clinical features is crucial in making an accurate diagnosis [4].

Other conditions that should be considered in the differential diagnosis of neonatal period electroclinical syndrome include:

  • Hypoxic-ischemic encephalopathy or vascular etiologies, which are common causes of seizures occurring within the first 24 hours of life [8].
  • Benign familial infantile epilepsy, which is a genetic condition that can cause seizures in infants and young children.
  • Metabolic and genetic testing have played an essential role in identifying the underlying causes of seizures in neonates, which suggests that these tests should be considered as part of the differential diagnosis [9].

References:

[4] by RA Machado · 2024 [5] by F Pisani · 2021 · Cited by 82 [6] [8] Apr 4, 2022 [9] by RA Machado · 2024

Additional Differential Diagnoses

Additional Information

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