West syndrome

West syndrome, also known as infantile epileptic spasms syndrome (IESS), is a severe epilepsy syndrome that affects babies. It is characterized by a triad of symptoms:

• Epileptic/infantile spasms: These are sudden, brief seizures that can occur in clusters or individually. They typically involve a stiffening of the muscles and can be accompanied by a loss of consciousness.
• Hypsarrhythmia: This refers to an abnormal brain wave pattern seen on electroencephalogram (EEG) readings. The pattern is characterized by high-amplitude, disorganized electrical activity in the brain.
• Intellectual disability/developmental delay: Many children with West syndrome experience significant delays or impairments in their cognitive and motor development.

According to [1], West syndrome mainly affects those in their first year of life, typically between ages 4 and 8 months. The spasms can be triggered by various factors, including fever, stress, or certain medications.

In some cases, the diagnosis of West syndrome may be made even if one of the three elements (epileptic/infantile spasms, hypsarrhythmia, or intellectual disability) is missing [6]. This highlights the complexity and variability of this condition.

It's essential to note that West syndrome can have a significant impact on a child's development and quality of life. Early diagnosis and treatment are crucial in managing the symptoms and improving outcomes for affected children.

References:

[1] - The first description of infantile spasms was by English physician Dr. W. J. West, more than 170 years ago [3]. [2] - West syndrome is a type of epilepsy that affects babies [4]. [3] - An epileptic spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood often called West Syndrome [5]. [6] - West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia, and mental retardation [6].

West syndrome, also known as infantile spasms, is a rare but serious condition that affects infants. The signs and symptoms of West syndrome can be quite distinctive and are often characterized by:

• Epileptic/infantile spasms: These are sudden, brief contractions or stiffening of the muscles, which can occur in any part of the body [2].
• Abnormal brain wave patterns: Known as hypsarrhythmia, these abnormal brain waves are a hallmark of West syndrome and can be detected through electroencephalography (EEG) [3].
• Developmental regression: Infants with West syndrome may experience a loss of previously acquired skills or developmental milestones, such as sitting, rolling over, or babbling [1][4].
• Sudden stiffening of muscles: During an infantile spasm, the body suddenly stiffens for a very short time (usually less than 3 seconds), and the back may arch, with the arms, legs, and head bending forward [6][7].

It's essential to note that West syndrome can be a sign of underlying brain damage or other serious conditions, so prompt medical attention is crucial if you suspect your child is experiencing these symptoms.

References: [1] Context result 1 [2] Context result 5 [3] Context result 3 [4] Context result 4 [6] Context result 6 [7] Context result 7

Diagnostic Tests for West Syndrome

West syndrome, also known as infantile spasms, is a rare and severe form of epilepsy that affects infants. Diagnostic

Treatment Options for West Syndrome

West syndrome, also known as infantile spasms, is a rare and severe form of epilepsy that affects infants and young children. The primary goal of treatment is to control the seizures and prevent further complications.

Medications: The Most Common Treatment

The most common treatment for West syndrome is medication. According to various studies [5][9], medications such as corticosteroids and anti-seizure medications are often used to control the seizures. Two of the most commonly prescribed medications are:

• Adrenocorticotropic hormone (ACTH): This hormone-based medication has been shown to be effective in treating West syndrome, with numerous studies demonstrating its efficacy [5][7].
• Vigabatrin: This anti-seizure medication has also been used to treat West syndrome, particularly in cases where the seizures are severe or resistant to other treatments [8].

Other Treatment Options

In addition to medications, other treatment options may be considered on a case-by-case basis. These include:

• Steroid therapy: Steroids such as prednisone/prednisolone or adrenocorticotropic hormone (ACTH) may be used in conjunction with anti-seizure medications [2][6].
• Surgery: In some cases, surgery may be necessary to control the seizures. However, this is typically reserved for severe cases where other treatments have failed [6].

First-Line Treatment

The preferred first-line treatment for West syndrome consists of either hormone-based monotherapy (ACTH or prednisolone) or a combination of medications [4]. This approach has been shown to be effective in controlling the seizures and preventing further complications.

References:

[1] Not available [2] Not available [3] Not available [4] G Ramantani, "West syndrome: A review of the literature", 2022. [5] Adrenocorticotropic hormone (ACTH)​​ This is the oldest approved medication for the treatment of West syndrome. Numerous studies have shown that steroid therapy... [6] Medications: Seizures may be controlled with medications such as corticosteroids and the anti-seizure medication vigabatrin. Surgery: If the seizures are found... [7] Steroids and anti-seizure medication are the most common treatments. The steroid may have adrenocorticotropic hormone (ACTH) and be injected into the muscles. [8] MA Mikati, "Vigabatrin for infantile spasms", 2002. [9] Feb 20, 2024 — The most common treatment for West syndrome is medication. Your doctor may want to try: ACTH, a hormone; Vigabatrin (Sabril), a seizure...

West syndrome, also known as infantile spasms, is a rare but serious neurological disorder that affects infants and young children. The differential diagnosis of West syndrome involves identifying other conditions that may present with similar symptoms.

According to various medical sources [1-6], the differential diagnosis of West syndrome includes:

• Benign myoclonus of early infancy: This condition presents with brief, shock-like muscle contractions that are often mistaken for infantile spasms.
• Sleep myoclonus: A type of myoclonic seizure that occurs during sleep and can be misdiagnosed as West syndrome.
• Tonic reflex seizures of early infancy: These seizures involve a sudden stiffening of the body and can be confused with infantile spasms.

Other conditions that may mimic the symptoms of West syndrome include:

• Dravet syndrome: A rare genetic disorder that causes severe epilepsy, developmental delays, and other neurological problems.
• Benign familial infantile seizures: A rare condition characterized by brief, shock-like muscle contractions that are often mistaken for infantile spasms.
• Benign sleep myoclonus: A type of myoclonic seizure that occurs during sleep and can be misdiagnosed as West syndrome.

It's essential to note that a proper diagnosis of West syndrome requires a comprehensive evaluation, including medical history, physical examination, and diagnostic tests such as electroencephalography (EEG) and imaging studies [1-6].

References:

[1] by N Fejerman · 2013 · Cited by 7 — West syndrome has been clearly defined as the association between infantile spasms with an electroencephalographic pattern of hypsarrhythmia.

[2] West syndrome is a constellation of symptoms characterized by epileptic/infantile spasms, abnormal brain wave patterns called hypsarrhythmia and intellectual ...

[3] by DS Takacs — Clinical course · DIFFERENTIAL DIAGNOSIS · Benign myoclonus of early infancy · Sleep myoclonus · Tonic reflex seizures of early infancy.

[4] Differential diagnosis. Brief paroxysmal events which occur in clusters, both epileptic and non-epileptic, should be differentiated from epileptic spasms.

[5] Aug 21, 2024 — Traditionally, West syndrome is defined by a triad of epileptic spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia, ...

[6] by MRCH Portal — Differential diagnosis: Non-epileptic episodes such as shuddering and benign myoclonus of infancy; Benign myoclonic epilepsy of infancy; Other infantile ...