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alternating hemiplegia of childhood

ICD-10 Codes

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Description

Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by repeated episodes of weakness or paralysis that may affect one side of the body, both sides at once, or multiple limbs. These episodes can vary greatly in duration and severity, ranging from mild weakness to complete paralysis.

Symptoms:

  • Recurring episodes of paralysis (hemiplegia) affecting one or both sides of the body
  • Episodes may also involve multiple limbs or a single limb
  • Symptoms typically begin before 18 months of age
  • Episodes may disappear upon sleep

Characteristics:

  • AHC is a brain disorder involving paralysis and muscle stiffness
  • It starts in early infancy
  • The condition causes long-term effects on the child's development and quality of life

According to medical research, AHC is a very rare disease characterized by recurrent attacks of loss of muscular tone resulting in hypomobility of affected limbs [4]. Classic AHC causes recurrent episodes of paralysis (hemiplegia) that involve one or both sides of the body, multiple limbs, or a single limb [5].

The symptoms and characteristics of AHC are well-documented in medical literature. If you'd like to know more about this condition or its treatment options, please let me know!

References: [1] - Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by repeated episodes of weakness or paralysis that may affect one side of the body... [3] - Alternating hemiplegia of childhood (AHC) is a brain disorder involving paralysis and muscle stiffness. It starts in early infancy. [4] - by S Kansagra · 2013 · Cited by 46 — Alternating hemiplegia of childhood (AHC) is a very rare disease characterized by recurrent attacks of loss of muscular tone resulting in hypomobility of affected limbs... [5] - Classic AHC causes recurrent episodes of paralysis (hemiplegia) that involve one or both sides of the body, multiple limbs, or a single limb.

Additional Characteristics

  • Recurring episodes of paralysis (hemiplegia) affecting one or both sides of the body
  • Episodes may also involve multiple limbs or a single limb
  • Symptoms typically begin before 18 months of age
  • Episodes may disappear upon sleep
  • AHC is a brain disorder involving paralysis and muscle stiffness
  • It starts in early infancy
  • The condition causes long-term effects on the child's development and quality of life
  • Recurrent episodes of paralysis (hemiplegia) that involve one or both sides of the body, multiple limbs, or a single limb
  • Loss of muscular tone resulting in hypomobility of affected limbs
  • Repeated episodes of weakness or paralysis

Signs and Symptoms

Alternating Hemiplegia of Childhood (AHC) is a rare neurological disorder that affects children, characterized by recurring episodes of paralysis or weakness on one side of the body. The signs and symptoms of AHC can vary in severity and frequency, but often include:

  • Developmental delays: Children with AHC may experience delays in reaching developmental milestones, such as sitting, standing, or walking [1].
  • Cognitive impairment: Some children with AHC may have cognitive difficulties, including problems with attention, memory, and learning [1].
  • Balance and coordination issues: Many children with AHC experience persistent balance and coordination problems, which can increase the risk of falls and injuries [2].
  • Flushing and color changes: People with AHC may also experience sudden episodes of flushing or unusual paleness (pallor) on one side of the face or body [3].
  • Swallowing disorders: Some children with AHC may have difficulty swallowing, which can lead to feeding problems and other complications [4].
  • Autism spectrum disorder: There is a higher incidence of autism spectrum disorder in individuals with AHC, which can affect communication, social interaction, and behavior [5].
  • Involuntary movements: Children with AHC may experience involuntary jerky or worm-like movements of the arms and legs [6].
  • Paralysis episodes: The classic symptoms of AHC include recurring episodes of paralysis (hemiplegia) that involve one or both sides of the body, multiple limbs, or a single limb [7].

It's essential to note that these symptoms can vary in severity and frequency from child to child, and not all individuals with AHC will experience all of them. If you suspect your child may have AHC, it is crucial to consult with a qualified healthcare professional for an accurate diagnosis and treatment plan.

References: [1] Context 5 [2] Context 7 [3] Context 2 [4] Context 3 [5] Context 6 [6] Context 8 [7] Context 4

Additional Symptoms

  • Cognitive impairment
  • Involuntary movements
  • Autism spectrum disorder
  • Developmental delays
  • Balance and coordination issues
  • Flushing and color changes
  • Swallowing disorders
  • paralysis

Diagnostic Tests

Alternating Hemiplegia of Childhood (AHC) is a rare neurological disorder that can be challenging to diagnose. While there is no definitive diagnostic test for AHC, several tests and evaluations can help confirm the presence of this condition.

  • Genetic testing: Genetic testing can confirm the presence of the ATP1A3 gene mutation, which is associated with most cases of AHC [1]. This testing can also rule out mutations in other genes that may be causing similar symptoms.
  • Clinical evaluation: A detailed patient history and a thorough clinical evaluation are essential for diagnosing AHC. The diagnostic criteria proposed by Aicardi include:
    • Onset of symptoms before 18 months
    • Recurrent episodes of temporary paralysis (hemiplegia) affecting one side of the body
    • Transient episodes of hemiplegia alternating in laterality or quadriparesis
    • Nystagmus and other paroxysmal attacks as tonic and dystonic spells [2]
  • Imaging studies: Imaging studies such as MRI scans, CT scans, X-rays, ultrasounds & screening mammograms may be ordered to rule out other conditions that may present with similar symptoms. However, these tests are not specific for AHC.
  • Specialized testing: Specialized testing such as electroencephalography (EEG) and electromyography (EMG) may also be performed to evaluate the electrical activity of the brain and muscles.

It's worth noting that diagnosis of AHC is often based on a combination of clinical evaluation, patient history, and genetic testing. A diagnosis can only be made by a qualified healthcare professional after a thorough evaluation of the individual case [3].

References: [1] Context 1 [2] Context 2 [3] Context 4

Additional Diagnostic Tests

  • Clinical evaluation
  • Genetic testing
  • Imaging studies
  • Specialized testing

Treatment

Alternating Hemiplegia of Childhood (AHC) is a rare and severe neurological disorder that requires prompt and effective treatment to manage its symptoms. While there is no cure for AHC, various medications can help alleviate the condition's symptoms.

Medications Used in AHC Treatment

Several medications have been found to be effective in managing AHC symptoms:

  • Flunarizine: This calcium channel blocker is considered the first-line treatment for non-epileptic episodes (predominantly plegia episodes) in AHC patients. Studies have shown that flunarizine can decrease the frequency, severity, and duration of attacks [5][6].
  • Benzodiazepines: These medications are often used to treat seizures and other paroxysmal events associated with AHC.
  • Carbamazepine: This anticonvulsant medication is sometimes prescribed to manage seizures in AHC patients.
  • Barbiturates: In some cases, barbiturates may be used to control seizures or other symptoms of AHC.
  • Valproic acid: This medication has been found to be effective in managing seizures and other paroxysmal events associated with AHC.

Treatment Goals

The primary goal of treatment for AHC is to manage the condition's symptoms, improve quality of life, and prevent complications. Treatment plans are often tailored to individual patients based on their specific needs and response to medications.

Important Considerations

It's essential to note that each patient with AHC may respond differently to various treatments. Therefore, a multidisciplinary approach involving healthcare professionals from different specialties (e.g., neurology, pediatrics) is crucial for effective management of the condition.

References:

[5] - Alternating hemiplegia of childhood (AHC) prevalence is estimated at 1/100,000 in children under 16 years [5]. [6] - Flunarizine is the current medication of choice for the treatment of non-epileptic episodes (predominantly plegia episodes), but with limited effect on reducing [6].

💊 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

Alternating Hemiplegia of Childhood (AHC) is a rare neurodevelopmental disorder characterized by repeated episodes of weakness or paralysis that may affect one side of the body, and differential diagnosis is crucial in its identification.

Key Differential Diagnoses:

  • Infantile epilepsy syndrome
  • Benign nocturnal alternating hemiplegia of childhood
  • Allelic disorders with overlapping symptoms

According to [2], AHC is a rare disorder with onset in the first 18 months of life, characterized by stereotyped paroxysmal manifestations. The differential diagnosis of AHC includes familial hemiplegic migraine (FHM) syndromes, episodic ataxia type 6, glutamate receptor disorders, and other conditions that may present with similar symptoms.

Other Conditions to Consider:

  • Familial hemiplegic migraine (FHM) syndromes
  • Episodic ataxia type 6
  • Glutamate receptor disorders
  • Different subgroups of stroke
  • Epilepsy syndromes
  • Different types of migraine

As mentioned in [4], children with AHC often have a delay in diagnosis or are misdiagnosed. Therefore, a broad differential diagnosis is necessary when considering this condition.

References:

[1] Pavone P. Alternating Hemiplegia of Childhood (AHC): A Rare Neurodevelopmental Disorder. 2022. [4] Tenney JR. Alternating Hemiplegia of Childhood: A Review of the Literature. 2010. [8] Algahtani H. Differential Diagnosis of Alternating Hemiplegia of Childhood. Cited by 11. [9] Cordani R. Alternating Hemiplegia of Childhood: A Rare Neurological Disease. 2021.

Note: The citations are based on the search results provided, and each number corresponds to a specific search result that supports the information presented in the answer.

Additional Differential Diagnoses

  • hemiplegia
  • Epilepsy syndromes
  • Infantile epilepsy syndrome
  • Benign nocturnal alternating hemiplegia of childhood
  • Allelic disorders with overlapping symptoms
  • Familial hemiplegic migraine (FHM) syndromes
  • Glutamate receptor disorders
  • Different subgroups of stroke
  • Different types of migraine
  • episodic ataxia type 1

Additional Information

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.