Alternating Hemiplegia of Childhood (AHC) Disease – Symptoms, Treatment

What is Alternating Hemiplegia of Childhood?

A neurological disorder known as “alternating hemiplegia of childhood” or AHC disease is characterized by recurrent episodes of momentary paralysis, frequently affecting one side of the body (hemiplegia). In certain cases, the paralysis affects both sides of the body simultaneously or alternately on one side of the body. These bouts normally start before the age of 18 months and persist for a few minutes to many days. It is a very rare condition that affects 1 in a million children.

AHC is generally diagnosed during early childhood, often before the age of 18 months. The exact cause of AHC is not fully understood, but it is believed to be related to dysfunction in the brain’s ion channels, which are responsible for regulating the flow of electrically charged particles (ions) in and out of nerve cells. This dysfunction disrupts the normal transmission of nerve signals and can lead to the characteristic symptoms of AHC.

What Leads to Hemiplegic Episodes?

Children’s hemiplegic attacks have various underlying causes. Attacks have been shown to be triggered by highly stressful activities, or upper respiratory issues. Brightness, weather, temperature fluctuations, and water exposure have an impact on children. Many attacks seem to have no purpose or reason.

Despite the fact that the phrase “of childhood” appears in the name AHC, there is no proof that a child will automatically become cured as they become older. As they grow older, many kids show an increased capacity to manage episodes or even avoid triggers, although this isn’t always the case.

Hemiplegic episodes in the context of Alternating Hemiplegia of Childhood (AHC) are thought to be caused by disruptions in the normal functioning of nerve cells in the brain. While the exact underlying mechanisms are not fully understood, researchers believe that the dysfunction of ion channels, which are responsible for regulating the flow of ions (charged particles) in and out of nerve cells, plays a significant role.
Here’s a general overview of the processes that contribute to hemiplegic episodes in AHC:

• Ion Channel Dysfunction: Mutations in specific genes that encode ion channels can disrupt the balance of ions, such as sodium, potassium, and calcium, across nerve cell membranes. These ion channels are crucial for generating and transmitting electrical signals (action potentials) between nerve cells. Dysfunctional ion channels can lead to abnormal firing of nerve cells and disruptions in communication between different parts of the brain.
• Impaired Nerve Signaling: The disrupted ion channel activity can lead to irregular patterns of nerve signaling in the brain. This can result in episodes of paralysis or weakness affecting one side of the body (hemiplegia). Depending on which areas of the brain are affected, these episodes may manifest as alternating hemiplegia, where the paralysis switches between the left and right sides of the body.
• Neurotransmitter Imbalance: The irregular nerve signaling caused by ion channel dysfunction can also impact the release and balance of neurotransmitters, which are chemical messengers that facilitate communication between nerve cells. This neurotransmitter imbalance can contribute to a variety of neurological symptoms, including seizures and movement abnormalities.

AHC Disease Symptoms

AHC in children has been associated with a variety of symptoms. These include:

• Seizures
• developmental delays
• mental disabilities
• tonic attacks (loss of muscle tone)
• dystonic postures (stiffening of the limbs)
• Nystagmus
• oculomotor abnormalities (eye problems).

Not all kids experience all of these related symptoms, and there is currently no proof that these symptoms are connected to or driven  by AHC.

What long-term Impacts does AHC Disease have?

Given its recent diagnosis, AHC would still be regarded as a “new” condition at this point. There is no evidence that the illness causes death or reduces life expectancy in any manner, but there isn’t enough supporting information. There is growing evidence that suggests AHC may have a progressive course and result in long-term neurological and mental impairments.

Alternating Hemiplegia of Childhood (AHC) is a complex and rare neurological disorder that can have significant long-term impacts on affected individuals. The severity and specific outcomes vary among individuals, but the disorder’s chronic and multifaceted nature can lead to various long-term challenges across different aspects of life. Some of the potential long-term impacts of AHC include:

• Motor Impairments: The alternating hemiplegic episodes and other movement abnormalities associated with AHC can lead to long-term motor impairments. These can affect mobility, coordination, and fine motor skills, which can impact activities of daily living and overall independence.
• Cognitive and Developmental Challenges: AHC can cause developmental delays, learning disabilities, and cognitive impairments. These challenges can persist into adulthood, affecting educational attainment, vocational opportunities, and overall quality of life.
• Speech and Communication Difficulties: Many individuals with AHC experience speech and language delays, making communication challenging. Speech difficulties can persist into adulthood, affecting social interactions, relationships, and participation in various settings.
• Seizures and Epilepsy: Seizures are a common feature of AHC and can continue to affect individuals throughout their lives. Managing seizures and epilepsy can impact daily activities, safety, and overall well-being.
• Behavioral and Emotional Issues: Behavioral challenges, mood disorders, and emotional difficulties can be long-lasting consequences of AHC. These issues can affect social interactions, relationships, and mental health.
• Sleep Disorders: Sleep disturbances are common in AHC and can lead to chronic sleep problems that impact overall health, cognitive functioning, and quality of life.
• Autonomic Dysfunction: Autonomic dysfunction can result in ongoing issues with body temperature regulation, breathing difficulties, and gastrointestinal problems.

Similarities with Autism and Cerebral Palsy

AHC patients have developmental delays, such as those in sitting, standing, walking, and the acquisition of social skills. Additionally, they have trouble speaking and understanding. Some children display symptoms like repeatedly asking the same question or making the same statement instead of responding. They frequently act rashly and are very busy. But unlike children with autism, they frequently interact with and even manipulate their carers, and given the correct circumstances, they are able to obey directions.

Children with AHC experience issues with mobility similar to CP, such as uncontrollable limb and body movements. Typically, people walk with their hands parallel to their chest in a dance-like motion. Some of them have uncontrollable walking movements or are unable to walk at all. When stimulated, they also show heightened reflexes. They struggle with chewing and swallowing food because of their reduced muscular tone and impaired motor coordination.

AHC Disease Treatment

As of right now, AHC disease is not specifically treated. Antiepileptic medications, however, are employed to lessen the incidence of movement disorder episodes. But most of the time, these medications are ineffective. Sleep is a key component of episode recovery. Therefore, the Neurologist may prescribe drugs to make you fall asleep, like Valium or Midazolam, or chloral hydrate.

In order to promote development and enhance quality of life, management of AHC involves maintaining the child’s nutritional level in a healthy state. Depending on the child’s development, physiotherapy, occupational therapy, speech therapy, and special education for learning difficulties may be provided.

Clinical trials and research will determine the future mostly. Numerous nations currently take an active role in AHC research. Recently, stem cell therapy has gained traction for managing various diseases like autism, cerebral palsy, or forms of ALS like anterior horns cell disease, etc. Therefore, it can be a hope for managing AHC pertaining to its regenerative properties.

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