Alternating Hemiplegia of Childhood

Introduction

Alternating hemiplegia in childhood is a neurological disorder that results in recurrent temporary paralysis, usually on one side of the body called hemiplegia. Paralysis usually occurs in one side of the body and can shift from one side to another. This typically occurs in the infancy stage before 18 months and can persist for about a few minutes to days. Along with paralysis, it can also result in some uncontrolled muscle movements like choreoathetosis (limb movements), dystonia (tension in the muscle), nystagmus (eye movements), or shortness of breath. The affected child can also experience flushing (warmth and redness) or pallor of the skin.

A typical feature of this condition is that when the affected child is asleep, all the signs and symptoms disappear, but these reappear in a short time once the child is awake. The frequency and duration of these episodes are more during the initial stages, which reduces gradually over time. The occurrence of episodes persists throughout life even though the frequency reduces, but the uncontrolled movements of the muscle cease completely. Read the article to know more.

What Are the Factors That Trigger Hemiplegia in a Child?

The triggering factor of alternating hemiplegia is not always certain, but some common factors which trigger hemiplegia are:

• Stress.

• Tiredness.

• Cold temperature.

• Bathing.

What Are the Other Complications Caused by Alternating Hemiplegia?

Alternating hemiplegia in childhood is a rare condition that tends to mimic other conditions, such as epileptic seizures (it is a condition of the brain which is characterized by repeated seizures). The stiffness caused due to alternating hemiplegia is similar to an epileptic seizure, where alternating hemiplegia of childhood is misdiagnosed as a seizure many times. Paralysis is misunderstood to be the weakness of the muscle, which usually occurs after an episode of a seizure.

Alternating hemiplegia in childhood usually starts in infancy and persists life long. This condition can also cause:

• Developmental Anomalies: Alternating hemiplegia in childhood can have an impact on learning, movement, and psychological development.

• Motor Control Spells: Spells occur as frequent episodes of paralysis and stiffness; very rarely, they are intermittent.

• Damages to Other Systems: Alternating hemiplegia in childhood also affects other systems like the heart, gastrointestinal tract, and sleep pattern.

What Are the Signs and Symptoms of Alternating Hemiplegia in Childhood?

Some common symptoms of alternating hemiplegia in childhood are:

1. Abnormality of the gastrointestinal tract.

• Episodic Hemiplegia: Alternating hemiplegia in childhood is characterized by the frequent recurrence of one-sided paralysis called hemiplegia, which can also alter from one side to the other or can affect both sides at the same time.

• Gastrointestinal Dysmotility: Gastrointestinal dysmotility is a condition where the muscles that line up the digestive system get damaged and alter speed and coordination.

• Abdominal Distension: When there is swelling of the abdomen beyond its normal size, it is called abdominal distension. Few people misinterpret it as bloating caused due to gas or digestive contents.

• Autonomic Dysfunction: When the autonomous nervous system responsible for regulating involuntary functions such as heart rate, blood pressure, sweating, and balance does not function properly, it is called autonomic dysfunction.

• Abnormal Movements of Eyes: When there are abnormal movements of eyes that occur when the subject does not want them to happen.

• Anorexia: Anorexia is an eating condition where people are more concerned about their weight. People with this condition always want to maintain less weight which is considered unhealthy for their age, the height, which is achieved through excessive weight loss.

• Ataxia: Damage in the brain and nerves result in impaired balance, speech, and coordination.

2. Other Abnormalities:

• Intellectual disability.

• Movement disorder.

• Abnormal movement of the eyes.

• Dystonia.

• Choreoathetosis.

What Are the Causes of Alternating Hemiplegia in Childhood?

Alternating hemiplegia in childhood is a genetic disorder, which means one or more genes are not functioning properly. Alteration in a specific gene like ATP1A3 can result in this condition. Most of the affected people, almost 80 percent, have mutated ATP1A3 gene, which is considered to be a new mutation, meaning this does not follow in the families. In very rare cases, the mutation in some genes like ATP1A2 or RhoBTB2 can result in alternating hemiplegia. In very rare cases alternating hemiplegia is caused due to no gene mutation. In such cases, the cause is unknown. Even though it is a genetic condition, it does not run in families.

How Is Alternating Hemiplegia in Childhood Diagnosed?

Some common criteria for diagnosing this conditions are:

• Signs and symptoms before 18 months of age.

• Frequent occurrence of hemiplegia, which alters from one side to the other.

• Occurrence of hemiplegia on both sides at the same time.

• The signs and symptoms cease while the child is sleeping and reoccur upon waking up.

• Occurrence of other disturbances like involuntary movements of eyes, weakening of muscles, or autonomic symptoms.

• Developmental delay.

• Neurological issues.

The tests that are performed to confirm this condition are:

• Genetic Tests: Genetic testing is done to rule out the presence of any mutation of the ATP1A3 gene, which is the main cause of this condition. This testing also helps to rule out other genetic mutations associated with neurological conditions.

• Electroencephalogram (EEG): EEG helps to measure the brain’s electrical activity and also helps to rule out epilepsy.

• Electrocardiogram: Electrocardiogram helps to find any abnormalities in the heartbeat.

• Sleep Studies: Sleep studies are conducted to find any sleep abnormalities like sleep apnea, central sleep apnea, etc.

• Image Testing: MRI (magnetic resonance imaging) can be used to rule out any structural abnormalities of the brain which may be a causative factor for the occurrence of paralysis and muscle stiffness.

How Can Alternate Hemiplegia in Childhood Manage?

There is no permanent cure for this condition, but certain medications and treatments help to manage the signs and symptoms caused. Some medications recommended are:

• Flunarizine is a medication that is used to manage the frequency and severity of paralysis, but this is not FDA (Food and Drug Administration) approved. Alternative medications are recommended to help manage the symptoms by inducing sleep when the signs and symptoms cease.

• Additional medication should be provided if the child experiences a seizure.

Some other methods to manage alternating hemiplegia are:

• Vagus Nerve Stimulation: People affected by alternating hemiplegia are more prone to experience epilepsy and seizure, which are unmanageable. Vagus nerve stimulation is a device that is implanted in the vagus nerve, which helps to control drug-resistant epilepsy.

• Ketogenic Diet: A diet that has a low content of carbohydrates and high content of fat is seen to reduce the intensity of seizures and epilepsy in children.

• Physical, Occupational, or Speech Therapy: People affected by this condition require physical, occupational, or speech therapy to enhance strength, balance, mobility, etc. Speech therapy is used for children who find it difficult to eat, speak, or swallow.

Conclusion

Alternating hemiplegia in childhood is a genetic condition that results in the occurrence of paralysis on one side of the body, which persists for some minutes or days. In some cases, the paralysis may shift to the other side. This is a genetic condition that is caused due to mutation of a gene. This condition does not have a permanent cure, but medication and other treatment modalities can help to manage the signs and symptoms.