Lance-Adams Syndrome: Symptoms, Diagnosis, and Treatment

Introduction:

Lance-Adams syndrome is a rare neurological condition that is characterized by involuntary jerking movements that occur days or weeks after cardiopulmonary resuscitation. Cardiopulmonary resuscitation is a lifesaving approach that involves fast and hard chest compressions on a person whose breathing or heartbeat has stopped due to a heart attack or drowning.

What Is Lance-Adams Syndrome (LAS)?

Lance-Adams syndrome (LAS) is a rare complication of cardiopulmonary resuscitation. It is accompanied by sudden, brief, involuntary, and shock-like body jerks. Lance-Adams syndrome is seen in patients who have regained consciousness after medical emergencies like a heart attack or being drowned in water. Usually, the involuntary jerks seen in Lance-Adams syndrome are triggered intentionally or by external stimuli. The involuntary jerking movements are stopped when the patient is asleep or under rest. Lance-Adams syndrome is different from post-hypoxic seizures, which take place when the patient is completely unconscious. Post-hypoxic seizures are seizures (sudden uncontrolled brain disturbances) that result from reduced oxygen supply to the brain.

What Is Posthypoxic Myoclonus (PHM)?

Posthypoxic myoclonus is a neurological complication that involves uncontrollable jerks following a hypoxic brain injury (brain injury due to lack of oxygen). Posthypoxic myoclonus is of two types: acute and chronic. Acute posthypoxic myoclonus occurs within twelve hours of hypoxic brain injury and is seen in completely unconscious patients. The chronic post-hypoxic myoclonus occurs days or weeks after hypoxic brain injury and is seen in patients who are conscious. The chronic variant of post-hypoxic myoclonus is also known as Lance-Adams syndrome.

What Are the Causes of Lance-Adams Syndrome?

The exact cause of Lance-Adams syndrome has not been found. However, researchers suggest that it occurs following a hypoxic brain injury. Hypoxic brain injuries are injuries of the brain which are caused due to restricted oxygen supply to the brain. Reduced oxygen supply to the brain leads to damage and death of the brain cells. The different causes of hypoxic brain injuries are as follows. They are

• Cardiac arrest (sudden stoppage of heart functioning and breathing accompanied by loss of consciousness).

• Strangulation or suffocation.

• Untreated low blood pressure.

• Untreated cases of severe anemia (a condition which is characterized by the lack of potent red blood cells in the blood to carry the required amount of oxygen to the body tissues).

• Drug overdose (misusing the prescribed dosage of drugs intentionally or using illicit drugs).

• Drowning.

• Electrocution.

• Smoke inhalation for a prolonged period of time.

• Carbon monoxide poisoning.

• Lung injury.

• Severe traumatic head injuries.

• Injuries caused due to bullets and explosions that may trigger bleeding by damaging the blood vessels.

• Severe asthmatic attacks.

• Certain surgical complications or problems that are caused due to anesthesia.

• Stroke (blockage in the blood supply to the brain or bursting of a blood vessel inside the brain).

What Are the Symptoms of Lance-Adams Syndrome?

There is no specific group of symptoms to identify Lance-Adams syndrome. However, the common features seen in patients with Lance-Adams syndrome are as follows. They are

• Repeated sudden shock-like jerking movements of the legs and hands.

• Difficulty in speaking.

• Lack of coordination.

• Loss of balance.

• Abnormal walking style.

• Difficulty in performing simple tasks like eating, buttoning the shirt, and writing.

• Change in levels of consciousness.

• Behavioral disturbances.

• Frequent falls.

• Difficulty in understanding spoken or written language.

• Poor memory.

• Difficulty in swallowing.

• Abnormal eye movements.

• Inability to pay attention or focus on one particular stuff.

• A feeling of tightness around joints and muscles which may be painful.

• Loss of bladder control (sudden urge to urinate).

• Difficulty in breathing.

How to Diagnose Lance-Adams Syndrome?

There is no specific test to diagnose Lance-Adams syndrome. However, certain tests help in finding out Lance-Adams syndrome by ruling out the possibilities of other conditions which have similar symptoms. They are as follows:

• The doctor makes a detailed examination of the jerking movements when the patient is completely conscious, with the postural imbalance and lack of coordination.

• Single Photon Emission Tomography (SPECT) Scan:

A single photon emission tomography scan of the brain helps in obtaining a three-dimensional image showing the blood flow activity inside the brain. This helps to identify the areas inside the brain which are having abnormalities in the blood supply. SPECT scans of patients with Lance-Adams syndrome may show areas with deficient blood flow in the brain.

• Computed Tomography (CT) Scan and Magnetic Resonance Imaging (MRI):

Computed tomography of the brain is an imaging technique that uses special X-rays and a computer to obtain images of various views of the brain. A CT scan of the brain helps to find any abnormal growth, bleeding, injuries, and other brain infections. Magnetic resonance imaging is a painless procedure that uses radio waves, a large magnet, and a computer to obtain detailed images of the brain. MRI of the brain gives detailed information about any abnormalities present in the brain and is considered to give more precise results than a CT scan. Normally MRI and CT scan does not show many changes in a person with Lance-Adams syndrome. However, these imaging techniques help to rule out the possibilities of other neurological conditions with similar symptoms.

• Electroencephalogram (EEG):

An electroencephalogram is a test that is used to measure the electrical activity of the brain using metal discs attached to the scalp of the patient. EEG helps to detect the areas of brain abnormalities like seizures, swelling, and injuries. Even though EEG is not much use in the diagnosis of Lance-Adams syndrome, in some cases, it helps to find out the areas of abnormal activity inside the brain.

How Is Lance-Adams Syndrome Treated?

There is no established treatment for Lance-Adams syndrome. However, some treatment options are being carried out to make the patient get relief from certain symptoms of the disease. They are as follows:

• Medications to stabilize the uncontrolled electrical activity of the brain are given to the patient.

• Medications to control the restlessness of the legs, behavioral disturbances, and to improve learning skills have shown effectiveness in some patients.

• Physical therapy has shown effective results in a few patients. It is a therapy in which trained professionals help the patients to improve their body movements and overcome their disabilities to lead a better life through certain exercises.

• In patients who are prone to frequent falls, additional supportive devices like a wheelchair that aid in the movement of patients is to be used.

Conclusion:

Early recognition and accurate diagnosis followed by proper management is the key to improving the symptoms experienced by the patient with Lance-Adams syndrome. Even though management of this condition is challenging, initiating medications and rehabilitation programs at the earliest helps the patient to lead a better life.