Hemiballismus

What is Hemiballismus?

Hemiballismus is a condition where a person can’t control their movements and make sudden, violent, and involuntary motions, often involving the same side of the body – arm and leg. This happens because of problems on the opposite side of the brain, in the part that controls our movements. It’s considered to be the most severe type within a group of similar conditions collectively known as chorea. Chorea is characterized by spontaneous, dance-like movements that affect one side of the body and aren’t repeated in a predictable pattern.

You might have heard that this kind of disorder is linked to a specific part of the brain called the subthalamic nucleus (STN) or the nucleus hypothalamicus/corpus Luys, especially if you’ve read medical textbooks. However, according to various research studies, the problem could also lie in other parts of the brain such as the basal ganglia and the caudate nucleus.

What Causes Hemiballismus?

Hyperkinetic syndromes, which involve too much uncontrolled movement, are typically caused by too much activity in the brain’s dopamine system and damage to parts of the brain called the basal ganglia. One specific type of this syndrome is called hemiballismus, and there are several ways it can happen:

• Brain hemorrhage
• Stroke (most common in patients over 65)
• Tumors
• Head injuries
• Trouble with metabolism (like unchecked type 2 diabetes)
• Brain infections (rare, but more often in younger people)
• Tuberculomas
• Complications from HIV that cause lesions in the basal ganglia
• Disorders where the body’s immune system attacks its own nerve tissues (like multiple sclerosis)
• Diseases that occur along with cancer (paraneoplastic syndromes)
• Vasculitis (like systemic lupus erythematosus)
• Toxic exposure to things like alcohol or heavy metals

Hemiballismus and chorea, which causes brief, irregular movements, are on a spectrum, with chorea being milder. Chorea can be caused by a variety of conditions, which range from inherited to those that develop later in life, and involve degenerative or developmental brain disorders. Some examples include diseases like Huntington disease, Wilson disease, or benign hereditary chorea. There’s also chorea that’s a secondary condition to metabolic disorders, exposure to dopamine-increasing drugs, Sydenham chorea that comes about from rheumatic fever in children, vitamin B1/B12 deficiency, toxic exposure, and pump-head syndrome, which can happen after certain types of surgery.

Risk Factors and Frequency for Hemiballismus

Ballismus and chorea syndromes, which involve abnormal body movements, are considered rare. It’s tricky to figure out exactly how many people have these ailments worldwide, but it’s thought to be about 1 to 2 people per million. These syndromes are often a rare side effect of other conditions. For those wanting to dive deeper, you can find more detailed information about both inherited and acquired hyperkinetic syndromes in medical literature.

Signs and Symptoms of Hemiballismus

Hemiballismus and hemichorea are conditions that cause involuntary, jerky, high-intensity movements on the side of the body opposite to the affected part of the brain. Aside from these movements, these conditions can present other symptoms too, especially when they are caused by a stroke or brain hemorrhage. These symptoms can include uneven pupil size, droopy eyelid, drooping of one side of the face, difficulty in speech, and headaches. In the case of a brain tumor, patients can become confused or disoriented and may struggle to follow instructions. Moreover, the tumor can lead to motor and sensory deficits depending on its size.

For younger individuals, it’s important to consider infection-related and inflammation-related causes. Information about risk factors, previous neurological events, associated health conditions, sexual history, and family history should be gathered. Additionally, information regarding food habits, work environment, and travel history might also be useful. Checking the patient’s medication history can help to investigate if a drug may have triggered their movement disorder.

A thorough neurological examination can also provide important clues. This should include a check of cognitive functions, cranial nerves, neck stiffness, motor tone, sense of touch, balance, and walking ability. In some cases, gathering information from family members could be necessary to complete the patient’s health history.

Testing for Hemiballismus

Hemiballismus, a condition that causes involuntary movements, can be due to a variety of disorders. A detailed medical history and physical examination by your doctor can help narrow down the possible causes. For older patients who have multiple risk factors, such as high blood pressure, diabetes, or a history of strokes, more advanced imaging, like an angiogram, might be needed to check for bleeding or a stroke. If a stroke is found, additional tests will be done to understand more about it.

Depending on what the doctor is suspecting could be the cause of the symptoms, they may do thorough routine blood tests. This could include a check on your overall metabolism and complete blood count, your long-term blood sugar levels (hemoglobin A1C), or any inflammation in your body (ESR and CRP). They can also look for risk of autoimmune diseases (with tests like ANA, ENA), deficiencies or excesses in certain vitamins or minerals (like B1, B12, or copper), exposure to heavy metals, drug use, or risk of HIV. If HIV is suspected, extra checks for infections related to a lowered immune system might be performed. Additionally, they can check for specific markers related to diseases like lupus (C3/C4, anti-histone, etc.) or other inflammation-related diseases (ANCA).

Your doctor might also use imaging technologies, such as a brain MRI, CTA, MRA, or DSA to evaluate for autoimmune diseases, physical abnormalities, or cancers.

If your doctor suspects an infection (such as bacterial, viral-HIV, or fungal-toxoplasmosis), or an inflammatory condition related to cancer, a lumbar puncture (a needle in the lower back to collect spinal fluid) could be performed. This can help your doctor to look for certain markers in the spinal fluid which could indicate these types of conditions. Similarly, if there is a suspicion of a hereditary cause, genetic tests might be performed to confirm this.

Treatment Options for Hemiballismus

Hemiballismus is a condition that causes uncontrollable, violent movements on one side of the body; treating the underlying cause is an important step in managing this condition. Over time, hemiballismus may improve naturally, so it’s important to manage any other health conditions the patient may be dealing with.

Medical treatment focuses on reducing these severe movement symptoms. This often involves using a variety of medications initially developed for other purposes. For example, the first and second-generation antipsychotic medicines, which work by impacting a certain type of receptor (D2) in the brain. Some of those medicines include risperidone, haloperidol, perphenazine, pimozide, and chlorpromazine. Other commonly used medications are benzodiazepines like clonazepam, anti-seizure drugs like topiramate, and a medicine called tetrabenazine, which were all found to ease severe hyperkinetic (uncontrolled) movements.

If these medical treatments don’t work, especially when the symptoms significantly affect daily life, surgery might become necessary. The preferred surgical procedure is called a ‘stereotactic posteroventral pallidotomy’. It reduces activity in a specific area of the brain, which in turn relieves the uncontrolled movements. Special equipment, such as micro-recorders and intraoperative monitors, is used during surgery to ensure accuracy. Using an intrathecal Baclofen has also shown promise as a treatment, particularly in cases of hemiballismus caused by injuries (post-traumatic).

However, deep brain stimulation — a procedure where electricity is used to stimulate parts of the brain — is not recommended for treating hemiballismus.

What else can Hemiballismus be?

It’s crucial not to mistake hemiballistic movements with other excessive movement disorders. Hemiballismus and chorea are types of movement disorders, and a thorough medical history and physical examination can help narrow down the diagnosis. This will then inform the neurologist on the type of tests to be conducted, which may include blood tests, brain scans, and sometimes studies on spinal fluid. Let’s look at several disorders that could present symptoms similar to hemiballismus:

• Side effects from medications or treatments
• Conditions related to blood vessels
• Conditions resulting from injury
• Infections
• Autoimmune conditions causing inflammation
• Brain tumors, primary or secondary
• Deficiencies in Vitamin B1 and B12
• Exposure to toxins, like alcohol or heavy metals
• Inherited conditions
• Metabolic disorders

What to expect with Hemiballismus

Hemiballismus, a condition characterized by sudden violent, flinging movements of the limbs, generally has a favorable outcome as symptoms often subside within a few weeks after starting treatment for conditions that can be addressed. However, for cases that do not respond to initial treatments, surgical intervention may become necessary.

Possible Complications When Diagnosed with Hemiballismus

Complications from treating hemiballismus, a condition resulting from damage to certain parts of the brain called the inhibitory pathways in the basal ganglia, are quite rare. This is mainly because the condition tends to improve on its own over time with proper symptomatic treatment. Importantly, treating the root cause is crucial, particularly in acute and serious conditions like an ischemic stroke. Doing so can prevent additional complications and the worsening of the patient’s physical condition.

• Hemiballismus tends to improve over time with proper symptomatic treatment
• Complications from treatment are rare
• Treating the root cause, especially in serious conditions like an ischemic stroke, can prevent additional complications
• Addressing the underlying issue is key to avoiding a worsening of physical condition

Preventing Hemiballismus

Hemiballismus, a condition characterized by involuntary flinging movements of the body, can have different causes depending on any underlying health problems. These may include brain injuries, stroke, cancer, metabolic issues, systemic or brain infections, autoimmune diseases, genetic disorders, side effects of certain medications, vitamin deficiencies, or exposure to harmful substances. Treating these root causes are extremely important. Medications to reduce the uncontrollable movements are sometimes recommended, while surgery is seldom considered.

It’s comforting to know that hemiballismus typically improves over time. Due to the emotional impact of dealing with such a condition, it may be necessary to consult with a social worker or psychologist to help manage the emotional stress associated with this condition.