Opisthotonus

What is Opisthotonus?

Opisthotonus is a condition where an unusual body posture is created due to extreme muscle contraction. This affects the muscles of the neck, back, and legs, leading to a major backward curve from the neck to the heel. In most situations, the person’s body raises a few inches off the floor. This condition can occur suddenly and might be consistent or come and go. Opisthotonus can be thought of as a specific type of posturing where the neck, back, and limbs are excessively extended back. It’s also sometimes called “arc de cercle.” In cases where the stomach muscles are more affected, the body might curve forwards, a situation referred to as emprosthotonus.

What Causes Opisthotonus?

Several conditions can cause general stiffness and spasms in your body, including tetanus, which often results in specific symptoms like arching of the back (opisthotonus), a stiff and grinning appearance (risus sardonicus), and difficulty opening the mouth (trismus). Noticeably, opisthotonus can present in many disorders and could sometimes be the only clear sign pointing to the underlying disease.

Infections like meningitis (an inflammation of the membranes covering the brain and spinal cord), encephalitis (an inflammation of the brain), tetanus, rabies, cerebral malaria (severe form of malaria affecting the brain), and neurosyphilis (a syphilis infection in the brain or spinal cord) can bring about these symptoms.

You can also experience these symptoms due to poisoning from substances like strychnine, phencyclidine, phenothiazines, lignocaine, methoxphenidine, bronchial inhalers taken excessively, and rarely propofol.

Certain developmental and metabolic conditions such as cerebral palsy (a condition affecting movement and coordination), Krabbe disease (a rare and often fatal disorder affecting the nervous system), hyperbilirubinemia (excess amounts of a substance called bilirubin in the blood), kernicterus (a severe form of jaundice), Gaucher disease (a rare genetic disorder), adenylosuccinate lyase deficiency (a rare metabolic disorder), and infant alcohol withdrawal syndrome (a condition newborns experience when the mother consumed large amounts of alcohol during pregnancy) could also lead to such symptoms.

Increased pressure within the brain due to conditions like intracranial hemorrhage (bleeding within the brain), subarachnoid hemorrhage (bleeding around the brain), hydrocephalus (a buildup of fluid in the brain), or a space-occupying lesion can trigger such symptoms.

Certain neurodegenerative syndromes which include neurodegeneration with brain iron accumulation, Wilson disease (a rare genetic disorder), cerebral palsy, or Parkinson disease along with neurometabolic disorders like maple syrup urine disease, glutaric aciduria, Lesch Nehan syndrome, dopa-responsive dystonias can also present these symptoms.

Psychogenic dystonia, a type of dystonia caused by mental or emotional processes, also known as hysteria, can result in these symptoms too.

Moreover, these symptoms can be seen after anoxic injury—lack of oxygen causing significant harm—such as after a near-drowning, cardiac arrest, suffocation, or birth asphyxia.

Lastly, miscellaneous conditions like stiff person syndrome, growth hormone deficiency, and Dandy-Walker malformation (a rare genetic brain malformation) could potentially cause similar symptoms.

Risk Factors and Frequency for Opisthotonus

Opisthotonus is a fairly rare condition that could be a symptom of several different health problems. It can affect people of all age groups, including infants, kids, and adults. It’s important to note that both males and females can experience it equally. Diagnosing opisthotonus can be particularly challenging among younger individuals. Since opisthotonus isn’t a disease or a disorder in itself, there isn’t any specific data about how often it occurs or how many people have it.

Signs and Symptoms of Opisthotonus

Opisthotonos is a condition where your back arches and your limbs extend rigidly due to intense and prolonged muscle contractions in your spine. This causes your head, neck, and trunk to bend backwards rigidly as both your ‘pushing’ and ‘pulling’ muscles contract at the same time.

The symptoms of this condition depend on what caused it. You might have a fever if it’s caused by infections like tetanus or meningitis, and a fear of water if it’s caused by rabies. Other symptoms could be a fast heartbeat, abnormal pupils, excessive sweating, heartbeat irregularities, changing blood pressure, and issues with breathing due to problems with your nervous system or muscles. It’s also possible to experience changes in your levels of awareness, vomiting, seizures, or to feel pain or discomfort because your muscles are contracting. Fatigue might also develop due to lack of food intake, increased muscle activity, fever, or vomiting. In children, diagnosis may be challenging as they might cry a lot, seem restless, or only have muscle contractions sometimes.

What symptoms you can expect, include:

• Back arching and limbs rigidly extending
• Fever (potential in cases of tetanus or meningitis)
• Fear of water (common with rabies)
• Fast heartbeat
• Issues with pupils
• Excessive sweating
• Irregular heartbeat
• Changing blood pressure
• Breathing issues
• Changes in awareness
• Vomiting
• Seizures
• Fatigue

Testing for Opisthotonus

Opisthotonus, a condition where spasms cause the body to arch with the head and heels touching the ground, can often be challenging to diagnose. This is because the symptoms are often quite general and can be linked to many different things, including infections, metabolic disorders, developmental issues, and neurological problems. Despite a physical exam revealing widespread stiffness and spasms, it could be hard to determine the exact cause without utilizing image testing and conducting blood tests.

Identifying the root cause of opisthotonus early is crucial, as delays in diagnosis could lead to severe health issues or even death. Trying to prevent whatever is causing the problem is also important. Because of this, collaborative care, where different health professionals work together, plays a critical role in providing efficient and effective health care. Although primary care providers are usually the ones who first screen for diseases, they may not always check for metabolic problems due to challenges like limited time and other priority health matters.

To confirm the underlying cause and monitor the disease, tests like full blood counts, metabolic profiles, and kidney and liver function will be conducted. Imaging tests such as brain MRIs or CT scans can also be done to detect any lesions that could be causing increased pressure within the skull (intracranial pressure). If these brain imaging results are normal, a lumbar puncture can be performed. This procedure involves drawing out a sample of spinal fluid, which is then tested to rule out infections.

Treatment Options for Opisthotonus

The best way to tackle opisthotonus, an extreme body tension condition, is to address the root cause of the problem. The cause could be anything from an infection to a disease like Parkinson’s or Gaucher disease. Therefore, doctors may provide treatments to fight against infections or manage other diseases present in the patient. Doing so can help alleviate the symptoms of opisthotonus.

Besides controlling the cause, doctors will also aim to support the overall health of the patient. This includes ensuring they stay hydrated, consume the right nutrients, and even provide breathing support if necessary. Also, creating a quiet and dark environment can help patients by reducing encounters with triggers such as loud noise or water sounds that can worsen their muscle spasms.

Medication can further help to control the symptoms of opisthotonus. For example, benzodiazepines and baclofen can be used to help relax the muscles. In some cases, baclofen can even be directly injected into the spinal canal to reduce muscle tension and stiffness, a condition often related to opisthotonus. Doctors may also provide medication for pain management. Anticholinergic drugs, which help to control saliva production and sweating, may further relieve patient discomfort by being administered through an injection into the muscle or the vein.

Beyond the treatment provided by healthcare providers, educating everyone involved in the patient’s care is vital. This includes the patients themselves, their caregivers, as well as other care providers. By recognizing the early signs and staying informed about opisthotonus, they can help manage the progression of the disease. This not only enhances the patient’s quality of life but can also prevent more severe health implications in older adults, particularly those who may forget to take their tetanus immunization, a significant preventive measure against a possible cause of opisthotonus.

What else can Opisthotonus be?

Here are some conditions that could be mistaken for each other due to similar symptoms:

• Dystonia
• Decerebrate posturing
• Hysteria
• Catatonia

What to expect with Opisthotonus

Opisthotonus, a condition where the body is abnormally tensed and arched, can be dangerous if not treated. Once the primary cause of this condition is managed or removed, the issue will typically resolve. However, the patient’s prognosis largely depends on the disorder causing it. 

For example, in the case of children with extreme muscle stiffness/dystonia, a treatment known as intrathecal baclofen can be used to decrease this stiffness and improve their condition. Similarly, if opisthotonus is caused by Parkinson’s disease, deep brain stimulation can provide good results, leading to a significant improvement in the patient’s quality of life.

Possible Complications When Diagnosed with Opisthotonus

Neurological problems are just some of the many issues that can occur. Other problems include extreme muscle pain and soreness, breathing difficulties, and trouble swallowing. Some people may encounter delayed development, malnutrition, and ulcers. Ongoing muscle stiffness, contractures (a condition where muscles or joints become tight or shortened), and scoliotic deformities (an abnormal sideways curve of the spine) are other potential problems. In the worst case scenarios, a person may fall into a coma or even die.

Possible Complications:

• Neurological problems
• Extreme muscle pain and soreness
• Difficulty breathing
• Swallowing problems
• Delayed development
• Malnutrition
• Ulcers
• Ongoing muscle stiffness
• Contractures (tight or shortened muscles or joints)
• Scoliotic deformities (sideways curve of the spine)
• Coma
• Death

Preventing Opisthotonus

Opisthotonus, a severe muscle spasm that causes an arching of the back, can be avoided by preventing the causes that might trigger it. To protect against one of these causes, neonatal tetanus, women are advised to get a tetanus vaccination during their pregnancy. This has greatly decreased the number of new cases of neonatal tetanus. Additionally, children should be given vaccines following current guidelines.

Another disease, meningitis, can be avoided by boosting immunity and getting vaccinations that can ward off certain bacterial infections. It’s also crucial to keep blood pressure and blood sugar at healthy levels.

General measures to maintain good health also play an important role. These include keeping hands clean, maintaining clean surfaces and environments, isolating sick individuals to prevent the spread of diseases, and quickly diagnosing any underlying illness.