What is Dejerine-Roussy Syndrome (Post-Stroke Pain)?
Central post-stroke pain is a rare type of pain that comes from damage to the nerves in the brain. It’s also known as Dejerine Roussy syndrome or thalamic pain syndrome. This condition usually happens after a specific part of the brain, called the ventroposterolateral thalamus, has a stroke or is damaged. Joseph Jules Dejerine and Gustave Roussy, two medical researchers, first wrote about this syndrome in 1906, and it was named after them after they died. The thalamus, where this condition is centered, is the brain’s communication hub for the sense of touch. However, any injury disrupting the spinal cord’s messages to the thalamus, which can happen in different parts of the brain including the subcortical area, capsular region, lower brain stem, and lateral medulla, can cause the symptoms of Dejerine-Roussy syndrome. This is also known as “pseudo-thalamic” pain. However, most professionals now use the term central post-stroke pain to talk about this type of nerve pain after a stroke, because not all central pains can be categorised as thalamic syndrome.
What Causes Dejerine-Roussy Syndrome (Post-Stroke Pain)?
Any damage or disease affecting the part of the central nervous system responsible for feeling pain can trigger these symptoms. This usually follows a lack of blood supply to the brain, known as ischemic stroke. However, the pain can also occur after a stroke caused by bleeding in the brain, either inside the brain tissue (intracerebral) or in the space around the brain (subarachnoid hemorrhage).
The arrangement of nerve fibers to the face, arm, trunk, and leg in a particular region of the brain, called the ventral posterior nucleus of the thalamus, makes it possible for this kind of stroke to precede the pain in the legs. People who have recently had a large stroke often experience this type of pain more frequently.
However, there isn’t any definitive finding on the brain scans of patient with this type of pain after stroke. When these patients’ brains are examined with MRI, areas of dead tissue (infarctions) are usually seen, suggesting a stroke. An imaging scan that measures blood flow, called a single-photon emission computed tomography, typically shows reduced blood flow, particularly to the left side of the thalamus.
Risk Factors and Frequency for Dejerine-Roussy Syndrome (Post-Stroke Pain)
After having a stroke, some people may start feeling pain. This can happen anywhere from a few days to several years after the stroke, but generally tends to start within the first six months. For some people, it might even take up to ten years for the pain to develop. However, the occurrence of this pain after a stroke varies widely: between 11% and 85% of stroke patients have reported changes in perception of pain and sensations, while the occurrence of characteristic central post-stroke pain fluctuates between 8% and 46%, according to historical studies.
A specific type of stroke, called the lateral medullary syndrome (or Wallenberg syndrome), sees a high percentage of sufferers of central post-stroke pain, with a quarter of such cases occurring within six months. In fact, such pain often develops just 4 weeks after the stroke, although it could take up to 24 weeks in some cases.
It’s important to note that measuring the exact number of people suffering from central post-stroke pain can be difficult. Other types of chronic pain, like shoulder pain, shoulder muscle stiffness, tension-type headaches, or joint pain (knees and hips), often occurring concurrently in stroke patients, can complicate the identification of the central post-stroke pain.
Lastly, it’s crucial to understand that anyone can suffer from central post-stroke pain. It does not depend on factors like the patient’s age, gender, or which side of their body was affected by the stroke.
Signs and Symptoms of Dejerine-Roussy Syndrome (Post-Stroke Pain)
Central post-stroke pain is a type of discomfort that often impacts the areas of the body affected by a stroke. This pain can feel sharp, like a burning sensation, or stabbing, and can range from mild to moderate intensity. Patients often find it hard to describe the exact nature of their pain, but it has been variously described as achy, prickly, sharp, or similar feelings. Some people even describe their pain as “troublesome,” “annoying,” or “tiring.” It’s important to note that over 90% of these patients also experience issues with either their ability to feel pain or temperature changes. Feeling vibrations or changes in touch sensation is less common.
Central post-stroke pain can be divided into three categories:
- The first is a constant pain that is often described as burning, aching, prickly, freezing, and squeezing.
- The second category, which appears in about 15% of cases, is spontaneous and intermittent. The pain intensity fluctuates and lasts anywhere from a few seconds to minutes, and is described as shooting and sharp. It usually comes and goes throughout the day, with periods of relief lasting a few hours.
- About two-thirds of patients experience a third type of pain. This is provoked by touch or pressure and leads to increased sensitivity to pain or touch, otherwise known as hyperalgesia, hyperesthesia, or allodynia.
Patients might experience one or more of these pain types.
Testing for Dejerine-Roussy Syndrome (Post-Stroke Pain)
The diagnosis of central post-stroke pain typically involves the following steps:
First, understanding the patient’s medical history is vital. This involves gathering information regarding the pain like its onset, location, intensity, duration, and any aggravating factors. Additionally, a detailed physical and sensory examination has to be performed to not only trace the abnormal sensory pattern but also to exclude other possible causes of the pain.
Secondly, imaging tests are used. This may include getting a brain CT scan or an MRI scan without contrast. These tests are helpful for confirming if a stroke happened, and also for determining the location and size of any brain lesions, if present.
Experts Dr. Henriette Klit, Dr. Nanna B. Finnerup and Dr. Troels S. Jensen suggest some criteria for diagnosing central post-stroke pain. These are divided into mandatory and supportive categories.
The mandatory conditions include pain that corresponds to an area affected by a nervous system lesion, a history that’s suggestive of stroke followed by the onset of pain, evidence of a central nervous system lesion through imaging or sensory signs confined to an area affected by the lesion, and ruling out other causes of pain.
Additional supportive conditions can include pain not primarily linked to movement, inflammation or other tissue damage, and specific sensory descriptors. Patients may describe the pain as burning, aching, or feeling like electric shocks or pins and needles, among other descriptors. Furthermore, they may experience pain when touched or when exposed to cold (a condition known as allodynia or dysaesthesia).
Treatment Options for Dejerine-Roussy Syndrome (Post-Stroke Pain)
Managing pain following a stroke often requires a team of medical professionals and a variety of treatments. These include medicines and other approaches.
Dr. Henriette Klit and his team suggest a method that proceeds in stages.
Antidepressants
Research indicates that amitriptyline (at a 75 mg dosage) can be more effective than carbamazepine, a medicine often used to treat nerve pain. The treatment typically starts with a low dose of 10 to 20 mg per day, slowly increasing each week until the pain lessens and the patient doesn’t experience side effects. Pain relief usually occurs 4 to 7 days after reaching this optimal dose. However, research hasn’t been conducted yet on the effects of selective serotonin reuptake inhibitors, a type of antidepressant, on post-stroke pain.
Anticonvulsants
If antidepressants aren’t successful in managing the pain, a medicine often used for seizures, such as carbamazepine, can be added to the treatment plan, especially if the pain is severe and sudden. Carbamazepine treatment starts at 100 mg per day (average dose is 800 mg per day), with the dose gradually increasing until the pain improves or side effects become too uncomfortable. Common side effects include drowsiness and dizziness. Gabapentin, another medication, is effective for neuropathic pain, the type of pain caused by damage to nerves, especially when the pain varies in intensity or worsens due to temperature changes. Long-term use of this medicine may lead to weight gain. Lamotrigine, another medication, has been found to moderately effective for post-stroke pain.
Opioids
If patients don’t respond to antidepressants and anticonvulsants alone or together, opioids may be the next step. Tramadol, for example, has been found effective for chronic post-stroke pain.
N-methyl D-aspartate Receptor Antagonist
Ketamine, taken orally three times per day, together with diazepam, can be effective. Ketamine can also be given intravenously for more severe cases of post-stroke pain.
Different Non-Pharmaceutical Techniques
Many non-pharmaceutical treatments are also available, but their effectiveness varies. These include repetitive transcranial magnetic stimulation, a noninvasive technique that stimulates the brain and offers long-lasting effects. Other options include electrical nerve stimulation, particularly when combined with social support and patient education, and deep brain stimulation, although this option is limited as it could potentially cause further brain damage including worsening the pain.
Future of Post-Stroke Pain Management
Other treatments, including special diets, medicines that prevent blood clots, and certain types of brain stimulation, may play a role in future treatments. Therapies involving behavior, coping strategies education for patients and families, and treating depression are also considered important. It is also recommended that patients with post-stroke pain participate in rehabilitation for the treatment of any additional health issues.
What else can Dejerine-Roussy Syndrome (Post-Stroke Pain) be?
When a patient is experiencing certain symptoms, doctors need to consider a variety of illnesses to ensure they arrive at the right diagnosis. Below are a few illnesses that share some symptoms:
- Herniated disc in the neck
- Multiple sclerosis (a disease affecting the brain and spinal cord)
- Syringomyelia (a rare disorder where a fluid-filled cyst forms within the spinal cord)
- Conversion disorder (a mental condition where a person has blindness, paralysis, or other nervous system symptoms that cannot be explained by medical evaluation)
The common thread among these conditions is that they can cause similar symptoms. This is why a thorough medical examination is crucial to ensure the correct diagnosis is made.
What to expect with Dejerine-Roussy Syndrome (Post-Stroke Pain)
Central post-stroke pain is a type of pain that continues to exist and might last for a person’s entire life.
Possible Complications When Diagnosed with Dejerine-Roussy Syndrome (Post-Stroke Pain)
These are the potential effects and risks associated with a specific health condition:
- Degrades the quality of life
- May result in depression or anxiety
- Can cause disturbances in sleep
- Increases likelihood of dependence on drugs
- Poor interaction with other people
- May lead to self-harm
- Increases the risk of suicide