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Dejerine-Roussy Syndrome - Causes, Symptoms, Diagnosis and Treatment

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Dejerine-Roussy syndrome is a rare neurological condition where a stroke in the thalamus results in excessive pain in response to mild stimulation.

Written by

Dr. K. Shobana

Medically reviewed by

Dr. Abhishek Juneja

Published At August 16, 2022
Reviewed AtMarch 28, 2023

What Is a Stroke?

A stroke occurs when blood flow to the brain stops. Even when blood flow stops for a short period, brain cells begin to die without blood or oxygen. Thus, brain functions are lost. The risk factors of stroke are smoking, diabetes, high blood pressure, heart disease, previous history of transient ischemic strokes, obesity, and lack of exercise.

What Is a Thalamic Stroke?

A thalamic stroke is a life-threatening form of intracranial hemorrhage (A severe medical condition where rupture of blood vessels in the brain causes bleeding in the brain).

  1. Thalamic stroke also occurs when there is restricted blood supply and oxygen to the lower part of the brain, known as the thalamus.

  2. Thalamic stroke also develops due to bleeding in the thalamus resulting from a severe injury.

  3. This stroke is also caused when profuse bleeding occurs from the ruptured artery that supplies oxygen and nourishment to the brain, including the thalamus.

What Is Dejerine-Roussy Syndrome?

Dejerine-Roussy syndrome is a rare neurological condition where the stroke occurring in a part of the brain called the thalamus develops excessive pain due to reduced sensation or mild stimulation. Face and limbs are the parts of the body that are commonly affected.

This syndrome is otherwise termed thalamic pain syndrome and central post-stroke pain syndrome. This syndrome was first described by Joseph Jules Dejerine, a French neurologist, and Gustave Roussy, a French neuropathologist, in 1906.

What Causes Dejerine-Roussy Syndrome?

Any condition that causes damage to the thalamus might cause Dejerine-Roussy syndrome. The conditions causing this syndrome are,

1. Parkinson’s disease.

2. Bleeding in the brain.

3. Disorders of the nervous system.

4. Circulatory system conditions.

Other conditions that can cause Dejerine-Roussy syndrome are head trauma, tumors, demyelination, and abscesses involving the thalamus. This syndrome can also be caused by hemorrhagic and ischemic lesions of the thalamus.

In ischemic causes of Dejerine-Roussy syndrome, the thalamogeniculate artery, a branch of the posterior cerebral artery supplying the thalamus's ventroposterior aspect, are commonly affected. This syndrome can also occur due to lesions developing at any level of the spinothalamic pathway. Lesions developing in these locations are also known as pseudo-thalamic pain syndrome.

What Is the Risk Factor Lying Behind Dejerine-Roussy Syndrome?

The risk factor of Dejerine-Roussy syndrome is a stroke where one part of the brain, including the thalamus, gets damaged. The thalamus sends sensory data to the cerebral cortex and different subcortical regions.

The sensory data from the surrounding organs are sent to the thalamus for processing. Later on, the data travels to the somatosensory cortex for people to understand. Finally, a person can feel, hear or see things as processed by the brain.

Thalamus is also responsible for tactile sensation in the body. When this thalamus gets damaged, it results in poor communication between the afferent cortex and pathways of the brain.

What Are the Symptoms of Dejerine-Roussy Syndrome?

The symptoms of Dejerine-Roussy syndrome are an abnormal sensation of pain felt due to a natural stimulus, tingling sensation on one side of the body, low muscle tone resulting in muscle wasting, and an odd feeling of heat or cold on one side of the body. Sometimes, this syndrome may occur accompanied by weakness in the face, arms, and legs on one side of the body. This weakness may resolve over time, and other symptoms may remain permanent.

The sensory symptoms of Dejerine-Roussy syndrome may begin immediately after a stroke or develop slowly in the subsequent weeks or months. Abnormal curving of spines, speaking difficulty, problems in walking and balancing the body may also occur.

How to Diagnose Dejerine-Roussy Syndrome?

Laser evoked potentials to diagnose the damage on the pain conducting pathways. But this test is rarely required. CT (computed tomography) and MRI (magnetic resonance imaging) can show an infarct or hemorrhage in the thalamus. Involvement of the ventroposterior portion of the thalamus confirms the diagnosis of Dejerine-Roussy syndrome.

How to Treat Dejerine-Roussy Syndrome?

Physiotherapy is the necessary treatment given for relieving the symptoms of this syndrome. A physiotherapist does a thorough assessment of the patient. After careful examination, the physiotherapist may provide a treatment plan, which is often focused on maintaining movements of the body.

Other medications given are antidepressants, opiates, anticonvulsants, and analgesic medications. In severe cases, people are given strong pain medicines such as Morphine and Methadone. People can administer medications orally or through periodic injections. Finding the proper medication regimen for a person takes time. Medications prescribed might differ from person to person according to the precursor disease that led to this syndrome.

People affected with this syndrome need to know their normal vitamin D levels as vitamin D deficiency might also increase the severity of this condition. Intake of vitamin D supplements improves the symptoms of this syndrome.

Surgical options also exist, but neurosurgery remains the last resort. Deep brain stimulation comes under surgical options. In this procedure, an electrode is implanted, and the stimulation is sent to the pain receptors, which reduces a person’s perception of pain.

What Are the Complications Faced Due to Dejerine-Roussy Syndrome?

Dejerine-Roussy syndrome causes problems when a normal sensory input such as sound, light, vibrations, movement, stress, emotions, and thinking is disturbed. A breeze, a light touch, and even clothes touching the skin might also cause intense burning pain.

Even touching a coffee mug, door handle, or steering wheel can cause pain in different body areas and the hand touching it. Temperature changes might also cause severe issues for people suffering from this syndrome.

Showering, sitting on a hot or cold car seat, walking outside in the daylight, touching warm steering in a car can also add pain to the sufferers of this syndrome.

What Is the Prognosis of Dejerine-Roussy Syndrome?

The prognosis of Dejerine-Roussy syndrome is not good. Patients with this syndrome seem to develop unbearable pain with high blood pressure. Patients are more prone to commit suicide due to this syndrome because this medical condition causes intolerable pain, which never vanishes and worsens within a short period. Hence, this condition is challenging to resolve.

What Lifestyle Changes Are Needed?

People needing recovery from this syndrome can initiate a physically active lifestyle for a lifetime after stroke rehabilitation. Developing a self-efficiency in following an active lifestyle helps better. But there is no better evidence against this measure.

Conclusion:

Preventing the formation of blood clots in the brain controls the development of Dejerine-Roussy syndrome. People thinking about avoiding this syndrome need to avoid the common risk factors of stroke as the blood clots might suddenly dislodge in the brain, causing a stroke.

Dr. Abhishek Juneja
Dr. Abhishek Juneja

Neurology

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