Dejerine–Roussy syndrome

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Dejerine–Roussy syndrome
Other namesThalamic pain syndrome
SpecialtyNeurology Edit this on Wikidata

Dejerine–Roussy syndrome or thalamic pain syndrome is a condition developed after a thalamic

hemorrhagic strokes can cause lesioning in the thalamus.[citation needed] As initial stroke symptoms (numbness and tingling) dissipate, an imbalance in sensation causes these later syndromes, characterizing Dejerine–Roussy syndrome. Although some treatments exist, they are often expensive, chemically based, invasive, and only treat patients for some time before they need more treatment, called "refractory treatment".[1]

Symptoms and signs

Dejerine–Roussy syndrome is most commonly preceded by numbness in the affected side. In these cases, numbness is replaced by burning and tingling sensations, widely varying in degree of severity across all cases.

dysaesthesia or allodynia. Less commonly, some patients develop severe ongoing pain with little or no stimuli.[3]

Allodynia is pain from a stimulus that would normally not cause pain.[4][5] For example, there is a patient who experiences unrelenting pain when a breeze touches his skin. Most patients experiencing allodynia, experience pain with touch and pressure, however some can be hypersensitive to temperature.[medical citation needed]

Dysaesthesia is defined as an unpleasant, abnormal sense of touch. It often presents as pain.[6] In this condition it is due to thalamic lesioning. This form of neuropathic pain can be any combination of itching, tingling, burning, or searing experienced spontaneously or from stimuli.[5]

Allodynia and dysaesthesia replace numbness between one week and a few months after a thalamic stroke. In general, once the development of pain has stopped, the type and severity of pain will be unchanging and if untreated, persist throughout life. Consequentially, many will undergo some form of pain treatment and adjust to their new lives as best they can.[medical citation needed]

Pain associated with Dejerine–Roussy syndrome is sometimes coupled with anosognosia or somatoparaphrenia which causes a patient having undergone a right-parietal, or right-sided stroke to deny any paralysis of the left side when indeed there is, or deny the paralyzed limb(s) belong to them. Although debatable, these symptoms are rare and considered part of a "thalamic phenomenon", and are not normally considered a characteristic of Dejerine–Roussy syndrome.[medical citation needed]

Mechanism

Although there are many contributing factors and risks associated with strokes, there are very few associated with Dejerine–Roussy syndrome and thalamic lesions specifically. In general, strokes damage one hemisphere of the brain, which can include the

somatosensory cortex for interpretation. The final product of this communication is the ability to see, hear or feel something as interpreted by the brain. Dejerine–Roussy syndrome most often compromises tactile sensation. Therefore, the damage in the thalamus causes miscommunication between the afferent pathway and the cortex of the brain, changing what or how one feels.[1] The change could be an incorrect sensation experienced, or inappropriate amplification or dulling of a sensation. Because the brain is considered plastic and each individual's brain is different, it is almost impossible to know how a sensation will be changed without brain mapping and individual consultation.[citation needed
]

Recently, magnetic resonance imaging has been utilized to correlate lesion size and location with area affected and severity of condition. Although preliminary, these findings hold promise for an objective way to understand and treat patients with Dejerine–Roussy syndrome.[7]

Diagnosis

Dejerine-Roussy is a rare pain syndrome. Individuals with emerging Dejerine–Roussy syndrome usually report they are experiencing unusual pain or sensitivity that can be allodynic in nature or triggered by seemingly unrelated stimuli (sounds, tastes). Symptoms are typically lateralized and may include vision loss or loss of balance (position sense). Workup should be performed by a neurologist and brain imaging to look for evidence of infarction or tumor should be obtained.[citation needed]

Treatments

Many chemical medications have been used for a broad range of

electrical stimulation of the brain and spinal cord and caloric stimulation have been explored as treatments.[citation needed
]

The most common treatment plans involve a schedule of physical therapy with a medication regimen. Because the pain is mostly unchanging after development, many patients test different medications and eventually choose the regimen that best adapts to their lifestyle, the most common of which are orally and intravenously administered.[medical citation needed]

Pharmaceutical treatment

Stimulation treatments

  • Electrode stimulation from surgically implanted
    Electric stimulation utilizing implants deliver specific voltages to a specific part of the brain for specific durations. More recently, research is being done in radiation therapy as long-term treatment of Dejerine–Roussy syndrome. In general, these studies have concluded initial efficacy in such implants, but pain often re-appears after a year or so. Long-term efficacy of stimulation treatments must be further tested and evaluated.[10]

Expensive and invasive, the above treatments are not guaranteed to work, and are not meeting the needs of patients. There is a need for a new, less expensive, less invasive form of treatment, two of which are postulated below.[citation needed]

  • Spinal cord stimulation has been studied in the last couple of years. In a long case study, 8 patients were given spinal cord stimulation via insertion of a
    thoracic spine. Between 36 and 149 months after the stimulations, the patients were interviewed. 6 of the 8 had received initial pain relief, and three experienced long-term pain relief. Spinal cord stimulation is cheaper than brain stimulation and less invasive, and is thus a more promising option for pain treatment.[11]

Epidemiology

8% of all stroke patients will experience central pain syndrome, with 5% experiencing moderate to severe pain. The risk of developing Dejerine–Roussy syndrome is higher in older stroke patients, about 11% of stroke patients over the age of 80.[1]

History

In 1906,

hemiplegic side, not yielding to any analgesic treatment".[1]

In 1911, it was found that the patients often developed pain and hypersensitivity to stimuli during recovery of function. And thus it was thought that the pain associated after stroke was part of the stroke and lesion repair process occurring in the brain.[medical citation needed] It is now accepted that Dejerine–Roussy syndrome is a condition developed due to lesions interfering with the sensory process, which triggered the start of pharmaceutical and stimulation treatment research. The last 50 years have been filled with refractory treatment research. As of the early 2000s, longer treatments lasting months to years have been explored in the continued search for permanent removal of abnormal pain.[1]

Eponym

Dejerine–Roussy syndrome has also been referred to as: "Posterior Thalamic Syndrome", "Retrolenticular Syndrome", "Thalamic Hyperesthetic Anesthesia", "Thalamic Pain Syndrome", "Thalamic Syndrome", "Central Pain Syndrome", and "Central Post-Stroke Syndrome".

genetic disorders.[14][15][16]

See also

References

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  13. ^ "Thalamic Syndrome (Dejerine Roussy)". National Organization for Rare Disorders. 2003.
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External links