View source for Medicine:Dejerine–Roussy syndrome

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| synonyms        = Thalamic pain syndrome
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'''Dejerine–Roussy syndrome''' or '''thalamic pain syndrome''' is a condition developed after a thalamic [[Medicine:Stroke|stroke]], a stroke causing damage to the [[Biology:Thalamus|thalamus]].<ref name = Klit>{{cite journal |author=Klit, H., Finnerup, N. B., Jensen, T. S. |year=2009 |title=Central post-stroke pain: clinical characteristics, pathophysiology, and management |journal=The Lancet Neurology |volume=8 |issue=9 |pages=857–868 |doi=10.1016/S1474-4422(09)70176-0|pmid=19679277 |last2=Finnerup |last3=Jensen |s2cid=7838197 }}</ref> Ischemic strokes and hemorrhagic strokes can cause lesioning in the thalamus.{{citation needed|date=June 2019}} 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".<ref name = Klit/>

==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.<ref name = Hadley>{{cite journal |author=Hadley, R. |year=2004 |title=Dejerine-Roussy Syndrome |journal=Clinical Chiropractic |volume=7 |issue=2 |pages=79–83 |doi=10.1016/j.clch.2003.11.003}}</ref> The majority of those reported are cases in which the symptoms are severe and debilitating.{{medcn|date=November 2017}} Burning and tingling can also be accompanied by hypersensitivity, usually in the form of dysaesthesia or allodynia. Less commonly, some patients develop severe ongoing pain with little or no stimuli.<ref name = Wang>{{cite journal |author=Wang, G., Thompson, S. M. |year=2008 |title=Maladaptive homeostatic plasticity in a rodent model of central pain syndrome: thalamic hyperexcitability after spinothalamic tract lesions |journal=Journal of Neuroscience |volume=28 |issue=46 |pages=11959–11969 |doi=10.1523/JNEUROSCI.3296-08.2008|pmid=19005061 |last2=Thompson |pmc=2627563 }}</ref>

Allodynia is pain from a stimulus that would normally not cause pain.<ref name = Quiton>{{cite journal |author=Quiton, R. L., Masri, R., Thompson S. M., Keller, A. |year=2010 |title=Abnormal activity of primary somatosensory cortex in central pain syndrome |journal=Journal of Neurophysiology |volume=104 |issue= 3|pages=1717–1725 |doi=10.1152/jn.00161.2010|pmid=20660417 |last2=Masri |last3=Thompson |last4=Keller |pmc=2944690 }}</ref><ref name = Bowsher>{{cite journal |author=Bowsher, D. |year=2005 |title=Allodynia in relation to lesion site in central post-stroke pain |journal=The Journal of Pain |volume=6 |issue=11 |pages=736–740 |doi=10.1016/j.jpain.2005.06.009 |pmid=16275597|doi-access=free }}</ref> 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.{{medcn|date=November 2017}}

Dysaesthesia is defined as an unpleasant, abnormal sense of touch. It often presents as [[Medicine:Pain|pain]].<ref>{{cite web|url=http://www.iasp-pain.org/AM/Template.cfm?Section=General_Resource_Links&Template=/CM/HTMLDisplay.cfm&ContentID=3058|title=IASP pain terminology|website=wayback machine internet archive|url-status=dead|archive-url=https://web.archive.org/web/20080512061229/http://www.iasp-pain.org/AM/Template.cfm?Section=General_Resource_Links&Template=%2FCM%2FHTMLDisplay.cfm&ContentID=3058|archive-date=12 May 2008|access-date=29 January 2020}}</ref> 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.<ref name = Bowsher/>

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.{{medcn|date=November 2017}}

Pain associated with Dejerine–Roussy syndrome is sometimes coupled with [[Medicine:Anosognosia|anosognosia]] or [[Medicine:Somatoparaphrenia|somatoparaphrenia]] which causes a patient having undergone a right-parietal, or right-sided stroke to deny any [[Engineering:Paralysis|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.{{medcn|date=November 2017}}

==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 [[Biology:Thalamus|thalamus]]. The thalamus is generally believed to relay sensory information between a variety of subcortical areas and the [[Biology:Cerebral cortex|cerebral cortex]].<ref name = Quiton/> It is known that sensory information from environmental stimuli travels to the thalamus for processing and then to 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 [[Biology:Afferent nerve fiber|afferent]] pathway and the cortex of the brain, changing what or how one feels.<ref name = Klit/> 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 [[Biology:Brain mapping|brain mapping]] and individual consultation.{{citation needed|date=February 2021}}

Recently, [[Physics:Magnetic resonance imaging|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.<ref name = Misra>{{cite journal |author=Misra, U. K., Kalita, J., Kumar, B. |year=2008 |title=A study of clinical, magnetic resonance imaging, and somatosensory-evoked potential in central post-stroke pain |journal=The Journal of Pain |volume=9 |issue=12 |pages=1116–1122 |pmid=18848810 |doi=10.1016/j.jpain.2008.06.013|last2=Kalita |last3=Kumar |doi-access=free }}</ref>

==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|date=February 2021}}

==Treatments==
Many chemical medications have been used for a broad range of neuropathic pain including Dejerine–Roussy syndrome. Symptoms are generally not treatable with ordinary analgesics. Traditional chemicals include opiates and anti-depressants. Newer pharmaceuticals include [[Chemistry:Anticonvulsant|anti-convulsants]] and Kampo medicine. As there is no scientific basis in the analgesic efficacy of Kampo medicine beyond placebo, mainstream methods are preferred. Pain treatments are most commonly administered via oral medication or periodic injections. Topical In addition, physical therapy has traditionally been used alongside a medication regimen. More recently, electrical stimulation of the [[Biology:Brain|brain]] and [[Biology:Spinal cord|spinal cord]] and caloric stimulation have been explored as treatments.{{citation needed|date=February 2021}}

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.{{medcn|date=November 2017}}

===Pharmaceutical treatment===
* Opiates contain the narcotics [[Biology:Morphine|morphine]], [[Chemistry:Codeine|codeine]], and [[Chemistry:Papaverine|papaverine]] which provide pain relief. Opiates activate μ-opioid receptors in the brain which alter the brain's perception of sensory input, alleviating pain and sometimes inducing pleasure for a short time period. When intravenously administered, opiates can relieve neuropathic pain but only for a time between 4 and 24 hours. After this time window, the pain returns and the patient must be treated again.<ref name = Klit/> Although this method of treatment has been proven to reduce pain, the repetitive use of opiates has also been linked to the activation of the brain's reward system and therefore poses a threat of addiction. Heavy doses of opiates can also cause [[Medicine:Constipation|constipation]], and respiratory depression.  More common [[Medicine:Adverse effect|side effect]]s include light-headedness, [[Medicine:Dizziness|dizziness]], [[Medicine:Sedation|sedation]], itching, nausea, [[Medicine:Vomiting|vomiting]], and sweating.<ref name="rxlist.com">{{Cite web |url=http://www.rxlist.com/cgi/generic/hydromorphone_ad.htm |title=Hydromorphone Monograph |access-date=2013-02-06 |archive-url=https://web.archive.org/web/20080213054025/http://www.rxlist.com/cgi/generic/hydromorphone_ad.htm |archive-date=2008-02-13 |url-status=dead }}</ref>{{ums|date=November 2017}}
* Anti-depressants are traditionally administered for treatment of mood disorders, also linked to the thalamus, and can be used to treat Dejerine–Roussy symptoms. Specifically, tricyclic anti-depressants such as [[Chemistry:Amitriptyline|amitriptyline]] and selective [[Chemistry:Serotonin|serotonin]] [[Chemistry:Reuptake inhibitor|reuptake inhibitor]]s have been used to treat this symptom and they are effective to some degree within a short time window.<ref name = Klit/><ref name = Ueda>{{cite journal |author=Ueda, K., Namiki, T., Kasahara, Y., Chino, A., Okamoto, H., Ogawa, K., Tersawa, K. |year=2011 |title=A case of thalamic pain successfully treated with kampo medicine |journal=Journal of Alternative and Complementary Medicine |volume=17 |issue=6 |pages=567–570 |pmid=21574822 |doi=10.1089/acm.2010.0390|last2=Namiki |last3=Kasahara |last4=Chino |last5=Okamoto |last6=Ogawa |last7=Terasawa }}</ref>
* Anti-convulsants reduce neuronal hyperexcitability, effectively targeting Dejerine–Roussy syndrome. [[Chemistry:Gabapentin|Gabapentin]] and [[Chemistry:Pregabalin|pregabalin]] are the most common anti-convulsants. They have significant efficacy in treatment of peripheral and central neuropathic pain. Treatments last 4–12 hours and in general are well tolerated, and the occurrence of adverse events does not differ significantly across patients. Commonly reported side-effects are dizziness, decreased intellectual performance, [[Medicine:Somnolence|somnolence]], and nausea.<ref name = Klit/>
* Topical treatment such as [[Chemistry:Lidocaine|lidocaine]] patches can be used to treat pain locally.

===Stimulation treatments===
* Electrode stimulation from surgically implanted electrodes has been studied in the past decade in hopes of a permanent pain treatment without refraction. Electric stimulation utilizing implants deliver specific voltages to a specific part of the brain for specific durations. More recently, research is being done in [[Physics:Radiation therapy|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.<ref name = Hayashi>{{cite journal |author=Hayashi, M., Chernov, M. F., Taira, T., Ochiai, T., Nakaya, K., Tamura, N., Goto, S., Yomo, S., Kouyama, N., Katayama, Y. Kawakami, Y., Izawa, M., Muragaki, Y., Nakamura, R., Iseki, H., Hori, T., Takakura, K. |year=2007 |title=Outcome after pituitary radiosurgery for thalamic pain syndrome |journal=International Journal of Radiation Oncology, Biology, Physics |volume=69 |issue=3 |pages=852–857 |pmid=17570607 |doi=10.1016/j.ijrobp.2007.04.043|last2=Chernov |last3=Taira |last4=Ochiai |last5=Nakaya |last6=Tamura |last7=Goto |last8=Yomo |last9=Kouyama |last10=Katayama |last11=Kawakami |last12=Izawa |last13=Muragaki |last14=Nakamura |last15=Iseki |last16=Hori |last17=Takakura }}</ref>

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|date=February 2021}}
* 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 [[Medicine:Percutaneous|percutaneous]] lead at the appropriate level of the cervical or 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.<ref name = Lopez>{{cite journal |author=Lopez, J. A., Torres, L. M., Gala, F., Iglesias, I. |year=2009 |title=Spinal cord stimulation and thalamic pain: long-term results of eight cases |journal=Neuromodulation |volume=12 |issue=3 |pages=240–243 |doi=10.1111/j.1525-1403.2009.00221.x|pmid=22151367 |last2=Torres |last3=Gala |last4=Iglesias |s2cid=35575713 }}</ref>

==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.<ref name = Klit/>

==History==
In 1906, Joseph Jules Dejerine and Gustave Roussy provided descriptions of central post-stroke pain (CPSP) in their paper entitled: "Le syndrome thalamique". The name Dejerine–Roussy syndrome was coined after their deaths. The syndrome included "…severe, persistent, paroxysmal, often intolerable, pains on the hemiplegic side, not yielding to any [[Biology:Analgesic|analgesic]] treatment".<ref name = Klit/>

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.{{medcn|date=November 2017}} 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.<ref name = Klit/>

==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".<ref name = Klit/><ref name = NINDS>{{cite web |year=2011 |title=Pain: Hope Through Research |publisher=[[Organization:National Institute of Neurological Disorders and Stroke|National Institute of Neurological Disorders and Stroke]] |url=http://www.ninds.nih.gov/disorders/chronic_pain/detail_chronic_pain.htm |access-date=2011-11-21 |archive-url=https://web.archive.org/web/20161215182202/http://www.ninds.nih.gov/disorders/chronic_pain/detail_chronic_pain.htm |archive-date=2016-12-15 |url-status=dead }}</ref><ref name = NORD>{{cite web |year=2003 |title=Thalamic Syndrome (Dejerine Roussy) |publisher=National Organization for Rare Disorders |url=http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/796/viewAbstract}}</ref> This condition is not associated with [[Medicine:Roussy–Lévy syndrome|Roussy–Lévy syndrome]] or [[Medicine:Dejerine–Sottas disease|Dejerine–Sottas disease]], both of which are genetic disorders.<ref name = Auer-Grumbach>{{cite journal |author=Auer-Grumbach, M., Strasser-Fuchs, S., Wagner, K., Korner, E., Fazekas, F. |year=1998 |title=Roussy–Lévy syndrome is a phenotypic variant of Charcot–Marie–Tooth syndrome IA associated with a duplication on chromosome 17p11.2 |journal=Journal of the Neurological Sciences |volume=154 |issue=1 |pages=72–75 |pmid=9543325 |doi=10.1016/S0022-510X(97)00218-9|last2=Strasser-Fuchs |last3=Wagner |last4=Körner |last5=Fazekas |s2cid=40475515 }}</ref><ref name = Haubrich>{{cite journal |author=Haubrich, C., Krings, T., Senderek, J., Zuchner, S., Schroder, J. M., Noth, J., Topper, R. |year=2002 |title=Hypertrophic nerve roots in a case of Roussy-Levy syndrome |journal=Neuroradiology |volume=44 |issue=11 |pages=933–937 |doi=10.1007/s00234-002-0847-2 |pmid=12428130|last2=Krings |last3=Senderek |last4=Züchner |last5=Schröder |last6=Noth |last7=Töpper |s2cid=13141793 }}</ref><ref name = Zubair>{{cite journal |author=Zubair, S., Holland, N. R., Beson, B., Parke, J. T., Prodan, C. I. |year=2008 |title=A novel point mutation in the PMP22 gene in a family with Roussy-Levy syndrome |journal=Journal of Neurology |volume=255 |issue=9 |pages=1417–1418 |doi=10.1007/s00415-008-0896-5 |pmid=18592125|last2=Holland |last3=Beson |last4=Parke |last5=Prodan |s2cid=13390074 }}</ref>

==See also==
* [[Medicine:Central pain syndrome|Central pain syndrome]]

==References==
{{Reflist}}

== External links ==
{{Medical resources
|   DiseasesDB     = 13002
|   ICD10          = {{ICD10|G93.8}} + {{ICD10|I69}}
|   ICD9           = {{ICD9|338.0}}
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|   MeshID         = D013786
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{{CNS diseases of the nervous system}}
{{Cerebrovascular diseases}}

{{DEFAULTSORT:Dejerine-Roussy syndrome}}
[[Category:Syndromes affecting the nervous system]]

{{Sourceattribution|Dejerine–Roussy syndrome}}