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Due to the high clinical variability in presentation of Déjerine-Roussy syndrome, it is impossible to predict which patients with a thalamic stroke will develop pain. Stroke and pain can sometimes result in Dejerine-Roussy syndrome. Available treatments include antidepressants, anticonvulsants, and. Improvement in neurological signs and symptoms of thalamic syndrome ( Dejerine-Roussy Syndrome) due to a stroke 20 years previously (in.

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NORD gratefully acknowledges Prof. Central pain syndrome is a neurological disorder caused by damage to the sensory pathways of the central nervous system CNS. Pain is often constant but can be intermittent and can be mild, moderate, or severe in intensity. Affected individuals may become hypersensitive to painful stimuli.

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The specific type of pain experience can vary from one individual to another based, in part, upon the underlying cause of the disorder and the area of the central nervous system affected. Central pain syndrome can develop following a variety of conditions including stroke, multiple sclerosis, spinal cord injury, brain tumors, or iatrogenic damage to the central nervous system.

Central pain was first described in by German neurologist L. For years, it was believed that the majority of cases of central pain syndrome were due to damage of the thalamus most often caused by a stroke. Later on, the disorder came dejerime be labelled Dejefine syndrome after the two French neurologists who described the so-called thalamic syndromewhich included a pain component. Thereafter, and up until recently, central pain became synonymous with thalamic pain syndrome.

However, researchers now know that damage to the pain-conducting pathways anywhere along the neural axis, from the spinal cord to the sensory cortex, can cause central pain syndrome, including cases following injury or a stroke.

Consequently, the current name for this group of disorders is central pain syndrome to acknowledge that damage to various areas of the CNS and not predominantly the thalamus can cause central pain and that a stroke is not necessarily the primary cause.

Central pain syndrome can be broken down into central pain of brain or brainstem origin or central pain of spinal cord origin. The term for the specific subtype of central pain syndrome caused by Russy damage due to a stroke is central post-stroke pain.

The onset of central pain syndrome can vary from days to weeks to months or more than a year after injury to the central nervous system. However, the disorder can also appear immediately denerine an injury or within a day.

In most cases, central pain syndrome remains a lifelong condition. Central pain syndrome can be limited to a specific area of the dejerinf such as the hands or feet or may be widespread over a large portion of the body.

Some areas of the body may be more intensely affected than other areas. Pain can fluctuate during the day and can be affected by several factors including touch, emotions such as stress, certain movements or overall level of activity, and temperature changes, especially cold temperatures. Rest and distraction may lessen symptoms. Central pain syndrome can vary greatly from one individual to another, in part, based upon the underlying cause of the condition.

In some cases, symptoms of central pain syndrome can be vague and difficult to characterize. A constant burning sensation is in several cases the most prominent symptom. Pain can be constant and unrelenting or it may come and go intermittent.

In most cases, pain is constant and usually moderate or severe in intensity. In some cases, pain has been described as agonizing. Some affected individuals may experience short bursts of sharp, excruciating pain, which has been compared to the pain that occurs when a dental probe strikes an exposed nerve.

Pain sensations associated with central pain syndrome are generally spontaneous, which means they occur despite no apparent cause or trigger. People with this disorder may be extra-sensitive or have a heightened response to acute painful stimuli hyperalgesia, hyperpathiawhich means that pain that would normally be small or minimal is felt to a far greater degree.


In addition, individuals may feel pain from stimuli that would normally not be painful allodynia. For example, affected individuals may experience pain when touched, even when lightly touched.

In severe cases, this can include pain caused by a strong breeze, the weight of a blanket, or even the clothes a person is wearing. Some individuals with central pain syndrome may initially experience impairment or distortion of sensation, especially of touch dysesthesia. Dysesthesia is described as vague, unpleasant sensations. Some individuals may experience a painful numbness, especially affecting the feet. Itching pruritus has also been reported in individuals with central pain syndrome.

Some individuals with central post-stroke pain may experience painful symptoms on one side of the body from head to foot ; other notable, accompanying symptoms include loss of sensation hemihypesthesiapartial or complete muscle weakness hemiparesis, hemiplegiaandon occasion, abnormal, involuntary, irregular jerky motions and slow, writhing movements hemichoreoathetosis. The disorder develops following damage to the central nervous system — the brain, brainstem or spinal cord.

Such damage is most often associated with a stroke, multiple sclerosis, spinal cord but also brain injury or brain tumors. Central pain syndrome can also develop after neurosurgical procedures involving the brain or spine.

Dejerine-Roussy Syndrome | Institute of Neurological Recovery

Central pain syndrome results from damage to the pain-transmission pathway from the level of the spinal cord up to the cortex, the grey matter that covers the cerebral hemispheres. Central pain syndrome is due to a disturbed communication between the sensory thalamus and the sensory cortex.

The thalamus is a structure deep in the brain that acts as the key hub in sensory processing, in synchrony with the cortex. CPS is thus due to a dysfunctional corticothalamic loop along the sensory pain-conducting pathways. Central pain syndrome is estimated to affect several million people worldwide. Symptoms of peripheral neuropathic pain e. Complex regional pain syndrome CRPSa. Its genesis is highly controversial, and several researchers deny its existence as a stand-alone syndrome; the American Medical Association —for one- believes that the sympathetic nervous system plays no direct role.

Instead, CRPS can be defined as peripheral neuropathic pain with a strong inflammatory component. In some individuals, CRPS may occur in one or both legs or it may be localized to one knee or hip. The skin over the affected area s may become swollen edema and inflamed. Affected skin may be extremely sensitive to touch and to hot or cold temperatures cutaneous hypersensitivity.

The affected limb s may perspire excessively and be warm to the touch vasomotor instability. CRPS may be associated with injury to the nerves, trauma, surgery, atherosclerotic cardiovascular disease, infection, or radiation therapy.

A diagnosis of central pain syndrome is based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized tests. Central pain syndrome is suspected in individuals who complain of pain or other abnormal sensations following injury to the central nervous system. Other conditions that cause pain may need to be excluded before a diagnosis of central pain syndrome is made. Clinical Testing and Workup Magnetic resonance imaging MRI is the technique of choice to visualize tumors, infarcts, cerebral hemorrhage, demyelinating plaques and other causal lesions.

An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Recent permutations of this technique such as magnetic resonance spectroscopy or default-state MRI have only research interest.

Laser evoked potentials are rarely needed to confirm damage to pain conducting pathways and are not available on a routine basis. Treatment Treatment for central pain syndrome remains unsatisfactory in that, although most patients find some relief, complete control remains infrequent.

Additionally, therapies that have proven beneficial in some individuals may have no benefit in others. Traditional pain medications e. NSAIDs generally provide little to no relief for individuals with central pain syndrome. However, drug therapy remains the first-line therapy for most affected individuals.


Two drugs, amitriptyline and lamotrigine, have proven beneficial in controlled trials, especially for individuals with central pain of brain origin.

Amitriptyline is an antidepressant. Lamotrigine is an anti-seizure drug anticonvulsant. Antiarrhythmics mexiletine and local anesthetics such as lidocaine can be effective. Gabapentinoids, another type of anti-seizure medication, although FDA approved for central pain, are not indicated as first line therapy for central pain: Although FDA approved for chronic pain, unbiased assessment of the entire database shows that use of ziconotide has a very low therapeutic index with lack of consistent benefit in a majority of patients.

Opioids such as morphine or levorphanol are not effective in the vast majority of cases and are actually contraindicated for most patients. In several cases, the effectiveness of drug therapies are limited due to associated side effects. Of special interest is virtual-reality-assisted hypnosis.

Supportive care, rehabilitation, and psychotherapy are recommended as well. Extradurally means that the paddle is placed on the dura mater, the outermost layer of the membranes meninges that line the brain and spinal cord. The paddle is inserted through two burr holes or via the temporary removal of a small piece of the skull craniotomy.

Studies show that this form of stimulation is more effective that placebo stimulation. Non-invasive forms of cortical stimulation such as transcranial magnetic stimulation TMS or transcranial direct current stimulation tDCS have been employed in the management of CPS, but data show that their efficacy is not on a par with neurosurgical intervention.

However, they may help select patients for surgical implantation. Extensive studies showed that deep brain stimulation is NOT indicated for the treatment of individuals with central pain syndrome, with few exceptions, and only after a trial of CS. During this surgical procedure, an electrode is placed into the thalamus and a thin wire is passed under the skin is connected to a small battery pack which is also placed underneath the skin.

The risk of lethal brain hemorrhage is small, but real. In resource-constrained contexts, spinal cord stimulation benefited a small percentage of patients with both CCP and BCP, but long-term control is rare. Some individuals experience pain relief through the direct injection of drugs into the cerebrospinal fluid that surrounds the brain and spinal cord intrathecal administration.

Drugs such as baclofen or midazolam have been used in this manner. A few individuals have experienced pain relief through the insertion of a morphine pump that delivers drugs intrathecally, but as mentioned should be considered as a last-resort.

Intrathecal drug delivery is not always effective for the treatment of individuals with central pain syndrome. However, much more research is necessary in this area. In the past, surgery has been used to treat individuals with central pain syndrome.

This includes invasive, drastic surgeries such as the surgical removal or destruction of targeted parts of the brain such as thalamus thalamotomy and mesencephalon mesencephalotomy. However, most of these surgeries were generally associated with low long-term success rates and a high risk of complications and morbidity.

Such invasive surgeries are rarely used for individuals with central pain syndrome anymore. During this procedure, a small lesion is made in certain areas of the white matter of the brain in order to interrupt the descending arm of the corticothalamic loop. In one case reported in the medical literature, this procedure immediately and completely relieved pain in the affected individual.

However, more research is necessary to determine the long-term safety and effectiveness of this procedure as a potential therapy for individuals with central pain syndrome.

Recent advances in the field of spinal cord injury suggest that removal of an injured segment of the spinal cord, wherever feasible, accompanied by vertebrectomy or multiple discectomies might benefit both motor and sensory recovery.