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Approximately 25% of all patients with sensory strokes due to thalamic lesions develop Déjerine-Roussy syndrome 1.
Déjerine-Roussy syndrome is characterized by chronic neuropathic pain that is present on the side of the body contralateral to the thalamic stroke 1-4.
The nature of the pain varies considerably between patients, but is often moderate to severe in intensity, can be either persistent or episodic, can be either spontaneous or evoked (e.g. by cold stimuli), and may have other accompanying sensory deficits (including both hyposensitivity and hypersensitivity) 1-4. The location of the pain also varies considerably and can affect a large part of the contralateral body or a smaller portion depending on the exact size and location of the thalamic lesion 1-4. Additionally, the onset of the pain also is extremely variable, with some patients reporting onset immediately, while others years after the stroke 1-3.
Depending on the size and location of the stroke, patients may have other clinical features that are not strictly part of this syndrome or due to thalamic involvement, including 2,4:
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 1-4. Furthermore, and partly because of this, this syndrome is considered by many authors to be a diagnosis of exclusion in patients with a known thalamic stroke 1-3.
Although the exact pathophysiology of how a lesion in the thalamus causes such a varied pattern of neuropathic pain is unclear, the syndrome can be caused by both ischemic and hemorrhagic lesions of the thalamus 1-4. Furthermore, tumors (including metastases), demyelination, and abscesses involving the thalamus can also cause an identical syndrome 5,6.
In ischemic causes of Déjerine-Roussy syndrome, the thalamogeniculate artery, a branch of the posterior cerebral artery which supplies the ventroposterior aspect of the thalamus, is commonly implicated 1. Indeed, multiple studies have cited involvement of the ventroposterior aspect in thalamic lesions as being crucial to the development of this syndrome 1-3. In particular, one study showed that involvement of the anterior pulvinar nucleus and surrounding nuclei as highly correlating with development of thalamic pain 1.
Although the thalamus is the location of the lesion implicated in this syndrome, central post-stroke pain can also occur due to lesions at any level of the spinothalamic pathway, such as in patients with the lateral medullary syndrome or with cortical lesions 2,7. Lesions in these other locations have occasionally been referred to as pseudo-thalamic pain syndrome 7.
CT and MRI can demonstrate an infarct or hemorrhage in the thalamus 1. Involvement of the ventroposterior portion of the thalamus would be characteristic and support the diagnosis of Déjerine-Roussy syndrome 1.
Treatment and prognosis
Déjerine-Roussy syndrome is often treatment-resistant, however numerous pharmacological (e.g. opiates, antiseizure medications) and surgical (e.g. thalamotomy, electrode stimulation) treatment strategies may be trialed 3,8.
In addition to treating the pain, general stroke management should also be employed.
History and etymology
The syndrome was first described by Joseph Jules Déjerine (1849-1917), a French neurologist, and Gustave Roussy (1874-1948), a French neuropathologist, in their 1906 seminal paper 9.