Dejerine–Roussy syndrome

Overview

Dejerine–Roussy syndrome, also known as thalamic pain syndrome, is a rare neurological condition that occurs after a stroke affecting the posterolateral thalamus, a part of the brain responsible for processing sensory information. First described by French neurologists Joseph Dejerine and Gustave Roussy in 1906, the syndrome is characterized by chronic, often severe pain on one side of the body, typically the side opposite the brain lesion. The pain can be debilitating and resistant to conventional treatments, making management challenging.

Causes

The primary cause of Dejerine–Roussy syndrome is a stroke involving the thalamus, particularly the ventral posterior nucleus, which is critical for sensory perception. Other less common causes include:

• Traumatic brain injury involving the thalamus

• Brain tumors affecting thalamic structures

• Multiple sclerosis or demyelinating diseases

• Infections or vascular malformations in the brain

The condition typically develops weeks to months after the initial brain injury, as the brain begins to heal and reorganize damaged sensory pathways.

Symptoms

Symptoms of Dejerine–Roussy syndrome usually affect the contralateral side of the body (opposite to the brain lesion) and may include:

• Chronic, burning, or stabbing pain: Often described as severe and unrelenting, typically affecting the face, arm, or leg.

• Allodynia: Pain triggered by stimuli that are normally non-painful, such as light touch or clothing.

• Dysesthesia: Abnormal, unpleasant sensations such as tingling, numbness, or electric shocks.

• Sensory loss: Numbness or decreased sensation to touch, temperature, or pain in the affected areas.

• Emotional distress: Due to chronic pain, patients often experience anxiety, depression, and reduced quality of life.

Diagnosis

Diagnosis of Dejerine–Roussy syndrome is based on clinical history, neurological examination, and imaging studies. Diagnostic steps include:

• History of stroke: Especially one affecting the thalamus or involving sudden onset of sensory changes.

• Neurological exam: Identifies sensory deficits, pain distribution, and response to touch or temperature.

• Neuroimaging:

  • MRI scan: Most effective for detecting thalamic stroke or lesion.

  • CT scan: May show older or larger strokes.

• Exclusion of other causes: Rule out peripheral neuropathy, spinal cord lesions, or other central pain syndromes.

Treatment

Treating Dejerine–Roussy syndrome can be difficult, as pain often persists and may be resistant to typical analgesics. A multidisciplinary approach is usually necessary, combining medications, therapies, and supportive care:

• Medications:

  • Antidepressants: Such as amitriptyline or duloxetine, which help modulate pain perception.

  • Anticonvulsants: Like gabapentin or pregabalin, effective for neuropathic pain.

  • Opioids: May be used in severe cases, but are generally avoided due to side effects and dependence risk.

  • Topical agents: Such as lidocaine or capsaicin creams for localized relief.

• Physical therapy: Helps with mobility, strength, and desensitization of affected areas.

• Cognitive-behavioral therapy (CBT): Addresses the psychological impact of chronic pain.

• Transcranial magnetic stimulation (TMS) or deep brain stimulation (DBS): Experimental treatments for severe, refractory cases.

Prognosis

The prognosis for Dejerine–Roussy syndrome varies. Some individuals experience gradual improvement with therapy and medications, while others suffer from persistent, disabling pain that significantly impacts their quality of life. Early recognition, comprehensive management, and psychological support are essential to help reduce suffering and improve functional outcomes. Despite ongoing research, complete recovery is rare, and treatment typically focuses on symptom control and pain relief rather than cure.