Background & Aims

Dejerine-Roussy syndrome (DRS), also known as central post-stroke pain or thalamic pain syndrome, is a rare form of centrally-mediated neuropathic pain that develops following damage to the ventroposterolateral thalamus [1]. While the mechanisms of DRS are not well characterized, focal disruption of the spinothalamic tract can result in profoundly bothersome and persistent burning, throbbing, stabbing, or shooting pain. Patients can experience increased sensitivity to external stimuli (allodynia and hyperalgesia) as well as spontaneous pain [2, 3]. DRS pain is often refractory to pharmacological and non-pharmacological therapy [3]. This syndrome has not been thoroughly studied, and misdiagnosis can impair timely and appropriate treatment. We describe an unusual case of Dejerine-Roussy syndrome with an accompanying movement disorder thought to represent seizure activity, which resolved on withdrawal of anticonvulsant therapy.

Methods

‘N-of-1’ Case report study: prospective monitoring, video EEG, and trial of anticonvulsant (levetiracetam) withdrawal with induction of pain-active pharmacotherapy (gabapentin and duloxetine); occupational therapy, and brief geriatric psychiatry counseling.

The patient was a 69 year-old male with intercurrent hypertension and hyperlipidemia having a remote history of right occipital stroke. He suffered a hemorrhagic stroke of the right ventroposterolateral thalamus (VPL) 6 months prior to this new admission for recurrent bouts of left arm movement and pain which had worsened over 3 weeks. He had previously been diagnosed with and treated for “focal aware motor seizures”; levetiracetam and topiramate were taken as prescribed.

CT scan in the emergency department was consistent with a sub-centimeter lacune in the right VPL and prior right occipital stroke.

Results

At hospital admission, pain and movements were episodic. Events began with burning-shock sensations running subcutaneously down the anterior forearm, followed by profound pressure sensations in the wrist. Dystonic posturing of the left wrist and hand then evolved, followed by 1 Hz flexion-extension movements at the elbow with the hand slamming into the bed for a variable period, this was followed by a brief subjective fatigue. The movements were distractable and semiology was variable. Episodes lasted from 2 to twenty minutes, often several times per hour, throughout the day. Sleep onset was delayed and sleep periods were markedly shortened, but movements remitted with sleep.

Continuous video EEG monitoring showed no epileptiform activity or peri-ictal findings. Levetiracetam was step-wise tapered over 48 hours with an immediate decrease in movement amplitude, bout frequency and duration. Gabapentin and duloxetine were initiated. The patient was much improved at discharge.

Conclusions

In the context of levetiracetam administration, we observed that post-stroke thalamic pain syndrome manifested with a functional movement disorder relieved by levetiracetam discontinuation. Levetiracetam has been associated with behavioral disruption, especially in patients with fixed brain lesions; consideration should be given to withdrawal of this agent in selected settings with substitution when appropriate. Continuous video-EEG monitoring and careful attention to event semiology is helpful to discern whether events are likely to represent epileptiform activity.

In this case, we concluded that the patient’s ‘focal seizure disorder’ was a functional motor response to severe pain as a sequela of thalamic stroke amplified in the context of an aggravating agent.

References

1.Jahngir MU, Qureshi AI. Dejerine-Roussy Syndrome. [Updated 2023 Jul 3]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK519047/
2.Jang SH, Kim J, Lee HD. Delayed-onset central poststroke pain due to degeneration of the spinothalamic tract following thalamic hemorrhage: A case report. Medicine (Baltimore). 2018 Dec;97(50):e13533. doi: 10.1097/MD.0000000000013533. PMID: 30558012; PMCID: PMC6319862.
3.Treister AK, Hatch MN, Cramer SC, Chang EY. Demystifying Poststroke Pain: From Etiology to Treatment. PM R. 2017 Jan;9(1):63-75. doi: 10.1016/j.pmrj.2016.05.015. Epub 2016 Jun 16. PMID: 27317916; PMCID: PMC5161714.

Presenting Author

Beth Hogans

Poster Authors

Beth Hogans

M.S. (Biomath), M.D., Ph.D.

Johns Hopkins SOM/ VA Maryland

Lead Author

E-mail

Matthew Yen

M.D.

University of Maryland School of Medicine, VA Maryland Health Care System

Lead Author

E-mail

Esther Xu

University of Maryland School of Medicine, VA Maryland Health Care System

Lead Author

E-mail

Anna Schoonover

University of Maryland School of Medicine, VA Maryland Health Care System

Lead Author

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Alanna Stefano

University of Maryland School of Medicine, VA Maryland Health Care System

Lead Author

E-mail

Omar Khan

M.D.

VA Maryland Health Care System

Lead Author

E-mail

Topics

  • Specific Pain Conditions/Pain in Specific Populations: Neuropathic Pain - Central