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E-135 Dejerine-roussy syndrome in the setting of right sigmoid sinus thrombosis: a unique case report
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  1. S Nittala1,
  2. C Jara2,
  3. D Raza1,
  4. J Cazzaniga3,
  5. D Filippi4
  1. 1Neurology, Larkin Community Hospital Palm Springs Campus, Hialeah, FL, USA
  2. 2Nova Southeastern University KPCOM, Davie, FL, USA
  3. 3Florida International University HWCOM, Miami, FL, USA
  4. 4Neurology, Design Neuroscience Center, Doral, FL, USA

Abstract

Introduction Dejerine-Roussy Syndrome (DRS), also known as central post-stroke pain, is an unusual neuropathic pain syndrome in the central nervous system. Symptoms can originate from any vascular lesion or disease affecting the central somatosensory system. Most frequently the etiology is due to acute arterial infarction, with a high prevalence in lateral medullary syndrome. Few cases have been reported of DRS secondary to venous thrombosis. This case presents evidence of DRS secondary to right sigmoid sinus thrombosis.

Case Presentation Here we report the case of a 37-year-old male with a past medical history of hypertension, tibial and fibular fracture, status-post open reduction and internal fixation (ORIF), and recent right-sided cerebral venous thrombosis of the sigmoid sinus with residual left-sided weakness from January 2023. The patient presented to the hospital in March 2023, due to the sudden onset of severe left-sided pain with left arm weakness. On exam, he was found to have allodynia of left hemi-body: severe pain to light touch LUE, moderate pain to light touch left face, left chest, left abdomen, and LLE respecting the midline. Imaging studies including Computerized Tomography (CT) Angiogram of the Head and Neck, CT Brain Venogram, and CT Brain without contrast showed reduced flow in the right sigmoid sinus and no flow in the adjacent jugular vein, confirming a right sigmoid sinus thrombus. Magnetic Resonance Imaging (MRI) showed a small sub-centimeter white matter focus of DWI hyperintensity in the left frontal centrum semi-ovale, presenting as a possible left hemispheric stroke, which does not account for the current symptomatology. Pertinent laboratory studies demonstrated an increase in thrombosis risk from Lupus anticoagulant PT at 13 and Antithrombin III Ag at 68 (L). He was treated with physical therapy/occupational therapy, and the pain was controlled gradually with amitriptyline 50mg nightly and Lyrica 50mg three times daily. Cardio-embolic workup was also completed including Transesophageal echocardiography (TEE), showing an intra-arterial aneurysm, but since it is present without a patent foramen ovale (PFO), it was deemed not significant.

Discussion This case illustrates the important clinical aspects and physical findings needed to diagnose DRS. The presence of focal allodynia and hyperalgesia with recent cerebrovascular incidents provides significant evidence for DRS. The purpose of this case is to provide awareness and to increase clinical suspicion of DRS during examination, especially in the setting of cerebral venous sinus occlusion as infarction alone may not present as atypical allodynia.

Disclosures S. Nittala: None. C. Jara: None. D. Raza: None. J. Cazzaniga: None. D. Filippi: None.

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