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Paraneoplastic Subacute Sensory Neuronopathy

MedGen UID:
233843
Concept ID:
C1335344
Disease or Syndrome
Synonym: Paraneoplastic Subacute Sensory Neuropathy

Definition

A sensory neuropathy caused by the effects of a distant neoplasm (usually a small cell carcinoma). It is usually caused by the production of anti-neuronal autoantibodies: anti-Hu (ANNA-1) or anti-CV2 (CRMP-5), which result in cytotoxic T-cell mediated destruction of dorsal root ganglia. It is the most common clinical presentation of paraneoplastic encephalomyelitis. Clinical signs include asymmetric paresthesia of the face, trunk and proximal extremities followed by lancinating pain, vibrioceptive and proprioceptive impairment, and muted reflexes. Clinical course usually follows a progressive neurologic deterioration which stabilizes at a level of severe, permanent disability. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVParaneoplastic Subacute Sensory Neuronopathy

Professional guidelines

PubMed

Vedeler CA, Antoine JC, Giometto B, Graus F, Grisold W, Hart IK, Honnorat J, Sillevis Smitt PA, Verschuuren JJ, Voltz R; Paraneoplastic Neurological Syndrome Euronetwork
Eur J Neurol 2006 Jul;13(7):682-90. doi: 10.1111/j.1468-1331.2006.01266.x. PMID: 16834698

Recent clinical studies

Etiology

Oh SJ, Gürtekin Y, Dropcho EJ, King P, Claussen GC
Clin Neurophysiol 2005 Jan;116(1):28-34. doi: 10.1016/j.clinph.2004.07.012. PMID: 15589180
Dropcho EJ, Stanton C, Oh SJ
Neurology 1989 Feb;39(2 Pt 1):249-51. doi: 10.1212/wnl.39.2.249. PMID: 2536905

Diagnosis

Cowley A, Pascoe S
BMJ Case Rep 2011 Jul 28;2011 doi: 10.1136/bcr.04.2011.4077. PMID: 22689835Free PMC Article
Bi WL, Bannykh SI, Martel M, Baehring JM
Obstet Gynecol 2006 Feb;107(2 Pt 2):504-6. doi: 10.1097/01.AOG.0000171115.93233.5f. PMID: 16449165
Kuntzer T, Antoine JC, Steck AJ
Muscle Nerve 2004 Sep;30(3):255-68. doi: 10.1002/mus.20100. PMID: 15318336
Wanschitz J, Hainfellner JA, Kristoferitsch W, Drlicek M, Budka H
Neurology 1997 Oct;49(4):1156-9. doi: 10.1212/wnl.49.4.1156. PMID: 9339709
Lövblad KO, Boucraut J, Bourdenet S, Burger D, Bernard D, Regli F, Steck AJ
J Neurol 1993 Jun;240(6):327-32. doi: 10.1007/BF00839961. PMID: 8393093

Therapy

Cowley A, Pascoe S
BMJ Case Rep 2011 Jul 28;2011 doi: 10.1136/bcr.04.2011.4077. PMID: 22689835Free PMC Article

Prognosis

Bi WL, Bannykh SI, Martel M, Baehring JM
Obstet Gynecol 2006 Feb;107(2 Pt 2):504-6. doi: 10.1097/01.AOG.0000171115.93233.5f. PMID: 16449165
Camdessanché JP, Antoine JC, Honnorat J, Vial C, Petiot P, Convers P, Michel D
Brain 2002 Jan;125(Pt 1):166-75. doi: 10.1093/brain/awf006. PMID: 11834602
Drlicek M, Bodenteich A, Setinek U, Tucek G, Urbanits S, Grisold W
Acta Neuropathol 2000 May;99(5):599-602. doi: 10.1007/s004010051168. PMID: 10805108

Clinical prediction guides

Oh SJ, Gürtekin Y, Dropcho EJ, King P, Claussen GC
Clin Neurophysiol 2005 Jan;116(1):28-34. doi: 10.1016/j.clinph.2004.07.012. PMID: 15589180
Camdessanché JP, Antoine JC, Honnorat J, Vial C, Petiot P, Convers P, Michel D
Brain 2002 Jan;125(Pt 1):166-75. doi: 10.1093/brain/awf006. PMID: 11834602
Drlicek M, Bodenteich A, Setinek U, Tucek G, Urbanits S, Grisold W
Acta Neuropathol 2000 May;99(5):599-602. doi: 10.1007/s004010051168. PMID: 10805108
Wanschitz J, Hainfellner JA, Kristoferitsch W, Drlicek M, Budka H
Neurology 1997 Oct;49(4):1156-9. doi: 10.1212/wnl.49.4.1156. PMID: 9339709
Kida E, Barcikowska M, Joachimowicz E, Michalska T, Siekierzyńska A, Walasik A, Roszkowski K, Figura E
Neuropatol Pol 1993;31(1-2):63-73. PMID: 8208442

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