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paraneoplastic brainstem encephalitis; paraneoplastic rhombencephalitis
Etiology:
1) mediator: unknown
2) KLHL11 autoimmunity [2,3]
3) associated neoplasms:
- small cell lung cancer
- testicular germ cell tumors [2,4]
Pathology:
1) neuronal loss in brainstem
2) perivascular & meningeal lymphocytic infiltration
Clinical manifestations:
1) evolution over weeks to months
2) nystagmus
3) diplopia
4) vertigo
5) ataxia
6) dysarthria
7) dysphagia
4) memory loss with progession to dementia
Interactions
disease interactions
General
encephalitis
paraneoplastic neurologic syndrome
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 624
- Devine MF, Gay PC, Dubey D
Ondine's Curse and Trismus in Association With Kelch-like Protein-11
Autoimmunity.
JAMA Neurol. Published online May 28, 2021
PMID: 34047764
https://jamanetwork.com/journals/jamaneurology/fullarticle/2780425
- Dubey D, Wilson MR, Clarkson B et al
Expanded Clinical Phenotype, Oncological Associations, and Immunopathologic
Insights of Paraneoplastic Kelch-like Protein-11 Encephalitis.
JAMA Neurol. 2020 Nov 1;77(11):1420-1429
PMID: 32744608
- Mandel-Brehm C, Dubey D, Kryzer TJ et al
Kelch-like Protein 11 Antibodies in Seminoma-Associated Paraneoplastic
Encephalitis.
N Engl J Med. 2019 Jul 4;381(1):47-54
PMID: 31269365 Free PMC article
- Lawn ND, Westmoreland BF, Kiely MJ, Lennon VA, Vernino S.
Clinical, magnetic resonance imaging, and electroencephalographic findings
in paraneoplastic limbic encephalitis
Mayo Clin Proc. 2003 Nov;78(11):1363-8.
PMID: 14601695