Autoimmunity to Kelch-like Protein-11 (KLHL11) can result in a disabling paraneoplastic neurologic disorder that may be refractory to treatment.
Pathogenesis: rhomboencephalitis (inflammation of the hindbrain = brainstem and cerebellum), with T-cell mediated inflammation with non-necrotizing granulomas
Patients possess antibodies to Kelch-like Protein-11.
Clinical symptoms nay include:
(1) ataxia
(2) visual changes (diplopia, nystagmus)
(3) vertigo
(4) hearing loss
(5) tinnitus
(6) dysarthria
(7) seizures
(8) neuropsychiatric dysfunction
(9) myeloneuropathy
(10) cervical amyotrophy
(11) limbic encephalitis
(12) anti-NMDAR encephalitis
(13) facial weakness
Underlying neoplasms:
(1) germ cell tumors (seminoma, teratoma, mixed)
(2) lung adenocarcinoma
(3) CLL
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