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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