Paraneoplastic encephalomyelitis revealing burned-out seminoma

Guilmot, Antoine; Pesch, Vincent Van; Duprez, Thierry; Jacquerye, P.

doi:10.1007/s13760-020-01447-7

Cited by 5 publications

(3 citation statements)

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“…Our report highlights the importance of detailed and repeated investigations for testicular cancer in male patients between the ages of 20-50 with suspected PNS, since it is the second most common malignancy in this patient cohort, and the first most common between 20 and 40 years of age (Ward et al, 2019). The number of reports describing PNS associated with burned-out testicular tumor is limited (Vogrig et al, 2021;Chen et al, 2021;Guilmot et al, 2021;Maldonado Slootjes et al, 2022;Onishi et al, 2014;Freifeld et al, 2018;Ishikawa et al, 2016;van de Warrenburg et al, 2007), and it is tempting to speculate that at least some of them might have been associated with KLHL11 autoantibodies (Maldonado Slootjes et al, 2022).…”

Section: Discussionmentioning

confidence: 72%

Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic “burned-out” seminoma – A scar(r)y phenomenon

Gyöngyösi¹,

Magyar-Stang²,

Takács³

et al. 2023

Journal of Neuroimmunology

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Section: Discussionmentioning

confidence: 72%

Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic “burned-out” seminoma – A scar(r)y phenomenon

Gyöngyösi¹,

Magyar-Stang²,

Takács³

et al. 2023

Journal of Neuroimmunology

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“…developed progressive vertigo, and truncal and appendicular ataxia almost 4 years after diagnosis of and therapy for a testicular seminoma [9]. So far, 112 patients with KLHL11-IgG-associated encephalitis have been reported in the literature (Table 1) [9,[11][12][13][14][15][16][17][18][19][20][23][24][25][26][27][28].…”

Section: Liter Ature Re Vie Wmentioning

confidence: 99%

“…So far, 112 patients with KLHL11‐IgG‐associated encephalitis have been reported in the literature (Table 1) [9, 11–20, 23–28].…”

Section: Literature Reviewmentioning

confidence: 99%

Anti‐kelchlike protein 11 antibody‐associated encephalitis: Two case reports and review of the literature

Betancourt

Schwarzwald

Millonig

et al. 2023

Euro J of Neurology

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Background and purpose: Kelchlike protein 11 antibodies (KLHL11-IgGs) were first described in 2019 as a marker of paraneoplastic neurological syndromes (PNSs). They have mostly been associated with testicular germ cell tumors (tGCTs).Methods: Two patients with KLHL11-IgG encephalitis are reported, and the literature is comprehensively reviewed. Results: Patient 1 had been in remission from a tGCT 10 years prior. He developed episodic vertigo and diplopia progressing over a few days. Treatment with corticosteroids (CSs) was started a few days after symptom onset. Patient 2 had transient diplopia, which resolved spontaneously. Visual problems persisted for 7 months, when he additionally developed a progressive cerebellar syndrome. One year after onset, CS treatment was started. Initial magnetic resonance imaging was unremarkable in both patients, but analysis of cerebrospinal fluid (CSF) revealed chronic inflammation. KLHL11-IgG was positive in both patients (Patient 1 only in CSF, Patient 2 in serum). Neoplastic screening has so far not revealed any signs of active underlying malignancy. We found 15 publications of 112 patients in total with KLHL11-IgG encephalitis. Most patients (n = 82) had a cerebellar syndrome with or without signs of rhombencephalitis. The most common symptoms were ataxia (n = 82) and vertigo (n = 47), followed by oculomotor disturbances (n = 35) and hearing disorders (n = 31). Eighty of 84 patients had a GCT as an underlying tumor. Conclusions: Our cases demonstrate classical symptoms of KLHL11-IgG encephalitis.Early diagnosis and therapy are imperative. As with other PNSs, clinical awareness is needed and further studies are required especially in regard to therapeutic management.

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Comment on “Paraneoplastic encephalomyelitis revealing burned-out seminoma”: confirmed case of anti-Kelch-like protein-11 encephalomyelitis

et al. 2021

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Paraneoplastic neurological syndromes occur in association with cancer and have an immune-mediated pathogenesis, but sometimes neuronal antibodies cannot be identified. Recently, Guilmot et al. [1] published a Letter to the Editor describing a case of paraneoplastic encephalomyelitis that led to the diagnosis of a burned-out seminoma, but the patient remained seronegative for all the onconeuronal antibodies known at that time. The 52-year-old patient presented with hearing loss, vertigo, diplopia, nystagmus, and ataxia, and later developed gait difficulties, limb incoordination, and left arm weakness. Repeated brain magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintensities in both thalami, brainstem, and right temporal lobe, while spinal cord imaging showed an extensive myelitis. Cerebrospinal fluid (CSF) studies showed lymphocytic pleocytosis, elevated protein levels, and CSF-specific IgG oligoclonal bands. Given the suspicion of a paraneoplastic syndrome, an extensive workup was performed and revealed a para-aortic nodal metastasis of a burned-out seminoma. Despite orchiectomy, radiotherapy, and multiples lines of immunotherapy, the patient died 16 months after symptom onset.Kelch-Like Family Member 11 (KLHL11) is an intra-cellular protein involved in ubiquitination [2]. Autoantibodies * Vincent van Pesch

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Paraneoplastic encephalomyelitis revealing burned-out seminoma

Cited by 5 publications

References 5 publications

Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic “burned-out” seminoma – A scar(r)y phenomenon

Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic “burned-out” seminoma – A scar(r)y phenomenon

Anti‐kelchlike protein 11 antibody‐associated encephalitis: Two case reports and review of the literature

Comment on “Paraneoplastic encephalomyelitis revealing burned-out seminoma”: confirmed case of anti-Kelch-like protein-11 encephalomyelitis

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