Image features of anti-SEZ6L2 encephalitis, a rare cause of ataxia and parkinsonism

Shen, Dingding; Zhou, Qinming; Meng, Huanyu; Zhang, Min; Peng, Lisheng; Chen, Sheng

doi:10.1007/s00415-023-11756-5

Cited by 2 publications

(1 citation statement)

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“…Patients developed subacute cerebellar syndrome that was frequently associated with symptoms of dysautonomia, parkinsonism, and cognitive impairment. Brain MRI was normal or showed a discrete cerebellar atrophy [24–26]. Only 23% (3/13) of patients made a clear response to immunotherapy [27,28 ▪ ].…”

Section: Cerebellar Ataxias and Neuronal Antibodies Against Surface A...mentioning

confidence: 99%

Neuronal antibodies in nonparaneoplastic autoimmune cerebellar ataxias

Saiz,

Graus

2024

Current Opinion in Neurology

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Purpose of review To describe relevant advances in nonparaneoplastic autoimmune cerebellar ataxias (ACA) with neuronal antibodies. Recent findings Apart from metabotropic glutamate receptor 1(mGluR1) antibodies, in recent years, the number of neuronal antibodies against surface antigens in ACA has increased with the description of glutamate kainate receptor subunit 2 (GluK2) antibodies in young patients with cerebellitis. Around 20% of patients with contactin-associated protein-like 2 (CASPR2) encephalitis also present prominent cerebellar ataxia. However, isolate cerebellar ataxia is unusual (<4%). Outcome in patients with neuronal antibodies against surface antigens remains suboptimal despite the cerebellar ataxia probably is antibody-mediated. Concerning neuronal antibodies against intracellular antigens, up to 25% of patients with glutamic acid decarboxylase (GAD) antibodies present transient episodes of vertigo or diplopia that antedate the development of the ACA. There is in-vitro evidence that septin-5 is partially exposed to the membrane and the antibodies may interfere with septin-5 function. The clinical significance of the remaining antibodies against intracellular antigens remains unclear. Summary The number of antibodies against surface antigens is increasing in ACA, but the response to the immunotherapy remains suboptimal. More studies are needed to clarify the role of most of the antibodies against intracellular antigens described in these patients.

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Section: Cerebellar Ataxias and Neuronal Antibodies Against Surface A...mentioning

confidence: 99%