TRIM46¶
Tripartite motif-containing protein 46¶
Description¶
TRIM46-IgG is newly described biomarker of a range of paraneoplastic CNS disorders including cerebellar syndromes, encephalopathy and seizures, limbic encephalitis, brainstem encephalitis, myelopathy, parkinsonism/dystonia.
Mechanism of action¶
Paraneoplastic syndromes
Associated clinical features¶
In an initial series of 22 patients subacute cerebellar syndrome (6 isolated; 9 with CNS accompaniments: encephalopathy (3), brainstem signs (2), myelopathy (2), parkinsonism (1)).
Other phenotypes included limbic encephalitis (3), encephalopathy with/without seizures (2), myelopathy (2).
Associated neoplasia¶
High risk
In an initial series of 22 patients, 18 (82%) had cancer:
-
Neuroendocrine carcinomas (9)
- Pancreatic (3)
- Small-cell lung (4)
- Oesophagus (1)
- Endometrium (1)
-
Adenocarcinomas (6)
- Lung (2)
- Ovarian (2)
- Endometrial (1)
- Breast (1)
- Sarcoma (2)
- Gastrointestinal tumour (1)
Neurological symptoms in three patients followed immune checkpoint inhibitor (ICI) administration.
See reference.1
Laboratory method¶
- Immunohistochemistry screening
- Western blotting
- Cell-based assay
Notes of performance characteristics¶
Grade¶
Next steps¶
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Valencia-Sanchez C, Knight AM, Hammami MB, Guo Y, Mills JR, Kryzer TJ, Piquet AL, Amin A, Heinzelmann M, Lucchinetti CF, Lennon VA, McKeon A, Pittock SJ, Dubey D. Characterisation of TRIM46 autoantibody-associated paraneoplastic neurological syndrome. J Neurol Neurosurg Psychiatry. 2022 Feb;93(2):196-200. ↩
Created: 2022-04-07