JNNP: Relationship between sub-tumor-related marginal encephalitis and olive nuclear degeneration under hypertrophobicity

Olive nuclear degeneration (HOD) is a disease caused by the interruption of the white beam in the Dentato-rubro-olivary path path (DROP), resulting in a high signal and MRI increase in the lower olive leaf.
olive atrophy can be seen as a hang-out of HOD.
HOD and cerebral stem infarction, tumors, demyelination and vascular lesions (e.g. spongy malformations).
, however, HOD can also occur without any lesions, sometimes idyrogenic encephalitis, which is an inflammation that affects the brain stem and/or the small brain and is rarely caused by a side tumor syndrome.
a number of neurosynthic antibodies are typically associated with sub-tumor encephalitis, including anti-Ma2 and anti-neuronal antibody type 2 (ANNA2 or anti-Ri).
Kelch-like protein 11 (KLHL11) IgG is another serum marker recently described as closely related to by-tumor encephalitis.
commonly associated with by-tumor encephalitis include testicular serum cell tumors, breast cancer, small cell lung cancer, and gynecological malignancies.
or brain MRI is usually normal or shows a contraction of the brain and the small brain.
MRIs in these patients also rarely showed high signals and enhancement of brain trunk T2.
recently reported case of KLHL11 encephalitis was accidentally found to have left HOD caused by tooth lesions.
, even in a comprehensive review, no clear link between sub-tumor encephalitis and HOD has been previously found.
this paper reported six cases of tumor nerve antibody-related side tumor patients with HOD encephalitis.
test patients serum and/or cerebrospinal fluid, autoantibodies are serum-positive: type 1 anti-neuronal antibodies (ANA1/anti-Hu), KLHL11, Ma2, ANNA-2 (anti-Ri), Poken wild cell antibody type 1 (PCA-1, anti-Yo), CRMP5 or anti-CV2.
177 patients were found (KLHL11, n=24; Ma2, n=11; ANNA-1, n=60; ANNA-2, n=14; PCA-1, n=32; biceps, n=9; CRMP5, n=22; PCA Tr, n=5).
3 patients were excluded due to lack of available brain MRIs (2 KLHL11 patients and 1 PCA-1 patient).
retrospective analysis of brain magnetic resonance imaging and clinical characteristics in 174 patients to evaluate its diagnostic value of HOD.
HOD was based on imaging results that showed high signals of lower olive leaf T2 and enlarged brain MRI.
review clinical records to determine any conclusive results, such as palate tremors, co-relief disorders, visual changes, or muscle weakness.
review of neurotypes and MRI brain images in 174 patients with positive tumor neuroantibodies included: by-tumor encephalitis (n-30), by-tumor limbic encephalitis (n-35), by-tumor cer cerebral degeneration (n-43), edge and encephalitis merging (n-5) ), para-tumor encephalopathy (n-10), sub-tumor neuropathy (n-33, including sensory motor neuropathy, polyneural root neuropathy or sensory neuropathy), sub-tumor gastric palsy and/or autonotic neuro dysfunction (n-8) and severe muscle weakness (n-7).
identified six patients, all clinically diagnosed as sub-tumor encephalitis, which developed into HOD during the course of the disease.
three are men and three are women, aged 30-62.
antibodies identified by the company include KLHL11 IgG, AntiMa2, ANA-2, and PCA-1.
five patients were diagnosed with malignant tumors within a year, including testicular germ cell tumors and breast cancer.
patient 3 had a history of pregenital peritina post-germ cell tumors and had successfully underwent chemotherapy a year ago.
all patients initially showed a months to years of aggressive gait disorder, with the exception of patient 2, all patients with gaze paralysis.
patient 1 also experienced hidden palate tremors.
all patients who detected potential malignancies were treated with primary tumor removal and chemotherapy.
patients were followed with fluoro deoxygenated glucose positive electron emission fault scans/CT every 3-6 months for 8 years without any signs of cancer recurrence.
patient 4 under the testicular ultrasound and body CT scan about 1 year after the initial diagnosis, no recurrence.
other patients did not have any routine malignant tumor screening.
patients began immunotherapy at a median age of 7.5 months after the on-the-on-the-appearance of symptoms.
HOD was 84 months after symptoms appeared, ranging from 7 to 120 months.
side HOD in patient 1 (Figure 1A) and one-sided HOD in patients 2-5 (Figure 1B-E).
patients with 6 had one-sided olive atrophy, consistent with chronic atrophic HOD sequelae.
patients with the exception of patient 3, all patients had aggressive double-sided small brain atrophy.
no patients in the drops.
recognized the clinical significance of the link between HOD and by-tumor encephalitis.
from a diagnostic point of view, our study recommends that the cause of the by-tumor be considered when identifying HOD patients, especially in the absence of a clear cause.
these patients may manifest subacute onset of cerebral and/or cerebral dysfunction, inflammation of cerebrospinal fluid, weight loss and hearing loss/tinnitus, especially in patients with KLHL11 encephalitis.
clinically highly suspected patients with sub-tumor encephalitis, 3t or 7t MRIs may be more sensitive in detecting HOD and DROP lesions, but further research is needed to determine this.
from a prognostic point of view, studies have shown that patients with hod's side tumor encephalitis may have a more difficult course of treatment.
further study of treatment options and comparison with patients without HOD will help determine whether HOD is a true marker of progressive or incurable diseases.
Madhavan A, Carr CM, Krecke KN, et al Association between paraneoplastic rhombenitis and hypertrophic olivary degeneration Journal of Neurology, Neurosurgery and Psymy Published Online First: 15 January 2021. doi: 10.1136/jnnp-2020-325569MedSci Original Source: MedSci Original Copyright Notice: All text, images and audio and video materials on this website that indicate "Source: Met Medical" or "Source: MedSci Original" are owned by Mets Medicine and are not authorized by any media, website or individual. "Source: Metz Medicine" shall be indicated at the time of authorization for reprint.
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