1.Therapeutic Outcomes and Electrophysiological Biomarkers in Anti-Myelin-Associated Glycoprotein Neuropathy:A Multicenter Cohort Study in South Korea
Young Gi MIN ; Hee-Jo HAN ; Ha Young SHIN ; Jong-Gyu BAEK ; Jun-Soon KIM ; Kyung-Seok PARK ; Seol-Hee BAEK ; Ilhan YOO ; So-Young HUH ; Young Nam KWON ; Seok-Jin CHOI ; Sung-Min KIM ; Yoon-Ho HONG ; Jung-Joon SUNG
Journal of Clinical Neurology 2024;20(1):50-58
Background:
and Purpose Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management.
Methods:
This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases.
Results:
The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792).
Conclusions
Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.
2.Recurrent Seizures in a Patient with Primary Hypoparathyroidism
Hyung-Kyum KIM ; Jung-Ju LEE ; Byung-Kun KIM ; Kyusik KANG ; Woong-Woo LEE ; Ilhan YOO ; Yong Soo KIM
Journal of the Korean Neurological Association 2024;42(3):274-277
Primary hypoparathyroidism is a rare disorder that presents with various psychiatric and neurological symptoms. A 50-year-old female patient visited hospital due to recurrent seizures. Despite treatment with phenytoin and diazepam, her seizures persisted. Laboratory tests revealed hypocalcemia, hyperphosphatemia, and decreased intact parathyroid hormone level. Following treatment with valproic acid and calcium supplementation, her seizures ceased. Thorough examination including laboratory tests play a crucial role in the accurate diagnosis of primary hypoparathyroidism in a patient with recurrent seizures.
4.Diagnosis of Lymphoid Malignancy by PCR for Analysis of Antigen Receptor Rearrangement after Blood Transfusion in a Dog with Acute Lymphocytic Leukemia.
Suhee KIM ; Hyunwoo KIM ; Soo Hyeon LEE ; Ilhan CHO ; Seongwoo KANG ; Junwoo BAE ; Woosun KIM ; Soomin AHN ; Jihye CHOI ; Sang Ki KIM ; Yoonjung DO ; Jae Gyu YOO ; Jinho PARK ; DoHyeon YU
Immune Network 2017;17(4):269-274
Acute lymphocytic leukemia (ALL) is uncommon lymphoid malignancy in dogs, and its diagnosis is challenging. A 14-year-old spayed female mixed breed dog was transferred to a veterinary medical teaching hospital for an immediate blood transfusion. The dog showed lethargy, pale mucous membranes, and a weak femoral pulse. Complete blood count revealed non-regenerative anemia and severe leukopenia with thrombocytopenia. ALL was tentatively diagnosed based on the predominance of immature lymphoblasts on blood film examination. For confirmation of lymphoid malignancy, PCR for antigen receptor rearrangement (PARR) on a peripheral blood sample and flow cytometry analysis were performed after blood transfusion. Flow cytometry analysis revealed that lymphocyte subsets were of normal composition, but PARR detected a T-cell malignancy. The dog was diagnosed with ALL and survived 1 wk after diagnosis. In conclusion, after blood transfusion, flow cytometry was not a reliable diagnostic method for an ALL dog, whereas PARR could detect lymphoid malignancy. Our results suggest that PARR should be the first-line diagnostic tool to detect canine lymphoid malignancy after a blood transfusion.
Adolescent
;
Anemia
;
Animals
;
Blood Cell Count
;
Blood Transfusion*
;
Diagnosis*
;
Dogs*
;
Female
;
Flow Cytometry
;
Hospitals, Teaching
;
Humans
;
Lethargy
;
Leukopenia
;
Lymphocyte Subsets
;
Methods
;
Mucous Membrane
;
Polymerase Chain Reaction*
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
;
Receptors, Antigen*
;
T-Lymphocytes
;
Thrombocytopenia

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