Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory disease that most often affects the optic nerves and spinal cord. We describe a case of 36-year-old woman presented at 13 weeks of gestation with 4 extremities paresthesia and weakness that had lasted for two months at her first visit to our hospital. She had two previous uncomplicated full-term vaginal deliveries and no significant medical or family history. Spine magnetic resonance imaging (MRI) showed extensive cervical cord lesion and aquaporin-4 antibodies were strongly positive, confirming the diagnosis of NMOSD. Initial management with high-dose corticosteroids and plasmapheresis was done and she showed substantial improvement, but she revisited hospital at 26 weeks of gestational age due to visual disturbance and aggravated weakness. Relapse of NMOSD was confirmed by spine MRI, so rituximab therapy was initiated at 28 weeks of gestational age for prevention of recurrence.The patient showed clinical improvement with no adverse effects and relapse of symptoms. She successfully delivered a healthy male infant at 39 weeks and 3 days of gestational age through uncomplicated vaginal delivery. This case demonstrates successful management of new-onset NMOSD during pregnancy using a multi-modal treatment approach including rituximab.

Lecanemab (product name Leqembi ® ) is an anti-amyloid monoclonal antibody treatment approved for use in Korea for patients with mild cognitive impairment (MCI) or mild dementia due to Alzheimer's disease. The Korean Dementia Association has created recommendations for the appropriate use of lecanemab to assist clinicians. These recommendations include selecting patients for administration, necessary pre-administration tests and preparations,administration methods, monitoring for amyloid related imaging abnormalities (ARIA), and communication with patients and caregivers. Lecanemab is recommended for patients with MCI or mild dementia who confirmed positive amyloid biomarkers, and should not be administered to patients with severe hypersensitivity to lecanemab or those unable to undergo magnetic resonance imaging (MRI) evaluation. To predict the risk of ARIA before administration, apolipoprotein E genotyping is conducted, and regular brain MRI evaluations are recommended to monitor for ARIA during treatment. The most common adverse reactions are infusion-related reactions, which require appropriate management upon occurrence. Additional caution is needed when co-administering with anticoagulants or tissue plasminogen activator due to the risk of macrohemorrhage. Clinicians should consider the efficacy and necessary conditions for administration, as well as the safety of lecanemab, to make a comprehensive decision regarding its use.

Background: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by tumors in multiple endocrine organs, caused by variants in the MEN1 gene. This study analyzed the clinical and genetic features of MEN1 in a Korean cohort, identifying prevalent manifestations and genetic variants, including novel variants. Methods: This multicenter retrospective study reviewed the medical records of 117 MEN1 patients treated at three tertiary centers in Korea between January 2012 and September 2022. Patient demographics, tumor manifestations, outcomes, and MEN1 genetic testing results were collected. Variants were classified using American College of Medical Genetics and Genomics (ACMG) and French Oncogenetics Network of Neuroendocrine Tumors propositions (TENGEN) guidelines. Results: A total of 117 patients were enrolled, including 55 familial cases, with a mean age at diagnosis of 37.4±15.3 years. Primary hyperparathyroidism was identified as the most common presentation (84.6%). The prevalence of gastroenteropancreatic neuroendocrine tumor and pituitary neuroendocrine tumor (PitNET) was 77.8% (n=91) and 56.4% (n=66), respectively. Genetic testing revealed 61 distinct MEN1 variants in 101 patients, with 18 being novel. Four variants were reclassified according to the TENGEN guidelines. Patients with truncating variants (n=72) exhibited a higher prevalence of PitNETs compared to those with non-truncating variants (n=25) (59.7% vs. 36.0%, P=0.040). Conclusion The association between truncating variants and an increased prevalence of PitNETs in MEN1 underscores the importance of genetic characterization in guiding the clinical management of this disease. Our study sheds light on the clinical and genetic characteristics of MEN1 among the Korean population.

Lecanemab (product name Leqembi ® ) is an anti-amyloid monoclonal antibody treatment approved for use in Korea for patients with mild cognitive impairment (MCI) or mild dementia due to Alzheimer's disease. The Korean Dementia Association has created recommendations for the appropriate use of lecanemab to assist clinicians. These recommendations include selecting patients for administration, necessary pre-administration tests and preparations,administration methods, monitoring for amyloid related imaging abnormalities (ARIA), and communication with patients and caregivers. Lecanemab is recommended for patients with MCI or mild dementia who confirmed positive amyloid biomarkers, and should not be administered to patients with severe hypersensitivity to lecanemab or those unable to undergo magnetic resonance imaging (MRI) evaluation. To predict the risk of ARIA before administration, apolipoprotein E genotyping is conducted, and regular brain MRI evaluations are recommended to monitor for ARIA during treatment. The most common adverse reactions are infusion-related reactions, which require appropriate management upon occurrence. Additional caution is needed when co-administering with anticoagulants or tissue plasminogen activator due to the risk of macrohemorrhage. Clinicians should consider the efficacy and necessary conditions for administration, as well as the safety of lecanemab, to make a comprehensive decision regarding its use.

Background: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by tumors in multiple endocrine organs, caused by variants in the MEN1 gene. This study analyzed the clinical and genetic features of MEN1 in a Korean cohort, identifying prevalent manifestations and genetic variants, including novel variants. Methods: This multicenter retrospective study reviewed the medical records of 117 MEN1 patients treated at three tertiary centers in Korea between January 2012 and September 2022. Patient demographics, tumor manifestations, outcomes, and MEN1 genetic testing results were collected. Variants were classified using American College of Medical Genetics and Genomics (ACMG) and French Oncogenetics Network of Neuroendocrine Tumors propositions (TENGEN) guidelines. Results: A total of 117 patients were enrolled, including 55 familial cases, with a mean age at diagnosis of 37.4±15.3 years. Primary hyperparathyroidism was identified as the most common presentation (84.6%). The prevalence of gastroenteropancreatic neuroendocrine tumor and pituitary neuroendocrine tumor (PitNET) was 77.8% (n=91) and 56.4% (n=66), respectively. Genetic testing revealed 61 distinct MEN1 variants in 101 patients, with 18 being novel. Four variants were reclassified according to the TENGEN guidelines. Patients with truncating variants (n=72) exhibited a higher prevalence of PitNETs compared to those with non-truncating variants (n=25) (59.7% vs. 36.0%, P=0.040). Conclusion The association between truncating variants and an increased prevalence of PitNETs in MEN1 underscores the importance of genetic characterization in guiding the clinical management of this disease. Our study sheds light on the clinical and genetic characteristics of MEN1 among the Korean population.

Lecanemab (product name Leqembi ® ) is an anti-amyloid monoclonal antibody treatment approved for use in Korea for patients with mild cognitive impairment (MCI) or mild dementia due to Alzheimer's disease. The Korean Dementia Association has created recommendations for the appropriate use of lecanemab to assist clinicians. These recommendations include selecting patients for administration, necessary pre-administration tests and preparations,administration methods, monitoring for amyloid related imaging abnormalities (ARIA), and communication with patients and caregivers. Lecanemab is recommended for patients with MCI or mild dementia who confirmed positive amyloid biomarkers, and should not be administered to patients with severe hypersensitivity to lecanemab or those unable to undergo magnetic resonance imaging (MRI) evaluation. To predict the risk of ARIA before administration, apolipoprotein E genotyping is conducted, and regular brain MRI evaluations are recommended to monitor for ARIA during treatment. The most common adverse reactions are infusion-related reactions, which require appropriate management upon occurrence. Additional caution is needed when co-administering with anticoagulants or tissue plasminogen activator due to the risk of macrohemorrhage. Clinicians should consider the efficacy and necessary conditions for administration, as well as the safety of lecanemab, to make a comprehensive decision regarding its use.