3.Efficacy and safety of cyclophosphamide as a sequential immunotherapy drug for anti-N-methyl-D-aspartate receptor encephalitis in children.
Wei-Wen ZHU ; Wei-Ping LIAO ; Yong-Hong YI ; Xing-Wang SONG
Chinese Journal of Contemporary Pediatrics 2017;19(6):668-671
OBJECTIVETo evaluate the efficacy and safety of cyclophosphamide as a second-line drug in the treatment of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
METHODSSix children with anti-NMDAR encephalitis, who showed poor response to steroids and intravenous immunoglobulin, were given cyclophosphamide as a second-line immunotherapy. Follow-up was performed to evaluate the efficacy and safety of cyclophosphamide.
RESULTSAfter first-line immunotherapy for 1-4 weeks, the six patients had reduced psychiatric symptoms, seizures, and involuntary movements; three patients had an improved level of consciousness and were able to make simple conversations. However, all the patients still showed slow response, as well as cortical dysfunction symptoms such as aphasia, alexia, agraphia, acalculia, apraxia, and movement disorders. The six patients continued to receive cyclophosphamide as a sequential therapy. They were able to answer simple questions 7 days after treatment. Three school-aged patients were able to make simple calculation, had greatly improved reading and writing ability, and almost recovered self-care ability 2-3 weeks later. The cognitive function of the six patients was almost restored to the level before the onset of disease, and their living ability returned to normal 2-3 months later. During the treatment period, there were no adverse reactions or abnormal results of routine blood test and liver and kidney function tests.
CONCLUSIONSChildren with anti-NMDAR encephalitis should be given appropriate immunotherapy as soon as possible. Cyclophosphamide as a sequential therapy has good efficacy and safety.
Adolescent ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis ; drug therapy ; physiopathology ; psychology ; Child ; Child, Preschool ; Cognition ; Cyclophosphamide ; therapeutic use ; Female ; Humans ; Immunotherapy ; Male
4.Neurologic Manifestations of Enterovirus 71 Infection in Korea.
Kyung Yeon LEE ; Myoung Sook LEE ; Dong Bin KIM
Journal of Korean Medical Science 2016;31(4):561-567
		                        		
		                        			
		                        			Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients' mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection.
		                        		
		                        		
		                        		
		                        			Acute Disease
		                        			;
		                        		
		                        			Brain/diagnostic imaging
		                        			;
		                        		
		                        			Central Nervous System Diseases/etiology/*pathology
		                        			;
		                        		
		                        			Child
		                        			;
		                        		
		                        			Child, Preschool
		                        			;
		                        		
		                        			Encephalitis/pathology
		                        			;
		                        		
		                        			Enterovirus A, Human/genetics/*isolation & purification
		                        			;
		                        		
		                        			Enterovirus Infections/drug therapy/*pathology/virology
		                        			;
		                        		
		                        			Feces/virology
		                        			;
		                        		
		                        			Female
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Immunoglobulins/administration & dosage
		                        			;
		                        		
		                        			Infant
		                        			;
		                        		
		                        			Injections, Intravenous
		                        			;
		                        		
		                        			Leukocytes/cytology
		                        			;
		                        		
		                        			Leukocytosis/cerebrospinal fluid/pathology
		                        			;
		                        		
		                        			Magnetic Resonance Imaging
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			RNA, Viral/genetics/metabolism
		                        			;
		                        		
		                        			Real-Time Polymerase Chain Reaction
		                        			;
		                        		
		                        			Republic of Korea
		                        			;
		                        		
		                        			Retrospective Studies
		                        			;
		                        		
		                        			Seasons
		                        			
		                        		
		                        	
5.Severe Chicken Pox in an Immunocompromised Host after Chemotherapy.
Jin Woong JUNG ; Jun Il KWON ; Sung Ae KIM ; Young Wook RYOO
Keimyung Medical Journal 2016;35(2):147-152
		                        		
		                        			
		                        			Chicken pox is highly contagious and occurs most often in childhood. In normal children, systemic symptoms are usually mild and serious complications are rare. But in adults, especially in immunocompromised patients, it is more likely to be associated with life-threatening complications such as pneumonia, encephalitis and sepsis. A 38-year-old woman was admitted due to headache, fever and multiple umbilicated vesicles on face, trunk and both extremities for 3 days. She underwent chemotherapy for the breast cancer 20 days prior to admission. Based on typical clinical presentation and skin biopsy, she was diagnosed with chicken pox. Despite 5 days of intensive intravenous antiviral therapy (Acyclovir® 30 mg/kg/day), fever and headache continued and epigastric pain occurred. Finally, the patient was recovered from varicella after additional 7 days of antiviral therapy. Generally, old age or immunocompromised hosts have declined cellular immunity, so the incidence of chicken pox is higher and the clinical course is more severe. Herein, we report a case of chicken pox in the patient who underwent chemotherapy for breast cancer, requiring intensive treatment and close observation for systemic symptoms.
		                        		
		                        		
		                        		
		                        			Adult
		                        			;
		                        		
		                        			Biopsy
		                        			;
		                        		
		                        			Breast Neoplasms
		                        			;
		                        		
		                        			Chickenpox*
		                        			;
		                        		
		                        			Chickens*
		                        			;
		                        		
		                        			Child
		                        			;
		                        		
		                        			Drug Therapy*
		                        			;
		                        		
		                        			Encephalitis
		                        			;
		                        		
		                        			Extremities
		                        			;
		                        		
		                        			Female
		                        			;
		                        		
		                        			Fever
		                        			;
		                        		
		                        			Headache
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Immunity, Cellular
		                        			;
		                        		
		                        			Immunocompromised Host*
		                        			;
		                        		
		                        			Incidence
		                        			;
		                        		
		                        			Pneumonia
		                        			;
		                        		
		                        			Sepsis
		                        			;
		                        		
		                        			Skin
		                        			
		                        		
		                        	
6.Paraneoplastic Limbic Encephalitis in a Male with Squamous Cell Carcinoma of the Lung.
Tamara SAURI ; Angel IZQUIERDO ; LLuis RAMIO-TORRENTA ; Angel SANCHEZ-MONTANEZ ; Joaquim BOSCH-BARRERA ; Rut PORTA
Journal of Clinical Neurology 2015;11(1):87-91
		                        		
		                        			
		                        			BACKGROUND: Paraneoplastic limbic encephalitis (PLE) is a rare syndrome characterized by memory impairment, symptoms of hypothalamic dysfunction, and seizures. It commonly precedes the diagnosis of cancer. Small-cell lung cancer is the neoplasm that is most frequently reported as the etiology underlying PLE. CASE REPORT: This report describes a male patient who presented with neurologic symptoms consistent with anterograde amnesia, apathy, and disorientation. MRI revealed diffuse hyperintensities located predominantly in the medial bitemporal lobes, basal ganglia, frontal lobes, and leptomeninges on fluid attenuated inversion recovery images, suggesting PLE. Study of the primary tumor revealed squamous cell carcinoma of the lung. The patient was treated with neoadjuvant chemotherapy followed by surgery and adjuvant chemoradiotherapy, which resulted in his neurologic symptoms gradually improving. CONCLUSIONS: PLE might be a rare debut of squamous cell carcinoma of the lung. Treatment of the primary tumor may improve the neurologic symptoms.
		                        		
		                        		
		                        		
		                        			Amnesia, Anterograde
		                        			;
		                        		
		                        			Apathy
		                        			;
		                        		
		                        			Basal Ganglia
		                        			;
		                        		
		                        			Carcinoma, Squamous Cell*
		                        			;
		                        		
		                        			Chemoradiotherapy, Adjuvant
		                        			;
		                        		
		                        			Diagnosis
		                        			;
		                        		
		                        			Drug Therapy
		                        			;
		                        		
		                        			Frontal Lobe
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Limbic Encephalitis*
		                        			;
		                        		
		                        			Lung Neoplasms
		                        			;
		                        		
		                        			Lung*
		                        			;
		                        		
		                        			Magnetic Resonance Imaging
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Memory
		                        			;
		                        		
		                        			Neurologic Manifestations
		                        			;
		                        		
		                        			Paraneoplastic Syndromes
		                        			;
		                        		
		                        			Seizures
		                        			
		                        		
		                        	
7.Ramsay Hunt Syndrome Complicated by Brainstem Encephalitis in Varicella-zoster Virus Infection.
Yao-Yao SHEN ; Ting-Min DAI ; Hai-Ling LIU ; Wei WU ; Jiang-Long TU
Chinese Medical Journal 2015;128(23):3258-3259
		                        		
		                        		
		                        		
		                        			Acyclovir
		                        			;
		                        		
		                        			therapeutic use
		                        			;
		                        		
		                        			Anti-Inflammatory Agents
		                        			;
		                        		
		                        			therapeutic use
		                        			;
		                        		
		                        			Antiviral Agents
		                        			;
		                        		
		                        			therapeutic use
		                        			;
		                        		
		                        			Brain Stem
		                        			;
		                        		
		                        			pathology
		                        			;
		                        		
		                        			virology
		                        			;
		                        		
		                        			Encephalitis
		                        			;
		                        		
		                        			complications
		                        			;
		                        		
		                        			diagnosis
		                        			;
		                        		
		                        			drug therapy
		                        			;
		                        		
		                        			virology
		                        			;
		                        		
		                        			Herpes Zoster
		                        			;
		                        		
		                        			complications
		                        			;
		                        		
		                        			diagnosis
		                        			;
		                        		
		                        			drug therapy
		                        			;
		                        		
		                        			virology
		                        			;
		                        		
		                        			Herpes Zoster Oticus
		                        			;
		                        		
		                        			diagnosis
		                        			;
		                        		
		                        			drug therapy
		                        			;
		                        		
		                        			etiology
		                        			;
		                        		
		                        			virology
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Male
		                        			;
		                        		
		                        			Methylprednisolone
		                        			;
		                        		
		                        			therapeutic use
		                        			;
		                        		
		                        			Middle Aged
		                        			
		                        		
		                        	
9.A case of limbic encephalitis with positive antibody to the GABAB receptor.
Yan ZHANG ; Ying-Ying SU ; Yuan GAO
Chinese Medical Journal 2013;126(18):3599-3600
10.Toxoplasma Encephalitis in an Allogeneic Hematopoietic Stem Cell Transplant Recipient in Korea.
Soo Kyung PARK ; Jong Ki CHOI ; Changhoon YOO ; Seong Joon PARK ; Tae Hoon LEE ; Je Hwan LEE ; Sung Han KIM
The Korean Journal of Internal Medicine 2012;27(2):235-238
		                        		
		                        			
		                        			No abstract available.
		                        		
		                        		
		                        		
		                        			Adult
		                        			;
		                        		
		                        			Anemia, Aplastic/*surgery
		                        			;
		                        		
		                        			Antiprotozoal Agents/therapeutic use
		                        			;
		                        		
		                        			Drug Therapy, Combination
		                        			;
		                        		
		                        			Encephalitis/diagnosis/drug therapy/*parasitology
		                        			;
		                        		
		                        			Female
		                        			;
		                        		
		                        			Hematopoietic Stem Cell Transplantation/*adverse effects
		                        			;
		                        		
		                        			Humans
		                        			;
		                        		
		                        			Immunosuppressive Agents/adverse effects
		                        			;
		                        		
		                        			Magnetic Resonance Imaging
		                        			;
		                        		
		                        			Parasitology/methods
		                        			;
		                        		
		                        			Polymerase Chain Reaction
		                        			;
		                        		
		                        			Republic of Korea
		                        			;
		                        		
		                        			Toxoplasma/genetics/*isolation & purification
		                        			;
		                        		
		                        			Toxoplasmosis, Cerebral/diagnosis/drug therapy/*parasitology
		                        			;
		                        		
		                        			Transplantation, Homologous
		                        			;
		                        		
		                        			Treatment Outcome
		                        			
		                        		
		                        	
            
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