| ObjectiveThe clinical data of 25 patients with antibodies against contactin-associated protein-like 2(CASPR2)encephalitis were sorted and analyzed,and the clinical characteristics of encephalitis were summarized.This study explored the treatment,prognosis and factors affecting the prognosis of the disease in order to improve the understanding of the disease and reduce the misdiagnosis rate.MethodsThe clinical data of 25 anti-CASPR2 antibody-associated autoimmune encephalitis patients who were admited to the Fifth and first Affiliated Hospital of Zhengzhou University from December 2017 to December 2020 were collected.The general situation,clinical manifestations,laboratory examination results,imaging data and electrophysiological,treatment plan and prognosis of the patients were summarized and analyzed.We grouped them according to the level of serum anti-CASPR2 antibody titer,the level of Modified Rankin Scale followed up for 3months after discharge,and different treatment options.We analyzed the collected data.Results1.General situation: Among the 25 patients,13 were male(52%)and 12 were female(48%),with a median age of 49 years(range,10-72 years).2 cases(8%)had predisposing factors,namely upper respiratory tract infection and periodontal abscess.11 cases(44%)had prodromal symptoms,including 3 cases of fever with headache,5cases of headache and 3 cases of fever.Patients may have other immune diseases and tumors: 12 cases of anemia,2 cases of thymoma,1 case each of optic neuritis,ankylosing spondylitis and lung cancer.2.Clinical manifestations: The first symptoms of the disease included 8 cases(32%)of epilepsy,6 cases(24%)of cognitive impairment,4 cases(16%)of limb pain,2 cases(8%)of limb muscle twitching,2 cases(8%)of mental behavior abnormality,1 case(4%)of cranial nerve damage,1 case(4%)of dizziness and 1 case(4%)of sleep disorder.The main symptoms of the disease included 13 cases(52%)of cognitive impairment,10 cases(40%)of epileptic seizures,10 cases(40%)of psychobehavioral disorders,2 cases(8%)of cerebellar ataxia,7 cases(28%)of muscle involuntary beat,7 cases(28%)of limb muscle pain or skin pruritus burning sensation,19 cases(76%)of autonomic nervous dysfunction,7 cases(28%)of insomnia,5 cases(20%)of increased sleep.Weight loss in 4 cases(16%),visual acuity decreased in 2 cases(8%),and cranial nerve injury in 2 cases(8%).3.Laboratory examination: 12 cases(48%)may have reduced red blood cell count and hemoglobin.7 cases(28%)had decreased serum potassium,and 4 cases(16%)had decreased serum sodium.The cerebrospinal fluid pressure is60-320 mm H2O,with a median of 140 mm H2O.In cerebrospinal fluid examination,the white blood cell count increased in 5 cases(20%),lymphocytosis in 8 cases(32%),protein quantitative increased in 5 cases(20%),Ig G index increased in 9 cases(36%),oligoclonal zone 3 cases(12%)were positive.The positive rate of serum anti-CASPR2 antibodies was 100%,of which 16 cases(64%)were positive,7 cases(28%)were strongly positive,and 2 cases(8%)were weakly positive;the cerebrospinal fluid positive rate was 32%,of which 5 cases(20%)were weakly positive and 3 cases(12%)were positive.4.Imaging examination: 5 cases(20%)showed high signal intensity on T2 WI and FLAIR in bilateral hippocampus,fronto-parietal temporo-occipital lobe,basal ganglia and thalamus on MRI.1 case of PET-CT showed bilateral frontal lobe and bilateral parietal lobe metabolism decreased.5.Neuroelectrophysiology: 16 cases(64%)had abnormal EEG,including 6cases(24%)of epileptic waves and 13 cases(52%)of localized or extensive slow waves.5 cases(50%)showed double,triple or multiple motor unit discharge activity on the EMG examination.6.Treatment: All patients were given first-line immunotherapy in the acute phase,15 cases(60%)were treated with glucocorticoid shock alone,5 cases(20%)were treated with hormone shock combined with IVIg,and 5 cases(20%)were treated with hormone shock combined with plasma exchange.21 patients(84%)received maintenance treatment,14(56%)of which were maintained with low-dose glucocorticoids,5(20%)were treated with rituximab,and 2(84%)were treated with low-dose hormones combined with mycophenolate mofetil.After discharge,5cases relapsed and 2 cases died.5 patients treated with rituximab did not relapse and basically returned to normal.The effect of rituximab is better than the treatment of low-dose hormone and mycophenolate mofetil.7.Antibody titer evaluation: There was no statistically significant difference in ADL score,admission m RS,length of stay in ICU,clinical manifestations,etc.between the high-titer and low-titer serum antibody groups(P﹥0.05).Evaluation of treatment effect: The difference in m RS score of the hormone combined plasma exchange group was significantly higher than that of the hormone alone group and the hormone combined IVIG group(P <0.05).Prognosis evaluation: Patients with age ≥60 years old and combined tumors are significantly more in the poor prognosis group than in the good prognosis group,which is statistically significant(P﹤0.05).There were no significant differences in gender,clinical manifestations and auxiliary examination results between the two groups(P﹥0.05).Conclusion1.There is no significant difference in the incidence of anti-CASPR2 antibody encephalitis between men and women,and it is more common in middle-aged and elderly people.Cognitive decline and epilepsy are more common in central system symptoms,and muscle twitching and nerve pain are more common in peripheral nerve symptoms.The disease can be combined with other immune diseases and tumors.2.T2 WI and FLAIR hyperintensity are more common in bilateral hippocampus on cranial MRI.Epileptic waves can be seen on EEG,and twitch potentials can be seen on EMG.3.The level of serum anti-CASPR2 antibody titer is not proportional to the severity of the disease.4.Hormone shock combined with plasma exchange is effective in the acute phase.Rituximab is recommended in the maintenance phase.The prognosis is generally better after treatment,but age ≥ 60 years old,combined with tumors may indicate a poor prognosis. |
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