Diagnosis And Treatment Of Two Special Types Of Hydrocephalus

Background and Objective:The clinical manifestations and classification of autoimmune diseases vary greatly which includes systemic lupus erythematosus(SLE),vasculitides,Sjogren’s syndrome,rheumatoid arthritis(RA),connective tissue disease and other diseases.Dysregulation of the immune system is the common mechanism and usually causes damage to single or multiple organs.The central and peripheral nervous systems can be involved at any time during the course of the disease,but few cases complicated with hydrocephalus is reported.Autoimmune disease accompanied by hydrocephalus is rare and has not been reported systematically.Many patients who suffer from autoimmune diseases complicated with hydrocephalus can be treated effectively to some extent with medications such as steroids,immunosuppressants and acetazolamide.This study retrospectively collected and analyzed cases of autoimmune disease complicated by hydrocephalus in Peking Union Medical College Hospital,in order to summarize the experience of the diagnosis and treatment of autoimmune disease-associated hydrocephalus and explore it’s possible pathogenesis.MethodsA retrospective study was conducted at Peking Union Medical College Hospital,Beijing,China.Files were retrieved from the hospital archives by screening records from Jan 1990 to Jan 2016.Medical records were screened for data regarding(1)the number of patients diagnosed with hydrocephalus associated with autoimmune diseases(2)the clinical manifestation of hydrocephalus associated with autoimmune disease(3)the outcomes of these patients treated with medication or ventriculoperitoneal shunt(VPS).Results:A total of 19 of 19,643 hospitalized autoimmune diseases patients were found to have hydrocephalus.Seven of the 19 patients had systemic lupus erythematosus(SLE),3 patients had Sjogren’s syndrome,2 patients had rheumatoid arthritis(RA),1 patient had connective tissue disease,1 patient had juvenile idiopathic arthritis(JIA),1 patient had Guillain-Barre syndrome(GBS),1 patient had systemic sclerosis,1 patient had Crohn’s disease,1 patient had relapsing polychondritis(RPC),and 1 patient had autoinflammatory disease(AID),Of the 19 patients,13 received medication treatment,and the most commonly used drugs were corticosteroids and mannitol.A total of 6 patients received both medication therapy and VPS treatment with a programmable valve.After average follow-up lengths of 11 months for patients who received VPS and 8.2 months for patients who received medical treatment,the clinical symptoms of patients treated by VPS or medication were improved(83%(5/6)vs 15.4%(2/13);respectively),more patients were in stable condition(17%(1/6)vs 30.8%(4/13),respectively),and mortality decreased(0%vs 53.8%(7/13),respectively).Conclusions:Autoimmune disease complicated with hydrocephalus is a special clinical disease,and it’s pathogenesis is complex.According to the literature reports and our experience,we speculate that the hydrocephalus may be caused by intracranial infection,aseptic inflammation of the meninges,narrowing of the midbrain aqueduct,etc.VPS combined with corticosteroids and immunosuppressive agents provides an effective treatment for patients with autoimmune disease-related hydrocephalus.Background and Objective:Normal pressure hydrocephalus(NPH),which was first reported by Hakim and Adams in 1965,attracted attention by the normal cerebrospinal fluid pressure and abnormal imaging findings such as enlargement of the ventricles,widening of the lateral fissure pool,at the same time,a syndrome of the clinical triad of gait disturbance,dementia and urinary dysfunction can occur.The epidemiological data of iNPH are currently lacking in China.The prevalence of iNPH in Japan is approximately 10.2 per 100 000;In Sweden,the prevalence of iNPH in elderly persons aged 70-79 is approximately 0.2%,and the prevalence of elderly people aged 80 and over is approximately 5.9%.NPH mostly occurs in the elderly and the disease progresses slowly.The clinical symptoms lacks specificity and is often misdiagnosed as brain atrophy,Alzheimer’s disease,and other neurodegenerative diseases.According to the reports,patients with longer duration have poor response to cerebrospinal fluid shunt surgery,and clinical symptoms such as ataxia,dementia,and urinary incontinence caused by the disease are difficult to be alleviated or reversed.Therefore,timely diagnosis and treatment are very important.This study collected and analyzed the clinical data of 33 patients with iNPH who received VPS from October 2010 to October 2016 in Peking Union Medical College Hospital,and explored the diagnosis and treatment strategies of iNPH by overviewing the latest literature.Subjects and methods:Patients diagnosed as idiopathic normal pressure hydrocephalus during October 2010 and October 2016 were included.A retrospective study was conducted to collect the data of patients undergoing ventriculoperitoneal shunt intervention,including the general condition at preoperative and postoperative,the iNPH scores,imaging data,etc.Then,we analyzed and evaluated the treatment effect of this surgical procedure on idiopathic normal pressure hydrocephalus patients.Results:33 patients were included in the group,of which,30 cases were presented as gait disturbance,27 cases cognitive impairment,23 cases urinary dysfunction,18 cases typical triads.All of the 33 cases received cerebrospinal fluid peritoneovenous shunt.iNPH scores of patients at 3 months after operation were significantly decreased compared with the pre-operation(P<0.01)and there were no differences among 3 month,6 month and 12 month post operation.The Evans index and the width of the three ventricles were also significant decreased compared with the pre-operation.There was 1 patient complicated with subdural hematoma and no patient complicated with infection,obstruction or death.Conclusion:1.The clinician needs to analyze comprehensively the patient’s medical history,clinical symptoms,imaging findings,and Tap Test results.Multidispinary cooperation among neurosurgery,neurology,imaging,and other department can ensure the correct diagnosis of iNPH;2.Ventriculoperitoneal shunt should be performed timely once diagnosed with idiopathic normal pressure hydrocephalus,which can significantly improve the prognosis of patients;3.Tap test is a good measure to predict the effect of surgery.