ABSTRACT
Objective To observe the clinical manifestations of POEMS syndrome, and to improve the early diagnosis and prognosis.Methods The clinical data of 61 POEMS syndrome patients were analyzed retrospectively. Results Among the 61 patients,there were 27 males and 34 females with an average age of 47.2 years.The average time from the primary admission to the confirmed diagnosis was 22.5 months.All the patients(100%)had peripheral neuropathy by various degrees,the majority showing the sensory dysfunction of lower limbs.Fifty-two cases (85.2%)were identified with organomegaly by imaging techniques,most presented with the enlargement of spleen and lymph nodes.Fourty-five cases(73.8%)were accompanied with endocrinopathy, reflected by diabetics along with the hypofunction of the pituitary, gonad, thyroid and the adrenal glands.Fifty cases(82.0%)were with positive serum M protein.Twenty-seven cases(44.3%)manifested evident skin changes including hair growth along the limbs and trunk, skin pigmentation and skin keratinization of the feet.In this group of patients, 59 patients mainly use dexamethasone treatment,some patients with horses flange or cyclophosphamide,lenalidomide treatment. The other 2 patients were treated by autologous stem cell transplantation therapy.The clinical outcome of patients with diagnosis time ≤10 months was significantly better than that of patients with diagnosis time 10 to 20 months and≥20 months(all P<0.05).Patients with diagnosis time ≥20 months had the worst clinical outcomes, with the highest death rate(all P<0.05).Conclusions The clinical manifestations of POEMS syndrome are complex, especially the peripheral nerve damage symptoms and merging other organs or systems.M protein detection is needed in order to make clear diagnosis.Early diagnosis and effective treatment for patients can get better prognosis.
ABSTRACT
Objective To investigate the clinical features of familial idiopathic basal ganglia calcification (FIBGC).Methods Clinical data of 4 FIBGC patients from 2 families were analyzed retrospectively.Results The average age of 4 patients was(62.7 ±13.4)years old.The first symptom of 2 female patients was depression and schizophrenia, while 2 male patients displayed dementia and Parkinson's syndrome.Patients treated with vitamin D capsules,follow-up period for 3 to 6 months.All patients showed symptomatic improvement.Conclusions Clinical symptoms of FIBGC are complicated,and progressive development.The main symptom of female is mental symptoms, and male patients accompanied with dementia and dyskinesia.There is still no effective therapeutic plan for FIBGC.