A Case of Normal Pressure Hydrocephalus in Adult-Onset Pompe Disease

摘要: A 65-year-old man, who was diagnosed with Pompe disease when he 50 years, developed memory loss and urinary urgency. His medical history showed severe left hip arthrosis osteonecrosis which caused intense pain functional limitations. The patient also affected by chronic obstructive pulmonary disease, benign prostatic hyperplasia, hyperamylasemia. Examination revealed a lack of strength in the deltoid muscles (bilateral F = 3), biceps brachii 4.5), gluteus maximus bilateral iliopsoas (F 4), tibialis anterior hyperlordosis, right winged scapula, anserine gait, only possible unilateral support, an inability to completely abduct arms. No extrapyramidal signs were observed except for postural instability, might be compatible weakness pelvic muscles. underwent psychometric tests Mini-Mental-State-Examination score equal 24 (adequate age schooling) short loss. Brain MRI increased dimension ventricular system reduced subarachnoid cavities periventricular hyperintensity, supporting normal pressure hydrocephalus and, moreover, atrophy Case Normal Pressure Hydrocephalus Adult-Onset Disease