[A young woman with headaches and skull anomalies: a long road to the diagnosis 'Paget's disease'].
A 37-year-old woman complained of headaches, dizziness and squeaking noises in her right ear that had been going on for about 3 months. After experiencing tingling sensations in the left side of her body she consulted a neurologist, who ordered an MRI scan of her head, on which skeletal abnormalities consistent with multiple metastases of the skull were seen. Extensive clinical screening showed no evidence of a primary tumour. Lab examination showed her serum alkaline phosphatase activity to be twice as high as normal. Bone scintigraphy showed increased uptake in the skull. Plain X-rays of the skull showed large osteolytic areas and a thickened and sclerotic vault of the skull, characteristic of osteoporosis circumscripta due to Paget's disease of the skull. Treatment with oral risedronate, 30 mg per day for a period of 2 months resulted in a gradual decrease of symptoms and a rapid normalisation of bone turnover parameters. Five years after treatment the patient was still in remission and repeat X-rays ofthe skull taken after three years showed clear improvement of the osteolytic but not of the sclerotic lesions. Because Paget's disease has a relatively high prevalence in the elderly, it is important to know that it is not always recognised as such on MRI and that a simple X-ray can lead to the correct diagnosis.
Zillikens
M C
MC
Erasmus MC, Postbus 2040, 3000 CA Rotterdam. m.c.zillikens@erasmusmc.nl
Ginai
A Z
AZ
Pols
H A P
HA
D000328:Adult; D000469:Alkaline Phosphatase / Q000378:metabolism*; D050071:Bone Density Conservation Agents / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D012968:Etidronic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D005260:Female; D006261:Headache / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D010001:Osteitis Deformans / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D011859:Radiography; D011877:Radionuclide Imaging; D000068296:Risedronic Acid; D012886:Skull / Q000000981:diagnostic imaging / Q000473:pathology*; D016896:Treatment Outcome
D050071:Bone Density Conservation Agents; D000469:Alkaline Phosphatase; D000068296:Risedronic Acid; D012968:Etidronic Acid