Cranial Paget’s Disease: A Case Report

Abstract

Scientific BACKGROUND: Paget’s disease is mostly asymptomatic. Neurological syndromes are uncommon but include headache, dementia, brain stem and cerebellar dysfunction, cranial neuropathies, myelopathy, cauda equina syndrome and radiculopathies. While she was in a symptomatic treatment by the diagnosis of cluster headache, our case was hospitalized to our clinic and reported after diagnosed cranial Paget’s disease according to the results of clinical, radiological and laboratory findings.

OBJECTIVES: In our case of cluster headache, we aim to discuss the headaches that have different characters and the other properties due to Paget’s disease.

RESULTS: While 61 year old female case was in the medical treatment of cluster headache, she was examined by the complaints of headache that was constant and localized on the right orbito-frontal region, tinnitus and hearing loss. On neurological examination, in addition to headache, she had mixed type hearing loss in the right ear, but sensorineural hearing loss in the left ear. Alcaline phosphatase levels were high in the serum and hydroxyprolin levels were high in the urine. In the cranial radiography, there was diffused osteoporosis, circumscripta, osteolytic areas, sclerosis in petrous bone, increasing diploe spaces in the skull base and diffuse cotton wool rough granular osteolytic spaces in the right lower part of frontal bone and basilar invagination. There was an appearance of bone-within-bone without any symptom in L2-L3 vertebrae. In her bone scintigraphy, other bones were normal but osteoblastic activity involvements in the head bones. After all the examinations of the headache, we thought that it was originated from two different causes like cluster headache and cranial Paget’s disease.

CONCLUSION: In Paget’s disease, cranial headaches are intermittent in early stages and may be confused by the primary headaches. In late stages, it is violent and continuous. Cranial headaches are due to different causes as cluster headache associated with cranial Paget’s disease like our case. We wanted to emphasize the other etiologic causes to be considered in cranial headaches.