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Paget's disease usually is found in patients past the age of 40. Early presenting symptoms include headache, deafness, tinnitus, and pain due to radicular compression. The diagnosis is confirmed by radiographic features and elevated levels of serum alkaline phosphatase and urinary hydroxyproline. Bony overgrowth results in pressure on nearby soft tissues such as the brain, spinal cord, and certain peripheral nerves. The abnormally soft quality of the calvarial bone permits distortion by the weight of the brain. Dorsal inclination of the plane of the foramen magnum and the projection of the odontoid process into the posterior fossa lead to stretching of the brain stem over the odontoid process and the ventral margin of the foramen. Obstructive hydrocephalus may result. Sarcoma of the crainial vault may develop in cases of Paget's disease. Once cervicomedullary or spinal compression has occurred, surgical decompression may be necessary. Three drugs--calcitonin, disodium etidronate, and mithramycin--have been used with some benefit in the treatment of Paget's disease.
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PMID:Paget's disease and the nervous system. 21 14

The present paper attempts to comment on the relation between diffuse concentric hyperostoses of the cranial vault and neurologic and psychiatric symptoms in a male aged 42 and a woman aged 23 when death occurred. In the past, similar discussions mainly concerned the hyperostosis frontalis interna (Hfi), although the demonstration of a true relation is difficult in the Hfi because of its late occurrence at an advanced age (60-70 years), in which mental disturbances due to other pathological circumstances are frequent as well. The diffuse concentric hyperostosis in young individuals has comparatively seldom been regarded, although in young individuals the age factor in the consideration of mental disturbances can be excluded. The 42 year old male had an 18 years history of mental disturbances such as a mental change and severe episodic psychiatric symptoms, which repeatedly required clinical treatment. Since 18 years he had also suffered from headaches which caused an abusus of phenacetinum. A diffuse concentric hyperostosis of the neurocranium with a long developmental course, as could be derived from microscopical examination, was considered likely to be the reason for the longstanding headaches and the mental change. The 23 year old female was a prostitute suffering from serious headaches and neurodystonia until she died suddenly and unexpectedly, without sufficient autopsy findings. The cranial vault displayed a severe concentric hyperostosis besides other striking changes.
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PMID:[Significance of cranial hyperostoses for the neurologic-psychiatric practice]. 100 3

The authors analyse 22 cases of suprasellar meningiomas, drawing attention to factors influencing on the surgical outcome. In all but one case, symptomatology began with progressive visual failure in one eye. Bilateral anosmia was noted in 4 patients with large tumour. Mental disorders were conspicuous in 5 cases and 3 patients suffered from epilepsy. Headache was severe in 5 cases. Endrocrinological disorders were observed in 3 patients. The sella turcica was of normal shape in all cases. Marked hyperostosis of the planum or tuberculum existed in 7 cases. The tumour was heavy-calcified in 2 cases. CT scanning showed everytime a marked enhancement of the tumour and in 4 cases, a large hypodense area surrounded the tumour. The patients were operated on through a bifrontal approach or a unilateral frontal flap. A partial anterior frontal lobectomy was regularly performed on one side. While the tumour is piecemeal exacavated, the dural attachment at the base is reached as quickly as possible. Complications consisted in rhinorrhea of CSF in 2 cases, once in a transitory diabetes insipidus and in a secondary hydrocephalus. Post-operative mortality remains high. Among the eleven cases of large tumours, a direct postoperative death occurred, due to a severe arterial bleeding. Two other patients died 4 and 6 weeks respectively after operation. An other patient died 8 years after operation, from meningitis. Among the 5 cases of medium-sized tumours, one post-operative death occurred in a young female, 30 of age, following urinary infection by Klebsiella, complicated by toxicemia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Management and surgical outcome of suprasellar meningiomas. 318 2

In this paper we report a 7.5-year-old physically normal boy with van Buchem disease (endosteal hyperostosis). Vague complaints of headache were the indication for X-ray examination. At the age of 2 months a left-side peripheral facial nerve palsy suddenly occurred in this boy. Skull X-rays gave normal results at that age, suggesting that encroachment of the cranial nerves in van Buchem disease may occur as early as in the postnatal period, even before sclerosis of the skull has become radiologically visible.
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PMID:Facial paralysis at the age of 2 months as a first clinical sign of van Buchem disease (endosteal hyperostosis). 327 28

We experienced two cases of "Osteosclerosis" who were 12 and 15 year old sisters. Previous reported cases of this disease are 50 cases and among them only one patient was reported in Japan. Osteosclerosis seems to be inherited as an autosomal recessive trait. Parental consanguinity is also observed. The peculiar facies are evident in infancy, characterized by broad, flat nasal bridge, ocular hypertelorism and prognathic, broadened mandible. Commonly, they have cutaneous syndactylies in bilateral hands and feet, especially between the second and third finger and toe. Roentgenographically, hyperostosis with osteosclerosis can be observed in systemic bones, particularly the calvarium is greatly thickened. Since such a bony change occurs most severely at the base of the skull, important clinical symptoms of this disease are cranial nerve palsies resulting from obliterations of unilateral or bilateral several cranial nerve foramina. In many cases deafness due to progressive encroachment upon the middle ear cavities and auditory nerve canals appears early in infancy. Transient palsy of the facial nerve occurs somewhat later, and bilateral facial paralyses are usually permanent in adulthood. In some cases optic atrophy and visual field defect due to compression of the optic nerves are late complications. Other ocular symptoms are strabismus, nystagmus and exophthalmos. Anosmia and trigeminal nerve palsy are less common. Lower cranial nerve symptoms can not be noted but the reason is unclear. Chronic headache, convulsion and mental retardation are occasionally present. They are considered as a result from increased intracranial pressure due to progressive diminution of the cranial capacity. By same mechanism, several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum in early adulthood. Then, some reporter puts emphasis on prophylactic opening of the foramen magnum in all adult cases.
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PMID:[Sibling case of osteosclerosis with cranial nerve symptoms]. 629 11

Sclerosteosis is one of the rare, potentially lethal, autosomal recessive, progressive, craniotubular sclerosing bone dysplasias. Syndactyly of the second and third or other fingers is evident at birth. Hyperostosis and sclerosis are most prominent in the skull and tubular bones, and are frequently associated with excessive height and weight. The typical facial deformity is apparent by the age of 5 years. The changes involving the temporal bone include a marked increase in overall dimensions, extreme sclerosis, and narrowing and constriction of the external ear canal, middle ear cleft, internal acoustic meatus, and falloppian canal. Impairment of hearing, as a rule bilateral, is a frequent presenting symptom which may manifest in early childhood. Initially it is an expression of interference with sound conduction; later it may become associated with a loss of sound perception. Impairment of facial nerve function is another salient feature which occasionally is present at birth. As a rule, it manifests initially as a unilateral, recurrent paresis, eventually progressing to a bilateral permanent partial loss of facial nerve function. Since impairments of hearing and facial nerve function are two of the salient features, present at birth or in early childhood, the responsibility for recognizing the disease often falls upon the otolaryngologist. The clinical and radiologic features permit not only early recognition of the disorder but also differentiation from similar bony dysplasias. Hyperosteosis and sclerosis of the skull lead to thickening and distortion of the calvaria, cranial base, and foramen magnum resulting in reduction of the intracranial volume, interference with the cerebral blood flow, resorption of cerebrospinal fluid, and gradual increase of intracranial pressure. Severe headaches resulting from this mechanism often develop in early adulthood, and several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum. Decompression of the transverse sigmoid sinus and jugular bulb may be lifesaving, combined with a posterior, and if necessary, an anterior, craniectomy for decompression. Early decompression of the internal acoustic meatus and falloppian canal may help in the preservation of cochlear and facial nerve function.
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PMID:Sclerosteosis involving the temporal bone: clinical and radiologic aspects. 667 95

We report a patient with Morgagni syndrome. The main aim of this paper is to discuss hyperostosis frontalis interna (HFI) and coexisting clinical feature and to describe the pathomorphology in detail on the basis of MRI images of the skull. The patient, a woman, was 82 years old when she first came to our hospital. She had a 20-year history of hypertension and chronic headache, and had been excessively obese till three years before. On admission she presented with a broad spectrum of clinical symptoms and signs including insomnia, disorientation to place, loss of memory, dementia, night delirium, reduced deep tendon reflexes in the lower extremities, urinary incontinence and upward gaze palsy. Because of a fair recovery within several days, it was suspected that so-called "treatable dementia" played a considerable role in the above-mentioned clinical state. Laboratory testing data, including hormone levels, were all within normal limits. EEG examinations showed slowed, diffuse, and poorly developed alpha-waves with no paroxysms. Cranial CT in horizontal sections disclosed a deformed frontal bone with convexlens-shaped thickening bilaterally and diffuse high density on both sides. MRI images revealed more detailed structures: the outer plate, diploe and inner plate of the skull, and abnormal ossifications. Based on these findings we diagnosed her illness as Morgagni syndrome. Recent reports, though few in number, have tended to focus on the EEG findings, hormones and psychiatric states in this syndrome, and descriptions of the HFI itself seem to be rare. The true cause of this syndrome is not yet known, so this rare presentation of MRI images of HFI is thought to be important in explaining this peculiar phenomenon in the skull.
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PMID:[MRI findings of hyperostosis frontalis interna--a case of Morgagni syndrome]. 875 3

The authors present a case report of a 59-year-old female suffering from hyperlipidemia who developed chronic vitamin A intoxication syndrome after ingestion of 30,000 IU retinol/daily over a period of six years. The patient's main complaints included severe headaches, morning nausea, myalgias and disability around the hip, knee, and ankle joints. Radiologically, hyperostosis of the acetabular circumference and the spine was demonstrated. Because of rapidly increasing pain, total hip replacement was performed. Histology of cross sections from the femoral head revealed destructive osteoarthritis. Since no other causative reason was found, retinol may not only be responsible for hyperostotic bone and soft tissue formations but may perhaps also account for rapid progressing of degenerative joint disease. Despite the cessation of vitamin A intake the clinical symptoms persisted due to hyperlipidemia. The enlarged number of chylomicrons and the higher fraction of very low density lipoproteins may represent a second retinyl ester pool in case of overloaded fat storing Ito-cells in the liver. Therefore, rheumatological treatment reducing risk factors such as hyperlipidemia is mandatory.
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PMID:Hyperostotic and destructive osteoarthritis in a patient with vitamin A intoxication syndrome: a case report. 891 26

The authors report a rare case of ecchordosis physaliphora (EP) in the prepontine region. A 51-year-old woman was admitted with a small cystic mass in the prepontine region, complaining of headache and an episode of transient double vision. Plain X-ray and lateral tomography films showed a protrusive hyperostosis at the middle clival region. The CT scan showed no abnormal densities in the retroclival region, and CT cisternography showed an isolated small round mass in the prepontine cistern. A small cystic mass with no enhancement with Gd-DTPA was revealed on MRI, mildly compressing the basilar artery and the rostral surface of the pons. The totally excised mass was pedunculated and was contiguous with the dorsal wall of the clivus via a small dural defect. The histologic diagnosis was EP, consisting of scattered physaliphorous cell nests, which were not positive for MIB-1 staining. The pedicle consisted of mature cartilaginous cells. The authors briefly reviewed the few previously reported cases of symptomatic EP and intradural chordoma, and discussed the differences between them. The histological features, especially the proliferation potential, may be pathognomonic. The histogenesis and the clinical features of symptomatic EP are also provided.
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PMID:[A surgical case of ecchordosis physaliphora]. 893 71

We report three elderly patients with hyperostosis cranii (HC). Patient 1 had two episodes of unconsciousness; patient 2, headache; and patient 3, dementia. On the basis of the classification of Moore using skull films, patients 1 and 2 showed hyperostosis frontoparietalis and patient 3 had hyperostosis frontalis interna. Electroencephalography showed transient generalized spike and slow wave complexes over the frontal lobes in patient 1. Magnetic resonance (MR) images showed frontal lobes compressed by the thickness of the frontal bones in all patients and thickened parietal bones in patients 1 and 2. Because findings in our series and in the literature suggest that HC may show unexpected neuropsychiatric symptoms, HC should be checked in elderly patients whose presenting symptoms include epilepsy, dementia, psychiatric disease, headache and so on. MR images should reveal the relationship between clinical symptoms and the deformation of brains by the skull.
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PMID:[Three elderly cases of hyperostosis cranii with various clinical symptoms]. 1130 37


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