[Hyperostosis frontalis interna and epilepsy]. / Hyperostosis frontalis interna et épilepsie.

We have analysed the clinical history of a 48-year old female patient who has been admitted to hospital after an epileptic fit. The tomodensitometry examination has shown a hyperostosis frontalis interna. This condition is frequent and is characterized by a progressive thickening of the frontal bone internal table. The aetiology is unknown; the most probable hypothesis is a deterioration of the androgen-estrogen ratio. This woman suffers from epileptic fits for 40 years now but also from hereditary polycystic kidney disease and CREST syndrome. In this analysis, we have applied the principle of parcimony and we have examined the role played by the sexual hormones in these pathologies.

Frontal Sinus Osteoma Accompanied by Intracranial Mucocele and Local Hyperostosis Frontalis Interna.

Frontal sinus osteoma accompanied by intracranial mucocele and local hyperostosis frontalis interna has never been reported. A 47-year-old woman presented with a 3-month history of intermittent headache. Physical examination revealed no neurologic abnormality. Contrasted magnetic resonance imaging showed a frontal heterogeneously enhanced lesion with adjacent nonenhanced cyst. Computed tomography showed a bone density mass, which was accompanied by local hyperostosis frontalis interna, which filled the left frontal sinus and extended intracranially. The patient underwent a left frontobasal craniotomy. Both the osseous mass and cyst capsule were removed totally via a frontal craniotomy, followed by skull base reconstruction. The postoperative course was uneventful. The final pathologic diagnosis was osteoma and mucocele.

Manejo de vía aérea en paciente con leontiasis ossea. Reporte de caso / Airway management in a patient with leontiasis ossea. Case report

Leontiasis ossea is an uncommon complication of advanced chronic kidney disease that alters the facial bone and the airway, making its perioperative management more complex. We present a clinical case of a female with Leontiasis ossea presenting a difficult airway which requires parathyroidectomy. Assessment, planning and management of the airway by awake intubation is described.

La leontiasis ossea es una complicación infrecuente de la enfermedad renal crónica avanzada que altera el macizo facial óseo y la vía aérea, complejizando su manejo perioperatorio. Presentamos caso clínico de mujer portadora de leontiasis ossea con vía aérea difícil requiriendo paratiroidectomía. Se describe valoración, planificación y manejo de vía aérea mediante intubación vigil.

Uremic Leontiasis Ossea in a Patient With Chronic Renal Insufficiency Demonstrated on Bone Scintigraphy.

A 37-year-old woman with chronic renal insufficiency underwent bone scintigraphy to evaluate renal osteodystrophy (ROD). Markedly increased uptakes were shown in the maxilla and the mandible, which suggested extensive maxillary and mandibular hypertrophy. CT image revealed that diffuse bony thickening and ground-glass appearance in the skull, maxilla, and mandible with poor distinction of the corticomedullary junction. Whole-body bone scintigraphy images also demonstrated various skeletal characteristics of ROD. This case emphasizes the utility of bone scintigraphy for the surveillance of the whole body in ROD.

Morgagni Stewart Morel syndrome--additional features.

A case of Morgagni Stewart Morel syndrome with progressive depression in frontal bone, headache, transient monoparesis, obesity; imbalance, neuropsychiatric symptoms and recurrent disc prolapse with absent right radial pulse is discussed. This syndrome was first mentioned 235 years back, but till now exact pathology is not known. Balance assessment using dynamic posturography was done, which revealed abnormal vestibular function. To our knowledge this is the first case examined for Dynamic Posturography.

[Gardner syndrome: clinical and epidemiologic up to date]. / Attualità cliniche nella sindrome di Gardner: contributo casistico.

In 1950 EJ Gardner first described a new syndrome characterized by (1) familial colonic polyposis, (2) multiple osteomas, (3) soft tissues cysts and (4) fibrous lesions. Thereafter, in 1975 Watne and coll. have demonstrated the occurence, in patients affected by Gardner syndrome, of the early onset of osteomas and dental inclusions in maxillary bones. Gardner syndrome is actually considered a severe life treathening condition due to the poor quality of life and the evolutive pattern of colonic polyps to colon cancer in 100% of cases. The aim of this paper is the review of the pathophysiologic and clinical aspects of Gardner syndrome, with report of institutional clinical data about epidemiology and clinical presentation of such condition, attempting to elaborate a clinical protocol for early detection of that.

Prevalence of hyperostosis frontalis interna in relation to body weight.

In a prospective study, hyperostosis frontalis interna (HFI) was found to be present in 49% of the subjects. The group included 259 nuns between the ages of 60 and 80. A prevalence of 84% of HFI was found in the obese subjects (greater than 40% over ideal body weight) whereas in the thin subjects HFI was found to be present in only 16%. The possible relationship between adipose tissue mass and HFI is discussed.

Cranial epidural fibrous tumor associated with hyperostosis: a case report.

This report describes an unusual frontal epidural fibrous tumor in an adult woman. There had been no prior surgical procedure, radiation therapy, or significant head trauma. Microscopically, the lesion consisted of hypocellular, sparsely vascular, dense fibrous tissue. The fibrous mass was accompanied by hyperostosis of the frontal bone. The clinical and pathological characteristics of this lesion are illustrated. The differential diagnosis is discussed with reference to the literature regarding intracranial fibrous lesions. We are unaware of previous report of similar lesions in this location.

A special form of hyperostosis frontalis interna.

The article describes a very severe case of hyperostosis of the frontal bone and discusses its possible differential diagnosis. What makes this case special is the fact that the osseous changes include all 3 layers of the bone. Macroscopically the lesion resembles an osteoma as described by Burkhardt (1970) and v. Eiselsberg (1906). The histological examination lead to the final diagnosis of hyperostosis frontalis interna. One must note that there were certain effects caused by a metastasis of a lobular carcinoma of the breast which influenced the osseous changes from the outer surface. The rough, spicula-like structure in the centre of the frontal squama should be put down to tumour erosion. The peripheral areas of the hyperostosis, however, still exhibit the original smoother texture of the disease. The final diagnosis in this unusual and possibly unique case was an intense hyperostosis frontalis interna with secondary changes due to the metastases of a lobular carcinoma of the breast.

[Is enostosis frontalis a disease process? (author's transl)]. / Welchen Krankheitswert haben frontale Enostosen?

2851 radiodiagnostic examinations of the skull made in a psychiatric hospital between 1958 and 1977 were reviewed for enostosis frontalis. Out of 1398 female patients there were 125 cases of enostosis frontalis, whereas out of the 1453 male there were only 5. Hence, in females, this is a frequent condition (9%) and shows no correlation with manifest hormonal disease or any particular neuro-psychiatric disturbance. Enostosis frontalis in females can be considered to be an inconstant secondary sex characteristic. In male patients this condition is extremely rare (0.32%). The case histories of the male patients are reported. Endocrine disturbances, cerebral dysplasia and psychiatric disease in connection with mental retardation seem to be associated with enostosis frontalis in men.

[Hyperostosis cranialis interna; a new syndrome with autosomal dominant inheritance]. / Hyperostosis cranialis interna; een nieuw syndroom met autosomaal dominante overerving.

A family is described which currently comprises nine individuals, spanning three generations, who are affected with a bone disorder which is confined to the skull and is accompanied by impaired function of the cranial nerves. Radiological examination showed intracranial hyperostosis and osteosclerosis of the calvaria and the base of the skull, without involvement of the mandible or other skeletal bones. Invariably, the main presenting symptom was recurrent facial nerve paralysis from late childhood onwards, but concurrent and variable involvement of the olfactory, optic, vestibular and acoustic nerves was seen; this could be attributed to nerve compression by the bony encroachment into the cranial foramina. Morphological investigations revealed increased formation of bone tissue with a normal structure. The pedigree suggests an autosomal dominant mode of heredity. A review of the literature did not disclose any previous reports on this disorder.

[A case of adult-onset spike-wave stupor associated with hypoparathyroidism and hyperostosis frontalis interna (HFI)].

A 34-year-old women without history of seizures was admitted to our hospital because of the diminished responsiveness and the repeated blinking for two days. Her past history showed neither surgery nor inflammation of the thyroid gland. General physical examination was normal. On neurological examination, the patient was blinking frequently and staring without any responsiveness. However, she occasionally became able to respond correctly to verbal orders, such as "open your eyes", "open your mouth", "stand up" and so on, which occurred abruptly just like the switch-on. She was otherwise normal neurologically. The laboratory data showed hypocalcemia, hyperphosphatemia, decreased level of parathyroid hormone and normal renal function, indicating the presence of idiopathic hypoparathyroidism. EEG showed the continuous generalized 2-4 Hz spike and wave complexes with the maximum intensity on frontal lobes. Skull roentgenograms and MRI CT of the head disclosed hyperostosis frontalis interna (HFI) and the compression of superior medial frontal lobes by the HIF. SPECT also showed the diminished circulation and hypometabolism in the superior frontal lobes. Based on clinical and EEG findings, the diagnosis of spike-wave stupor was made. She was successfully treated with valproic acid and ethosuximide. Spike and wave complexes on EEG completely disappeared after administration of alfacalcidole. It is assumed that both hypoparathyroidism and HFI were deeply involved in the development of spike-wave stupor in this adult case.

[A case of Morgagni-Morel-Stewart syndrome with violent headaches predominant in the clinical course treated surgically]. / Przypadek zespolu Morgagniego-Morela-Stewarta z bólami glowy dominujacymi w obrazie chorobowym leczony operacyjnie.

The authors present a case of Morgagni-Morel-Stewart syndrome with violent headaches predominant in the clinical course. Surgical removal of hypertrophic frontal bone followed by dura and bone reconstruction was performed. The pains ceased immediately after the operation. The authors discuss surgical treatment especially in cases with high intensity headaches.

[The Stewart-Morel syndrome in the differential diagnosis of patients with frontal headache]. / Síndrome de Stewart-Morel en el diagnóstico diferencial del paciente con dolor de localización frontal.

Hyperostosis frontalis interna is the name generally applied to skull thickening more or less restricted to the squamous portion of the frontal bone and involving, in particular, its subdural or inner surface. The association of this calvarial thickening with virilism and obesity is a clinic-anatomic complex often know as the Morgagni's syndrome and is also referred to as the Stewart-Morel syndrome or metabolic craniopathy, when accompanied by mental disorders. Hyperostosis frontalis interna is being discussed here mainly in connection with the Stewart-Morel's syndrome of which it is the pivotal feature. Frontal headache is a common complaint and is severe in some cases. In addition various neuropsychiatric disturbances may be manifested.

[Morgagni-Steward-Morel syndrome accompanied by venous stasis on the edge of the optic disc]. / Zespól Morgagniego-Stewarda-Morela przebiegajacy z objawami zastoju zylnego w obrebie tarczy nerwu wzrokowego.

A case of a 41-year-old woman with Morgagni-Steward-Morel syndrome is presented. The first symptom of the disease was papilledema. The results of clinical examination and additional tests eliminated the presence of intracranial tumor and revealed the hypertrophy of the internal laminal of frontal bone. The patient also suffered from glaucoma, hormonal disorders and considerable obesity. Both the clinical picture and the results of laboratory and radiological tests permitted to diagnose Morgagni-Steaward-Morel syndrome.

Full penetrance of Morgagni-Stewart-Morel syndrome in a 75-year-old woman: case report and review of the literature.

CONTEXT: Morgagni-Stewart-Morel syndrome is defined as the presence of hyperostosis frontalis interna, variably associated with metabolic, endocrine, and neuropsychiatric disorders. The possible cause-effect relationship of these associations remains uncertain. CASE PRESENTATION: A 75-year-old woman presented with severe frontal headache and a history of psychotic disorders. On instrumental examination she was found to have extensive frontal hyperostosis and cortical atrophy. These findings, associated to the metabolic and neuropsychiatric pattern of the patient, are consistent with a high penetrance of Morgagni-Stewart-Morel syndrome. EVIDENCE ACQUISITION AND SYNTHESIS: In this clinical case seminar, we summarize the current understanding of the association between hyperostosis frontalis interna and Morgagni-Stewart-Morel, based on a MEDLINE search (case reports, original articles, and reviews published between 1928 and 2011) on this topic. Possible pathophysiological mechanisms underlying both the headache and the hyperostosis frontalis interna are discussed. CONCLUSION: A case of full penetrance of Morgagni-Stewart-Morel syndrome is reported, presenting many of the clinical features described in the literature. Metabolic and endocrine dysfunctions should be interpreted not only as isolated components of the syndrome, but also as the reason behind its pathogenesis. Endocrine or nutritional disorders may have led to an altered bone metabolism with frontal bone apposition. On the other hand, the severity of our patient's neurological and psychiatric symptoms correlates well with the severity of her hyperostosis frontalis interna and the cortical atrophy.