Lateral sellar angiolipomas: a therapeutic challenge. Our experience on two cases and review of the literature.

Angiolipomas are rare benign mesenchymal tumours, most commonly found in the subcutaneous tissue of the extremities. These neoplasms are rarely located in the central nervous system (CNS), with spinal localization being the most common in this group. Intracranial location is extremely infrequent, and only ten cases have been described in the sellar region. We report on two more patients with skull-base (intracranial) angiolipomas, the former presented with a long history of headache and the latter complained diplopia. Both patients were postoperatively verified as angiolipoma. Neuroimaging studies and peroperative features are presented and the role of microsurgery is discussed. On the basis of these tumours' characteristics, the management strategy of choice usually does not consist on surgical total removal, often a partial excision could be satisfying to improve the patient symptomatology. Nowadays, Gamma Knife surgery is standing out as an effective, additional and/or alternative, treatment modality.

Spontaneous intraparenchymal tension pneumocephalus triggered by compulsive forceful nose blowing.

The case is described of a 50-year-old man, treated for 10 years in an outpatient psychiatric clinic for an obsessive compulsive disorder, who presented with acute loss of consciousness after forceful nose blowing. A CT scan revealed an intraparenchymal air collection with tension signs in the left frontal lobe and a bone defect in the roof of the ethmoid sinus. After emergency left frontal craniotomy and dura opening, the gaseous collection was evacuated by a ventricular catheter inserted into the brain and the bone defect was repaired with pericranium flap and muscle. The postoperative course was uneventful with neurocognitive improvement and regained motility. Spontaneous tension pneumocephalus is a rare life-threatening condition which is often caused by a bone defect near the tegmen tympani. This case illustrates both an unusual cause and a unique surgical treatment for spontaneous tension intraparenchymal pneumocephalus. It can be a dangerous entity with potential for early mortality and long-term morbidity if not promptly decompressed. The pathogenesis, diagnosis and surgical strategies for spontaneous tension pneumocephalus are briefly discussed.

Lung cancer single intramedullary metastasis vs delayed radionecrosis. A case report.

Intramedullary metastases are rare, accounting for 0.9-5% of spinal metastases. Radiation myelopathy is considered one of the most distressing complications of radiotherapy. In both cases symptoms are aspecific, and there are no characteristic neuroradiologic findings. We describe a case of single intramedullary metastasis from lung microcytoma in a 55-year-old man with a history of malignancy, treated by radiotherapy five years previously. The patient returned to our observation complaining of pain and paraesthesia in the left C7 area. Spinal MRI and rachicentesis findings were aspecific. Ten days later a new MRI showed that the lesion size had increased, and neoplastic cells were found in CSF. Intramedullary metastases are extremely rare, accounting for 0.1-0.4% of all CNS tumors. The risk of developing delayed radionecrosis varies with the total dose administered. In both cases diagnosis is histological, while contrast-enhanced MRI is highly sensitive and specific in identifying and characterizing the lesion. In case of metastatic lesions the prognosis is unfavorable. Differential diagnosis is important because it has a strong effect on patient management.

Neurofibromatosis type 1 and optic pathway glioma. A long-term follow-up.

AIM: Optic pathway gliomas (OPG) are the predominant intracranial tumours associated with neurofibromatosis type 1 (NF1). The aim of this study was to evaluate the prevalence and the outcome of OPG in 200 NF1 patients (122 males and 78 females, aged 1-25 years) followed up to 16 years (mean of 6 years). METHODS: All children were evaluated by a detailed physical, neurological and ophthalmological examination. Fifteen out of 200 (7.5%) of these patients (7 males, 8 females) were identified with evidence of optic pathway tumours. RESULTS: Nine children had symptoms such as endocranial hypertension, seizures, headache; 4 patients only showed anomalies at ophthalmological examination; 2 patients had no symptoms or signs. All children had evidence of optic pathway tumour on magnetic resonance imaging. Three had a prechiasmal tumour, 2 had a chiasmal tumour, 1 had prechiasmal/chiasmal tumour, 2 had a prechiasmal/chiasmal and postchiasmal tumour, 2 had a chiasmal and postchiasmal tumour, 4 had a massive involvement of the optic system, 1 child exhibited a bilateral involvement of the optic nerves with additional impairment of the chiasm. Four patients had partial and/or subtotal spontaneous regression. CONCLUSIONS: Because optic pathway tumours arise in children younger than 6 years of age, all NF1 children should undergo yearly ophtalmologic examination and growth assessment to monitor signs of precocious puberty.

A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management.

We report a case of ecchordosis physaliphora, an uncommon benign lesion originating from embryonic notochordal remnants, intradurally located in the prepontine cistern, that unusually presented associated with symptoms. MRI detected and precisely located the small mass. At surgery, a cystic gelatinous nodule was found ventral to the pons, contiguous with the dorsal wall of the clivus via a small pedicle. Histological examination diagnosed the lesion as an ecchordosis physaliphora. Here we focus on the analysis of the neuroradiological aspects that play a crucial role from both a diagnostic and a therapeutic standpoint.

Giant intracranial chordoma: neuroradiological and radiotherapeutic aspects.

We describe a rare case of giant intracranial chordoma, emphasizing the patient's long survival and his excellent response to radiotherapy that led to a progressive regression of neurological symptomatology up to disappearance, in the absence of cerebral white matter damages.

Post traumatic retropharyngeal pseudomeningocele.

The authors report a case of a 33-year-old man who presented, during recovery from coma due to severe head injury, dysphagia and respiratory failure. Magnetic resonance, retrograde radionuclide myelography and computerized tomographic myelography identified a pseudomeningocele in the retropharyngeal space due to a tear of the left C2 radicular sleeve. After failed medical management, the patient underwent lumbo peritoneal shunt. Magnetic resonance controls showed progressive collapse of the collection. After 3 months the patient was able to breathe spontaneously and to swallow. The authors describe pathogenesis, diagnostic strategy and principles of treatment of traumatic retropharyngeal pseudomeningoceles.

Unusual manifestation of vertebral osteoid osteoma: case report.

We report the case of a 64 year-old man with a clinical history suggesting a low thoracic-cord involvement, in which an unexpected vertebral osteoid osteoma was discovered. The patient underwent MRI of the thoraco-lumbar spine, which included sagittal and axial T1-weighted images, and sagittal double-echo T2-weighted images. Subsequently, CT scan was carried out with 2-mm-thick axial sections, aimed at T10 vertebra. Magnetic resonance imaging disclosed an extra-axial mass at T10 level. Computed tomography scan suggested an osteoid osteoma of the tenth thoracic vertebra, involving the lamina with marked sclerosis and prevalently endocanalar extension. Histology following surgical resection confirmed the diagnosis. In the reported case CT scan provided the correct pre-operative diagnosis of osteoid osteoma despite its unusual clinical--anamnestic presentation. Magnetic resonance imaging was useful in establishing the relationship of the neoplasm with the spinal cord.

[The recrudescence of spondylodiscitis due to migratory flows. The role of magnetic resonance as a method of first instance in diagnosis and follow-up]. / Recrudescenza della spondilodiscite da incremento dei flussi migratori. Ruolo della Risonanza Magnetica come metodica di prima istanza nella diagnosi e nei controlli.

PURPOSE: We report 15 cases of spondylodiscitis due to infective processes of different etiology and stress the role of MRI in the diagnosis and follow-up of this condition, whose high epidemiologic recrudescence is probably related to an increased westward migratory flow. PATIENT AND METHODS: February through December, 1996, fifteen patients with acute spinal pain were submitted to MRI (1.0 T superconductive magnet, Magnetom SP42E, Siemens, Erlangen, Germany). In all cases both the painful spinal tract and the rest of the spine were studied to detect any infiltrative processes elsewhere in the spine. All patients underwent MR follow-up, according to the evolution of subjective symptoms, until complete recovery of the infections. RESULTS: MRI always permitted the correct diagnosis to be made and the correct evaluation of infection evolution, allowing adequate (medical and/or surgical) treatment to be carried out in the early stage of infections. DISCUSSION: MRI can be considered the best diagnostic tool study osteomedullary inflammations and, particularly, to diagnose and follow-up spondylodiscitis. This technique permits clear differentiation between the different evolution stages of the infections, as well as the evaluation of bone, disks, epidural spaces and spinal cord involvement. Moreover the MR findings permit adequate treatment choices, which results in much fewer complications and therefore better prognosis. CONCLUSIONS: The role of MRI in the diagnosis and follow-up of spondylodiscitis has been fully discussed in the literature. Our report is aimed at stressing the important role of MRI as the technique of choice for the diagnosis of suspected spondylodiscitis, especially considering the high recrudescence of this condition in Western populations which is probably related to increased westward migrations.

Association of primary antiphospholipid syndrome with inherited activated protein C resistance.

A 65-year-old man had had arterial thromboses of the lower limbs and cerebral region for several years; tests revealed anticardiolipin, antiphosphatidylserine, anti-beta2-glycoprotein I antibodies, and lupus anticoagulant. As well, both phenotypic and genotypic resistance to activated protein C was found. Antiphospholipid antibodies have been reported to interfere in different ways with the functions of protein C; in our patient the simultaneous existence of inherited resistance to activated protein C could account for the thrombophilic status underlying the diffuse and serious arterial thromboses.

MRI "fogging" in cerebellar ischaemia: case report.

Subacute cerebral infarcts may appear normal on T2-weighted MRI as an area isointense with surrounding normal tissue. This MRI "fogging effect" has been described in only a few cases. We present a further case of fogging observed during the evolution of a cerebellar infarct.

Transient "cerebellar" mutism in lesions of the mesencephalic-cerebellar region.

Four patients aged from 20 to 48 years with transient mutism are presented: 3 patients underwent surgery for midline tumours of the mesencephalic-cerebellar region (medulloblastoma in two cases and pinealoblastoma in one), at times attached to one or both lateral recesses of the IV ventricle. One patient was hospitalized and treated for brain-stem ischemia. All patients developed mutism 48 to 72 hours after surgery; in the patient with brain-stem softening mutism appeared 72 hours after admission. All the patients had unimpaired consciousness and no deficits of lower cranial nerves. Speech, always normal in the first hours after surgery, was regained after a period of 6-16 weeks. Various hypotheses for this speech disorder are analyzed.

Cerebral cavernous angioma. Diagnostic considerations.

Eighteen cases of cerebral cavernous angiomas are reported, and their histopathological, clinical and radiological features are reviewed. MR complements TC in characterizing cavernomas and in distinguishing them from similar-appearing lesions. However, when such lesions are seen with atypical features, discrimination from hemorrhagic tumors remains problematic. More malignant lesions present, on MRI, a much larger area of abnormality and make themselves obvious in a shorter period. Gadolinium administration may allow a more specific interpretation of small lesions.

Herniated disk in adolescents.

Twenty-one patients (9 female and 12 male) aged from 13 to 22 years were observed for lumbago and lumbo-sciatica caused by a discopathy which was subsequently confirmed by radiology and surgery. None of these patients had a history of trauma. It must be underlined that the painful symptoms exhibited and the young age of the patients suggest such pathologies as infections, tumours or malformations, but the possibility of discopathy must always be considered. It is also interesting to note the differences observed in symptomatology between adults and adolescents and to consider the various pathogenetic factors which might cause discopathy in adolescents while it was until now believed to be reserved to adults.

Surgical indications for intracranial arachnoid cysts.

Diagnostic work-up and management of intracranial arachnoid cysts are still controversial. The authors have standardized a therapeutic protocol based on the information derived from CSF contrast flow studies. The report concerns 16 cases of intracranial arachnoid cysts treated according to their protocol.

[The 75-seleno-homocholic acid-taurine test (SeHCAT). A useful method for detecting the idiopathic malabsorption of bile salts in chronic functional diarrhea]. / Il test al 75-selenio-omotauroglicolato (SeHCAT). Un utile metodo per rilevare il malassorbimento idiopatico di sali biliari nella diarrea cronica funzionale.

The 75-SeHCAT test has been used for identifying, within a group of patients with chronic functional diarrhea, a subgroup of cases with ileal bile acid malabsorption. Thirty-four subjects were studied: 10 healthy subjects and 24 patients with chronic functional diarrhea. Findings are expressed as percentage of basal abdominal radioactivity after 7 days. Normal values in our control subjects were: over 25% on day 4 and over 10% on day 7. Pathological results were found in 9 of 24 patients (37.5%) on day 4 and of 24 patients (41.7%) on day 7. A significant correlation was found between the percentages of retention on days 4 and 7 (p less than 0.001). In SeHCAT-positive patients cholestyramine administration led to clinical improvement. According to our experience, the SeHCAT test is a useful tool for the evaluation of bile acid malabsorption in the differential diagnosis of chronic functional diarrhea.

Thallium-201 per rectum for the diagnosis of cirrhosis in patients with asymptomatic chronic hepatitis.

In normal subjects, thallium-201, administered per rectum, is taken up mainly by the liver (heart/liver ratio in normal subjects: 0.04 to 0.12). It has been claimed that an increased heart/liver ratio is suggestive of portal-caval shunting and portal hypertension. To evaluate the possibility of using thallium-201 as a test to diagnose cirrhosis, we administered this substance per rectum to 33 patients with biochemical evidence, but no clinical symptoms, of liver disease. Laparoscopy and liver biopsy revealed chronic active hepatitis without cirrhosis in 18 patients, and chronic active hepatitis with cirrhosis in the others. The results of conventional liver function tests were similar in both groups. A significant difference, however, was found between the means of fasting serum bile acid concentrations (9.8 +/- 3.2 and 18.3 +/- 4.2 microM per liter) in chronic active hepatitis without cirrhosis and cirrhotic patients, and between the means of the heart/liver ratios 20 min after thallium-201 administration (heart/liver: 0.09 +/- 0.03 and 0.54 +/- 0.13, respectively). Unlike the serum bile acid concentration which gave some overlapping values, the thallium-201 test clearly distinguished the chronic active hepatitis without cirrhosis group from the cirrhotics. In the cirrhotic group, there was a significant correlation between the heart/liver ratio and signs of portal hypertension such as esophageal varices, increased diameter of the vena porta and hypersplenism. The thallium-201 test is therefore useful in discriminating between chronic active hepatitis with and without cirrhosis in clinically asymptomatic subjects with biochemical evidence of moderate liver function impairment. A heart/liver uptake ratio much higher than normal (above 0.30) strongly suggests the development of hepatic cirrhosis.