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Biomedical subjects

C Mottolese

Publications and source records attributed to C Mottolese.

At least 37 records · Page 2Linked to original sources

Middle meningeal artery aneurysm associated with meningioma.

The aneurysms of the middle meningeal artery are extremely rare. 30 pseudoaneurysms of the middle meningeal artery (MMA) are reported in literature. These are post-traumatic lesions different from the true aneurysms that have a different origin. In fact they can be associated with arteriovenous malformations, Paget's disease and tumors; nevertheless in some cases the genesis remains unknown. Sometimes the berry aneurysm of MMA can cause non traumatic extradural hematomas. The association meningioma-aneurysm of the middle meningeal artery is extremely rare: only a case has been signalled till now in literature. In our case the patient was a 69 year-old woman admitted in our department following an epileptic seizure. Neuroradiological studies (post-contrast CT and RM) showed a meningioma of the right pterion. A preoperative angiography evidenciated a big blush from the afferences of meningioma by the middle meningeal artery and, proximally a small berry aneurysm of this artery. Selective embolization of the tumor and the aneurysm was performed preoperatively with polyvinyl particles and straight platinum coils. A craniotomy a surgical resection of the meningioma were performed. The role of the endovascular treatment in the management of these lesions is underlined to decrease the risks of morbidity.

Aged↗

Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (Ollier's disease).

Among patients with enchondromatosis, those with Ollier's disease are usually considered to be at a lower risk for extra-osseous malignancy than those with Maffucci's disease. However, several reports suggest that Ollier's disease may also be associated with gliomas. We report here the youngest patient in the literature (16 years) to be detected with a brain tumor and Ollier's disease. This is also the first case with diffuse brain stem involvement. Thus, counselling of patients with Ollier's disease may become more difficult than initially thought.

Adolescent↗

[Ventriculostomy of the third ventricle and diabetes insipidus].

We report the case of a 2-year-old child who sustained a permanent diabetes insipidus following a third ventriculostomy for hydrocephalus from a Dandy-Walker syndrome. Ventriculostomy, used for therapy of non-communicating hydrocephalus, can cause complications such as diabetes insipidus. The latter has rarely been reported and is usually transient. Following ventriculostomy, a close postoperative surveillance is essential, especially in children.

Child, Preschool↗

Painful seizures with allodynia in an 11-year-old boy.

An 11-year-old boy's epileptic seizures started with a feeling of impending crisis, dizziness, headache, and a bad taste in the mouth. This was followed by swallowing and a burning sensation in the left hand. At the same time, other parts of the body experienced allodynia. MRI and CT scans showed a right anteromesial temporal lesion which proved at neuropathology to be a ganglioglioma. Lesionectomy resulted in complete cessation of seizures. Seizures were absent at an 18-month follow-up. Allodynia is discussed in relation to the locality of the lesion.

Child↗

[Clinical experience with the Sp[hy adjustable valve in the treatment of adult hydrocephalus. A series of 147 cases].

OBJECTIVE: Evaluate the advantages, results and drawbacks of the Sophy adjustable valve (SV) in the treatment of hydrocephalus in the adult. MATERIAL AND METHODS: One-hundred and forty-seven adult hydrocephalic patients (mean age = 64.5 years) shunted with a SV between 1990 and 1997 were retrospectively reviewed. Etiologies of hydrocephalus were: "normal pressure" chronic hydrocephalus (NPH) in 124 and tumoral hydrocephalus with intracranial hypertension in 23 patients. One-hundred fifty shunts were established in 147 patients: 14 ventriculoatrial and 136 ventriculoperitoneal shunts. The initial pressure settings of the SV were: high pressure: 12 times (8%), medium pressure: 136 times (90.6%) and low pressure: 2 times (1.3%). RESULTS: Sixteen patients were lost to follow-up. Two patients died within hours following the procedure. So, the results were analyzed in the 129 remaining patients, with a follow-up of 16.7 months (+/- 17.8). Three patients died from shunted-related causes (2.3%). Eighty-four patients had good results (65%), 25 had fair results (19.5%) and 20 failed to improve or deteriorated (15.5%) after shunting. Fifty-three reoperations were performed in 44 patients (34%), including 37 procedures for mechanical obstruction and 6 for septic complications. Subdural effusions (SDE) occurred in 16 patients (12.5%): 10 surgical evacuations were required in 7 patients; 8 patients were successfully treated using uprating of the SV pressure alone; one was managed conservatively. Fifty-eight pressure readjustments were performed in 47 patients. The pressure settings were uprated 28 times in patients showing clinical or computerized tomographic (CT) features suggesting overdrainage; 20 of those proved to be successful (71.5%). The pressure settings were lowered 30 times in patients showing a lack of improvement after shunting, attributed to underdrainage; only 12 of those proved to be successful (40%). Spontaneous changes of the pressure settings were observed in 14 patients (10.8%). After a MRI exposure, SV pressure changes were observed in 64% of patients. DISCUSSION: The technological refinements that have been proposed to eliminate the overdrainage which occurs in most usual cerebrospinal fluid shunts are reviewed. The SV valve has allowed non-invasive adjustments of its opening pressure in patients showing complications ascribed to inappropriate drainage. The need for additional surgery was eliminated in 20% to 25% of patients of the present series. Because of frequent spontaneous and magnetic resonance-induced pressure changes, the need for repeated controls of valve pressure is to be reemphasized. CONCLUSION: This experience suggests that the SV represents a substantial improvement over standard fixed-pressure valves.

Adult↗

[Thoracic spinal cord compression by a gouty tophus. Case report. Review of the literature].

An unusual case of thoracic spinal cord compression caused by extradural tophaceous deposits is reported in a 59-year-old female with a long-standing history of gout involving the metatarsophalangeal joints. T1 and T2 magnetic resonance images of the spine illustrated an extradural hyperintense signal extending from T2 to T9. A decompressive laminectomy disclosed a white caseum-like material in the extradural space, together with a small organized hematoma. Histologic examination showed areas of amorphous substance containing urate crystals surrounded by inflammatory cells, which was diagnosed as a gouty tophus. The patient made an uneventful recovery after surgery. Fifteen similar cases of the literature are reviewed. Although spinal involvement by gout seems relatively common, a compression of the spinal cord or of the cauda equina in gout patients seems exceptional. The diagnosis should be considered in patients showing a relevant history of gout, but spinal cord compromise may also represent the initial manifestation of the disease.

Arthritis, Gouty↗

Prognostic factors in pediatric spinal cord astrocytoma.

BACKGROUND: Factors that could optimize the management of pediatric spinal cord astrocytoma remain unclear and controversial. METHODS: To determine the factors that influence the prognosis of pediatric patients with spinal cord astrocytomas, a series of 73 consecutive patients at 13 French treatment centers with histologically proven spinal cord astrocytomas was retrospectively reviewed. Hospital records, operative records, and results of radiologic investigations were available in all cases. Follow-up was achieved in 94% of cases. RESULTS: Seventy percent of the patients had low grade (1 or 2) tumors. Total or subtotal surgical resection was achieved in 43%. Thirty-six patients were irradiated following surgery. Fifty-one patients were alive at a median follow-up of 54 months. Twenty-three patients relapsed. Univariate analysis showed that good outcome was correlated with male gender, age younger than 7 years, duration of presenting symptoms longer than 2 months, the presence of spinal deformities, and low grade histology, whereas sensory loss was associated with decreased survival. Multivariate analysis using the Cox proportional hazards model confirmed that histology (relative risk [RR] = 7.69) and the interval between first symptoms and diagnosis (RR = 4.93) were significant independent prognostic factors. The extent of surgery or radiotherapy had no clear influence on survival. CONCLUSIONS: This review sheds light on the prognoses of pediatric patients with spinal cord astrocytomas and may help to determine therapeutic strategies based on patients' clinical, radiologic, and pathologic features.

Adolescent↗

Immunohistochemical, ultrastructural, biochemical and in vitro studies of a pineocytoma.

Using both tumor specimen and cultured tumor cells, we have studied the differentiation of a pineocytoma by light and electron microscopy (EM) and immunohistochemical demonstration of glial, neuronal and neuroendocrine markers. Only interstitial cells were labeled with anti-glial fibrillary acidic protein and anti-S100 protein antibodies. Synaptophysin, neurofilaments and tau labeling was found in cells forming the pineocytomatous rosettes. Some cells also bound the anti-tryptophan hydroxylase antibody (TPOH), but no staining was seen after application of anti-chromogranin A or S-antigen antibodies. EM provided evidence for neurosensory differentiation demonstrating the presence of vesicle-crowned rodlets, cilia (9+0) and fibrous filaments. In culture, tumor cells proliferated slowly and showed positive immunolabeling for vimentin and TPOH. Expression of mRNA coding for TPOH, serotonin N-acetyltransferase, hydroxyindole-O-methyl-transferase and c-myc was found in the tumor using reverse transcriptase-polymerase chain reaction. These results demonstrate neuronal differentiation of this pineocytoma and suggest that the neoplastic pineal cells are capable of synthesizing serotonin and melatonin.

Acetylserotonin O-Methyltransferase↗

Low brain tissue oxygen pressure: incidence and corrective therapies.

In 16 head injured patients, the monitoring of brain tissue oxygen pressure (ti-pO2) show 22 episodes of low ti-pO2 (< or = 12 mmHg). Mean episode duration was 16 h. At time of the lowest ti-pO2 value, cerebral perfusion pressure (CPP), was < 60 mmHg in 5 cases, 4 of them with impending brain death. Oxygen saturation values of the jugular venous blood (svjO2) remained in the normal range (55-85 mmHg) in 12 cases and exceeded 85 mmHg in 3 cases, 2 of them with impending brain death. Lactate-oxygen index (LOI) was normal (< 0.08) in 7/10 cases and at very high level (> 0.60) in 3 cases including 2 cases of impending brain death. A first group of low ti-pO2 episodes was clearly related to an insufficient CPP level (n = 13), comprising 4 cases of parallel decrease in CPP and ti-pO2 until brain death, and 9 cases in which ti-pO2 was restored along with a significant increase in CPP (p < 0.001). In 5 patients, low ti-pO2 episodes were due to another cause: vasospasm (2 cases), hypoxemia, anemia and premature interruption of anesthesia. Appropriate treatments were effective in restoring ti-pO2 with no change in CPP. In 4 patients, the cause of low ti-pO2 was not identifiable and episodes resolved spontaneously. The results confirm the critical influence of CPP and ti-pO2. Patients in whom elevation of CPP improved ti-pO2 have normal range CPP during the episode. Optimal CPP should therefore be sometimes higher than recommended. ti-pO2 monitoring appears a good method to define the optimal CPP level in individual patient. The duration of the artefactual period after catheter placement is to clarify.

Adolescent↗

Brain metastases in children with solid tumors.

BACKGROUND: Brain metastases are uncommon among children with solid tumors. However, improvements in survival have increased the period of time during which children are at risk for developing these metastases. The authors reviewed brain metastases in children with solid tumors treated at the Centre Léon Bérard during the 9 years between 1987 and 1995. METHODS: Among 486 patients with solid tumors, 162 eventually developed distant metastases in their disease process, including 12 brain metastases detected by imaging. The tumor type, clinical setting, imaging characteristics, treatment modalities, and outcome were assessed for each patient. RESULTS: The most common tumors causing brain metastases were Ewing's sarcoma (in three patients), neuroblastoma (in three patients), and osteogenic sarcoma (in three patients). At the time of initial diagnosis, 9 of the 12 patients had metastatic disease. All but one patient initially received intensive multiagent chemotherapy, including high dose chemotherapy with bone marrow rescue in six patients. The median time from initial diagnosis to the detection of brain metastases was 15 months. These metastases were clinically detectable in 10 patients and subclinical in 2 patients. Brain metastases were present at the time of first relapse in five patients. In two patients, the brain was the only site of relapse. All other patients had extensive systemic disease. Seven patients had multiple brain metastases. Two children underwent surgical resection of solitary metastases, and eight were irradiated. One child achieved complete remission following chemotherapy and irradiation. All other children died, mostly of their systemic disease, within a median period of 3 months. CONCLUSIONS: The introduction of effective systemic chemotherapy has changed the patterns of brain metastases in children. The increasing incidence of these metastases in patients with sarcoma and neuroblastoma suggests that the brain is a pharmacologically protected site in patients initially diagnosed with metastatic disease.

Adolescent↗

Spinal cord compression by secondary epi- and intradural metastases in childhood.

Over a 9-year period, 35 out of 614 children with malignant tumours who were treated at the Centre Léon Bérard developed spinal metastases. Of these, 18 with known malignancies before the development of spinal cord compression are reviewed. The most common tumours causing spinal metastases were Ewing's sarcoma, neuroblastoma and renal tumours. Cord compression occurred 5-88 months after the diagnosis of systemic cancer. The median interval from first symptoms to the diagnosis of compression was 17 days. There were 16 patients with neurological deficit, including 5 with paraplegia. Specific imaging procedures were performed in 16 patients. Treatment included operation in 8 patients, followed by chemotherapy (6 patients) and/or radiotherapy (4 patients); 9 of the 10 non-operated patients received radiotherapy. Only 6 patients had a significant neurological improvement. All patients but 1 died within a median time of 2 months. Early diagnosis might prevent permanent disability in these children with a short survival expectancy.

Adolescent↗

Etoposide and thiotepa followed by ABMT (autologous bone marrow transplantation) in children and young adults with high-grade gliomas.

The addition of conventional chemotherapy to irradiation has improved the outcome of children with malignant gliomas. The application of high-dose chemotherapy has been proposed as a possible way to increase the response rate and thus the survival in children with malignant brain tumours. High-dose etoposide (500 mg/m2/day x 3) and thiotepa (300 mg/m2/day x 3) followed by bone marrow transplantation were given to 22 patients (age range 4-20 years) with newly diagnosed or recurrent high-grade glioma. The response rate in the 14 assessable patients was 29% with one complete and three partial responses. 5 patients had stable disease, and 5 progressive disease. 2 patients died of treatment-related toxicity. Only 3 patients remain alive disease free 54, 60 and 65 months after high-dose therapy. For children with high-grade gliomas, survival using high-dose chemotherapy is no better than that reported with conventional treatments.

Adolescent↗

[Chiari I malformation with syringomyelia treated by decompression of the cranio-spinal junction and tonsillectomy. Apropos of 8 cases].

We report a series of 8 patients with syringomyelia and associated Chiari I malformation treated between 1992 and 1996 using a cranio-vertebral decompression with tonsil resection. The follow up period ranged from 6 months through 4 years (mean: 28 months). Neurological symptoms and signs improved in 7 patients (87%), and were stabilized in 1 (13%). At 6 months, postoperative MRI showed that the syringe has decreased in all patients. We emphasize the respect of the different stages of surgical procedure (limited foramen magnum opening, arachnoid preservation, tonsillar subpial aspiration, arachnoid suture and dural closure). Other of treatment and their results are reviewed.

Adolescent↗

Complications after multidisciplinary treatment of cerebral arteriovenous malformations.

PATIENTS AND TECHNIQUES: A series of 67 patients treated for cerebral AVMs with a multidisciplinary approach is reported, with special attention for the complications due to treatment. The malformations were classified after the Spetzler Grading Scale, with 67% low-grade and 33% high-grade AVMs. Three modes of treatment were used: surgical resection, endovascular embolization, and radiosurgery (linear accelerator technique). The actual treatment was: resection alone (25% of cases), embolization plus resection (24%), embolization alone (21%), and radiosurgery (30%), either alone or after embolization or surgery. The following eradication rates were obtained: overall 80%, after resection (with or without embolization) 91%, after embolization alone 13%, after radiosurgery 87%. CLINICAL OUTCOME: The outcome was evaluated in terms of deterioration due to treatment. A deterioration after treatment occurred in 19 patients (28%), and was a minor deterioration (19%), a neurological deficit (4%), or death (4%). As far as the mode of treatment is concerned, surgical resection was responsible for deterioration (minor) in 17% of all cases operated upon. Radiosurgery was followed by a minor deterioration in 10% of irradiated cases. Embolization gave a complication in 25% of all embolized cases (minor or neurological deficit, or death). The mechanism of the complications was: resection or manipulation of an eloquent area during surgery, radionecrosis after radiosurgery, ischaemia and haemorrhage (50% each) following embolization. In most cases of haemorrhage due to embolization, occlusion of the main venous drainage could be demonstrated. DISCUSSION: The haemodynamic disturbances to AVMs and to their treatment are reviewed in the literature. The main haemodynamic mechanisms admitted at the beginning of a complication after treatment of cerebral AVMs are the normal perfusion pressure breakthrough syndrome, the disturbances of the venous drainage (venous overload or occlusive hyperaemia), and the retrograde thrombosis of the feeding arteries. CONCLUSIONS: According the authors' experience, the emphasis of treatment for cerebral AVMs has now shifted from surgical resection to endovascular embolization. One of the explanations is that endovascular techniques are now employed in the most difficult cases (high grade AVMs). As severe complications of endovascular embolization may also occur for low-grade malformations, the question arises whether surgery or radiosurgery should not be used first for this low-grade group even if embolization is feasible.

Adult↗

Management of unruptured cerebral aneurysms.

A series of 62 patients treated surgically for one or several unruptured intracranial aneurysms is reported. 83 aneurysms were treated in 65 operations. The main locations of the aneurysms were: MCA 35%, ICA (posterior communicating) 22%, carotido-ophthalmic segment 12%, carotid bifurcation 11%, anterior communicating artery 11%, verterbro basilar artery 5%. The circumstances of discovery were: incidental 28%, multiple aneurysm 22%, headache 18%, ischemic episode 9%, mass effect 8%, seizures 6%. Overall, 8% of these unruptured aneurysms were certainly symptomatic, 58% were certainly asymptomatic, and for 34% the relationship with the mode of discovery was uncertain. The overall outcome of surgery was: good recovery 94%, moderately disabled 1.5%, severely disabled 1.5%, and death 3%. The post-operative complications were related to surgical technique in 2 cases, to a severe atherosclerotic state of the ICA in 1 case, and to the general arteriopathy of the patient in 1 case. The discussion reviews in the literature the various arguments developed in favor of an active treatment of the unruptured cerebral aneurysms. Three arguments are proposed. 1. The overall severity of the aneurysm rupture, with a mortality rate over 60%. 2. The cumulative risk of rupture of an unruptured aneurysm, which may be high in young patients (from 16 to 30% lifetime risk). 3. The good outcome of the surgical treatment of the unruptured aneurysm (mortality rate under 4%, morbidity rate approximately 6%). The operative risk is higher for large or giant aneurysms, for a patient with a history of ischemic cerebrovascular accident as mode of discovery, for elderly patients with arteriosclerotic thickening of ICA wall and aneurysm neck. The decision to treat or not to treat may be easier (mass-effect, multiple aneurysm, acute headache) or more difficult (chronic headache, hemorrhage of other origin, seizures, incidental discovery). The endovascular treatment with occlusion of the aneurysms sac by means of coils is more and more an alternative to surgical treatment, but requires a long follow-up to ensure the absence of reexpansion of the coil-embolized aneurysms. The screening for unruptured aneurysms, especially in cases with familial intracranial aneurysms is more and more often proposed. The authors' opinion now is surgical clipping of small and middle-sized aneurysms in young patients, without severe associated pathology, and clearly agreeing with surgery. The limit of age for surgery is usually 65 years except for those aneurysms discovered after a mass-effect. Elderly patients, giant aneurysms, patients with contra-indication for surgery, are proposed for endovascular treatment.

Adult↗

[Therapeutic risk in multidisciplinary approach of cerebral arteriovenous malformations].

PATIENTS AND TECHNIQUES: A series of 67 patients treated for cerebral AVMs using a multidisciplinary approach is reported, paying special attention to the complications due to treatment. The malformations were classified according to the Spetzler Grading Scale, with 67% low-grade and 33% high-grade AVMs. Three modes of treatment were used: surgical resection, endovascular embolization, and radiosurgery (linear accelerator technique). The actual treatment was: resection alone (25% of cases), embolization plus resection (24%), embolization alone (21%), and radiosurgery, (30%) either alone or after embolization or surgery. The following eradication rates were obtained: 80% overall, 91% after resection (with or without embolization), 13% after embolization alone, 87% after radiosurgery. CLINICAL OUTCOME: The outcome was evaluated in terms of deterioration due to treatment. Treatment-related deterioration occurred in 28% of cases and consisted of a minor deterioration (19%), a neurological deficit (4%), or death (4%). As far as the mode of treatment is concerned, surgical resection was followed by deterioration in 20% of all operated cases (minor 17%, deficit 3%). Radiosurgery was followed by a minor deterioration in 10% of irradiated cases. Embolization resulted in complication in 25% of all embolized cases (minor 12.5%, neurological deficit 5%, or death 7.5%). The mechanism of the complication was: resection on manipulation of a functional area and the haemorrhage for cases treated by surgery, radionecrosis for radiosurgery, ischemia and haemorrhage (50% each) for embolization. In 4 out of the 5 cases of haemorrhage due to embolization, an occlusion of the main venous drainage could be demonstrated. DISCUSSION: The haemodynamic disturbances relating to AVMs and to their treatment are reviewed in the literature. The main haemodynamic mechanisms at the origin of a complication after treatment of cerebral AVMs are the normal perfusion pressure breakthrough syndrome, venous drainage defects (venous overload or occlusive hyperemia), and retrograde thrombosis of the feeding arteries. CONCLUSIONS: Improved treatment of cerebral AVMs has been achieved through the multidisciplinary approach, and especially through the endovascular embolization technique. Such an improvement is especially visible in the field of high-grade malformations, which are the most difficult and the most dangerous to treat. As a consequence, the risk of the treatment has naturally shifted from surgical resection towards endovascular embolization which is the first procedure to be performed in difficult cases. Careful consultation between the various specialists is necessary in this pathology, particularly as some of these AVMs are a good indication for each of the three available methods of treatment.

Adult↗

Level of consciousness and age as prognostic factors in aneurysmal SAH.

The prognostic value of the level of consciousness and the patient's age for the outcome of aneurysmal subarachnoid haemorrhage (SAH) is studied in 74 patients admitted on day (D) 0 to D3 after aneurysm rupture. For the level of consciousness three groups of patients are compared: grade I+II (alert patients), grade III+IV (drowsy patients), and grade V (comatose patients). For the age, two groups are compared: patients aged under 50, and patients aged 50 and over. The timing of surgery was: D0-D3 51%, D4-D6 20%, D7 and later 18%, and No surgery 11%. The overall management results were: Good (satisfactory result) 43%, Fair (moderately disabled) 18%, Poor (severely disabled+vegetative survival) 19%, and Death 20%. The outcome was strongly related to the level of consciousness, the rates of Good result decreasing from 71% (grades I-II) to 14% (grades III-IV) and to zero (grade V), and the mortality rates increasing respectively from 5% to 14% and 61%. The relationship between outcome and age was less marked: 54% Good result under 50 and 30% over 50. Out of the Grade V group, 56% could be operated upon and 44% died before surgery. No patient from the other two groups died before surgery. The literature concerning the Grading Systems published so far and the various prognostic factors are discussed.

Aged↗