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Notes: Abstract Clinical data and autopsy findings in a case ¶of new variant Creutzfeldt-Jakob disease (vCJD) are reported. this case, the first histologically confirmed case described outside the United Kingdom, very much resembles the cases described by Will et al. [(1996) Lancet 347 : 921–925] and Zeidler et al. [(1997) Lancet 350 : 903–908, 908–910]. Neuropathological studies failed to reveal any conspicuous clues that could be relevant for understanding the pathophysiology of the disease. For epidemiological surveillance, neuropathologists should scrutinize suspected cases keeping in mind the possibility of vCJD.

Notes: Summary The management of the ruptured intracranial aneurysm is studied in two consecutive series: an earlier series, including 328 patients admitted from 1972 through 1984, for which the general attitude was delayed surgery, and a later series, including 140 patients admitted from 1985 through 1989, in which selected patients were submitted to early surgery and other patients were postponed for delayed surgery, according to two main parameters: the clinical status and the patient's age. When we compare both series, the overall management results demonstrate an improvement of 10% of satisfactory results and a decrease of 10% in the death rate in favour of the later series; for the surgical results, the figures are respectively 6% and 5% in favour of the later series. The relationship between age and outcome shows a considerable improvement: over 50 years of age, we observed plus 25% of satisfactory results and minus 22% in death in favour of the later series. Similarly the relationship between state of consciousness and outcome, demonstrated a great improvement; for drowsy and stuporous patients the figures are respectively plus 22% and minus 21% in favour of the later series. When we consider the later series alone, the patients were admitted at 4 intervals of time from SAH (D0-3, D4-6, D7-15, D16 and over). The most favourable outcome was observed for those patients admitted late (after D7) and already stabilized. Patients admitted early (D0-3) were operated on at four intervals of time (D0-3, D4-6, D7-15, D16 and over). The most favourable outcome was observed for those patients operated on early (D0-3) or very late (D16 and over). For patients admitted early and being under 50 years of age, the results were: satisfactory 92%, poor 2.5%, death 5%. The relationship between age and outcome shows a very small difference between patients under or over 50 years of age. The relationship between level of consciousness and outcome still demonstrates an appreciable difference: plus 22% (satisfactory) and minus 7% (death) in favour of alert patients. Rebleeding was the cause of disability or death in 2.8% of the overall later series and 2.7% of patients admitted early; as for vasospasm the figures are respectively 4.2% and 5.4%. These results are presented with reference to those of the Co-operative Study. After this experience, the author's general attitude for the timing of surgery is neither systematic early surgery, nor systematic delayed surgery, but modulated surgery, based upon the evaluation of the operative risk: minor risk, major risk, intermediate risk. Schematically the authors propose: early surgery in alert patients and under 50 years of age (minor risk), late surgery in patients with disturbances of consciousness and over 50 years of age (major risk); preferably early surgery in younger patients even with disturbances of consciousness (intermediate risk); preferably late surgery in older patients, even being alert (intermediate risk).

Notes: Summary The prognostic value of the Spetzler's grading system is studied in a series of 52 AVMs treated by a combined management, using one or several of the 3 available techniques: surgical resection, endovascular embolization, radiosurgery. The symptoms at the time of treatment were haemorrhage 50%, seizures 31%, headache and deficit 19%. Three grade groups were considered: I and II (31%), III (33%), IV and V (36%). Overall, AVMs were managed as follows: resection alone 25%, embolization plus resection 23%, embolization alone 23%, radiosurgery with various combinations 29%. According to the grade groups, the most frequently used technique was resection alone for grade I–II AVMs (44%), radiosurgery for grade III AVMs (41%) and embolization alone for grade IV–V AVMs (42%). The clinical outcome was evaluated in terms of deterioration due to treatment. The best results were obtained in grade I–II AVMs (81% with no deterioration) then in grade III AVMs (65%) and in grade IV–V (58%). However, when we consider the outcome in terms of favourable results (no or only minor deterioration) we obtained a similar outcome for grade I–II and grade III AVMs (94% each), and only 79% for grade IV–V malformations. The angiographic outcome showed a better eradication rate in grade III AVMs (88% complete eradication), than in grade I–II AVMs (75%) and in grade IV–V (47%). Our conclusion is that the Spetzler's grading system in this series was well correlated with both the clinical and the angiographic outcome. However, we found no real difference between grade I–II and grade III AVMs. So, in terms of prognostic value, the grade I, II, and III AVMs could be considered together as low-grade malformations, with a better prognosis than the high-grade malformations (grade IV and V).

Notes: Abstract 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.

Notes: Summary The authors report a series of 37 cases of unruptured aneurysms, admitted and operated upon over a 5 year period (1985–1990), which represents an incidence of 18% of the total number of aneurysm patients operated upon during this period. These unruptured aneurysms were discovered in 4 types of circumstances: 1) Associated with a ruptured aneurysm but treated in a second procedure (9 cases); 2) After a transient ischaemic attack (6 cases); 3) After a cerebral haemorrhage of a different origin (3 cases), 4) After the onset of various neurological symptoms other than SAH (19 cases). Giant aneurysms (over 2.5 cm in diameter) are excluded from this series. Overall these 37 patients harboured 52 aneurysms, and 1 patient was operated upon on both sides. 27 aneurysms (52%) were located on the right side, 15 (29%) on the left side, and 10 (19%) on the midline. In the immediate post operative period, 1 patient died (2.6%) and 8 patients (21%) presented various complications. The outcome at 6 months was: death 2.6%, moderately disabled 8%, good recovery 89%. The arguments in favour of, or against, the surgical treatment of unruptured aneurysms are discussed in view of the literature. In favour of prophylactic surgery are: 1) The rather poor overall outcome following aneurysm rupture (including deaths before admission); 2) The rather good outcome of surgery in published series of unruptured aneurysms. The data of the natural history of the unruptured aneurysm are more questionable: in this view, surgery seems to be recommended in young patients with an easily accessible aneurysm and being in a good clinical condition. Several contra indications should be strictly accepted: severe associated diseases, age (over 65 and sometimes over 60), patient's refusal or reluctance. In cases of unruptured aneurysms to be operated upon in a second procedure after a ruptured aneurysm, the authors usually wait for 2 months or more before the second operation.

Notes: Summary The CO2 laser technique has been routinely used from 1988 through 1992 for the resection of 93 cerebral tumours (meningiomas 58%, gliomas 15%, neurinomas 9%, miscellaneous 18%). The CO2 laser technique was found the more effective 1) in tumours of hard consistency, 2) in large or giant tumours, 3) in tumours with scarce vascularization. Meningiomas were the indication of choice (54 cases that is 58% of all tumours treated with CO2 laser, and 64% of all meningiomas operated on during the same period). Among the meningiomas treated with the CO2 laser, 54% were located on the skull base. The CO2 laser beam provides good haemostasis of small vessels during the vaporization process. When attached to the operative microscope, the other advantages of the CO2 laser technique are: the absence of a handle-piece, the absence of manual manipulation of the tumour, the coaxiality of the laser beam with the visual beam. The disadvantages are: the rigidity of the coupled microscope-Laser arm, the smoke produced by the vaporization of hard tumours, the noise of the device.

Notes: Summary A series of 100 patients treated for a cerebral arteriovenous malformation (AVM) is presented. Patients were admitted between 1985 and April 1992. Two groups are considered: the first group including 52 patients treated before the availability of radiosurgery (1985–1988), and the second group including 48 patients treated after the availability of radiosurgery (1989–1992). AVM's were classified in five grades according to the Spetzler's Grading System. Three techniques of treatment were used: surgical resection, intravascular embolization (with cyanoacrylate), and radiosurgery (linear accelerator). These three techniques were used either alone or in association, giving four types of management: surgical resection alone, embolization and resection, embolization alone, and radiosurgery (alone, or after embolization, or after surgical resection). From 1989 on, the availability of radiosurgery was responsible for the decrease of the “embolization and resection” group, which until then was predominantly used as well for low-grade (I, II, III) as for high-grade AVM's (IV, V). Overall, for the low-grade AVM's, the treatment of choice was surgical resection (79% of cases), with pre-operative embolization in one-half of these cases; the other low-grade AVM's were irradiated, with various combinations. For the high-grade AVM's, the treatment of choice was intravascular embolization (95% of cases), either alone, or followed by resection (45%) or radiosurgery (9%). Results were evaluated in terms of deterioration following treatment, in five groups: no deterioration (59%), minor deterioration (20%), long-lasting deficit (10%), major deterioration (5%), and death (6%). Overall, results improved after 1989: favourable outcome (no deterioration and minor deterioration) increased from 67% to 90%. Results were not related to the patients' age. More favourable results were obtained for low-grade AVM's (93%) than for high-grade AVM's (60%). For the low-grade AVM's the evolution from 1989 on (favourable outcomes increasing from 89% to 96%) occurred with the lowering of the mortality rate. For the highgrade AVM's, the evolution from 1989 onwards (favourable outcome increasing from 46% to 78%) occurred with the decrease of the cases with deficits. The angiographic results were strongly related to the management: 95% of complete eradication after surgical resection and 5% only after embolization alone. Concerning the results in irradiated cases, the follow-up is not long enough. The review of the neurosurgical literature since 1972 demonstrates progressive modifications in the therapeutic attitude as regards AVM's. The surgical management which was predominantly used at the beginning gave way progressively to a combined management, with a combination of embolization, surgery, and lately radiosurgery. The authors' present attitude is in favour of combined treatment using the three techniques. Direct surgical resection is proposed for small and readily accessible AVM's. Direct radiosurgery is proposed for small but deep AVM's or those located in highly functional areas. Intravascular embolization is proposed in every other situation. After embolization has been completed, totally eradicated AVM's are left in place; no further treatment is proposed for AVM's which are still large with high surgical risk. AVM's which are sufficiently reduced in size are either operated on (if accessible) or irradiated (if deeply situated). When the results of radiosurgery are assessed with long enough follow-up (in terms of eradication and clinical outcome) the authors' attitude may either increase the role of radiosurgery or return towards surgical resection, depending of the quality of the results.

Notes: Summary 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.

Notes: Summary A series of 12 patients with tectal plate gliomas, is presented treated by direct surgery. Mean age was 19 years. All patients presented with signs of raised intracranial pressure and supratentorial hydrocephalus on CT scan. Diplopia was the most common local sign. CT scan and MR imaging showed 4 intrinsic, 6 exophytic, and 2 ventrally infiltrating tectal tumours. The histological diagnosis was low-grade astrocytoma in 7, high-grade astrocytoma in 2, oligodendroglioma in one, oligo-astrocytoma in one, and ependymoma in one case. The suboccipital supra- and transtentorial approach was used in every cases. Tumour resection was generous at the level of the superior colliculi, but on the contrary, resection was limited at the level of inferior colliculi due to the auditory risk. Tumour removal was total (macroscopically) in 9 cases and partial in 3 cases. There were 4 surgical complications and one death related to surgery. Parinaud's syndrome was the most-common postoperative sequelae. Auditory hallucinations and the acoustic neglect syndrome were seen once. In three cases additional radiotherapy and chemotherapy were given once with severe sequelae. The treatment of tectal plate gliomas is controversial. The role of different therapeutic options remains open. We consider the tectal plate as a relatively safer territory for surgery than the ventral part of the midbrain. The brain stem auditory evoked potentials (BAEPs) and middle latency potentials (MLPs) monitoring can help to determine the appropriate limit of surgery.

Notes: Summary Between 1979 and 1989, 7 patients were admitted, with cerebral arteriovenous malformations (AVM) and associated aneurysms (7% of the AVM patients and 2% of the aneurysm patients admitted during the same period). 6 of these patients were admitted because of an intracranial haemorrhage (in 3 of them the AVM, angiographically occult, was discovered at surgery). The last patient was referred for seizures. Preoperatively it was supposed that the haemorrhage was related to the aneurysm in 3 cases, and to the AVM in 3 cases. But surgery allowed one to correct this supposition. Haemorrhage was due to AVM rupture in all 6 cases, and no aneurysm had ruptured. Overall three situations were demonstrated in this series: aneurysm and occult AVM (3 cases); AVM and independent aneurysm in the same area (2 cases); large AVM and aneurysm on a feeding artery (2 cases). All 6 patients admitted for haemorrhage were operated upon, at one operation in 5 of them. Both the malformations were excluded in these six patients. For the patient admitted for seizure, intra-vascular embolization of the AVM was performed, the aneurysm was not treated. The pathogenesis of the association AVM-aneurysm is discussed. In the authors' opinion, haemodynamic relationship should be considered in two cases (large AVM and aneurysm on a feeding vessel). For the other 5 cases, both the AVM and the coexisting aneurysm may be the end-result of a common congenital vascular malformation syndrome.