[Craniopharyngiomas]. / Les craniopharyngiomes.

Craniopharyngiomas are rare benign epithelial tumors, arising from the pituitary stalk or gland and developing in the sellar and suprasellar region, affecting both adults and children. Incidence is 0.5 to 2 new cases per million inhabitants. Clinical features essentially include visual disturbances, endocrine deficiencies, and neurological signs. Initial signs are often visual loss and increased intracranial pressure in children, growth and pubertal delay in teenagers, visual disturbances or cognitive impairment in adults. Diagnosis is made on MRI and CT scan, demonstrating a sellar or suprasellar tumor, heterogeneous, with frequent calcifications. Craniopharyngiomas can be classified depending on their locations from the sella, the diaphragma sellae, and upon their origins from the pituitary stalk or the infundibulum. They can also be classified depending on the location from the optic chiasm and the third ventricle. This classification allows surgical series comparison, which is of importance since developments and extensions of the tumor can explain surgical difficulties. The management of this lesion is still controversial. Because it is an extra-cerebral benign lesion, the ideal goal of treatment should be complete tumor removal with improvement of altered visual functions, minimal deterioration of endocrine function, and no neuropsychological impairment. But the situation of the tumor, its relationship with third ventricle, hypothalamus, optic tract, vascular structures make its removal often difficult. However, great progresses have been realized in surgical treatment, resulting in a dramatic improvement of the prognosis of craniopharyngiomas. Nowadays, one can expect total removal in 60 to 70% of patients, subtotal removal in 20 to 30%, and partial removal in 10%. When total removal is impossible, radiotherapy may reduce the risk of a poor evolution. Recurrences are a problem in 15% of patients with total removal, 35% in subtotal removal, 70% in partial removal. If radiotherapy has not been performed as first treatment, it is efficient in 80% of recurrences. Long term follow up is necessary in these patients, due to medical management of endocrine, visual and psychological problems, and risk of late recurrence. With close involvement in this management, most of patients may enjoy a good outcome, 80% being able to return to normal active life.

Giant and large peripheral cerebral aneurysms: etiopathologic considerations, endovascular treatment, and long-term follow-up.

BACKGROUND AND PURPOSE: Giant/large peripheral cerebral aneurysms beyond the circle of Willis and middle cerebral artery (MCA) bifurcation are rare lesions, their etiopathogenesis is not completely elucidated, and their treatment is often difficult. We reviewed the etiopathologic findings available in the literature and report the results and long-term follow-up in 10 patients with a giant/large peripheral aneurysm treated by parent artery occlusion. METHODS: Four aneurysms were on the MCA, 2 on the anterior cerebral artery, and 4 on the posterior cerebral artery (PCA). Two patients presented with bleeding. An occlusion test was performed in 7 patients. Occlusion of the parent artery was performed by using coils in 8 patients; and liquid coils and glue, in 2. Imaging follow-up was available from 1.5 to 4.5 years; and clinical outcome, from 1.5 to 7 years. RESULTS: All aneurysms were excluded. One patient developed a partial homonymous hemianopsia after PCA occlusion. A transient deficit was observed in 2 other patients. In the partially thrombosed aneurysms, follow-up imaging showed shrinkage of the thrombosed compartment of the aneurysm and disappearance of the mass effect. No patient experienced progression of symptoms and/or bleeding during the follow-up period. At long-term follow-up, 6 patients presented with a modified Rankin Scale score of 0; 3, with score of 1; and 1, with score of 2. CONCLUSION: The endovascular occlusion of the parent artery appears a relatively safe and efficacious technique in the treatment of these lesions. Long-term follow-up studies confirm persistent exclusion of the aneurysm and good clinical tolerance to the vessel occlusion.

[A clinical study of the number processing system: decimal size effects on reading numbers in patients with left parieto-occipital gliomas]. / Effet de la grandeur décimale sur la lecture de chiffres dans les gliomes du carrefour gauche.

An increasing number of studies are focusing on the anatomo-functional organisation of number processing and some cognitive models have been recently developed. Nevertheless, relationships between areas implicated in number processing, and language areas and circuits remain unclear. Recently, Dehaene and Cohen, in their "triple-code model of number processing", (Dehaene and Cohen, 1995) distinguished two alternative number representation and processing systems: one depending on verbal processes, the other representing a quantity manipulation. According to this model, the retrieval of "arithmetical facts" (AF), learned by rote at school and memorised in a verbal form (such as the multiplication table or simple addition problems) can be considered as a verbal automatism; conversely, subtraction problems, which require mental manipulation of the quantities, represent an abstract, semantic elaboration: "Actual Calculation" (AC). The anatomical correlate of the retrieval of AF (depending on automatic verbal associations) seems to correspond to the left-hemispheric perisylvian areas, while impairment of the actual calculation (AC) depends on the intraparietal region, particularly in the left dominant hemisphere. The present study describes the neuropsychological assessment of three patients, tested after surgery for left parieto-occipital tumors. Two of them were affected by an anaplasic glioma, the third by a low-grade glioma. The cognitive evaluation included: words of Rey, numeral (directed and reversed) span, reading of "simple" numbers (from 1 to 10) and of "complex" numbers (many decimals), writing (dictation) and reading a standard text, finger denomination and right-left distinction. All patients showed language disturbances, dysgraphia and severe dyslexia. In reading numbers, we identified two types of errors: lexical and syntactic. "Lexical errors" consisted in a wrong choice among words in the number's lexicon. For instance, all patients made errors in reading "complex" numbers composed by many decimals, switching single numbers but respecting the decimal size and the structure of the whole number (such as 69107 instead of 68107). On the other hand, only one patient committed syntactic errors, misunderstanding the decimal size and the structure of the number. We considered lexical errors as verbal errors, and syntactic errors as semantic errors, affecting the notion of quantity. We tried to explain verbal disturbances as well as lexical errors as a consequence of lesion of the left-hemispheric perisylvian areas, while syntactic errors as a consequence of impairment of the intraparietal region.

[Optochiasmal cavernoma: a rare cause of unilateral visual loss]. / Baisse visuelle rapide unilatérale associée à un cavernome optochiasmatique.

INTRODUCTION: Optic pathways cavernomas are rare vascular hamartomas that can present either with an acute chiasmal syndrome or slowly progressive visual loss. OBSERVATION: A 29-year-old patient presented with mild unilateral visual loss of rapid onset and monocular left temporal hemianopia. MRI disclosed a heterogenous enhancing optochiasmal lesion. Work-up found no evidence for an inflammatory, infectious or tumoral disease and therefore a neurosurgical approach of the lesion allowed diagnosis of cavernoma, confirmed by pathological examination. Successful resection resulted in partial recovery of the visual field. CONCLUSION: Optochiasmal cavernomas are rare, removal can improve visual outcome.

Intracranial extension of an idiopathic orbital inflammatory pseudotumor.

Idiopathic orbital inflammatory pseudotumor (IOIP) with endocranial extension is very unusual. The authors used CT and MR to diagnose IOIP and demonstrate the presence of intracranial extension of orbital and lacrimal gland lesions. While providing additional evidence of IOIP having intracranial extension, this case report emphasizes the need to include IOIP as a possible differential diagnosis when radiologic explorations reveal lesions extending from the orbit to intracranial structures.

Craniopharyngiomas in two consanguineous siblings: case report.

OBJECTIVE AND IMPORTANCE: We describe a double case of craniopharyngioma in consanguineous siblings, suggesting the disease is sometimes genetic. CLINICAL PRESENTATION: Two typical adamantine craniopharyngiomas were observed in two consanguineous siblings. The brother and the sister, whose parents were first cousins, developed the tumors at the same age. INTERVENTION: The male patient was operated on using a frontopterional approach, and the tumor was completely resected. The patient remained free from recurrence 9 years after surgery. His older sister died after tumor removal was attempted at another institution. CONCLUSION: To our knowledge, such a connection has never been reported in the literature. It suggests that craniopharyngioma, which is usually sporadic, can also be transmitted in an autosomal recessive manner.

Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel-Lindau disease. Case report.

This report describes a patient with von Hippel-Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.

Correspondence between functional magnetic resonance imaging somatotopy and individual brain anatomy of the central region: comparison with intraoperative stimulation in patients with brain tumors.

OBJECT: The goal of this study was to determine the somatotopical structure-function relationships of the primary motor cortex in individual patients by using functional magnetic resonance (fMR) imaging. This was done to assess whether there is a displacement of functional areas compared with anatomical landmarks in patients harboring brain tumors close to the central region, and to validate these findings with intraoperative cortical stimulation. METHODS: One hundred twenty hemispheres in 60 patients were studied by obtaining blood oxygen level-dependent fMR images in patients while they performed movements of the foot, hand, and face on both sides. There was a good correspondence between anatomical landmarks in the deep portion of the central sulcus on axial slices and the somatotopical organization of primary motor areas. Pixels activated during hand movements were centered on a small characteristic digitation; those activated during movements in the face and foot areas were located in the lower portion of the central sulcus (lateral to the hand area) and around the termination of the central sulcus, respectively. In diseased hemispheres, signal-intensity changes were still observed in the projection of the expected anatomical area. The fMR imaging data mapped intraoperative electrical stimulation in 92% of positive sites. CONCLUSIONS: There was a high correspondence between the somatotopical anatomy and function in the central sulcus, which was similar in normal and diseased hemispheres. The fMR imaging and electrical stimulation data were highly concordant. These findings may enable the neurosurgeon to locate primary motor areas more easily during surgery.

Intra-orbital Castleman's disease.

Castleman's disease is a rare lymphoproliferative process found in either a focal or multicentric localization. Most cases occur as mediastinal and abdominal mass, although nodal and extranodal locations have been reported. Eleven cases of Castleman's disease involving the central nervous system have been reported, but only one is a proven instance of primary intra-orbital localization. The present report concerns a clinico-pathologic Castleman's disease case, to our knowledge the second orbital case described in the literature.

[Chronology of diagnostic and therapeutic activities in the 3 weeks following rupture of an intracranial saccular aneurysm (series of 316 cases)]. / Chronologie des actes diagnostiques et thérapeutiques dans les trois semaines suivant la rupture d'un anévrisme sacculaire intra-cranien (d'après 316 cas).

The authors advocate to operate grades I, II and III patients on the 6th or 7th day following the onset of SAH, that is to say between the "oedema" period and the rebleeding period. They study all investigations and therapy which are necessary previously to the operation and advocate to perform a CT scan as the first investigation as well as to control the intraventricular pressure. Their opinion is based on the study of 316 cases admitted over a period of 5 years 218 have been operated upon by a team of 7 neurosurgeons with an overall mortality of 18.3 p. 100. The mortality in grade I and II patients has been 13.3 p. 100. This percentage includes all fatalities occuring in the six months following surgery. The senior author assumes that a good audio-visual teaching of the trainees can improve the results.

[Preoperative direct cortical and sub-cortical electric stimulation during cerebral surgery in functional areas]. / Intérêt des stimulations électriques corticales et sous-corticales directes peropératoires dans la chirurgie cérébrale en zone fonctionnelle.

Indications of surgical treatment for lesions in functional cerebral areas depend on the ratio between the definitive neurological deficit and the beneficial effect of resection. Detection of eloquent cortex is difficult because of important individual variability. Peroperative direct cortical and subcortical electrical stimulations (DCS) provide the most precise and reliable method currently available allowing identification and preservation of neurons essential for motricity, sensitivity++ and language. We report our preliminary experience with DCS in surgery of intracerebral infiltrative tumors with a consecutive series of 15 patients operated from November 96 through September 97 in our institution. Presenting symptoms in the 15 patients (8 males, 7 females, mean age 43 years) were seizures in 11 cases (73%) and neurological deficit in 4 cases (27%). Clinical examination was normal in 11 patients and revealed hemiparesia in 4. Magnetic resonance imaging (MRI) with three-dimensional reconstruction showed a precentral tumor in 10 cases, central lesion in one patient, postcentral lesion in two cases, right insular tumor (non-dominant hemisphere) in one case. All patients underwent surgical resection using DCS with detection in 13 cases of motor cortex and subcortical pathways under genera anesthesia, in one case of somatosensory area under local anesthesia, and in one case of language areas also under local anesthesia. The tumor was recurrent in two patients had been operated earlier but without DCS. Resection, verified by postoperative MRI, was total in 12 cases (80%) and estimated at 80% in 3 patients. Histological examination revealed an infiltrative glioma in 12 cases (8 low grade astrocytomas, 3 low grade oligodendrogliomas, and one anaplastic oligodendroglioma), and metastases in 3 cases. Eight patients had no postoperative deficit, while the other 7 patients were impaired, with, in all cases except one, complete recovery in 15 days to 2 months. Direct cortical and subcortical electrical stimulations offer a reliable, precise and safe method, allowing functional mapping especially useful in case of infiltrative cerebral tumors in eloquent areas. This technique allows improvement in the quality of tumoral resection and concurrently a minimization of the risk of definitive postoperative neurological deficit.

[Total removal through the ventricle in 2 cases of pallidocaudal arteriovenous abnormalities under deep hypotension]. / Exérèse totale par voie ventriculaire de deux malformations aftério-veineuses pallido-caudées sous hypotension profonde

Two cases of A.V.M. situated in the pallido-caudal area and directly nourrished by the anterior perforated space arteries have been investigated using three angiographic techniques: selective internal carotid angiography, angiotomography including pneumoencephalography and angiography under deep hypotension at 30 mm Hg (mean pressure) induced by sodium nitroprusside. These investigations led to the conclusion that the A.V.M. could be removed through the lateral ventricle. Both were radically removed using the retrograde technique under deep hypotension and the operative microscope. Both patients are alive without additional neurological deficit.

[Intracranial cholesterol granulomas. Report of 2 cases]. / Granulomes cholestéroliques intracrâniens. 2 observations.

Two cases of cholesterolic granuloma, one intraorbital and the other of the petrous apex, are reported. The xanthogranulomatous and/or cystic process localized in the intracranial cavities or in the brain parenchyma are discussed. A simple topographical and histological classification is attempted in answer to a rather ambiguous terminology.

[Discussion of terminology concerning disorders of the optic nerve and disc (author's transl)]. / Discussion sur la terminologie concernant les affections du nerf optique et de la papille.

Progress over the past few years in knowledge concerning disorders of the optic nerve and disc leads to changes in their classification. A new terminology is proposed. It uses a minimum of terms, their very general sense being understandable by any nonophthalmologist physician. It is based upon clinical symptoms and signs, thus being immediately applicable in clinical practice.

[A case of tuberculoma of the orbit with sphenoidal origin]. / A propos d'un cas de tuberculome de l'orbite à point de départ sphénoïdal.

We report the case of an orbital and sphenoidal tuberculosis observed in a young Indian man living in France. The orbital disease is presenting as an orbital mass with a radiologic bony lytic aspect, without systemic manifestation. A malignant process was suspected. The diagnosis was obtained by the culture for Mycobacterium tuberculosis of the biopsy specimen. Orbital involvement in tuberculosis is a rare manifestation in developed countries. Most cases reported in the literature were reported from Asia and Africa. Orbital tuberculosis may become established in one of two ways: primarily by heamatogenous spread to cause a periostitis or a tuberculoma of the orbital tissues, or secondarily by direct extension from neighbouring structures. It occurs in patients with or without associated pulmonary tuberculosis. Mycobacterium tuberculosis should be considered in the differential diagnosis of inflammatory orbital diseases. This diagnosis is based on clinical feature, positive tuberculin skin test and positive culture in resected tissues. The complete resolution of the disease with the specific antituberculous drugs emphasizes the point that tuberculosis must be evocated.

[Computed x-ray tomographic aspects of intra-orbital hydatid cyst. Apropos of 10 cases]. / Aspects tomodensitométriques du kyste hydatique intra-orbitaire. A propos de 10 observations.

Echinococcus taenia occurs in human accidentally after contamination by infected dust of dog. Although it may localize in all parts of the body as a hydatic cyst, liver, lung and kidney are the most frequent sites. Intra-orbital localization is rare since all statistical studies estimate it to be one per cent of orbital tumours. Using computerized axial tomography (C.A.T. Scanner) ten cases of intra-orbital hydatic cyst originating from Algiers, Tunis Hospitals and C.N.O. des Quinze-Vingts of Paris were studied. The inclusion of C.A.T. scanning in the orbital evaluation enables more positive preoperative diagnosis and facilitates the choice of the most efficient surgical approach.

[Mucigenous cysts of the pituitary gland. 3 cases]. / Les kystes mucigènes de l'hypophyse. Trois observations.

Three cases of mucous cysts, or Rathke's cyst, of the pituitary gland are reported. The clinical and biological features were comparable to those of a non-secreting adenoma. Although extremely rare, these cysts must be recognized since their particular and, in theory, specific characteristics (wall made up of a ciliated epithelium with mucus cells and cavity filled with a mucoid substance) distinguish them from other intra or suprasellar pathological formations, especially craniopharyngiomas. The 62 cases reported in the literature suggest that they have a favourable prognosis.

[Radionecrosis of the optic chiasma. A clinical and radiological study of 3 cases]. / Radionécrose du chiasma. Etude clinique et radiologique. A propos de trois cas.

The paper reports three cases of optic chiasmal radionecrosis due to radiotherapy of the sellar region. These three cases are compared with 34 ones found in literature. The use of M.R.I. is shown to dramatically improve diagnosis. Corticoids are useful in the treatment. The gravity of the complication, which implies visual prognosis, focuses on a strict use of radiotherapy. For benign or slowly evoluting lesions, surgery and/or regular controls by modern imaging techniques would often be more eligible than systematic earliest irradiation.

[Craniopharyngiomas in adults and children. Study of a surgical series of 106 consecutive cases]. / Crâniopharyngiomes de l'adulte et de l'enfant. Etude d'une série chirurgicale de 106 cas consécutifs.

This work is devoted to a 20-year retrospective study of 106 surgical cases of craniopharyngiomas in adults and children treated and followed by the same neurosurgeon. The mean follow-up was 7.2 years. In this homogeneous series, the aim was total microsurgical removal of the tumor, without post-operative radiotherapy. The operation was usually performed through a fronto-pterional approach, and, in some particular cases, through a transsphenoidal approach. The tumor removal was total in 59 cases, subtotal in 31 cases, partial in 16 cases. Even when the removal was not total, radiotherapy was not systematically administered, but was reserved for relapses. We have studied clinical signs, operation characteristics, ophthalmological, endocrinological and functional outcome, as well as recurrence risk and long-term survival. The functional results of our patients were excellent in 82%, good in 9%, fair in 8% (usually due to ophthalmological sequellae), and poor in 1%. Twenty-five patients recurred, but the salvage treatment, by reoperation or radiotherapy, was successful in almost 75%. The actuarial survival was 91% after 5 years, and 80% after 10 years. These results compare favorably with the literature data, suggesting that radical surgery of craniopharyngiomas allows good outcome in terms of survival, full recovery and quality of life, for both adults and children.

[Value of the intracranial pressue measurement in the craniosynostoses detected after the age of 1 year]. / Intérêt de la mesure de la pression intra-crânienne dans les craniosténoses découvertes apres l'âge d'un an

Notwithstanding the fact that there is a general agreement on the necessity of surgery in the first year of life in craniosynostosis, the problem is more difficult for older children. Deterioration of clinical status is, in general, in relation with an increase in intracranial pressure (ICP), which may happen abruptly following different causes (slight head injury, for example). The purpose of this study was to determine, in different varieties of synostosis, if a high intracranial pressure could exist without clinical signs and consequently, if a surgical opening of the sutures would be necessary. IPC has been measured through a ventricular catheter connected with a transducer and recorder for 24 hours. In half of the cases (11 out of 22) a high ICP (above 20 mmHg) was recorded either permanently or during sleep. This increase in ICP should lead to a surgical decompression, event without clinical signs. On the other hand, psychomotor retardation, abnormal EEG, increased digitation should not be considered as an indication for surgical treatment in cases with normal ICP. The measurement of decreased ICP after operation on a long term basis would be a great value.