Whipple disease confined to the central nervous system in childhood.

In a case of pediatric Whipple disease confined to the central nervous system, white matter lesions initially appeared as areas of very low signal intensity on T1-weighted MR images and as areas of hyperintensity on proton density-weighted and T2-weighted images, and showed slight peripheral enhancement on delayed contrast-enhanced T1-weighted images. On MR studies obtained 3 and 6 months after antibiotic therapy, the lesions had decreased in size and no longer enhanced. They became progressively less hypointense on T1-weighted images and less hyperintense on T2-weighted images.

Sciatic neuropathy at the popliteal fossa: clinical, ultrasonographic and computed tomographic diagnosis.

A 22-year-old female had been suffering from sciatica-like pain in the left leg for four years. Clinical findings strongly directed further investigations to the popliteal fossa. Ultrasonography located a hypoechogenic mass in the upper lateral popliteal space. Guided by these data, computerized tomography (CT) with vertical reconstructions made the tentative diagnosis of a common peroneal nerve tumor, which was confirmed at operation. Microscopic examination showed a neurinoma of the mixed neurilemmoma-neurofibroma type. In the presence of atypical features of sciatica, a high index of suspicion seems advisable. Emphasis is laid on the complementary contribution of ultrasonography and CT in this type of ill-defined lower limb pain.

[Long-term results of the surgical treatment of myelopathy in cervical arthrosis]. / Résultats à long terme du traitement chirurgical des myélopathies cervicarthrosiques.

After a lapse of not less than two years the authors study the functional and clinical evolution of 62 patients presenting myelopathy in cervical arthrosis treated by surgery. A scoring system for evaluating the functional deficit of the patients is used for quantitative assessment of the disorders. Surgery brought a frank improvement in 38 patients: it left the sensorimotor deficits of 19 patients unchanged. The handicap continued to deteriorate in three patients. Two patients died from non-neurological disorders. The data are compared with those in the literature which indicate an improvement rate of the same order of magnitude. Within certain limits, age and the degree of initial handicap do not seem to influence the long-term result of surgery. However, the time lapse between the appearance of the first signs of deficiency and surgery seem to constitute an important prognostic factor. The shorter the time lapse, the better the result.

Intraneural ganglionic cyst of the common peroneal nerve: case report and review of the literature.

The authors report a case of compression of the peroneal nerve by an intraneural ganglionic cyst. The chief complaint is generally a lateral upper leg pain occasionally associated with foot drop and swelling of the upper tibiofibular joint. In our case, the disease was revealed by a tentative intraarticular injection of betamethasone. Early microsurgical treatment remains the only way to avoid permanent motor sequellae.

[Craniostenosis and craniofacial dysmorphia]. / Craniosténoses et dysmorphies cranio-faciales.

The paper gives a brief historical survey of craniostenosis and the first operative approaches. The authors describe a classification of various types of cranio-facio-stenosis and facio-cranio-stenosis and present a detailed study of their experience in this area. Operative results are discussed in function of the selected surgical procedure, the immediate per- and post-operative complications and the short and long term morbidity. The authors present their current attitude for each dysmorphic type. They underline the heavy nature of this long-lasting and bleeding surgical procedure that concerns small children. They have recorded 31 incidents in relation with surgical procedure, 10 complications at short and long term, and 1 death. Such surgery requires an experienced and vigilant team, associating surgeons, anesthetists and intensive-care physicians.

[Bilateral triple aneurysms operated in two stages: two case studies (author's transl)]. / Anévrysmes triples bilatéraux, opérés en deux temps. Deux observations.

The authors discuss the approach to adopt in a case of multiple cerebral aneurysm, taking into account the patient's condition and the appearance of the aneurysmal wall. The size of the aneurysm is not an absolute criterion on which the base an assumption as to the site of the rupture and the haemorrhage. Therapy should be based on plastic coating together with clipping of the aneurysm.

Craniofaciosynostosis: anesthetic and perioperative management. Report of 71 operations.

Faciocranial dysmorphisms are at the present time corrected during very complex procedures aiming at functional, esthetic and psychological improvement. The authors present their 9 years experience of 71 operations. The age at which those operations are performed is between 6 and 12 months for 14 patients (20%) and under 6 months for 34 patients (48%), the youngest patient being 6 weeks old. Per- and post-operative problems are reviewed. Mean surgical time is 210 minutes. The anesthetic approach aims at reducing the size of the brain by the use of osmotic diuresis, slight hyperventilation, moderate induced hypotension and semi-sitting position. The hemodynamic monitoring includes for all patients an arterial line and a central venous catheter. Blood loss can be large and sometimes sudden; it amounts in average to 62% of patient's estimated blood volume (12 to 200%). The most frequently encountered complications are cardiac dysrhythmias (mainly bradycardia 15 cases), accidental venous sinus opening (2 cases), post-extubation glottic edema (5 cases) and postoperative cerebrospinal leak (2 cases). No long-term sequelae persist from those incidents. There is neither infection, nor perioperative mortality. From those results, the authors consider that infants with craniofaciosynostosis can be operated upon and their dysmorphy corrected with a low perioperative risk. However successful prevention of complications and functional result depend essentially on a multidisciplinary approach, in well-equipped hospitals.

[Success and failure of the ventriculoperitoneal shunt in the treatment of hydrocephalus]. / Succes et échecs de la dérivation ventriculo-péritonéale dans le traitement de l'hydrocéphalie

In ventriculoperitoneal shunting, the intraperitoneal location of the drainage site can be an additional cause of failure. In our series of 70 ventriculoperitoneal shunts, performed in infants and adults for treatment of hydrocephalus from various cause and of various type, failures of this kind manifested themselves usually early in the postoperative period either as a reflux of liquid, not attributable to the operative technique, into the subcutaneous pericatheter tract, or as a reappearance of the intracranial hypertension, without faulty functioning of the ventricular drain. The total success rate was 61% (43/70) but the age of the patient made a clear difference. In patients aged less than 5 months, we observed 11 successes in 33 cases (33%), in those aged less than 1 year, 14 successes in 36 (39%); patients with myelomeninogocele, wherein the shunting was usually done during the first month of life, fared badly; the role of age seems herein predominant. In patients aged more than 1 year, 29 successes in 34 cases (85%) were obtained; results were notably good in nearly all hydrocephalus of tumoral origin. We think the possible role of the "tightness" of the hydrocephalus has to be invoked as also the possibility of spontaneous remission. Ventriculoperitoneal shunting, devoid of the serious complications of ventriculocardiac shunting, is a simple and effective procedure in many cases of hydrocephalus. Means for more accurate prediction of the chances of success in the individual case are needed.

[Arnold-Chiari malformation and myelomeningocele. Multiple paralysis of cranial nerves (author's transl)]. / Paralysies multiples des nerfs craniens dans la malformation d'Arnold-Chiari associée a la myéloméningocèle.

Multiple paralysies of cranial nerves are reported in two infants with myelomeningocele and Arnold-Chiari malformation. In the first case, there was a shunt dysfunction and its correction cured the troubles. In the second, posterior fossa decompression was successful. The indications of the latter intervention are discussed.

[Subdural or sub-arachnoid pericerebral effusions in the infant with subdural or subarachnoid localisation (author's transl)]. / Les épanchements péricérébraux du nourrisson à localisation sous-durale ou sous-arachnoïdienne.

Twenty-seven "subdural" effusions in infants were treated, according to our previously suggested method: a simple external drainage, followed ater one week by an internal peritoneal shunt, if the effusion did not dry up by this time. Daily electrophoresis of the fluid obtained by external drainage has been performed in 20 cases, either until the disappearance of the effusion or until the peritoneal shunting after 7 days. In 8 persistent and in 5 rapidly disappearing effusions, cerebro-spinal fluid was immediately present or appeared during the first week, simultaneously with a lowering of the total protein content. In the other 7 rapidly disappearing effusions, the protein content remained high and a conspicuous plasmatic transsudation was disclosed; the possibility that cerebro-spinal fluid could be present even in some of those last cases is discussed. The fact that some effusions display, from the onset, the characteristics of cerebro-spinal fluid, leads to the question of their subarachnoid rather than subdural localisation. For this reason, we prefer to use the less committed denomination of pericerebral effusions, in which we may distinguish subdural effusions, with or without cerebro-spinal fluid, and subarachnoid effusions. Nevertheless, it is not always easy to establish the pathogenic character of an effusion revealed by CT-scan. Pressure measurements by fontanometry appear to be a useful examination for that purpose.

[Juxtabulbar neurinoma of the spinal accessory nerve]. / Neurinome juxtabulbaire du nerf spinal.

A 27-year-old woman presented with right spinal accessory juxtabulbar schwannoma, associated with hydrocephalus. The only specific clinical symptom was long-standing weakness of the right trapezius. C.T. scan evoked a cerebellar tumor, whilst the jugular foramen appeared normal. Vertebral angiography was not decisive. M.R.I. suggested an extra-axial tumor. Post-operative evolution was entirely favourable. Schwannomas of the 9th, 10th and 11th cranial nerves are generally located at the level of the jugular foramen but can also be observed along the extracranial path of these nerves. An intracranial paramedial, or so-called "intracisternal" localization is rare and is best diagnosed by magnetic resonance imaging.

[Postmeningitic pericerebral effusion in infants]. / Les épanchements péricérébraux postméningitiques du nourrisson.

Agar-gel electrophoresis of the pericerebral fluid and more recent data obtained from CT-Scan examination suggest the presumably important role of external subarachnoid hydrocephalus in postmeningitic pericerebral effusions of infancy. Postmeningitic subarachnoid hydrocephalus results very likely from an impeded resorption at the level of the arachnoid villi and venous sinuses; in some cases, however, there could be a coincidental occurrence of meningitis and pericerebral effusion from a distinct origin. The presence of a pericerebral effusion correlates badly with the seriousness of the clinical condition, whilst its spontaneous tendency to resorption is obvious in many cases, leading to a more conservative attitude. Fontanometry seems helpful when discussing the indication to operate. If surgery is required, it will usually be limited to a minor procedure; we perform, in such cases, an external drainage that can be transformed, if necessary, into an internal shunt.

[Progressive enlargement and thrombosis of an anterior communicating artery aneurysm. Clinical, radiological and histological study (author's transl)]. / Accroissement progressif et thrombose d'un anévrysme de la communicante antérieure. Etude clinique, radiologique et histologique.

A non operated aneurysm of the anterior communicating artery enlarged considerably over a seven years period; it finally thrombosed and behaved like a suprasellar tumor. The clinical course, the angiographic evolution and the anatomical examination allowed, in this case, to account for the increase in volume by progressive stretching of the wall, and not by incorporation of a false aneurysmal sack resulting from an encapsulated haematoma. Histologically, the wall of this giant aneurysm showed various changes : on the inner aspect, progressive thickening occurs, due to fibrous organization of mural thrombosis; in the depth, far from the nutrient sources, necrotic atheromatous like foci occur, which isolate an inner leaflet, prone to become nectrotic at a later stage. Distension of such a weakened wall may lead to several consequences; a progressive increase in volume of the aneurysm, partial ruptures with haemodissection starting from the aneurysmal lumen, or even complete rupture of the wall and subarachnoid haemorrhage. On the other hand, circulatory stasis, due to the important increase in volume, may facilitate progressive thrombosis of the aneurysm. The living character of the wall is emphasized by the coexistance of these unfavourable (degeneration, ruptures) and favourable (thrombosis, fibrous organization) features, which continuously influence the spontaneous evolution of the aneurysm.

[Papilloma of the choroid plexus of the lateral ventricle without generalized hydrocephalus]. / Papillome du plexus choroïde du ventricule latéral sans hydrocéphalie généralisée.

A benign papilloma of the choroid plexus of the left lateral ventricle is found in a five-month old infant. An important dilatation of the isolated occipital horn is observed, without generalized enlargement of the ventricles. The clinical signs are limited to a moderate macrocephaly with deviation of head and eyes to the left. The absence of global hydrocephalus is rather exceptional in such cases, where overproduction of cerebrospinal fluid, in an amount proportional to the tumoral mass, is generally admitted. Nevertheless, the literature offers no absolute proof of the role of oversecretion alone in the origin of hydrocephalus, whilst an eventual obstruction of the circulatory pathways and resorption areas of the C.S.F. must be kept in mind. The absence of hydrocephalus, in the present case, suggests that the subarachnoid spaces and arachnoid villi remained patent.

[Empyema and subdural effusion after meningitis. 2 cases of unusual location]. / Lémpyème et l'épanchement sous-duraux post-méningitiques. Deux observations à localisation rare

A 12-year old child and a 2-month old infant developed, in the wane of a purulent meningitis, the former, an infratentorial subdural empyema, the latter, a large, encapsulated, haemoorhagic, aseptic subdural effusion, in the right parieto-temporo-occipital region. In both cases, signs of intracranial hypertension dominated the clinical picture. Neuroradiological investigations permitted diagnosis and localisation of the expansive processes, whose subdural position was recognized at operation and confirmed by histopathological examination. According to the literature, purulent meningitis is a rare cause of subdural empyema, except in infants; the solely infratentorial location is also unusual. Sterile subdural effusion is a more common complication of purulent meningitis in infancy, but the unilateral posterior supratentorial location is also a peculiar feature. Subdural collections after memingitis may be aseptic and possibly haemorrhagic, or septic and purulent; these different modes of presentation correspond perhaps to different degrees or stages of subdural pathological changes in the neighbourhood of leptomeningeal infection.

[Spontaneous hematoma of the cerebellum]. / L'hématome spontané du cervelet.

Forty spontaneous cerebellar hematomas have been observed in 39 patients whose age ranged from 13 to 82 years, 26 being older than 60. Arterial hypertension is the most frequent aetiological factor. Amongst the other causes, vascular malformations, which may also be present in older people, anticoagulant therapy, metastatic tumor, sepsis were found; one case remained unsolved. Diagnosis is no longer a challenge since the introduction of C.T. scan, whilst M.R.I. may give most valuable informations concerning aetiology, especially when a vascular malformation is present. In this study, the influence of the hematoma size and of hydrocephalus on consciousness was demonstrated; development of hydrocephalus is favoured by the size and by a rather median location of the hematoma. In the future, M.R.I. should give better precisions concerning an eventual extension of the hemorrhage to the brainstem. The purpose of surgical treatment is twofold: alleviating brainstem compression and correcting hydrocephalus; yet, divergent opinions have been put forward in retrospective studies, concerning evacuation of the hematoma and drainage of hydrocephalus. In this series, direct attack doesn't seem to be indicated in hematomas less than 3.5 cm diameter, unless a causal lesion has to be removed. The ideal moment for hematoma evacuation is, finally, dictated by the clinical status and its evolution; however, the authors prefer, if possible, to delay it for at least 48 hours. Amongst the 39 patients, 14 deceased; of the 25 survivors, 19 were autonomous, 7 being nearly asymptomatic, 6 patients remained handicapped. Consciousness is a main prognostic factor, whilst coma in the first hours has a clearly unfavourable significance.

Electrophysiological evaluation of cross-face nerve graft in treatment of facial palsy.

A cross-face autogenous single graft was performed in eight patients with facial palsy. Several electrophysiological techniques have been used in a follow-up study of one to three years' duration. Clinical results are extremely disappointing. The only recovery observed could not be ascribed to the graft. The investigations and the motor unit count in the facial muscles led to the conclusion that the surgical technique employed is a failure. The orbicularis oculi muscle in particular has no chance whatsoever of being satisfactorily reinnervated. Various reasons for this poor recovery are analysed: too small a number of properly grafted axons, syncinesis, muscle degeneration and poor myelination. These observations nevertheless suggest some experimental fields which may lead to improvements in the technique to a point where it may become clinically useful.