[Lymphocytis meningitis: Listeria monocytogenes is a potential risk in a immunocompetent child]. / Méningite lymphocytaire: Listeria monocytogenes, une étiologie à ne pas négliger chez l'enfant immunocompétent.

UNLABELLED: Meningoencephalitis due to Listeria monocytogenes is a rare and serious form of brainstem infection in childhood. OBSERVATION: We report the case of a 7 year-old girl presenting lymphocytic meningitis with a high CRP level. Parenteral antibiotics combining ceftriaxone and vancomycine led initially to clinical improvement. Ten days later, secondary brainstem inflammation with hydrocephalus appeared and led to the detection of L. monocytogenes during external ventricular bypass. CONCLUSION: This observation of paediatric lymphocytic meningoencephalitis suggests a prescription of amoxicillin in association with first line antibiotics, particularly when an important inflammatory syndrome exists, immunocompetent children included.

Mutations within the MGC4607 gene cause cerebral cavernous malformations.

Cerebral cavernous malformations (CCM) are hamartomatous vascular malformations characterized by abnormally enlarged capillary cavities without intervening brain parenchyma. They cause seizures and focal neurological deficits due to cerebral hemorrhages. CCM loci have already been assigned to chromosomes 7q (CCM1), 7p (CCM2), and 3q (CCM3) and have been identified in 40%, 20%, and 40%, respectively, of families with CCM. Loss-of-function mutations have been identified in CCM1/KRIT1, the sole CCM gene identified to date. We report here the identification of MGC4607 as the CCM2 gene. We first reduced the size of the CCM2 interval from 22 cM to 7.5 cM by genetic linkage analysis. We then hypothesized that large deletions might be involved in the disorder, as already reported in other hamartomatous conditions, such as tuberous sclerosis or neurofibromatosis. We performed a high-density microsatellite genotyping of this 7.5-cM interval to search for putative null alleles in 30 unrelated families, and we identified, in 2 unrelated families, null alleles that were the result of deletions within a 350-kb interval flanked by markers D7S478 and D7S621. Additional microsatellite and single-nucleotide polymorphism genotyping showed that these two distinct deletions overlapped and that both of the two deleted the first exon of MGC4607, a known gene of unknown function. In both families, one of the two MGC4607 transcripts was not detected. We then identified eight additional point mutations within MGC4607 in eight of the remaining families. One of them led to the alteration of the initiation codon and five of them to a premature termination codon, including one nonsense, one frameshift, and three splice-site mutations. All these mutations cosegregated with the disease in the families and were not observed in 192 control chromosomes. MGC4607 is so far unrelated to any known gene family. Its implication in CCMs strongly suggests that it is a new player in vascular morphogenesis.

[Tophaceous gout of the cervical spine, causing cord compression. Case report and review of the literature]. / Compression médullaire cervicale par tophus goutteux. Revue de la littérature à propos d'un cas.

We present the case of a 74-year-old patient with long-standing gout who developed C4-C5 tophaceous gout causing cord compression. The patient had undergone 4 years earlier for a disco-osteophytic cord compression (anterior C4-C5 and C5-C6 discectomy). When admitted, the patient presented quadriparesia which had developed during the previous week in association with acute tophaceous gout on the knees, and the metarsophalangeal articulations, in the context of a bronchial infection. The diagnosis of spondylodiscitis was initially retained because of the clinical features of severe infection and the radiological data (C4-C5 cord compression, with anterior epidural lesions in MRI). The intervention allowed decompression and pathological diagnosis of tophaceous gout. Spinal gout is well-known, and very rarely responsible for cord compression: only 15 cases of cervical gout have been described in the literature. Radiological findings are not specific, and treatment is surgical in the event of medullar compression despite medical treatment.

[Dermoid cyst of the lateral ventricle associated with ethmoidal dermal sinus. Report of a case]. / Kyste dermoïde des ventricules latéraux associé à un sinus dermique ethmoïdal. A propos d'un cas.

We report a case of a ventricular dermoid cyst associated with a dermal sinus connected with the ethmoidal cells in a patient who developed rapid symptoms of raised intracranial pressure. Computed tomography showed a cystic mass in the right lateral ventricle with a hydrolipidic image in the left frontal horn of the ventricle and associated hydrocephalus. Magnetic resonance imaging showed a heterogeneous T1 hyperintense mass with a fistulous tract communicating with the ethmoid cells. A cerebrospinal fluid ventriculo-peritoneal shunt was initially established, which required further revision. A right sided transventricular approach was undertaken in a second stage, allowing resection of a dermoid cyst. Obliteration of the dermal sinus tract was obtained using pericranial duraplasty. Clinical and imaging features are discussed. The need for total resection including the tumor capsule and occlusion of the fistula are emphasized if recurrence and infection are to be prevented.

[Krouamania causing an acute subdural hematoma of arterial origin]. / Hématome sous-dural aigu d'origine artériolaire et krouamanie.

We report a case of arteriolar subdural hematoma caused by Jedba assimilated to Krouamania. We discuss the pathophysiological mechanism of subdural bleeding, the clinical symptoms, the characteristics of such hematomas and the therapeutical management.

[Isolated intramedullary cysticercosis. Case report]. / Cysticercose intramédullaire isolée. A propos d'un cas.

BACKGROUND AND PURPOSE: Cysticercosis is the most common parasitic disease affecting the central nervous system. Although it is still very rare in Europe, the frequency will increase due to the influx of immigrants from the endemic areas and increasing trips in these countries. Spinal intramedullary cysticercosis is an uncommon manifestation of neurocysticercosis. CLINICAL PRESENTATION: We report a case of pure intramedullary cysticercosis in a young white French girl, presenting as a progressive paraplegia with a cystic lesion in T4 on MRI. The diagnosis was made only after surgery by pathological examination. CONCLUSIONS: A preoperative diagnosis of spinal intramedullary cysticercosis must be suspected not only in an endemic area in the presence of multiple soft tissue calcifications and segmental lesions revealed by myelography or MRI studies, but also for all cystic lesion of central nervous system even in no endemic area. Surgery is the unique treatment which can be used for spinal intramedullary cysticercosis and with the use of the microsurgical techniques for medullar surgery the outcome is not as dismal as reported earlier.

[XII cranial nerve pseudoneurinoma due to spontaneous fibrous transformation of an aneurysm of the vertebral artery]. / Pseudoneurinome du XII par transformation fibreuse spontanée d'un anévrisme de l'artère vertébrale.

The authors report an exceptional case report of tumor like evolution of a completely thrombosed aneurysm of the right vertebral artery suggestive of neurinoma of the XII nerve. We describe CT, MRI, MR-angiography. The diagnosis has been established by pathologic study after surgical extraction. Our case demonstrate the possibility of growth of totally thrombosed aneurysms and we discuss various mechanisms.

Idiopathic granulomatous hypophysitis: clinical and imaging features.

Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery.

[Simple intraparenchymal cysts of the cerebellum. Apropos of 2 cases]. / Kystes simples intra-parenchymateux du cervelet. A propos de 2 cas.

INTRODUCTION: Cysts of the posterior fossa are common in the literature, but the simple cyst is rarely considered in the differential diagnosis of these cysts. METHOD: The cases of two female patients with clinical symptoms of expansive cerebellar lesion are described. These patients were explored by MRI. They were operated upon and an anatomico-pathological diagnosis was made. RESULTS: Both patients had an intraparenchymal cystic lesion of the cerebellum. No mural nodule and no enhancement after gadolinium injection were noted. The anatomico-pathological examination confirmed that the lesion was an intra-parenchymal simple cyst the walls of which showed no tumoral features. CONCLUSION: Although this anatomico-pathological entity has not previously been described in the literature, such rare diagnosis should be borne in mind in patients presenting with a cystic tumour of the posterior fossa. MRI is of paramount importance in the evaluation of these lesions, but the anatomico-pathological diagnosis is indispensable to exclude a possible tumour.

[Solitary oligodendroglioma of the optic nerve. Apropos of a case]. / Oligodendrogliome solitaire du nerf optique. A propos d'un cas.

Proptosis, decreased vision and oculo-motor palsies developed in the left eye of a 14-year-old girl, without neurofibromatosis signs. Computed tomographic (CT) Scans of the head and orbit showed a large fusiform expansion of the left optic nerve without intracranial involvement. Magnetic resonance imaging improved contrast resolution over CT Scanning. A neurosurgical approach was performed and the optic nerve was resected from the back of the globe to the intracranial portion. Microscopic sections demonstrated oligodendroglioma, an unusual form of optic nerve glioma.

[Epidermoid cyst of the 4th ventricle. Apropos of a case in a child and review of the literature]. / Kyste épidermoïde du 4e ventricule. A propos d'un cas chez un enfant et revue de la littérature.

A 6 year-old child was admitted for a recurrent bacterial meningitis at Proteus Morganii. She was treated with antibiotics. The C.T. Scanner showed a midline low-density lesion, with a mild annular contrast uptake in the posterior fossa. M.R.I. showed the lesion and revealed a skin-bone-dura fistula. The surgical resection was complete. It was an epidermoid tumor. Intracranial epidermoid tumors constitute about 1% of brain tumors and are considered to be congenital. Epidermoid tumors of the 4th ventricle are exceedingly rare in childhood. The authors reviewed the literature and discuss the pathogeny, the radiographic diagnosis and the treatment of these tumors.

[Role of embolization in the emergency treatment of ruptured cerebral arteriovenous malformations]. / Place de l'embolisation dans le traitement en urgence des malformations artério-veineuses cérébrales rompues.

Embolization of cerebral arterio-venous malformations (A.V.M.s) is nowadays recognized as belonging to the therapeutic panel applied to these lesions. After an hemorrhagic accident, embolization is most often performed several weeks later. We report three cases of intracerebral hematoma due to A.V.M. rupture (cortico-ventricular rolandic, lateral ventricle subependymal, cortical anterior temporal A.V.M.s). These were treated by endovascular methods, using fluid material (N.B.C.A.), in emergency or semi emergency (hours, days). This had been done for direct preoperative purposes or medical reasons (heparinization in thrombophlebitis). If, at Bicêtre, the treatment of A.V.M.s by embolization is daily performed, this type of emergent treatment is rare (3 cases on the 350 patients treated till now). No complication was reported for these three patients. The efficiency of such an "emergent procedure" is unknown and cannot be compared to similar experiences. The empiric data we obtained make us think that embolization, necessarily done without heparin, is possible and may constitute an important help to surgery without delaying it. It reduces the risk of rebleeding by targetting the treatment on angio-architectural weakness points (false aneurysms). It redistributes the arterial blood, lowers the venous hyper-pressure, improves the locoregional perfusion and finally leads to a better final neurological status.

Jugular foramen arteriovenous shunt with subarachnoid hemorrhage.

The authors report the case of a 37-year-old man with an extracerebral arteriovenous fistula at the skull base, revealed by subarachnoid and intraventricular hemorrhage. The malformation was fed by the neuromeningeal trunk of the ascending pharyngeal artery and drained into left laterobulbar veins. Embolization with bucrylate was performed and occluded totally the shunting zone. A 1-year follow-up angiogram confirmed the good stability of the result, the patient being asymptomatic. This case emphasizes the quality of results that can be obtained with bucrylate in arterioverious fistulas presenting with hemorrhage. It confirms that the external carotid artery must be studied when dealing with intracranial hemorrhage. On the other hand, magnetic resonance imaging and angiography may depict vascular abnormalities but do not always indicate the shunting area, thus the pathologic type of the malformation.

Vein of Galen aneurysmal malformations. Report of 36 cases managed between 1982 and 1988.

The authors report a series of 36 vein of Galen aneurysmal malformations (VGM) diagnosed in the paediatric (78%) and adult (22%) populations that were referred to them for therapeutic management between 1982 and 1988. The clinical signs leading to the diagnosis were variable: 36% of systemic manifestations, 22% of neurological symptoms, 17% of hydrocephaly and 11% of intracranial haemorrhage. 30 angioarchitectural analyses could be obtained and allowed to classify these VGMs into 5 different types: 44% parenchymatous AVMs, 20% mural AVFs, 30% choroidal arteriovenous fistulas, 3% dural AVFs, 7% vein of Galen varices. This series demonstrates that the paediatric population is most sensitive to shunt effect whatever its type. Systemic manifestations and hydrocephaly are the most common signs encountered in the newborn and infants; whereas neurological signs and symptoms and haemorrhage belong mostly to the adult symptomatology. Because of the poor outcome of VGMs, all authors believe that these malformations have to be treated aggressively. However, we found contra-indications to be represented by pretherapeutic demonstration of cerebral tissue damage, or uncontrollable systemic failure, thus treatment is indicated to compensate for cardiac failure previously responding (even partially) to medical treatment. Secondly, appearance of sub-cortical calcifications, resistance to medication or clinical deterioration will also lead to urgent treatment. The endovascular method represents at present the best treatment with an overall low mortality (13%) and a 0% technical morbidity in children compared to the surgical one of (91% mortality in newborns and 38% in infants). The results achieved by embolization in this series were as follows: 27% satisfactory results with complete or almost complete occlusion of AV Shunt, 53% significant clinical improvement, 7% of patients were unchanged. The authors believe fundamentally that these patients (specially those belonging to the paediatric population) have to be treated in a centre where a paediatric intensive care unit, neurological, neurosurgical and surgical neuro-angiographic departments coexist, in order to assure the best possible management of these children.

Cerebral micro arteriovenous malformations (mAVMs). Review of 13 cases.

In a personal series of 152 cerebral vascular malformation, 13 patients had small (less than 1 cm) parenchymal arteriovenous malformations (mAVMs) with small nidus or fistula and a single normal-sized feeding artery and draining vein. All 13 patients presented with intracerebral haematomas (ICHs). The average age in this group was 31 years with no sex dominance; 8 patients had no antecedent symptoms. In 11 patients the small AMV could be demonstrated angiographically, with the remaining 2 malformations evident at surgery. In addition, all these mAVMs, being superficial (95% cortical), were surgically removable with no perioperative morbidity. They were not accessible by endovascular approach. This population group narrows the concept of occult vascular lesions if high quality angiographic studies are performed. mAVMs are by nature CT and MRI occult.

MRI features of craniopharyngiomas at 1.5 Tesla. A series of 13 cases.

Thirteen patients with craniopharyngioma were explored with a 1.5 Tesla magnetic resonance imager. The results were compared with those of CT and with operative findings. The MRI signals were correlated with the biochemical composition of the cysts (proteins, lipids, iron, LDH) in 5 cases; 2 patients were studied after an intravenous injection of gadolinium DOTA. MRI proved vastly superior to CT to evaluate the spread and identify the various components of craniopharyngiomas (cysts, fleshy parts, haemorrhages), except for calcifications. Gadolinium improved the detection of fleshy parts and "active" cysts. The signals emitted by cysts were extremely variable on T1-weighted sequences. The correlation between MRI and biochemical data was complex, but it appeared that protein, lipid and iron concentrations had a major influence on cyst signals. MRI with gadolinium may be envisaged as first-line examination to improve the evaluation of spread, determine the prognosis and detect recurrences of craniopharyngiomas. CT could be reserved to difficult cases with invasion of bones and sinuses.

Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). Case report.

A 49-year-old woman presented with orthostatic hypotension. Vertebral angiography and ventriculography revealed a tumor of the left cerebellar hemisphere. Ventriculoperitoneal shunting was followed by complete disappearance of the orthostatic hypotension. The tumor was subsequently removed and microscopic study showed Lhermitte-Duclos disease. Orthostatic hypotension has been rarely reported in association with tumors of the posterior fossa except for those tumors destroying the medullary centers and interrupting the baroreceptor reflex arc. This case is of interest because the tumor was restricted to the cerebellum. The authors have found no previous case in which orthostatic hypotension was a presenting symptom of Lhermitte-Duclos disease.

Effects of extra-intracranial arterial bypass on cerebral blood flow and oxygen metabolism in humans.

Twelve patients, eleven with a carotid obstruction and one with an occlusion of the middle cerebral artery, were studied before and after a successful unilateral extra-intracranial arterial by-pass, (EIAB) using PET and the 15-0 steady-state technique to measure regional cerebral blood flow (CBF), oxygen extraction fraction and oxygen metabolic rate (CMRO2). In the whole group of patients, both CBF and CMRO2 increased significantly on both cerebral hemispheres after EIAB, returning toward control levels defined in age-matched subjects. Mean oxygen extraction fraction, on the other hand, was not affected. Individually, three different effects of EIAB emerged: 1) Alleviation of a state of long standing unilateral "misery-perfusion", as reported earlier; 2) parallel increase of CBF and CMRO2 bilaterally, which appeared due to improvement of a hemodynamic depression of metabolism, the precise mechanism of which remains obscure; 3) Complex, unexpected changes in the CBF-CMRO2 couple again resulting in increases in CMRO2. This metabolic improvement afforded by EIAB in our patients has not been reported before; it suggests that long-standing hemodynamic failure may induce a metabolic depression that is still potentially reversible by surgical revascularization.