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Cerebrotendinous xanthomatosis is a rare lipid storage disorder due tocaused by an autosomal recessive inherited defect of the hepatic mitochondrial sterol 27 hydroxylase. It's characterized by accumulation of cholestanol and cholesterol in many tissues, in particular tendons and brain, with tendon xanthomas, juvenile cataracts, and neurological abnormalities. MR imaging showed typical bilateral and symmetrical involvement of the dentate nuclei. Early and long- term treatment may improve neurologic function. The authors present a case of cerebrotendinous xanthomatosis and describe ultrasound, computed tomography, and magnetic resonance findings.

Resume Nous rapportons un nouveau cas de meningiome agressif sans attache durale chez une enfant âgee de trois ans. Nous soulignons la rarete de ces tumeurs chez l'enfant et l'absence de predominance feminine, et nous insistons sur certains aspects particuliers, anatomopathologiques et radiologiques, des meningiomes.

Cavernous angioma or cavernoma is a vascular malformation that may affect any area in the neuraxis. Epidural location is very rare and therefore seldom considered in the differential diagnosis of spinal cord compression. We report two cases of epidural cavernous angiomas. The first case is a solitary and purely epidural dorsal cavernous angioma without foraminal expansion or bone modification causing spinal cord compression in a 35 year old woman. The second case is a solitary epidural dorsal cavernous angioma with foraminal extension causing spinal cord compression in a 56 year old woman. Histological confirmation is available for both cases. We describe the MRI features of this lesion insisting on its differential diagnosis on imaging.

Resume Le syndrome de Dyggve-Melchior-Clausen (SDMC) est une pathologie osseuse rare, appartenant au groupe des dysplasies spondylo-epimetaphysaires. Cliniquement, le SDMC se caracterise par un nanisme progressif et un retard mental de severite variable. Les caracteristiques radiologiques consistent en une platyspondylie, une dysplasie epiphysometaphysaire et un aspect festonne des ailes iliaques. Nous rapportons un cas de SDMC chez un enfant de neuf ans.

Resume Introduction La sclerose concentrique de Balo est une forme particuliere aigue de sclerose en plaques decrite par Balo en 1928. Ce terme resulte de l’aspect concentrique des lesions avec alternance de zones de demyelinisation et de zones de myeline normale. Observation Nous rapportons l’observation d’un patient nord-africain porteur d’une sclerose concentrique de Balo diagnostiquee par imagerie par resonance magnetique. Les localisations etaient a la fois encephaliques et medullaires et l’evolution clinique favorable apres traitement medical. Conclusion Autrefois fait en post-mortem, le diagnostic de la sclerose concentrique de Balo repose actuellement sur l’imagerie par resonance magnetique qui permet un diagnostic precoce et une prise en charge rapide de cette affection.

Resume Les kystes epidermoides intracrâniens sont des tumeurs relativement rares qui se caracterisent generalement a l’imagerie par une masse tumorale de densite et de signal proches de celui du LCS. Les kystes epidermoides denses sont exceptionnellement rapportes dans la litterature. L’observation d’une patiente de 12 ans, presentant un kyste epidermoide spontanement hyperdense de la fosse cerebrale posterieure, responsable de signes neurologiques focaux est rapportee. Le diagnostic a ete confirme a l’examen anatomo-pathologique.

Cerebrotendinous xanthomatosis is a rare lipid storage disorder due tocaused by an autosomal recessive inherited defect of the hepatic mitochondrial sterol 27 hydroxylase. It's characterized by accumulation of cholestanol and cholesterol in many tissues, in particular tendons and brain, with tendon xanthomas, juvenile cataracts, and neurological abnormalities. MR imaging showed typical bilateral and symmetrical involvement of the dentate nuclei. Early and long- term treatment may improve neurologic function. The authors present a case of cerebrotendinous xanthomatosis and describe ultrasound, computed tomography, and magnetic resonance findings.

We report two cases of cerebral deep venous thrombosis in female adults. The first patient presented with headaches and confusion 20 days postpartum. Deep cerebral internal venous thrombosis was suspected based on the CT scan showing bilateral hypodensity in the thalamus and internal capsules. The diagnosis was confirmed on cerebral angiography. The patient was treated but died within 3 weeks of the onset of symptoms. The second patient had no obvious predisposing factors. She presented with confusion and fever. The diagnosis was strongly suggested by the CT scan, which showed spontaneous high density in the deep venous system associated with bilateral hypodensities in the thalamus and internal capsules. Venous MR angiography revealed obliteration of internal cerebral veins and the great vein of Galen. Ten days after appropriate therapy, the patient recovered completely.

The nuclear syndrome of the oculomotor nerve, first described in 1981, is characterized by ipsilateral third nerve palsy and elevation paresis of the contralateral eye.A 59-year-old man suddenly developed diplopia and headache. Neurological examination showed nuclear ophthalmoplegia of the left third nerve without any other neurological abnormality. MRI findings demonstrated a typical, popcorn-like, smoothly circumscribed left posterior lesion. Surgical resection was not performed because of the deep localization. Spontaneous bleeding developed.This neuro-ophthalmological complication of a cavernous angioma, as seen in our patient, has rarely been described in the literature. The clinical presentation and anatomic elements of the nuclear oculomotor syndrome are discussed. This exceptional localization provides a useful illustration of anatomoclinical correlations.

We report a case of sphenoidal plasmocytoma in a 57-year-old male revealing multiple myeloma. MRI showed a tumor located in the sphenoid sinus with local extension. Diagnosis was made by histology after transsphenoidal resection. We discuss the imaging features of plasmocytomas and review the principal differential diagnoses.

Balo's concentric sclerosis is a rare variant of multiple sclerosis described by Balo in 1928. It is characterized by alternating rings of demyelination and spared myelin.We report a case of Balo's concentric sclerosis diagnosed by the typical MRI findings of concentric rings of demyelination. Medullar and brain localisation were found and clinical course was good under intravenous corticosteroids.MRI provides the best diagnostic information for Balo's concentric sclerosis, allowing early diagnosis and treatment.

Intracranial epidermoid cysts are relatively rare tumors. These cysts are generally nearly isodense to CSF at CT and nearly isointense to CSF on T1W and T2W MR imaging. Spontaneously hyperdense epidermoid cysts are exceptional. We report the case of a 12-year-old girl with a spontaneously hyperdense epidermoid cyst of the posterior fossa presenting with focal neurological symptoms. The diagnosis was confirmed at histological examination.

Idiopathic granulomatous hypophysitis is a chronic inflammation of the pituitary gland. It is a rare occurrence and is diagnosed only on pathologic examination. We report a case of a 23-year-old female patient presenting with polyuria and polydipsia associated with a pituitary failure and hyperprolactinemia. MRI study showed a space occupying lesion of the pituitary gland with a marked enhancement after gadolinium injection. Pituitary biopsy was consistent with the diagnosis of idiopathic granulomatous hypophysitis. The aim of this work is to discuss the different clinical and radiological aspects of the disease with a special emphasis on differential diagnosis.

We report radiological features of a biopsy-proven early infantile form of Nasu-Hakola disease in two Tunisian sisters with new bony and cerebral findings.

Resume Introduction Le syndrome nucleaire du nerf oculomoteur commun, initialement decrit en 1981, est defini par l’association d’une atteinte partielle ou complete du III ipsilateral a la lesion et d’une paralysie controlaterale du muscle droit superieur. Nous rapportons le cas d’un cavernome mesencephalique revele par un syndrome nucleaire isole du III. Observation Un patient, âge de 59 ans, fut hospitalise en octobre 2003 pour l’apparition brutale, trois jours avant l’admission, d’une diplopie gauche et de cephalees intenses. L’examen montra une atteinte complete intrinseque et extrinseque du nerf oculomoteur commun gauche associee a une paralysie complete de l’elevation du regard vers le haut de l’œil droit. L’IRM encephalique revela l’existence d’une lesion pedonculaire gauche en hypersignal T1, T2 et IR T2, associee a un hyposignal T2 en anneau evoquant un cavernome. Aucune resection chirurgicale n’a ete faite en raison de la localisation profonde de la lesion. L’evolution a ete marquee par l’apparition trois semaines plus tard d’une hemorragie spontanee et d’un coma. Conclusion Cette complication neuro-ophthalmologique d’un cavernome a ete rarement rapportee dans la litterature. Les caracteristiques semiologiques en fonction de la localisation anatomique seront discutees dans cet article.

Objectifs Dans ce travail, realise sous forme d’un kit radiologique et concu sur un support informatique multimedia interactif, nous avons d’une part rappele la radio-anatomie normale du lobe temporal et d’autre part decrit la semiologie en imagerie IRM des differentes lesions pouvant entrainer ce type d’epilepsie. Nous proposons au lecteur un quiz compose de 22 cas cliniques lui permettant de tester ses connaissances. Materiels et methodes Vingt-deux patients explores par une imagerie par resonance magnetique completee, pour certains, par une angiographie cerebrale. Resultats L’analyse semiologique, confrontee aux donnees cliniques a permis de faire le diagnostic positif de la lesion et de preciser son siege exact et son extension. De plus l’analyse du signal des differentes composantes de la lesion permet l’approche de sa nature. Cette analyse doit etre complete a la recherche d’anomalies associees, encephaliques extra-temporales. Cette etude permet de definir la meilleure attitude therapeutique, le type d’intervention et l’etendue de la resection, si un geste chirurgical est envisage. Une fois la lesion traitee, l’IRM reste la technique de choix pour evaluer l’etat postoperatoire. Conclusion L’IRM constitue l’examen de reference dans l’exploration de l’epilepsie temporale. Pour cela, elle necessite une bonne connaissance de la radio-anatomie normale de cette region et la realisation d’un protocole rigoureux.

The authors reports a case of malignant schwannoma of sciatic nerve. Clinically, this tumor was manifested by a progressive tumefaction of the leg, and moderate pain, without functional signs. The origin of the sciatic nerve has been suspected by computed tomography, because its location, the vascular contacts, and the lesion's extension. Biopsy leads to confirm the nerve tumor, and precised the histologic type: malignant schwannoma. Local, regional and general extension did not premise treatment.

The authors report a retrospective study of 50 CT Scan made for patients suffering from Behçet's disease with neurological manifestations. Neuro-Behçet can affect all the central nervous system structures with particular predilection to the brain stem, internal capsula and basal brain ganglia. The authors emphasize on the importance of CT Scan for the diagnosis, the severity evaluation of the neurological impairment and for the Neuro-Behcet's prognosis.

A case of intralobar pulmonary sequestration in an infant is reported. The lesion presented as recurrent bronchopulmonary infections. The chest film showed a density in the right lower lobe, in which air-filled images and air-fluid levels developed rapidly. Aortography demonstrated systemic blood supply to the lesion from three arteries stemming from the thoracic aorta. The pathological study of the operative specimen confirmed the diagnosis. Postoperative outcome was satisfactory.

Spinal osteoid osteomas are rare. We report 21 cases, that have been studied and treated since 1985. The tumor affects young people, who are less than 25 years old. The pain is the most common sign. Diagnosis is difficult, but can be made by radiology. X ray sometimes enables to suspect the diagnosis. Scintigraphy shows a focus hyperfixation more suggestive. Computed tomography enables the diagnosis by showing a bony lacuna surrounded by osteosclerosis. Moreover, computed tomography localizes very well the nidus of the osteoid osteoma, and guides the surgical treatment. Since computed tomography, other techniques like angiography and tomography are less used for diagnosis.

Lipoma of the corpus callosum is a rare congenital intracranial tumor. Although only 153 cases had been reported as of March 1992, the incidence of this tumor is underestimated (some cases are unpublished and others are asymptomatic). Three cases of lipoma of the corpus callosum diagnosed in children are reported herein. These tumors may be either discovered fortuitously in an asymptomatic patient or responsible for manifestations of which the most common are seizures, headache, and mental disorders. Diagnosis rests on imaging techniques. The main investigation is computed tomography which discloses a mass composed of fat, with or without peripheral calcifications, and also identifies any other malformations such as dysgenesis of the corpus callosum.

We report a case of double primary amoebic brain abscess in a 22 year old man, with signs of intracranial hypertension and deficit. Computed tomography found two hypodense lesions, not suggestive of pyogenic abscesses. Aspiration of one of the two abscesses, revealed the organism, and the two abscesses regressed in response to medical treatment. The authors insist on the rarity of these abscesses, their gravity, and the difficulty of the diagnosis.

The authors report 5 cases of cerebral miliary tuberculosis studied by computerized tomography and review 5 cases of the literature. Computerized tomography can see very small tuberculomas, of several millimeters. Chest miliary tuberculosis is found in all their cases, and 3 of the 5 cases of the other authors. Because of the discretion of neurological signs and the association with chest miliary, we suggest to make a computed tomography of brain of all patients who have chest miliary tuberculosis despite the absence of neurological signs and we think that we will find out other cases of brain miliary tuberculosis.

The authors report 3 cases of skull base chordomas. In 2 cases, skull radiographies and computed tomography found lytic lesions of sphenoid and clivus, with calcifications into the tumors. In the third case, radiological findings suggest a naso pharynx tumor.

The authors report about 3 new cases of cutaneous-mucosal hyalinosis, or Urbach-Wiethe disease, with cerebral sites. The intracranial calcifications were detected without any neurological presenting features, and were discovered incidentally on radiographs of the skull. The CT examination allows specifying the site of these calcifications, in the gyri of the hippocampus.

We report two cases of trigeminal neuroma in patients with Von Recklinghausen neurofibromatosis (NF1). Despite the high frequency of intracranial neuroma in neurofibromatosis, the localization on the fifth cranial nerve is an uncommon findings. In our two cases, neuromas were asymptomatic clinically and electrophysiologically, but computed tomography showed a small nodular lesion in the cerebellopontine cistern, independent of the seventh and eight nerve and which can only correspond to the fifth nerve. MR imaging very clearly demonstrates intracranial neuromas of all cranial nerves.

Biballism is an infrequent hyperkinetic disorder characterized by involuntary, intermittent, violent, uncontrollable contractions of the proximal muscles of the limbs. Biballism is classically ascribed to a lesion in the controlateral subthalamic nucleus or its connections but other causes have been reported. These include infections (bacterial, viral parasitic), cerebrovascular lesions, tumors, toxics, and systemic disease (systemic lupus erythematosus). Although poorly understood, the pathophysiology of hemiballism is widely believed to involve hyperactivity of the dopaminergic system. The prognosis of these abnormal movements, formerly poor, has been improved by the use of neuroleptics and drugs acting on the different neurotransmitter systems. A unique case of biballism at resolution of a febrile coma in a 4 1/2 year old is reported. The EEG showed diffuse slow waves. A hyperdense lesion was visible in the right thalamic region on the cerebral CT scan. The magnitude of the abnormal movements decreased under haloperidol. The etiology of this case of biballism is discussed.

Objectifs Souligner la richesse pathologique a l’origine de la nevralgie symptomatique du trijumeau et preciser le role de l’imagerie en particulier celui de l’IRM dans le diagnostic etiologique de cette nevralgie. Materiels et methodes Patients presentant des nevralgies du V resistantes au traitement medical, isolees ou associees a d’autres atteintes neurologiques. Ces patients ont ete explores par une IRM et/ou une tomodensitometrie. Resultats Les lesions retrouvees sont variees : tumorales, vasculaires (conflits vasculo-nerveux, malformations anevrysmales, lesions ischemiques), inflammatoires et infectieuses. Elles interessent les differentes portions anatomiques du nerf trijumeau. Conclusion Le nerf trijumeau peut etre touche en differents points de son trajet depuis le tronc cerebral jusqu’aux branches de division. La possibilite d’une etude multiplanaire en haute resolution et la diversite des differentes lesions font de l’IRM l’examen de choix pour le diagnostic precis des differentes lesions pouvant toucher le V dont certaines exclusivement revelees par cette technique.