Acute disseminated encephalomyelitis following herpes simplex encephalitis.

Acute disseminated encephalomyelitis is an acute immunomediated demyelinating disorder of the central nervous system, usually encountered in children or adolescents and characterized by multifocal neurologic deficits of rapid onset. It is often preceded by various infectious diseases or vaccination, but acute disseminated encephalomyelitis developing after herpes simplex encephalitis is rare. We report such a case in a 2-year-old girl which illustrates the importance of MRI features to consider acute disseminated encephalomyelitis in a patient who develops neurologic signs after the onset of HVS encephalitis.

[Canavan disease or N-acetyl aspartic aciduria: a case report]. / La maladie de Canavan ou acidurie N-acétyl aspartique: à propos de 1 cas.

Canavan disease or N-acetyl aspartic aciduria, is an autosomal recessive leukodystrophy characterized by spongy degeneration of brain. The disease is an inborn error of metabolism caused by aspartoacylase deficiency resulting from accumulation of N-acetyl aspartic acid in the brain. The authors report a case in a 10-month-old boy who presented with developmental delay and megalencephaly noticeable after 4 months of age. Magnetic resonance imaging of the brain showed diffuse white matter degeneration. The diagnosis of Canavan disease was confirmed by nuclear magnetic resonance spectroscopy and gas chromatography-mass spectrometry.

[Discoid menisci in children: ultrasonographic features]. / Le ménisque discoïde chez l'enfant: aspects échographiques.

OBJECTIVE: Discoid meniscus is a rare congenital pathology affecting mainly the lateral meniscus. Radiological diagnosis, initially based on arthrographic findings, is now established on well defined MRI criteria. In this study, ultrasonography (US) was the modality proposed for diagnosing discoid meniscus and compared to the normal sonographic meniscal pattern. Subjects and methods. 8 children aged from 6 to 11 years, with a symptomatology highly suggestive of meniscal pathology, were investigated by US of both knees, followed by MRI in 3 and arthroscopy in all cases. RESULTS: In all 8 cases, a meniscus was diagnosed as discoid when it no longer had its normal triangular shape, was abnormally elongated and thick and demonstrated a heterogeneous central pattern. Associated lesions (fracture, cystic degeneration) were well demonstrated on US. The lateral meniscus was involved in all 8 cases, the pathology was bilateral in 5 cases and MRI/US correlation was good in 3 cases. Arthroscopy confirmed US findings in all cases. CONCLUSION: US, a more widely available imaging modality, is a reliable technique for the diagnosis of discoid meniscus in children.

MGMT methylation assessment in glioblastoma: MS-MLPA versus human methylation 450K beadchip array and immunohistochemistry.

PURPOSE: The MGMT gene encodes a DNA repair enzyme that counteracts with chemotherapy efficiency, specifically with alkylating agents such as temozolomide (TMZ). It is well established that MGMT methylation should be screened as a predictive marker for TMZ in glioblastoma, and we thus aimed to determine a reliable and practical diagnostic method of MGMT methylation detection. PATIENTS AND METHODS: 55 glioblastomas were investigated for MGMT methylation status using methylation-specific multiplexed ligation probe amplification (MS-MLPA), illumina human methylation 450K BeadChip array (HM450 K) analysis, and compared to MGMT protein expression by immunohistochemistry (IHC) staining. The methylation status of promoter, intron and all MGMT CpG targeted sites were separately correlated to patient's survival. RESULTS: In addition to MS-MLPA and 450 K concordance, our results showed significantly higher overall survival (OS) of patients receiving TMZ and presenting MGMT methylated promoter (mean OS = 21.5 months, p = 0.046). Including all glioblastoma cases and regardless of chemotherapy, MS-MLPA showed significant survival difference between MGMT methylated and unmethylated cases (mean OS = 13, p = 0.021). CONCLUSION: We concluded that in glioblastoma, MGMT promoter methylation predicts TMZ sensitivity. This current comparative analysis leads to consider that MS-MLPA is a valuable as HM450 K array for MGMT methylation status screening.

[Biliary cystadenocarcinoma: a case report disease]. / A propos d'un cas de cystadénocarcinome biliaire.

Biliary cystadenocarcinoma is a rare tumour arising in a healthy liver. Prognosis is better than other malignant tumours of the liver. It frequently develops in a pre existing benign biliary cystadeonoma and usually occurs in middle-aged women. This tumour is difficult to diagnose because of the lack of specificity of clinical, biological and radiological features. We report the sonographic, computed tomographic and angiographic features of a case of biliary cystadenocarcinoma. A review of the literature is presented as well. The sonographic and CT findings suggestive of biliary cystadenocarcinoma are summarized. Treatment is surgical and diagnosis is confirmed at histopathology.

[Calcification of the cervical ligamentum flavum. Case report and review of the literature]. / Calcification du ligament jaune du rachis cervical. A propos d'un cas et revue de la littérature.

Calcification of the cervical ligamentum flavum is a rare entity observed exclusively in Japanese people. We report a new case in a 65-year-old man from Tunisia who presented with symptoms of cervical myelopathy with mild tetra paresis, sensory abnormalities and dysuria. Magnetic resonance imaging (MRI) showed a posterior compression of the spinal cord at C3-C4. CT-scan showed a calcification of the ligamentum flavum at level C3-C4, compressing the left postero-lateral aspect of the spinal cord. C3-C4 laminectomy was performed with removal of abnormal ligamentum flavum tissue. The postoperative course was uneventful and all symptoms resolved. Calcification of the cervical ligamentum flavum is a rare entity; the diagnosis is easy but the pathogenesis remains unclear. Literature regarding this pathology is reviewed.

[A rare cause of recurrent meningitis in children: cochleovestibular dysplasia]. / Une cause rare de méningite récurrente chez l'enfant: la dysplasie cochléovestibulaire.

Recurrent bacterial meningitis is an uncommon disease of childhood. It occurs most often in children who have an underlying predisposing disorder that can result from anatomic fistula or immunodeficiency. Cochleovestibular dysplasia is a rare malformation of the inner ear that is often associated with translabyrinthine cerebrospinal fistula and then can cause recurrent bacterial meningitis. We report an unusual case of recurrent meningitis revealing cochleovestibular dysplasia in a 9-year-old child. The malformation was confirmed by imaging and the child had surgery. The outcome was favourable with no recurrence of meningitis during the 3 years after the operation.

Adult recurrent pilocytic astrocytoma: Clinical, histopathological and molecular study.

BACKGROUND: PA is a grade I glial tumor that mostly occurs in children. However, although apparently similar to paediatric PA, adult PA presents a different clinical follow-up that could arise from specific molecular alterations. A variety of genetic alterations have been identified as diagnostic or prognostic glioma molecular markers. MATERIAL AND METHODS: We describe a right infratentorial tumor that occurred in a 58-year-old man. Neuroimaging and neuropathological examination suggested PA as an initial diagnosis. The tumor was completely resected. Unexpectedly, two years later, a rapidly growing tumor on the operative site was observed with a second location in the pineal region. Immunohistochemical reactions (IHC), Multiplex ligation probe amplification (MLPA) and fluorescence in situ hybridization (FISH) was performed in both primary and relapse tumor. RESULTS: Neuroimaging and neuropathological examinations suggested an unusual diagnosis for adult patients: a recurrent PA. Both MLPA and FISH analysis contribute to diagnostic confirmation by KIAA1549: BRAF fusion detection. Additional genetic results revealed interesting findings that justified the tumor aggressivity. CONCLUSION: Molecular analysis of adult PA cases should be routinely combined with histopathological and neuroimaging examination to further refine prognostic diagnoses.

[Computed tomography features in aorto-digestive fistula after abdominal aortic surgery. Report of two cases]. / Apport de la tomodensitométrie dans les fistules aorto-digestives compliquant la chirurgie de l'aorte abdominale. A propos de deux cas.

Aorto-enteric fistula is an uncommon late complication of aortic reconstructive surgery. Morbidity and mortality remain high despite progress in diagnosis and therapeutic procedures. We report two cases of complications of aortic interventions and present the diagnosis contribution of the CT scans. Our patients were two men aged 62 and 68 years. Both presented with abdominal pain and gastrointestinal bleeding. Endoscopy and CT scan with contrast enhancement were performed providing the diagnosis of aorto-enteric fistula before surgery. Both patients died after surgery. The fundamental principle in the management of late complications of abdominal aortic surgery is early and aggressive surgery. Cross sectional imaging and particularly CT scan plays an important role in diagnosis and in determining the extent of these complications. CT must be performed for life-long follow-up of these patients to allow assessment of early diagnosis of aortic reconstruction surgery complications.

[Neonatal ultrasonic detection of medullary nephrocalcinosis associated with Butler-Albright distal tubular acidosis. Apropos of a case]. / Détection échographique néonatale de la néphrocalcinose médullaire associée à l'acidose tubulaire distale de Butler-Albright. A propos d'une observation.

Butler-Albright's distal tubular acidosis was confirmed by ultrasound imaging in the neonatal period, image characteristics showing persistent medullary hyperechogenicity. This case is remarkable by the very early-stage diagnosis of the nephrocalcinosis.

[Aicardi syndrome with favorable outcome]. / Syndrome d'Aicardi d'évolution favorable.

BACKGROUND: Aicardi syndrome is usually associated with a very poor outcome. CASE REPORT: We report a particular case of Aicardi syndrome characterised by a partial agenesis of the corpus callosum associated with an arachnoidal interhemispheric cystic formation, and by a complete control of epilepsy and a favorable outcome regarding visual and psychomotor performances. CONCLUSION: A better prognosis could be expected in Aicardi syndrome when spasms occur lately and are not preceded by partial seizures, when agenesis of corpus callosum is partial and especially when chorioretinal lacunae are small, isolated, few and spare the macula.

[Tuberculous mesenteric lymphadenitis: a case report]. / La lymphadénite mésentérique d'origine tuberculeuse: à propos d'un cas.

The authors report a case of mesenteric tuberculous lymphadenitis which mimic a pancreatic cystadenoma. They emphasize ultrasound and CT scan features which lead to the recognition of tuberculosis and permit a percutaneous fine needle biopsy. Laparotomy seems the most reliable method for a positive diagnosis. The place of different imaging methods is discussed.

[Bone anomalies in von Recklinghausen disease]. / Anomalies osseuses dans la maladie de Von Recklinghausen.

28 cases of neurofibromatosis are reported. Principals bone anomalies are reviewed. The skeletal manifestations are numerous and varied. These anomalies interest specially cranial vault, spheno-orbital area and vertebral spine. It is imperative to keep in mind skeletal manifestations because neurologic and cutaneous signs can be absent.

[Subcapsular hematoma complicating acute pyelonephritis]. / Hématome sous-capsulaire compliquant une pyélonéphrite aiguë.

Spontaneous subcapsular renal hematoma is rare and essentially associated with malignant, or benign tumor vascular diseases, inflammatory processes. In few cases, no cause was detected. We observed one additional case in a diabetic women. She presented fever, and bilateral flank pain. Ultrasonography showed bilateral subcapsular hematoma. Computed tomography confirmed these findings and demonstrated multiple area of acute focal pyelonephritis.

[Primary lymphoma of bone: imaging features]. / Lymphomes osseux primitifs: apport de l'imagerie médicale.

Primary lymphoma of bone (PLB) has benefited from recent advances in medical imaging and immunohistochemical study that are of prognostic and therapeutic value. From 3 case reports of PLB involving the iliac bone and the skull base, we review the definition of PLB and their main features. The importance of complete initial workup for staging in order to exclude secondary bone lymphomas, more frequent and of worse prognosis, is emphasized. Diagnosis of PLB involving the skull base is more difficult to obtain and nearly always requires neurosurgical biopsy unlike PLB of peripheral bones that are more readily amenable to percutaneous biopsy.

[Pyogenic abscess of the psoas]. / Les abcès à pyogènes du psoas.

11 cases (7 primary and 4 secondary) of pyogenic psoas abscesses are reported. In all cases Staphylococcus aureus was isolated on blood culture or pus drained under ultra-sonographic control. US is very useful for diagnosis and the follow up of psoas pyogenic abscesses. Percutaneous aspiration and drainage of psoas abscesses under ultrasonographic control combined to antibiotherapy is an effective therapeutic procedure. Our experience suggests that antibiotherapy alone is an effective therapy of presuppurative pyogenic psoas abscesses and even in collected and non complicated primary psoas abscesses.

[Radioclinical profile of cardiopericardial hydatid: report of 17 cases]. / Profil radioclinique du kyste hydatique cardiopéricardique: à propos de 17 cas.

Hydatid cysts concerning the heart are rare, accounting for 0.5 to 2% of all hydatic sites. The risk of serious complications in this location makes rapid diagnosis and surgical treatment essential. The aim of our study is to clarify the role of imaging in the diagnosis of the disease and to propose an adequate strategy. We report on 17 patients who underwent surgery for cardiopericardial hydatid cyst in the cardiovascular and thoracic unit of Sahloul hospital in Sousse from January 1988 to December 1998. Clinical investigation included in all cases chest X ray, ECG and ultrasonography (US). A computed tomography (CT) scan was performed in 14 cases, magnetic resonance imaging in three cases, transesophageal US in five cases, and coronary angiography in fsix cases. Examination for other hydatic sites was realized in all cases, and brain CT was performed in four cases. The hydatid cyst was variably localized in the left ventricle wall (five cases), the right ventricle (five cases), the pericardium (five cases), the interventricular septum (four cases), the right auricle (one case) and the left auricle (one case). The existence of other cardiac hydatid sites was found in 12 cases. All patients underwent surgery. Outcome was favorable in 14 cases, with a mean of 3 years and 5 months survey. Three patients died. The combination US-CT scan allowed a precise topographical inventory, reducing the need for MRI to the complicated cases and to the rare cases of inconclusive results by US-CT scan.

[Primary cardiac sarcoma: report of 3 cases and review of the literature]. / Les sarcomes primitifs du coeur: revue de la littérature, illustrée par 3 cas.

Primary cardiac sarcoma is a rare tumor with a poor prognosis. We report 3 cases with a review of literature about this disease. There were 2 males and 1 woman. The main symptoms were thoracic pain. The clinical features were various and the thoracic ultra sonography exam allowed the diagnosis in the 3 cases. All patients had surgical remove of their cardiac tumor followed by chemotherapy. All of them died within 13 to 36 months after the diagnosis. Primary cardiac sarcoma has a poor prognosis with a mean survival less than 12 months.

[Giant lobar emphysema. The cause of a compressive hemithoracic opacity in a newborn infant]. / Emphysème lobaire géant. Cause d'une opacité hémithoracique compressive chez le nouveau-né.

The authors describe a case of congenital lobar emphysema of the upper right pulmonary lobe discovered at birth with an opaque hemithorax and a shift of mediastinum. The diagnosis was confirmed by a CT-scan. A lobectomy was performed at 6 weeks of age. The different diagnosis and physiopathological mechanisms are discussed.

[Purulent retention in the upper urinary tract and pregnancy. Apropos of 3 cases]. / Rétention purulente dans la voie excrétrice supérieure et grossesse. A propos de trois cas.

The purulent retention of the upper urinary tract is fortunately a rare urologic complication for the pregnant women. It decompensates a congenital or acquired uropathy. It is an important urologic emergency in which the diagnosis of obstruction, and the drainage of urines must be of immediate necessity as important as antibiotherapy, to avoid pyonephrosis and the destruction of the kidney. Concerning 3 cases, treated in the stage of pyonephrosis in the department of urology in Susa, we emphasize a premature diagnosis, which can only be done through an ultrasonography, the only guarantee to preserve the kidney. When possible, percutaneous nephrostomy constitutes an excellent means of drainage, while expecting a final disappearance of the obstruction.