[Case No. 4. Ossifying fibroma of the ethmoid].
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Biomedical subjects
Publications and source records attributed to J P Cottier.
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Torticollis in children must always instigate a search for trauma. Many other etiologies can be found. The authors report a case of postmedicinal atlantoaxial rotatory pseudoluxation (AARP) occurring in a child. This child had fallen down in the morning with head trauma followed by headache. Clinically, a stiff neck with an irreducible right rotation of his neck, and an osteotendinous hyperreflexia were noted. There was a C1-C2 rotatory dislocation with no traumatic lesion on the cervical CT scan. After a few hours, the torticollis spontaneously reduced and then reappeared on the left side. This clinical fluctuation and the absorption of metoclopramide (Primpéran) started in the morning for acute gastroenteritis provided the diagnosis of AARP. This entity was confirmed by the good clinical and radiological follow-up and was caused by the substantial ligament laxity of the craniovertebral junction encountered in children. The analysis of medical imaging and the systematic search for a medicinal cause helped make the right diagnosis.
Malformations of cortical development are increasingly recognized as important causes of epilepsy, developmental delay and other neurological disorders. Our purpose is to present the relevance of the MRI in these pathologies with the clinical, genetic and therapeutic aspects. This classification is based on the three fundamental events of cortical formation: proliferation of neurons and glie in the periventricular zone, migration of postmitotic neurons to the periphery, subsequent cortical organization. MR analysis evaluates particularly the cortical thickness, sulcal and cortical morphology, gray-white matter junction, and looks for gray matter in abnormal location. These data coupled with the familial history, the seizure characteristics and genetic findings should allow an appropriate classification of the lesions. MR imaging allows the detection and classification of cortical malformations. MR imaging findings are primordial to consider surgery when the epilepsy becomes refractory to the anti-epileptic drugs. An adequate classification of these malformations should help to provide to the family an appropriate counseling both in terms of genetics and outcome.
We report the case of a patient with streptococcal mitral endocarditis discovered following an ischaemic cerebrovascular accident. The clinical evolution was marked by the progressive development of a mycotic aneurysm. Surveillance was performed with repeated angio-MRI which provided an indication for embolisation. We demonstrate the value and the current quality of angio-MRI for the diagnosis of mycotic aneurysms, and the value of active management of these lesions which present a high risk of rupture and which are associated with a significant excess mortality. Embolisation can allow earlier cardiac surgery for haemodynamically unstable patients and for those whose neurological state until now contra-indicated intervention.
Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment.
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Cerebrospinal fluid (CSF) fistulae can produce leakage through a defect in the bony skull and meninges into the contiguous air-filled cavities at the base of the skull. The major risk is central nervous system infection. When abundant clear rhinorrhea or otorrhea is present, the diagnosis is obvious and imaging is used to localize the fistula. Computed tomography (CT) with millimetric slices and magnetic resonance imaging (MRI) are the most effective diagnostic tools. CT cisternography, an invasive procedure, should only be used when the diagnosis remains uncertain following CT scan and MRI. When CSF leakage is sparse or intermittent, the diagnosis can be made by measuring beta-2 transferrine in the escaping fluid. CT scan followed by MRI are also useful for making the diagnosis and locating the fistula when exterior leakage is absent. CT scan alone is effective for assessing isolated otorrhea. If the diagnosis remains uncertain after all these studies have been used, the patient should be closely followed clinically and isotopic study or surgery should be considered.
PURPOSE: To evaluate the efficacy of percutaneous sclerotherapy for the treatment of venous malformations (VMs) with regards to cosmetic and functional outcome as a function of their size and to review the complications. MATERIALS AND METHODS: A retrospective study was performed between January 1997 and January 2002 on 68 patients (45 females and 23 males) ranging in age from 3 to 60 Years at the CHRU of Tours. RESULTS: Percutaneous sclerotherapy was a very effective treatment for small and medium-size VMs, for which the aim was to achieve cure. Aetoxisclerol and Ethibloc are the sclerosing agents used. They were associated with minimal side effects and no major complication. For larger lesions, the treatment was more complex and combined stronger and also more dangerous agents like absolute ethanol and Histoacryl. The aim was then a decrease of cosmetic and functional problems. CONCLUSION: Percutaneous sclerotherapy with Aetoxisclerol, Ethibloc, absolute ethanol or Histoacryl, either alone or before surgery, is a safe and effective method of managing soft-tIssue venous malformations.
Ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle disorder. This condition usually presents in neonates or children. This report describes the clinical case of a 21 year old woman who was diagnosed in adulthood during the course of an unexplained coma. After recovery from the coma, she presented very unusual neuropsychological disorders involving memory and the meaning of certain words, suggesting a semantic deficit. The discovery of OTCD in adulthood is rare and the neuropsychological consequences may be unique.
All patients with aneurysms treated with Guglielmi detachable coils (GDC) are undergo angiography to assess long-term stability of aneurysm exclusion or to show recurrence of the aneurysm sac, which may require further treatment. We prospectively compared the plain-film appearance of the coil-mass, 3D time-of-flight MR angiography (TOF MRA) and digital subtraction angiography (DSA) for the detection of aneurysm recanalisation during follow-up. We studied 60 patients with 74 intracranial aneurysms treated with Guglielmi detachable coils. We used the unsubtracted image of the angiograms performed at the completion of any embolisation procedure and at follow-up as the plain radiographs. Recanalisation was considered if loosening, compaction or reorientation of the coil mass was apparent. TOF MRA was performed to assess the presence and size of a neck remnant. DSA was regarded as the definitive investigation. Comparison of the techniques showed good agreement as regards aneurysm recanalisation. MRA was more accurate than plain radiography and could replace DSA for long term follow- up. The initial follow-up examination should, however, include both modalities. In cases of contraindications or limitations to MRA, the interval between follow-up angiographic examinations could be increased if there is no change in the plain-film coil-mass appearances.
BACKGROUND: Selective embolization is an effective and reputedly safe method of managing pregnancy-related bleeding. However, we report an ischemic uterine necrosis after arterial embolization. CASE: The patient had heavy postpartum bleeding treated by embolization of the uterine arteries using polyvinyl alcohol particles (diameter 150-250 and 300-600 microm) and gelatin sponge pledgets. Her postoperative recovery was complicated by menorrhagia and pelvic pain. Because of the persistent menorrhagia and risk of infection, a hysterectomy was performed. Histopathology of the hysterectomy specimen revealed massive ischemic myometrial necrosis. CONCLUSION: This complication is most likely related to the small size of the particles used. In the management of postpartum bleeding by arterial embolization, the material of choice is gelatin sponge pledgets, and the use of small particles should be avoided.
OBJECTIVES: To evaluate sonographic features following uterine artery embolization and to assess using ultrasound the efficacy of embolization as the primary treatment of fibroids. DESIGN: Fifty-eight women (mean age, 44.5 years; range, 33-65 years) suffering from symptoms due to fibroids (menometrorrhagia, bulk-related symptoms, pelvic pain) were followed-up after uterine artery embolization by ultrasound examination at 3 months, 6 months, 1 year and 2 years with assessment of volume and vascularization of fibroids as well as uterine vascularization. RESULTS: Fifty-eight patients were examined at 3 months, 46 at 6 months, 36 at 1 year and 19 at 2 years. Most patients were improved or free of symptoms at 3 months (90%), 6 months (92%) and 1 year (87%) and all monitored patients were free of symptoms at 2 years. Clinical failure of treatment occurred in only two cases (3%). Progressive significant reduction in fibroid size with reference to the baseline was demonstrated during follow-up from 3 months (-29%) to 24 months (-86%). Absence of intrafibroid vessels was observed in all except three cases as early as 3 months, whereas perifibroid vessels persisted in 21 cases. No changes in uterine vascularization or uterine artery resistance were noted. CONCLUSIONS: Uterine artery embolization is a valuable endovascular method for the treatment of fibroids, resulting in marked reduction in fibroid size and disappearance of intrafibroid vessels without reduction in uterine vascularization which is well depicted by sonography.
We studied the morphometric MRI findings and their correlation with cognitive functions in a population of 10 young adults shunted for congenital hydrocephalus related to spina bifida. Morphometric MRI analysis included measurement of the ventricular dilatation index, frontal and occipital parenchymal thickness and the size of the corpus callosum. The neuropsychological status was evaluated, notably to look for a discrepancy between verbal and performance skills, a finding which has previously been described in hydrocephalic children. We also investigated whether there was a correlation between cognitive function and cerebral morphometric indexes. In each case, MRI demonstrated the structural changes associated with the Chiari II malformation. The size of the lateral ventricles varied, ranging from important dilatation to small ventricles. Six patients had only partial development of the corpus callosum. All patients had a normal global IQ. In our population of young adults, we did not observe any discrepancy between verbal and visuospatial performances as has been described in children with hydrocephalus. We found no relationship between cognitive function and ventricle dilatation or parenchymal thickness or between the size of the corpus callosum and callosal transfer.
123I-Iodolisuride has high specific affinity for binding on dopamine D2 receptors in the striatum and has been used in a few single photon emission computed tomography (SPECT) studies of extrapyramidal disorders. The diagnosis of Parkinson's disease (PD) is very difficult in the first 5 years of evolution, with 15-25% false positive diagnoses. The aim of this study was therefore to determine the value of iodolisuride SPECT in discriminating Parkinson's from the most frequent Parkinson-plus syndromes (PPS). Seventeen patients with an extrapyramidal syndrome had a SPECT examination 1 h after injection of 180-185 MBq of 123I-iodolisuride. They were followed under dopaminergic treatment for at least 2 years. After 2 years, they were separated in two groups according to specific clinical criteria and sensitivity to dopaminergic treatment: nine patients had PD (age = 59.8+/-8.8 years; Hoehn and Yahr = 1.8+/-0.7; evolution = 4.3+/-3 years) and eight had PPS (age = 71.6+/-7.3 years; Hoehn and Yahr = 2.9+/-2.0; evolution = 4.1+/-1.5 years). The binding potential of iodolisuride in the striatum was assessed by considering the striatum (S)/occipital lobe (O) ratio at the pseudo-equilibrium 1 h after injection. The S/O ratio was statistically different between PD and PPS (1.97+/-0.3 vs. 1.65+/-0.2 (P<0.02)). Iodolisuride SPECT could differentiate both groups with a sensitivity of 88.8% and a specificity of 75%. Iodolisuride is a good specific D2 receptor ligand for SPECT and complements specific clinical criteria for the diagnosis of Parkinson's disease and differentiation between different extrapyramidal disorders.
BACKGROUND AND PURPOSE: The management of wide-necked aneurysms or aneurysms with a neck-to-body ratio close to 1 is a difficult challenge for the interventional radiologist because of the risk of coil migration or coil protrusion into the parent vessel. Our objective was to evaluate the efficacy and safety of balloon-assisted coiling as well as the follow-up results of occlusion for those difficult aneurysms in which conventional treatment with Guglielmi detachable coils (GDCs) had failed. METHODS: A nondetachable balloon was used in 49 procedures performed in 44 patients (35 women and nine men) who underwent GDC coiling of aneurysms. Every aneurysm had either a wide neck or a sac diameter/neck size ratio (SNR) of 1.5 or less. RESULTS: In four (8%) of the procedures, balloon placement failed, leaving a total of 45 aneurysms treated with balloon-assisted coiling. Final results consisted of total occlusion in 30 cases (67%), subtotal occlusion in 11 cases (24%), and incomplete occlusion in four cases (9%). We found a correlation between the diameter of the sac and the occlusion rate, but not between the size of the neck or the SNR and the occlusion rate. Two thromboembolic complications occurred, but neither had clinical consequences. No aneurysmal rupture was observed during treatment. Final angiographic follow-up time ranged from 3 months to 5 years (mean, 16 months). CONCLUSION: Balloon-assisted coiling is an important adjunct in the treatment of aneurysms with a wide neck or low SNR. In our experience, this technique allowed safe and efficient treatment of aneurysms when conventional GDC treatment had failed.
BACKGROUND: Cognitive disorders have been described in amyotrophic lateral sclerosis, but usually after the diagnosis has ben established. CASE REPORT: A 57-year-old man was intubated for acute respiratory distress subsequent to pneumonia and diaphragm palsy. He had a 2-year history of drug-resistant depression and deterioration of cognitive functions. A pyramidal syndrome associated with biopsy-proven chronic neurogenic atrophy led to the diagnosis of amyotrophic lateral sclerosis. The electromyogram did not contribute to diagnosis. Brain MRI only evidenced moderate bilateral frontal-temporal atrophy. Brain SPECT demonstrated major perfusion defects in the frontal lobes. DISCUSSION: This patient had amyotrophic lateral sclerosis and frontal-temporal dementia with an unusually late onset clinical presentation: cognitive disorder was the inaugural sign. Brain SPECT and muscle biopsy enabled us to identify the cortical and peripheral motor neurone involvement in this uncooperative intensive care patient totally dependent on mechanical ventilation.