Marcello Longo

Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.

Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified.

Contrast-enhanced MR imaging with fat suppression in adult-onset septic spondylodiscitis.

Abstract. Spinal infections typically involve vertebrae as well as discs, and for this reason they are called septic spondylodiscitis. Magnetic resonance imaging is the most sensitive imaging method for the evaluation of this group of spinal diseases. The use of contrast-enhanced T1-weighted sequences with fat suppression, if correctly applied, may increase information provided by MRI. Firstly, this technique allows the primary vertebral focus, which often precedes disc involvement, to be identified at a very early stage. When the disease spreads, T1-weighted fat-suppressed gadolinium dimeglumine (Gd-DTPA) enhanced images provide macroscopic details of the primary vertebral focus, disc involvement patterns, and pathways of infection diffusion. All this information, when correlated with laboratory tests, may be useful in identifying the infectious agents (tuberculous vs piogenic forms), thus enabling a suitable therapy to be started. This technique is also useful in the assessment of the real extension of the disease, providing a clear depiction of paravertebral space involvement and of psoas muscle abscesses. Dangerous complications, such as meningitis, myelitis, and epidural abscesses, may be more promptly diagnosed and fully evaluated with fat-suppressed post-contrast T1-weighted images. Finally, this imaging technique may help to differentiate infectious processes from degenerative disorders, extradural neoplastic processes, and rheumatic diseases.

Cerebral neoplastic enhancing lesions: Multicenter, randomized, crossover intraindividual comparison between gadobutrol (1.0 M) and gadoterate meglumine (0.5 M) at 0.1 mmol Gd/kg body weight in a clinical setting

OBJECTIVE Two macrocyclic extracellular contrast agents, one-molar neutral gadobutrol and ionic gadoterate meglumine, were compared to determine the overall preference for one or the other in a clinical setting. MATERIALS AND METHODS Multicenter, randomized, single-blind, intra-individually controlled, comparison study with a corresponding blinded read. Efficacy analysis was based on 136 patients who underwent identical MRI examinations: group A first received 1.0M gadobutrol followed by 0.5M gadoterate meglumine 48 h to 7 days later; group B had a reversed administration order. Three independent blinded readers assessed off-site their overall diagnostic preference (primary efficacy parameter) based on a matched pairs approach. RESULTS Superiority of gadobutrol over gadoterate meglumine was demonstrated for the qualitative assessment of overall preference across all readers by a statistically significant difference between both contrast agents for this primary endpoint. Preferences in lesion enhancement (secondary endpoint) were also found significantly in favor of gadobutrol. For preference in lesion delineation from surrounding tissue/edema and for internal structure only a trend towards a higher proportion for gadobutrol was found (except for internal structure reported by one reader, which showed a result of statistical significance). Lesion contrast and relative lesion enhancement (quantitative parameters) were statistically significantly higher for gadobutrol compared to gadoterate meglumine. CONCLUSION Contrast-enhanced MRI of neoplastic brain lesions at a dose of 0.1 mmol Gd/kg body weight, assessed in a standardized off-site blinded reading, results in a significantly higher qualitative and quantitative preference for gadobutrol compared to gadoterate meglumine.

Bulbar Compression by an Ectatic Vertebral Artery: A Novel Neurovascular Construct Relieved by Microsurgical Decompression

OBJECTIVE: Brainstem compression caused by vascular abnormalities has rarely been reported in the literature. We describe five cases of large ectatic vertebral artery causing compression and distortion of the medulla oblongata with pyramidal tract signs and low cranial nerve dysfunction. Microvascular decompression by retracting the vertebral artery and anchoring it to the dura has been the treatment of choice. METHODS: Five patients, four male and one female, presented with progressive myelopathic features and lower cranial nerve dysfunction, especially dysphonia and dysphagia. Four patients were affected by systemic arterial hypertension. Magnetic resonance imaging showed impingement of the right vertebral artery in three patients and the left vertebral artery in two patients, on the right and left lateral medulla, respectively. In two patients, hypoplasia of the contralateral vertebral artery was documented. RESULTS: All patients underwent neurovascular decompression of the medulla oblongata. The ectatic and tortuous vertebral artery was detached from the medulla, shifted away, and repositioned by anchoring to the nearby dura mater using a Gore-Tex vascular slip. Postoperatively, all patients but one had improvement of their previous neurological symptoms. CONCLUSION: Brainstem dysfunction caused by a tortuous ectatic vertebral artery might be less uncommon than expected. It should be considered a new distinct clinical entity, the real incidence of which needs to be carefully evaluated by an appropriate diagnostic protocol, which includes primarily magnetic resonance imaging with specific three-dimensional sequences. Awareness of this condition is necessary to ensure the appropriate treatment. Surgical microvascular decompression seems very effective.

Autosomal recessive congenital cerebellar atrophy: A clinical and neuropsychological study

Congenital cerebellar atrophy associated with a non-progressive cerebellar syndrome and mild cognitive retardation is described in seven cases, four of them familial. Their occurrence is consistent with an autosomal recessive inheritance. Clinical and neuroimaging data seem to exclude supratentorial changes. Even though it is not possible to definitely rule out a possible role of the forebrain in determining the mental defect, the neuropsychological study supplies arguments stressing the relationship between cerebellar defect and cognitive development.

Temozolomide-Induced Shrinkage of a Pituitary Carcinoma Causing Cushing's Disease — Report of a Case and Literature Review

Temozolomide (TMZ) is an alkylating chemotherapeutic agent that has recently been used in some cases as a new therapeutic tool for pituitary carcinomas and aggressive pituitary adenomas. In this report, we present the case of effective TMZ treatment in a 42-year-old man with ACTH-secreting carcinoma. The tumor grew progressively over 4 years, from 2.2 to 31.1 cm3, despite three surgical approaches and gamma-knife treatment. Ki-67 increased from 2 to 18%. An intradural metastasis at the foramen magnum was detected by MRI after the third operation. Thereafter, four cycles of 5-day TMZ administration (200 mg/m2/day during the first, and 150 mg/m2/day during the following cycles) induced dramatic tumor size reduction (>90%). Clinical conditions improved progressively and, after 17 months from the beginning of TMZ administration, the patient is still alive. The treatment was well tolerated except for a transient thrombocytopenia (grade 4 WHO).

Sclerotherapy for cervical cystic lymphatic malformations in children. Our experience with computed tomography-guided 98% sterile ethanol insertion and a review of the literature

PURPOSE The treatment of cystic lymphatic malformations of the neck and mediastinum is controversial. Surgical management may be limited by the invasiveness of the procedure, the complex anatomy of this region, and the high recurrence rate. An alternative therapeutic method is sclerotherapy. We report our experience in the treatment of cystic lymphatic malformations of the neck by computed tomography (CT)-guided instillation of 98% sterile ethanol in children. METHODS Eight children with clinical suspicion of cervical cystic lymphatic malformation were assessed by ultrasonography (US) and magnetic resonance imaging (MRI) to define the location, size, and number of cystic cavities. The CT-guided instillation of 98% sterile ethanol was performed. Cystic fluid was analyzed by fine-needle aspiration cytology. Clinical and US or MRI follow-up was performed after 1 and 3 months and at 1 and 2 years. RESULTS The results were excellent with complete disappearance of the lesion in 7 (87.5%) of 8 patients. One patient (12.5%) with satisfactory results required a second alcohol injection with an excellent outcome. No allergic reactions or complications were observed. CONCLUSIONS The CT-guided 98% sterile ethanol sclerotherapy is a good alternative to surgical therapy. This procedure seems accurate, minimally invasive, safe, low cost, and reliable without untoward complications. Moreover, it does not exclude later surgical treatment.

Ct findings in hepatic involvement by echinococcus granulosus

The CT findings in hepatic involvement by Echinococcus granulosus are described. Pathognomonic signs of the hydatid nature of a cystic lesion are visualization of the cystic wall on plain CT, calcification of the cyst wall, daughter cysts, and membrane detachment.

MRI Tractography of Corticospinal Tract and Arcuate Fasciculus in High-Grade Gliomas Performed by Constrained Spherical Deconvolution: Qualitative and Quantitative Analysis

BACKGROUND AND PURPOSE: MR imaging tractography is increasingly used to perform noninvasive presurgical planning for brain gliomas. Recently, constrained spherical deconvolution tractography was shown to overcome several limitations of commonly used DTI tractography. The purpose of our study was to evaluate WM tract alterations of both the corticospinal tract and arcuate fasciculus in patients with high-grade gliomas, through qualitative and quantitative analysis of probabilistic constrained spherical deconvolution tractography, to perform reliable presurgical planning. MATERIALS AND METHODS: Twenty patients with frontoparietal high-grade gliomas were recruited and evaluated by using a 3T MR imaging scanner with both morphologic and diffusion sequences (60 diffusion directions). We performed probabilistic constrained spherical deconvolution tractography and tract quantification following diffusion tensor parameters: fractional anisotropy; mean diffusivity; linear, planar, and spherical coefficients. RESULTS: In all patients, we obtained tractographic reconstructions of the medial and lateral portions of the corticospinal tract and arcuate fasciculus, both on the glioma-affected and nonaffected sides of the brain. The affected lateral corticospinal tract and the arcuate fasciculus showed decreased fractional anisotropy (z = 2.51, n = 20, P = .006; z = 2.52, n = 20, P = .006) and linear coefficient (z = 2.51, n = 20, P = .006; z = 2.52, n = 20, P = .006) along with increased spherical coefficient (z = −2.51, n = 20, P = .006; z = −2.52, n = 20, P = .006). Mean diffusivity values were increased only in the lateral corticospinal tract (z = −2.53, n = 20, P = .006). CONCLUSIONS: In this study, we demonstrated that probabilistic constrained spherical deconvolution can provide essential qualitative and quantitative information in presurgical planning, which was not otherwise achievable with DTI. These findings can have important implications for the surgical approach and postoperative outcome in patients with glioma.

Endothelial Functions in Pathophysiology of Thrombosis and Fibrinolysis: Late Spontaneous Recanalization of an Occluded Internal Carotid Artery: A Case Report

The use of oral contraceptives is a potential cause of ischemic stroke in young women. The risk of stroke is higher when contraceptives contain high levels of estrogens. A thrombotic occlusion of the right internal carotid artery, seen on ultrasound, developed in a patient who was taking high-dose estrogen contraception. Recanalization occurred several months later by spontaneous thrombolysis and was confirmed by cerebral angiography. This case suggests that the activation of endothelial spontaneous antithrombotic mechanisms may allow the dissolution of a thrombus, once the cause of the thrombosis has been identified and removed and when the endothelium has maintained its functional integrity.