Robert Semnic

Dystonia in Wilson's disease.

The frequency and type of dystonic movements, as well as brain abnormalities, as depicted with magnetic resonance imaging (MRI), which might correlate with dystonia, were studied in 27 consecutive patients with a neurologic form of Wilsons disease (WD) and optimized treatment. Dystonia was found in 10 patients (37%), being generalized in half of them, while two patients had segmental, two patients multifocal dystonia, and one patient bilateral foot dystonia. Dystonia was a presenting sign in four patients and developed later in the course of the disease in six patients, despite the administered therapy for WD. Putamen was the only structure significantly more frequently lesioned in dystonic (80%) in comparison to WD patients without dystonia (24%), suggesting a relation between abnormalities in this brain region and dystonic movements in WD.

MR and MRS Characteristics of Intraventricular Meningioma

Meningiomas are frequent intracranial, non‐glial tumors of adults. We present the unusual left lateral ventricular localization of meningioma in a 51‐year‐old man. The magnetic resonance (MR) images showed well demarcated, large mass of the atrium of the left lateral ventricle with transependymal extension into the left temporal lobe. MR spectroscopy revealed the presence of “choline only” spectrum, typical for extra axial neoplasms. The mass was completely resected. The diagnosis of transitional type intraventricular meningioma, with psammoma bodies, histologic grade I was made. Progesterone and estrogen receptors were negative.

HIV-associated neurodegeneration and neuroimmunity: multivoxel MR spectroscopy study in drug-naïve and treated patients

AbstractObjectivesThe aim of this study was to test neurobiochemical changes in normal appearing brain tissue in HIV+ patients receiving and not receiving combined antiretroviral therapy (cART) and healthy controls, using multivoxel MR spectroscopy (mvMRS).MethodsWe performed long- and short-echo 3D mvMRS in 110 neuroasymptomatic subjects (32 HIV+ subjects on cART, 28 HIV+ therapy-naïve subjects and 50 healthy controls) on a 3T MR scanner, targeting frontal and parietal supracallosal subcortical and deep white matter and cingulate gyrus (NAA/Cr, Cho/Cr and mI/Cr ratios were analysed). The statistical value was set at p < 0.05.ResultsConsidering differences between HIV-infected and healthy subjects, there was a significant decrease in the NAA/Cr ratio in HIV+ subjects in all observed locations, an increase in mI/Cr levels in the anterior cingulate gyrus (ACG), and no significant differences in Cho/Cr ratios, except in ACG, where the increase showed trending towards significance in HIV+ patients. There were no significant differences between HIV+ patients on and without cART in all three ratios.ConclusionNeuronal loss and dysfunction affects the whole brain volume in HIV-infected patients. Unfortunately, cART appears to be ineffective in halting accelerated neurodegenerative process induced by HIV but is partially effective in preventing glial proliferation.Key Points• This is the first multivoxel human brain 3T MRS study in HIV. • All observed areas of the brain are affected by neurodegenerative process. • Cingulate gyrus and subcortical white matter are most vulnerable to HIV-induced neurodegeneration. • cART is effective in control of inflammation but ineffective in preventing neurodegeneration.

Perineural tumor spread — Interconnection between spinal and cranial nerves

The secondary neoplastic involvement of the cervical plexus in patients with head and neck malignancies is extremely rare. MR examination of the neck revealed the diffuse neoplastic infiltration of the right C2 root, in a 57-year-old patient with several months long pain in the right ear region and a history of the tongue squamous cell carcinoma. Associated perineural tumor spread and consequent distal involvement of great auricular nerve and vagus nerve were evident. Best of our knowledge, this is the first reported involvement of the cervical plexus in patients with head and neck cancers, associated with the clearly documented interconnection between the cervical plexus and cranial nerves via great auricular nerve.

Magnetic Resonance Imaging Morphometry of the Midbrain in Patients With Wilson Disease

In this study, the focus is on midbrain magnetic resonance imaging morphometric measures (transverse diameter of the midbrain peduncle [Tp], transverse diameter of the tegmentum, anteroposterior diameter of the midbrain, interpeduncular distance [IPD], and interpeduncular angle [IPA]) in a group of 47 consecutive patients with neurologic (37 patients) and hepatic (10 patients) forms of Wilson disease (WD). Morphometric measures were significantly different between the group of patients with WD and healthy controls (51 subjects) as well as patients with Parkinson disease (15 patients) and multiple sclerosis (15 patients). Among the studied variables, IPA, Tp, and IPD were particularly useful in differentiating patients from healthy subjects (probability reaching 93%).

A novel Notch3 Gly89Cys mutation in a Serbian CADASIL family

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable cause of stroke and vascular dementia in adults. We present a family from Serbia presenting with stroke and depression in the lack of vascular risk factors, with brain MRI indicating CADASIL. A novel NOTCH3 Gly89Cys mutation was located in exon 3. This report illustrates that in the setting of a positive family history with typical clinical and MRI features, even with an atypical form of pedigree, a high suspicion of CADASIL should lead to genetic testing.

Segmental cavernous carotid ectasia in a patient with cluster-like headache

INTRODUCTION Cluster headache (CH) is a primary headache with severe, unilateral periorbital or temporal pain lasting 15-180 min, accompanied with various cranial autonomic features. A diagnosis of cluster-like headache can be made whenever underlying cause of CLH is present. METHODS AND RESULTS We report a case where an ectatic cavernous segment of the internal carotid artery triggered CHL, most probably due to compression of the ophthalmic nerve within cavernous sinus. The pathological substrate of a vessel ectasia is degeneration of the tunica intima as a consequence of atherosclerosis and hypertension. On the other hand, cavernous sinus is unique space where parasympathetic, sympathetic and nociceptive fibers are in intimate relationship which is of great importance for understanding of CH pathophysiology. CONCLUSION Magnetic resonance imaging and MR angiography are mandatory imaging tools used for precise localization of pathological changes in the cavernous sinus, especially in the group of secondary headaches attributed to vascular disorders.

DIFFERENCES BETWEEN MULTI-INFARCT DEMENTIA AND SMALL-VESSEL DISEASE DEMENTIA IN ATTENTION AND WORKING MEMORY DEFICITS

P3-268 DIFFERENCES BETWEENMULTI-INFARCT DEMENTIA AND SMALL-VESSEL DISEASE DEMENTIA IN ATTENTION ANDWORKING MEMORY DEFICITS Marija D. Semnic, Robert R. Semnic, Vojislava V. Bugarski Ignjatovic, Tijana Stankov, Sanela B. Popovic, Dusko B. Kozic, Faculty of Medicine University of Novi Sad, Novi Sad, Serbia; Neuroradiology Department, Academical Hospital, University of Uppsala, Uppsala, Sweden; Faculty of Medicine University of Novi Sad, Novi Sad, Serbia. Contact e-mail: memoryns@gmail.com

Atypical, polyarticular lipoma arborescens in a child

Introduction Lipoma arborescens is a rare, tumor-like lesion commonly involving synovial joints and less commonly bursae and synovial tendon sheaths. Case Outline We report a case of a 12-year-old boy with symmetric involvement of the bicipitoradial bursae, synovial sheaths of extensor compartments of both hands and medial ankles. The diagnosis of polyarticular lipoma arborescens was proposed on magnetic resonance (MR) imaging and this diagnosis was histologically proven after biopsy of the bursae and later by open surgery of the synovial sheath of the right ankle tendons. Literature search was performed and twelve cases with polyarticular involvement were analyzed. Lipoma arborescens commonly involves suprapatellar recess of the knee and very rarely other joints or bursae. Histological analysis revealed an accompanying non-necrotizing granulomatous synovial inflammation. Conclusion Polyarticular lipoma arborescens is a rare entity and symmetrical involvement of the joints other than the knees is exceedingly rare. MR imaging plays a significant role in the diagnostic protocol, and the characteristic fatty signal on MR imaging is highly suggestive of lipoma arborescens.

Diffusion restriction of posterior uveal melanoma on MR imaging.

A 55-year-old male with clinical and opthalmological signs of retinal ablation was referred for orbital magnetic resonance imaging (MRI). MR scan revealed a small tumor nodule in the posterolateral part of left eye globe as a cause of ablation. Tumor showed high signal on transverse diffusion weighted image (DWI) (Fig. A, arrow), low signal on apparent diffusion coefficient image (Fig. B, arrow), consistent with restricted diffusion. The nodule had intermediate signal on T1 sagittal weighted unenhanced image (Fig. C, arrow). The patient underwent left eye enucleation with histopathologically proved uveal melanoma.