1/111. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/111. Multifocal fibrosclerosis presenting as Grave's orbitopathy. Bilateral exophthalmos associated with retroperitoneal and sellar fibrosis.BACKGROUND: Multifocal fibrosclerosis (MF) is a rare disease that may be misdiagnosed as Graves' orbitopathy. The combination of localisations of MF presented here has not been reported before. CASE REPORT: A 44-year-old man was referred with progressive bilateral exophthalmos. CT of his chest and abdomen revealed an intrathoracic and retroperitoneal peri-aortal soft-tissue process with hydronephrosis. Histological examination of orbital masses showed a fibrous process with low inflammatory activity. Later the sella was found to be involved as well. A diagnosis of MF was made. CONCLUSION: MF should be considered in the differential diagnosis of bilateral exophthalmos.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
3/111. Hippocampal sclerosis with hypertrophy of end folium pyramidal cells.Mesial temporal lobectomy for the treatment of intractable temporal lobe seizures may show dual pathologies for example hippocampal sclerosis (HCS) combined with a malformation. In a lobectomy specimen from a 40-year-old female with typical radiological and pathological features of HCS, an additional histopathological finding was the presence of hypertrophic pyramidal cells in the dentate hilus, in which cytoplasmic accumulations of phosphorylated neurofilament were demonstrated. Although these cells closely resembled dysplastic nerve cells of cortical dysplasia, we argue that the cytoskeletal abnormalities observed are a result of ongoing alterations to hippocampal circuitry in an evolving HCS.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
4/111. osteosclerosis, hypoplastic nose, and proptosis (Raine syndrome): further delineation.We describe a newborn girl with a lethal sclerosing bone dysplasia leading to prenatal skeletal alterations and microcephaly, proptosis, hypoplastic nose and midface, small jaw, cleft palate, hypertrophied gums, intracranial calcifications, and generalized osteosclerosis. There is a remarkable similarity between our patient and six previously reported infants subsequently categorized as having a distinct entity: Raine syndrome. Autosomal recessive inheritance is postulated based on parental consanguinity in several of the previous cases and in our patient.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
5/111. White-matter change in mesial temporal sclerosis: correlation of MRI with PET, pathology, and clinical features.PURPOSE: To assess the magnetic resonance imaging (MRI), positron emission tomography (PET), pathology, and clinical findings of patients with the MRI feature of white-matter change (WMC) in the anterior temporal lobe. methods: Fifty-six patients with pathologically proven mesial temporal sclerosis were included in this study. MRI and 18F-2-deoxyglucose-(FDG) PET images were obtained before surgery in all patients. The patients were divided into two groups according to the presence of WMC on their MRI. WMC consists of an indistinct gray-white matter demarcation and an increased signal intensity of the anterior temporal lobe on T2-weighted images. The two groups were then compared in terms of MRI, PET, pathology, and clinical features. RESULTS: The MRI feature of WMC was observed in 18 (32%) of the 56 patients. PET images of those patients revealed more severe hypometabolism of the ipsilateral temporal lobes (p< 0.05). In terms of histologic findings, larger numbers of heterotopic neurons were observed in the anterior temporal lobe white matter of these patients who also shared the following clinical features: earlier seizure onset, frequent history of febrile convulsions, and favorable surgical outcomes. CONCLUSIONS: The MRI feature of WMC is an additive sign for correct seizure lateralization and may be related to a favorable surgical outcome in patients with temporal lobe epilepsy.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/111. Ictal vomiting in partial seizures of temporal lobe origin.We report 3 cases presenting ictal vomiting during partial seizures of temporal lobe origin. Two patients had complex partial seizures accompanying vomiting characteristics. Ictal vomiting occurred early in the course of the seizure when rhythmic discharges involved predominantly the left hemisphere, the language dominance hemisphere. The other patient had ictal vomiting in simple partial seizures which originated from the right temporal lobe or the language nondominant side. All 3 patients underwent anterior temporal lobectomy with promising outcomes. Pathologic diagnosis included hippocampal sclerosis in 2 patients and astrocytoma in 1 patient. In our patients, ictal vomiting does not lateralize temporal lobe epilepsy and is not specific to pathology.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/111. Temporal lobe focal cortical dysplasia: MRI imaging using FLAIR shows lesions consistent with neoplasia.Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children's Hospital of Eastern ontario, four patients with temporal lobe FCD have recently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/111. Intracardiac tumour and brain lesions in tuberous sclerosis. A case report of antenatal diagnosis by ultrasonography.A case of antenatal tuberous sclerosis was diagnosed by ultrasonography. Intracardiac tumour (highly suspected rhabdomyoma) with transitory heart failure and multiple brain lesions were observed. After delivery, echocardiography, spiral CT and MR imaging diagnosis of tuberous sclerosis was confirmed by typical skin lesions (depigmented macules) and development of seizures.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
9/111. multiple sclerosis simulating a mass lesion.The cases of two young women with a homonymous hemianopia are described. Both women had a progressively enlarging mass lesion that was seen with neuroimaging studies. One patient had neurologic deterioration despite intravenous corticosteroid treatment. In each case, results of a stereotactic biopsy showed demyelination that was consistent with multiple sclerosis. multiple sclerosis infrequently presents as a mass lesion. The atypical clinical and radiographic features of large demyelinating plaques may lead to an erroneous diagnosis of a brain tumor, infection, or demyelination from other causes.- - - - - - - - - - ranking = 6keywords = sclerosis (Clic here for more details about this article) |
10/111. susac syndrome: microangiopathy of the retina, cochlea and brain.BACKGROUND: susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. methods: Analysis of two male and two female cases of susac syndrome recognized in australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behcet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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