Occipital epilepsies in children

PubMed ID: 12374578Occipital lobe seizures, as defined by subjective symptoms and objective signs, can be recognized by clinical seizure characteristics in most cases. Visual symptoms such as hallucinations and amaurosis are the most common occipital lobe seizure symptoms. The patients must be classified in order to be able to define the prognosis. In this study, we classified patients with occipital epilepsy as childhood epilepsy with occipital paroxysms (19 patients), idiopathic photosensitive occipital epilepsy (10 patients) and symptomatic occipital epilepsy (25 patients). They were evaluated according to clinical, electrographic and neuroimaging characteristics

Primary Sjögren's Syndrome Presenting As Neuromyelitis Optica

PubMed ID: 17162200This report presents a patient with Devic's neuromyelitis optica associated with primary Sjögren's syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjögren's syndrome could not be made until the second decade because it was not suspected. © 2007 Elsevier Inc. All rights reserved

POEMS syndrome: Case report [POEMS sendromu: Olgu sunumu]

Presented with characteristic polyneuropathy and multisystemic manifestations, POEMS syndrome is a rare plasma cell disorder. Vascular endothelial growth factor, secreted by plasmacytoma, is considered responsible for these symptoms. The first symptoms in this patient were arthralgia, distal sensory impairment ascending proximally and motor impairment of distal lower extremities. By immunoelectrophoresis, M protein in serum and urine was detected. In addition to polyneuropathy and monoclonal gammopathy, the patient presented with organomegaly, endocrine dysfunction and skin changes, and was diagnosed as POEMS syndrome. This rare syndrome should be included in the differential diagnosis of acquired neuropathies associated with multisystemic manifestations

Childhood-onset optic neuritis as the first manifestation of Behçet's disease

A 15-year-old girl presented with sudden blurring of vision in both eyes, which was recurring for the third time. The fundoscopy demonstrated bilateral optic disc edema with macular star exudates. The diagnosis of Behçet's disease (BD) could be made 5 months after the first visual disturbance, when the other manifestations of the disease occurred. Although ocular involvement is frequent, primer optic neuritis has rarely been reported in BD. Childhood onset of the disorder is very uncommon as well. To our knowledge, this is the first report of optic neuritis as the first manifestation of BD in the pediatric age group. Copyright © Taylor & Francis Group, LLC

Progressive multifocal leukoencephalopathy in a case without clear evidence of immunodeficiency [Belirgin İmmunyetmezligi Olmayan Bir Olguda Progressif Multifokal Lökoensefalopati]

Introduction: Progressive Multifocal Leukoencephalopathy(PML) is a rare demyelinating disease of the central nervous system due to reactivation of a latent infection with JC papovavirus. PML predominantly occurs in severely immunosupressed patients, mostly as a late complication of acquired immunodeficiency syndrome and lymphoproliferative disorders. Clinically it is manifested by motor and sensory deficits, ataxia, cognitive impairment and dementia, corresponding to central nervous system (CNS) lesions. It is difficult to distinguish PML clinically from immune-mediated diseases of the CNS, such as multiple sclerosis, neuropsychiatric sistemic lupus erythematosus and CNS vasculitis. Case Report: A 19-year-old woman presented with ataxia and slurred speech rapidly progressing to quadriparesis and confusion. The diagnosis of PML was made by detecting the JC virus in the cerebrospinal fluid by polymerase chain reaction. Discussion: PML predominantly occurs in severely immunosupressed patients. However, it must also be kept in mind in patients without an apparent immunodeficiency when the clinical and radiological features are typical

Headache in primary Sjögren's syndrome: A prevalence study

PubMed ID: 18355394Objectives - To determine the prevalence of headache in patients with primary Sjögren's syndrome (pSS) and to examine the relationship between headache types and clinical, serologic features of the disease. Methods - The study enclosed 133 patients with the diagnoses of pSS and 97 healthy controls. A questionnaire designed to assess the presence of headache and if present to classify it according to the criteria of the International Headache Society was used. Results - In 133 of the pSS patients evaluated, 104 had headache. No association was present between types of headache and the clinical and laboratory manifestations of the disease. Both migraine and tension-type headache were more common in patients with pSS when compared with healthy controls (P < 0.001). Conclusions - The high prevalence of migraine in pSS patients might be explained by a vascular headache triggered by immuno-mediated disease activity without an obvious clinic or laboratory marker. Copyright © 2008 The Authors

Multimodal evoked potentials in primary Sjögren's syndrome without neurological manifestations

We evaluated multimodal evoked potentials in patients with primary Sjögren's syndrome without clinical neurological manifestations. While brainstem auditory evoked potentials (BAEP) were performed in ninety patients visual (VEP) and somatosensory evoked potentials (SEP) could be evaluated in fifty-eight patients. The control group included 20 healthy adults matched for sex and chronological age. VEP and AEP studies did not reveal any abnormality. In SEP; N9-N13 interpeak latencies were significantly prolonged in the patients group. However, the latency of N9 potentials recorded from Erb's point was normal as well as the N13-N20 interpeak latencies. This result indicates that central branches of the primary sensory neurons are involved in Sjögren's syndrome