1/47. Dacrystic seizures reconsidered.Ictal lachrymation has not been reported in children as a main seizure manifestation. We report an infant with tuberous sclerosis who presented with seizures characterised by lachrymation without apparent emotional change associated with a left posterior temporal epileptic focus. We discuss the pathophysiological implications, and propose a clinically orientated classification of crying seizures.- - - - - - - - - - ranking = 1keywords = tuberous sclerosis, sclerosis (Clic here for more details about this article) |
2/47. 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 = 0.11542897251285keywords = sclerosis (Clic here for more details about this article) |
3/47. tuberous sclerosis presenting for laparotomy.A 30-year-old female patient with tuberous sclerosis presented for anaesthesia and surgery for haemorrhagic renal angiomyolipoma. The anaesthetic management of this case was tailored to the prevention of seizures. Diagnostic features and possible complications of the disease are also described.- - - - - - - - - - ranking = 1.0923431780103keywords = tuberous sclerosis, sclerosis (Clic here for more details about this article) |
4/47. Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases.Neurofibromatosis and tuberous sclerosis are the most well-recognized of the congential phakomatoses, a group of six hereditary neuro-oculo-cutaneous disorders. Although easily diagnosed at birth by a parasagittal line of facial sebaceous naevi, the linear naevus sebaceous syndrome (LNSS) of Jadassohn is the rarest phakomatosis, one often characterized by airway and anaesthetic considerations that do not apply to the other phakomatoses. In addition to its obvious cutaneous manifestations, LNSS is characterized by hemifacial asymmetry, an anatomic predictor of difficult trachael intubation, and intractable seizure activity, a condition that limits selection of anaesthetics. The perioperative management challenges of LNSS are depicted in the presentation of two cases of LNSS with different outcomes and contrasted with the major anaesthetic considerations in the perioperative management of other, more common phakomatoses.- - - - - - - - - - ranking = 1keywords = tuberous sclerosis, sclerosis (Clic here for more details about this article) |
5/47. Human herpesvirus 6 limbic encephalitis after stem cell transplantation.central nervous system complications are common in stem cell transplant recipients, but selective involvement of the medial temporal area is unusual. The 5 patients reported here presented after stem cell transplantation with increased hippocampal T2 signal on magnetic resonance imaging and increased hippocampal glucose uptake on [F-18]fluorodeoxyglucose-positron emission tomography (FDG-PET) associated with short-term memory loss, insomnia, and temporal lobe electrographic seizure activity. The initial scalp electroencephalograms (EEGs) failed to detect seizure activity in these patients, although the memory dysfunction along with the magnetic resonance imaging and FDG-PET findings suggested subcortical seizure activity. However, extended EEG monitoring revealed repetitive temporal lobe electrographic seizure activity. Follow-up MRIs in 2 patients and postmortem findings on 1 patient suggested that hippocampal sclerosis had developed following the clinical syndrome. cerebrospinal fluid studies revealed the presence of human herpesvirus 6, variant B, dna in all of 3 patients who had lumbar punctures. Immunohistochemical staining for the P41 and P101 human herpesvirus 6 protein antigens showed numerous immunoreactive astrocytes and neurons in the hippocampus of 1 of the patients who died from other causes. Because of its subtle clinical presentation, this syndrome may be underrecognized, but can be diagnosed with appropriate magnetic resonance imaging techniques, EEG monitoring, and cerebrospinal fluid viral studies.- - - - - - - - - - ranking = 0.023085794502569keywords = sclerosis (Clic here for more details about this article) |
6/47. Another autosomal recessive form of focal glomerulosclerosis with neurological findings.We report four patients in a consanguineous family with focal segmental glomerulosclerosis (FSGS), early onset nephrotic syndrome, eventual end-stage renal failure, psychomotor retardation, seizures and microcephaly or brain atrophy without hiatus hernia. Other characteristic dysmorphic features were convergent strabismus and narrow forehead. One patient had enamel hypoplasia of the upper incisors and deviation of bilateral thumbs to palm side. We could not detect an NPHS2 mutation in this family. We propose that this may be another autosomal recessive syndrome with FSGS and neurological findings.- - - - - - - - - - ranking = 0.11542897251285keywords = sclerosis (Clic here for more details about this article) |
7/47. Pathological effect of seizures on the hippocampus in cases with temporal lobe epilepsy caused by brain tumors.The cause of Ammon's horn sclerosis in temporal lobe epilepsy has not yet been clarified. In the present study, the pathological effect of epileptic seizures on the hippocampus was investigated in surgically treated patients with brain tumor-induced temporal lobe epilepsy. Tumors involving the hippocampus were identified as the foci of epilepsy in 13 patients (seven male and six female) and resected after epileptic discharges were found at the hippocampus on intraoperative electrocorticogram. The mean age at operation was 29.8 /- 11.5 years and the mean age of seizure onset was 19.9 /- 10.8 years. Because only three of the 13 patients who underwent temporal lobectomy for brain tumors involving the hippocampus had Ammon's horn sclerosis pathologically, it was concluded that it was very unlikely that the Ammon's horn sclerosis was produced by the epileptic seizures. Two of the three patients with pathological signs of Ammon's horn sclerosis had episodes of coma, covulsion, high fever and cyanosis in their past histories that might have portended the appearance of Ammon's horn sclerosis later in their lives.- - - - - - - - - - ranking = 0.11542897251285keywords = sclerosis (Clic here for more details about this article) |
8/47. seizures and extrapyramidal symptoms in a patient with Tourette's syndrome, Asperger's syndrome, and multiple sclerosis treated with interferon beta-1a and clomipramine.Seizure activity is a known complication associated with multiple sclerosis; however, it may also result from side effects of the treatments for the disease. A 21-year-old man with Tourette's syndrome, pedophilia, Asperger's syndrome, and multiple sclerosis experienced seizures after receiving therapy with interferon beta-1a. Adjustments in his drug regimen led to the discovery of pseudoparkinsonism and other extrapyramidal symptoms. This case report illustrates how pharmacodynamic properties of drugs can complicate the treatment of neurologic disorders. Clinicians must be aware of the delicate balance between the signs and symptoms of disease states and the effects of drugs.- - - - - - - - - - ranking = 0.13851476701542keywords = sclerosis (Clic here for more details about this article) |
9/47. Transmantle dysplasia in tuberous sclerosis: clinical features and surgical outcome in four children.In the literature, several malformations of cortical development have been described as additional lesions in tuberous sclerosis complex. Among these lesions, a very large focal cortical dysplasia has peculiar magnetic resonance imaging features: a signal abnormality that extends radially inward toward the lateral ventricle from the pachygyric cortical surface plus a homogeneous clinical picture. Affected patients have early-onset drug-resistant epilepsy and severe developmental delay. We describe the clinical, genetic, neurophysiologic, and neuroradiologic characteristics of four patients affected by tuberous sclerosis and this type of cortical dysplasia these patients are of special interest because they have been operated on for their dysplastic lesions. Total control of seizures has been achieved in the three children who underwent a complete lesionectomy. This result cannot be permanent, however, because of the presence of other cortical tubers which could become epileptogenic. All things considered, our choice was to give these children at least temporary relief from severe epilepsy and possibly support for developmental progression.- - - - - - - - - - ranking = 6keywords = tuberous sclerosis, sclerosis (Clic here for more details about this article) |
10/47. Mesial temporal sclerosis--a late complication in four allogeneic pediatric recipients with persistent seizures after an acute episode of cyclosporine-A neurotoxicity.Mesial temporal sclerosis (MTS) is a common finding in patients with intractable temporal lobe epilepsy (TLE). In this report, we retrospectively reviewed the neuroimaging results of four children who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT), and who developed recurrent, partial, intractable seizures following a first event caused by cyclosporine-A (CSA) neurotoxicity. Neuroradiologic findings of MTS were demonstrated in all these patients. We suggest that MTS may be a consequence of CSA neurotoxicity, which induces repeated seizures, associated with other predisposing conditions, as well as being a consequence of the underlying disease and its treatment, and of severe graft-versus-host disease (GvHD).- - - - - - - - - - ranking = 0.11542897251285keywords = sclerosis (Clic here for more details about this article) |
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