1/76. Leukoencephalopathy in childhood acute lymphoblastic leukemia with t(1;19).To clarify the incidence of leukoencephalopathy in patients with t(1;19) and their clinical characteristics, we studied 239 acute lymphoblastic leukemia (ALL) cases. The 1;19 translocation was found in 20 (8.5%) of the 239 children with ALL. Leukoencephalopathy occurred in 2 (10%) patients with t(1;19) during the early first remission and in one case with t(1;19) at the time of central nervous system (CNS) relapse. Leukoencephalopathy was not found during the early first remission in patients lacking t(1;19), but did develop in 4 patients lacking t(1;19) at the time of CNS relapse. There were no differences in age, sex, leukocyte count, platelet count or serum lactate dehydrogenase level between t(1;19) patients with and without leukoencephalopathy. Our results suggest the incidence of leukoencephalopathy in patients with t(1;19) during the early first remission to be 10%, but we can not predict which patients will develop leukoencephalopathy.- - - - - - - - - - ranking = 1keywords = leukoencephalopathy (Clic here for more details about this article) |
2/76. Myeloneuropathy following nitrous oxide anesthaesia in a patient with macrocytic anaemia.The neurological condition triggered by anaesthesia with nitrous oxide involves the cyanocobalamine pathway and is characterised by progressive demyelination and axonal lesions of the peripheral nerves and cervicothoracic spinal cord (posterior and anterolateral columns) giving a peripheral neuropathy and very frequently subacute combined degeneration of the spinal cord. It is possible to show these demyelinating lesions by MRI of the spine, allowing early diagnosis and follow-up. We describe a case of myeloneuropathy with onset a few hours after nitrous oxide anaesthesia in a patient with macrocytic anaemia and possible subclinical vitamin B(12) deficiency and MRI evidence of a lesion of the cervical spinal cord. Neurological and haematological improvement followed cyanocobalamine replacement.- - - - - - - - - - ranking = 8.5672740933993E-5keywords = posterior (Clic here for more details about this article) |
3/76. A case of leukoencephalopathy with vanishing white matter.A 6-year old Turkish boy with a recently defined entity: "leukoencephalopathy with vanishing white matter" is described. He was born to consanguinous parents. His psychomotor development was normal till he first presented with fever and generalized tonic-clonic seizures at the age of 2.5, followed by rapid motor and mental deterioration. Decerebrate posture and marked spasticity subsequently developed. The initial MRI examination showed diffuse involvement of white matter, including subcortical U-fibers, with signal intensity parallel to CSF on all sequences. The white matter appeared swollen. The ventricles were slightly enlarged and there was cavum septi pellucidi et vergae. The posterior crus of the internal capsule, external and extreme capsules were affected. Cerebellar hemispheres and vermis showed atrophy. The involvement pattern of brainstem was noteworthy in that pontine tegmentum and cruri cerebri were affected. Follow-up MRI obtained after three years did not show any interval change. brain biopsy showed thinned cortex with relatively preserved cortical layering and neuronal structure. There was rarefaction of the white matter with cystic degeneration. Fibrillary gliosis and increased number of oligodendroglial cells were observed within the cerebral white matter.- - - - - - - - - - ranking = 1.2500856727409keywords = leukoencephalopathy, posterior (Clic here for more details about this article) |
4/76. heroin-induced spongiform leukoencephalopathy: value of diffusion MR imaging.The diffusion-weighted (DW) magnetic resonance (MR) imaging findings of a patient with subacute stage of heroin-induced vacuolating myelinopathy are reported. The diffuse decrease of apparent diffusion coefficient (ADC) of the white matter on DW imaging is attributed to restricted water diffusion, which is known to be caused by fluid entrapment within the myelin lamellae without demyelination.- - - - - - - - - - ranking = 1keywords = leukoencephalopathy (Clic here for more details about this article) |
5/76. Symptomatic paroxysmal hemidystonia due to a demyelinating subthalamic lesion.We present a case of paroxysmal hemidystonia in a patient with an isolated demyelinating lesion in the subthalamic region, involving the posterior arm of the internal capsule and extending to the subthalamic nucleus and mesencephalon, possibly due to multiple sclerosis. Compared with similar reports in the literature, in our case there was a paucity of lesions, permitting a more direct clinico-anatomical correlation. The role of the subthalamic region and basal ganglia circuitry in the genesis of symptomatic dystonia is discussed.- - - - - - - - - - ranking = 8.5672740933993E-5keywords = posterior (Clic here for more details about this article) |
6/76. Non-progressive leukoencephalopathy with bilateral temporal cysts.We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.- - - - - - - - - - ranking = 1.25keywords = leukoencephalopathy (Clic here for more details about this article) |
7/76. Cranial root injury in glossopharyngeal neuralgia: electron microscopic observations. Case report.Optical and electron microscopic examinations were made of a biopsy sample of the ninth and 10th cranial nerves obtained during posterior fossa surgery for the relief of pain in a patient suffering from glossopharyngeal neuralgia (GN). Pathological findings, which were restricted to a small fraction of fascicles in the nerves, included large patches of demyelinated axons in close membrane-to-membrane apposition to one another and zones of less severe myelin damage (dysmyelination). These observations, in the light of similar morphological changes observed in biopsy samples excised from patients with trigeminal neuralgia, and new information on the pathophysiological characteristics of injured peripheral nerve axons, can account for much of the symptomatology of GN.- - - - - - - - - - ranking = 8.5672740933993E-5keywords = posterior (Clic here for more details about this article) |
8/76. Neuroaxonal leukoencephalopathy with axonal spheroids.A 51-year-old woman with no significant family history exhibited progressive presenile dementia followed by right-sided spastic hemiplegia and died 27 months after the onset of her illness. brain MRI demonstrated a widespread abnormality in the cerebral deep white matter and corpus callosum. Neuropathologically, extensive destruction of axons and myelin and abundant axonal spheroids were found in the deep white matter, preferentially of the frontoparietal areas. The corpus callosum was also severely damaged. The cerebral cortex and subcortical U fibers remained intact. The pathological features were different among the lesions, reflecting the sequential degenerative processes of the white matter. This case is, to our knowledge, the third sporadic case of neuroaxonal leukoencephalopathy with axonal spheroids.- - - - - - - - - - ranking = 1.25keywords = leukoencephalopathy (Clic here for more details about this article) |
9/76. Tensor diffusion imaging in B12 leukoencephalopathy.anisotropy measurements were obtained from periventricular foci of T2 prolongation and adjacent normal-appearing white matter in a case of B12 leukoencephalopathy. Measurements were compared with mean values from two age-matched control subjects. anisotropy was greatly reduced in the lesions evident on T2-weighted images and in the normal-appearing adjacent white matter (WM), indicating that the extent of WM tract disruption was greater than could be identified on routine MR sequences.- - - - - - - - - - ranking = 1.25keywords = leukoencephalopathy (Clic here for more details about this article) |
10/76. Central-peripheral sensory axonopathy in a juvenile case of Alpers-Huttenlocher disease.Peripheral ataxia is reported in a juvenile case of Alpers-Huttenlocher disease (AHD). Neurophysiological and neuropathological investigations revealed a central-peripheral axonopathy, affecting the deep sensation carried by the peripheral nerve fibres and the posterior tracts of the cord, due to neuronal loss of the sensory ganglia. Involvement of the sensory pathways is regarded as a major feature of juvenile AHD.- - - - - - - - - - ranking = 8.5672740933993E-5keywords = posterior (Clic here for more details about this article) |
| | Next -> |