1/284. Steroid-responsive multifocal demyelinating neuropathy with central involvement.We describe 2 patients with associated central and peripheral demyelination. Electrophysiological studies revealed a demyelinating polyneuropathy with sensory and motor conduction blocks. Visual evoked potentials were abnormal. Motor evoked potentials showed abnormal central conduction time in 1 patient. magnetic resonance imaging revealed regions of abnormal high signal in the spinal cord and brain; sural nerve biopsy disclosed a demyelinating neuropathy. Both patients showed clinical and electrophysiological improvement after steroid therapy.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/284. Neuro-ophthalmic, radiographic, and pathologic manifestations of adult-onset alexander disease.A 61-year-old woman had a 3-year history of imbalance. eye movement studies revealed square-wave jerks, gaze paretic nystagmus, rebound nystagmus, impaired smooth pursuit, impaired optokinetic nystagmus, and abnormal fixation suppression of vestibular nystagmus. A brain magnetic resonance imaging study showed extensive areas of increased signal from the middle cerebellar peduncles and dentate nuclei, which enhanced with gadolinium. Histopathological analysis of a needle biopsy specimen of the left cerebellar peduncle revealed diffuse gliosis in the presence of symmetrically distributed areas of demyelination. There were associated Rosenthal fibers. Clinicopathologic correlation supported a diagnosis of alexander disease. An adult patient with a history of progressive imbalance, ocular motility abnormalities consistent with cerebellar and/or brainstem dysfunction, and diffuse, symmetric hyperintense magnetic resonance imaging signals in brainstem and cerebellar white matter should suggest a diagnosis of adult-onset alexander disease.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
3/284. Clinical, pathologic, and neurochemical studies of an unusual case of neuronal storage disease with lamellar cytoplasmic inclusions: a new genetic disorder?A child of first-cousin Puerto Rican parents had global developmental delay, failure to thrive, and hypotonia since early infancy. At 1 1/2 years of age, she developed clinical and electrophysiologic evidence of progressive motor and sensory neuropathy. At 2 1/2 years, she developed visual impairment and optic atrophy followed by gradual involvement of the 7th, 9th, 10th, and 12th cranial nerves. Uncontrollable myoclonic seizures began at 4 years and she died at 6 years of age. Motor nerve conduction velocities were initially normal and later became markedly slowed. Sensory distal latency responses were absent. Lysosomal enzyme activities in leukocytes and fibroblasts were normal. sural nerve and two muscle biopsies showed only nondiagnostic abnormalities. Electron microscopy of lymphocytes, skin, and fibroblasts showed cytoplasmic inclusions. light microscopy of frontal cortex biopsy showed neuronal storage material staining positively with Luxol fast blue, and electron microscopy showed cytoplasmic membranous bodies in neurons, suggesting an accumulation of a ganglioside. At autopsy, all organs were small but otherwise normal and without abnormal storage cells in the liver, spleen, or bone marrow. Anterior spinal nerve roots showed loss of large myelinated axons. The brain was small and atrophic; cortical neurons showed widespread accumulation of storage material, most marked in the pyramidal cell layer of the hippocampus. Subcortical white matter was gliotic with loss of axons and myelin sheaths. In cortical gray matter there was a 35% elevation of total gangliosides, with a 16-fold increase in GM3, a three- to four-fold increase in GM2 gangliosides, and a 15-fold elevation of lactosyl ceramide. GM3 sialidase activity was normal in gray matter at 3.1 nmols/mg protein per hour and lactosyl ceraminidase I and II activities were 70% to 80% of normal. In white matter, total myelin was reduced by 50% but its composition was normal. Phospholipid distribution and sphingomyelin content were normal in gray matter, white matter, and in the liver. These biochemical findings were interpreted as nonspecific abnormalities. The nature of the neuronal storage substance remains to be determined.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
4/284. Osmotic demyelination syndrome with two-phase movement disorders: case report.Osmotic demyelination syndrome (ODS) is characterized by regions of demyelination throughout the brain, which are most prominent in the pons. This demyelinating disease is associated with electrolyte disturbances and typically occurs in patients who are alcoholic or malnourished. movement disorders are not frequently recognized in patients with ODS. This report describes a 22-year-old woman with ODS after correction of profound hyponatremia. The main neurologic symptom was two-phase movement disorder. First, she had acute onset dystonia, then the movement disorder transformed to generalized rigidity and tremors in the delayed second phase. magnetic resonance imaging in the first phase revealed demyelinating lesions in the central pons, bilateral thalami and basal ganglia. In the second phase, the previous myelinolysis had been partially resolved. The clinical course of the two-phase movement disorder did not correlate with the resolving feature of neuroradiologic findings. During the second-phase movement disorder, the patient had a good response to propranolol and trihexyphenidyl.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/284. 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 = 1.5732937376727keywords = nervous system (Clic here for more details about this article) |
6/284. optic atrophy and chronic acquired polyneuropathy.Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, multifocal disorder usually defined as limited to the peripheral nervous system. Multifocal motor neuropathy, an acquired demyelinating neuropathy with conduction block affecting motor neurons only, may be a pathogenically distinct syndrome or a predominantly motor variant of chronic inflammatory demyelinating polyneuropathy. central nervous system demyelination including optic neuropathy has been reported uncommonly previously in these entities. We report two cases and review the literature on the possible association of optic neuropathy and chronic acquired polyneuropathy.- - - - - - - - - - ranking = 3.1465874753454keywords = nervous system (Clic here for more details about this article) |
7/284. 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 = 4.3317325921919keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/284. Lesion development in Marburg's type of acute multiple sclerosis: from inflammation to demyelination.We report a patient who suffered from acute inflammatory CNS demyelination and underwent two consecutive diagnostic stereotactic brain biopsies during the early disease course. The first lesion was drawn 33 days after the onset of disseminated neurological symptoms. macrophages and T lymphocytes diffusely infiltrated small vessel walls and the white matter. mRNA for tumor necrosis factor alpha (TNFalpha) and inducible nitric oxide synthase (iNOS) was abundantly expressed. Myelin sheaths were entirely preserved. The second biopsy 76 days later showed confluent demyelinating lesions with a diffuse infiltration of macrophages that were positive for myelin debris, activation markers and TNFalpha and iNOS mRNA. IgG and C9neo deposits were found along myelin sheaths. The patient had received intravenous immunoglobulins (IVIG) prior to biopsy. Findings from this single patient affirm that demyelination follows the migration of inflammatory cells from the circulation into the white matter with subsequent inflammation and demyelination. inflammation alone may be sufficient to cause significant clinical deficits without demyelination. Inflammatory mediators such as TNFalpha and NO are involved at very early stages in the pathogenetic process. IVIG treatment may lead to the deposition of immunoglobulins and to the activation of the complement cascade, but the clinical relevance of this particular finding remains uncertain.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/284. Demyelination of retinal myelinated nerve fibers in Behcet's disease.A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone.- - - - - - - - - - ranking = 4.3317325921919keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/284. Chemotherapy for pancreatic cancer: a neuroimaging clinicopathologic correlation.This 52-year-old male without a significant medical history was receiving chemotherapy with diethylnorspermine (DENSPM), a polyamine analogue, for a partially resected pancreatic adenocarcinoma. Ten months after his initial diagnosis, he was admitted to an outside hospital for evaluation of altered mental status. Over the course of the next few days the patient developed progressive neurologic signs and symptoms including lethargy, tonic deviation of his eyes to the left, asymmetic pupils, and right-sided decerebrate posturing elicited by painful stimuli. neuroimaging studies revealed multiple lesions scattered in the periventricular white matter, thalamus, midbrain pons, and cerebellar peduncles. The clinical and neuroimaging differential diagnoses are discussed, and postmortem neuropathologic correlation is presented.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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