1/54. vitamin e deficiency ataxia associated with adenoma.Vitamin E is one of the most important lipid-soluble antioxidant nutrient. Severe vitamin e deficiency (VED) can have a profound effect on the central nervous system. VED causes ataxia and peripheral neuropathy that resembles Friedreich's ataxia. We report here a patient presenting this syndrome, but also a prolactin and FSH adenoma. Both the neurological syndromes and the adenoma regressed after treatment with alpha-tocopherol. Although, the presence of the prolactinoma in this patient may not be related to his vitamin e deficiency, alpha-tocopherol treatment seems to be beneficial and might usefully be tested in patients with hypophyseal secreting other forms of adenoma.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/54. Increased density of oligodendrocytes in childhood ataxia with diffuse central hypomyelination (CACH) syndrome: neuropathological and biochemical study of two cases.We report neuropathological, biochemical and molecular studies on two patients with childhood ataxia with diffuse central nervous system hypomyelination (CACH) syndrome, a leukodystrophy recently defined according to clinical and radiological criteria. Both had severe cavitating orthochromatic leukodystrophy without atrophy, predominating in hemispheric white matter, whereas U-fibers, internal capsule, corpus callosum, anterior commissure and cerebellar white matter were relatively spared. The severity of white matter lesions contrasted with the rarity of myelin breakdown products and astroglial and microglial reactions. In the white matter, there was an increase in a homogeneous cell population with the morphological features of oligodendrocytes, in many instances presenting an abundant cytoplasm like myelination glia. These cells were negative for glial fibrillary acidic protein and antibodies PGM1 and MIB1. Some were positive for myelin basic protein, proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein, but the majority were positive for human 2'-3' cyclic nucleotide 3' phosphodiesterase and all were positive for carbonic anhydrase ii, confirming that they are oligodendrocytes. Myelin protein and lipid content were reduced. The PLP gene, analyzed in one case, was not mutated or duplicated. The increased number of oligodendrocytes without mitotic activity suggests an intrinsic oligodendroglial defect or an abnormal interaction with axons or other glial cells. This neuropathological study supports the notion that CACH syndrome constitutes a specific entity.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/54. Asymmetric sweating in a child with multiple sclerosis.A 10-year-old male with multiple sclerosis complained of excessive sweating on the right side of the forehead and shoulder on relapse 3 months after the onset of multiple sclerosis. Because the neurologic evaluation revealed no abnormalities in the sudomotor function, it is likely that the hyperhidrosis resulted from a lesion in the central or preganglionic sympathetic nervous system. magnetic resonance imaging demonstrated a high-intensity lesion involving the left hypothalamus on T(2)-weighted imaging. Thus hypothalamic involvement might be the reason for the hyperhidrosis in this patient.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/54. propafenone-induced ataxia: report of three cases.propafenone is an effective antiarrhythmic drug used widely for the treatment of supraventricular and ventricular arrhythmias. Although it is generally well tolerated, 30 to 45% of patients may experience adverse cardiac effects. In 15 to 20% of patients, adverse effects may involve other organ systems. A wide variety of adverse central nervous system effects have been reported in association with propafenone; dizziness is the most common. ataxia caused by propafenone has been reported to the pharmaceutical companies and drug monitoring agencies, but has not been well described or emphasized in the medical literature. We describe 3 elderly patients with moderate to severe ataxia that occurred while they were taking propafenone.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/54. A female with central anticholinergic syndrome responsive to neostigmine.Central anticholinergic syndrome is a rarely observed condition in children. The occurrence of this syndrome after ingestion of solanum pseudocapsicum is infrequent because findings tend to be milder and localized to the gastrointestinal system, without central nervous system involvement. Most patients do not present with diagnostic problems because their relatives can usually report any ingestion of poisonous agents; however, when drug poisoning or plant ingestion is uncertain, a differential diagnosis with encephalitis must be considered. physostigmine salicylate is the specific antidote because it crosses the blood-brain barrier because of its tertiary ammonium group. neostigmine methylsulfate has a quaternary ammonium group, which prevents its penetration through the blood-brain barrier; hence its primary influence is believed to be due to its action on the peripheral nervous system. We describe a female with central anticholinergic syndrome caused by ingestion of solanum pseudocapsicum. A slow intravenous infusion of neostigmine methylsulfate (0.03 mg/kg) immediately resolved the clinical picture. To our knowledge, this case is the first reported of central anticholinergic syndrome occurring after ingestion of solanum pseudocapsicum in a child and the first report of a complete and rapid remission after intravenous neostigmine methylsulfate administration.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
6/54. Presentation of a previously asymptomatic Chiari I malformation by a flexion injury to the neck.Flexion injury and/or whiplash injury to the neck in car accidents are usually trivial injuries with no serious neurological deficits. Our intention was to point out the importance to proceed with diagnostic procedures if neurological deficits do occur in order to reveal the true cause of the deficit. The paper presents the case of a 35-year-old woman who sustained a flexion injury to the neck. A relatively trivial injury to the neck promoted a progressive neurological deterioration. The standard diagnostic procedures (x-ray, computed tomography scan) were normal. Further diagnostics with magnetic resonance imaging was required to reveal an underlying Chiari I malformation. Finally, the operative decompression of the craniocervical junction was performed. Following the surgical treatment, the patient's clinical symptoms regressed. One year after her discharge, she remains in good physical condition. To our knowledge, this case is the first report of the manifestation of Chiari I malformation in the adult as a result of a flexion or whiplash injury of the neck. This unusual case suggests that in a trivial flexion injury to the neck sustained in a car accident, which presents with serious neurological dysfunction, and where the standard diagnostic procedures are normal, the possibility of underlying congenital abnormality, such as Chiari I malformation should be considered.- - - - - - - - - - ranking = 0.13416770398113keywords = malformation (Clic here for more details about this article) |
7/54. Episodic coma in a new leukodystrophy.Among the leukodystrophies of a hypomyelinating nature, childhood ataxia with diffuse central nervous system hypomyelination exhibits the unique feature of rapid decrease in mental status after relatively minor head injuries or otherwise noncomplicated febrile illnesses. This article reports the case of a child with progressive spastic quadriparesis in whom unconsciousness developed repeatedly as a result of minor head trauma and required prolonged critical-care nursing. Although cognition is believed to be relatively preserved in this disorder, this child developed progressive cognitive decline. A detailed review of the literature is presented along with discussion of the potential mechanisms of neurologic deterioration.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/54. Blue rubber bleb nevus syndrome in a patient with ataxia and dementia.Blue rubber bleb nevus syndrome (BRBNS), an uncommon disorder characterized by cavernous hemangiomas, most often of the skin and gastrointestinal tract, is usually diagnosed during childhood and young adulthood. We made this diagnosis in an octogenarian referred to a geriatric medicine clinic because of concerns about his ability to live independently. ataxia, dementia, focal neurologic signs, and bluish/purplish vascular nodules on his lips, buccal mucosa, tongue, chest, and neck were noted on physical examination. magnetic resonance imaging (MRI) revealed an old left parietal infarction, multiple cavernous hemangiomas most densely concentrated in the subcortical structures and cerebellum, and areas of hemosiderin deposition. skin biopsy findings were consistent with hemangioma. The physical examination, MRI, and skin biopsy made a diagnosis of BRBNS likely. The patient's ataxia, dementia, and other neurologic signs can be explained by previous hemorrhage from the vascular malformations in his brain. Blue rubber bleb nevus syndrome is an uncommon cause of a relatively common geriatric syndrome presentation.- - - - - - - - - - ranking = 0.019166814854448keywords = malformation (Clic here for more details about this article) |
9/54. A case of miller fisher syndrome: atypical findings and therapeutic considerations.The miller fisher syndrome (MFS) is generally considered to be a disease of the peripheral nervous system. In some cases contemporary involvement of the central nervous system has been described (CNS). We report a case in which it was possible to prove involvement of cranial nerves VII, VIII, IX and X and to exclude CNS involvement. We discuss the possible role of early plasmapheresis treatment on disease evolution.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
10/54. Chiari I malformation with syrinx.The Chiari I congenital malformation is characterized by caudal displacement of the cerebellar tonsils through the cervical canal. Although this malformation is often asymptomatic, coexisting syringomyelia can result in neurologic symptoms. We report a case of progressive ataxia with brainstem dysfunction in an adolescent female manifesting a severe Chiari I malformation with syrinx. Chiari decompression 4 years after initial presentation led to rapid improvement in most of her long-term symptoms. This case demonstrates the importance of consideration of Chiari I with syringomyelia in the differential diagnosis of progressive ataxia and brainstem symptoms.- - - - - - - - - - ranking = 0.13416770398113keywords = malformation (Clic here for more details about this article) |
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