N Kumar

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..This report also reviews the literature on the relationship between SS, dural defects, and CSF hypovolemia...

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..This review discusses the role of multimodality imaging in evaluation of superficial siderosis and the therapeutic implications of identified associations...

..The significance of the association between superficial siderosis and idiopathic intracranial hypotension, and potential therapeutic and pathophysiological implications, are the subject of this report...

..Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified...

..An abbreviated version of the original film is provided as Supplementary material...

..Low serum copper can also be observed in some carriers of the Wilson disease gene and aceruloplasminemia. This study was undertaken to determine the clinical significance of low serum copper...

..The site of communication was identified with dynamic CT myelography and the defect was surgically repaired...

..Despite extensive investigations, the cause of bleeding is frequently undetermined...

..The presence of cerebral hemiatrophy and hemorrhagic or cystic components is suggestive. Measurement of serum and cerebrospinal fluid markers such as human chorionic gonadotropin may suggest the diagnosis...

..Cognitive impairment is well described in DM1 and is being recognized in SCA8. The significance of these associations is discussed...

..This article describes patients with copper deficiency myelopathy seen at the Mayo Clinic in Rochester, Minn, and reviews the literature on neurological manifestations of acquired copper deficiency in humans...

..This article presents some "clinical pearls" in the evaluation and management of spinal cord diseases in context of these recent developments...

..The hematologic picture can resemble sideroblastic anemia or myelodysplastic syndrome. Hyperzincemia can be an accompanying abnormality even without exogenous zinc ingestion. The reason for the copper deficiency may not be evident...

..The third sections includes neurologic presentations caused by nutrient deficiencies in the setting of alcoholism. The concluding section addresses neurologic deficiency diseases that have a geographic predilection...

..Myelopathy due to acquired copper deficiency is not a well-recognized entity in humans, although myelopathy due to copper deficiency is well documented in some animal species...

..The preventable and potentially treatable nature of these disorders makes this an important subject. Prognosis depends on prompt recognition and institution of appropriate therapy...

..The significance of these associations and possible similarities between ataxia related to superficial siderosis and ataxia and intraparenchymal hemosiderin is discussed...

..The authors suggest that Rassmusen's encephalitis may in fact be a spectrum with the typical descriptions representing one end. The importance of multimodality imaging in evaluating patients with cerebral hemiatrophy is discussed...

..Scleromyxedema is a dermatologic disorder associated with a monoclonal gammopathy and neurologic manifestations. The reason for occurrence of cutaneous reactions in interferon treated patients is unknown...

..The neurological consequences of acquired copper deficiency in humans are not well described...

..The limited literature on neurological manifestations of acquired copper deficiency suggests that the clinical presentation resembles the myeloneuropathy seen with vitamin B12 deficiency...

..Copper deficiency in ruminants is known to cause an ataxic myelopathy. Copper deficiency as a cause of progressive myelopathy in adults is underrecognized...

..The presence of coexisting hematologic abnormalities should prompt consideration of a bone marrow biopsy. Early diagnosis may direct therapy and lead to a more favorable prognosis...

..A similar finding has also been reported in patients with craniospinal hypotension. This review discusses the role of multitechnique imaging in the work-up of patients with SS and focuses on recent developments...

..They discuss the association between migraine and cerebral hemiatrophy and suggest that the spectrum of acquired cerebral hemiatrophy may be broader than what has been commonly recognized...

..Acquired copper deficiency is not a well-recognized complication of gastric surgery. In Menke's disease a defect in enterocyte transport of absorbed copper results in increased copper content in the duodenal mucosa and hypocupremia...

..Subclinical evidence of brachial plexus involvement may be present. The prognosis for patients with bilateral phrenic neuropathy may be more favorable than reported previously...

..With onset from 40 to 59 years of age, the incidence was higher at 50%. These incidence data may overestimate the functional importance, because patients with minimal or reversible dyskinesias were counted...

..Manganese accumulation may be responsible for the imaging and clinical findings...

..Across all three groups, the neuropsychological profiles suggest frontal and subcortical cognitive impairment, with more widespread abnormalities occurring in the non-welding groups...

..CONCLUSIONS: MPZ mutation His39Pro may be associated with acute-onset neuropathy, early-onset hearing loss and restless legs. The relationship with multiple sclerosis in the proband remains uncertain...

..Increased T1 MRI signal in the basal ganglia is a biologic marker of manganese accumulation. Recent studies have associated welding and parkinsonism, but generally without MRI corroboration...

..Compulsive behaviors provoked by dopamine agonists often go undetected in clinical series, especially if not specifically inquired about...

..This population-based study suggests dyskinesia risk may not be a major concern for most Parkinson disease patients...

..The clinical course can mimic a demyelinating illness. A high index of suspicion and a search for sarcoidosis at extraneural sites are required for an early diagnosis. Steroid treatment is associated with a favorable outcome...

..All 5 patients had neurologic complications of copper deficiency and 3 patients also presented with hematologic abnormalities. We also review the literature regarding copper deficiency in celiac disease...

..Although newer antipsychotics have favourable side effect profiles, there is increasing evidence that EPS develop more frequently than previously thought...

..The purpose of this report is to describe the broad range of visual symptomatology in a patient with HvCJD...

..This association could be either direct with viral vessel infiltration leading to the arteritis or by an indirect dysimmune route. A pathophysiological association with VZV leading to the development of GCA is proposed...

..Herein, we report three idiopathic cases of berign ipsilateral headache with hypoglossal nerve palsy...

..We selected cases on the basis of evidence of brain manganese accumulation, and this may be a crucial component of these syndromes. Further studies are necessary to explore this issue...

..Due to these diagnostic difficulties, POLG-related syndromes are definitively diagnosed based on the presence of deleterious mutations in the POLG gene...

..Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive...

..Copper deficiency is an uncommon but very treatable cause of hematologic abnormalities...

..To describe the clinical features, treatment, and outcomes of patients with antiphospholipid antibody (aPL)-associated chorea...

..Sensitivities and specificities found in the present study validate the use of algorithms to help to prioritize specific SCA gene testing, which will help to reduce costs for gene testing...

..Their structures were elucidated mainly on the basis of IR, 1D, 2D NMR (HMQC, HMBC, COSY), HR-ESI-QTOF-MS and EI mass spectral studies...

..Their structures were elucidated on the basis of 1D- and 2D-NMR techniques and high-resolution mass spectrometry...

..The midline group also experienced more discomfort than the paramedian group. We conclude that, with the patient sitting with an unflexed spine, it is usually possible to insert an epidural catheter with the paramedian approach...

..If confirmed in independent datasets, these results may be of importance in at-risk individuals to distinguish rapid from delayed progression to clinical CD...

..Biocompatibility of polymer was evaluated by injecting microspheres subcutaneously into Sprague-Dawley (SD) rat and no local histopathological abnormalities were found...

..The structures were established on the basis of UV/vis, 1D, 2D NMR (HMQC and HMBC) and ESI-QTOF-MS/MS spectroscopic methods and chemical evidences...