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Condition: Ataxia

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Total 492 results found since Jan 2013.

Electrolyte imbalance triggering relapse of inflammatory neuropathy
We describe a case of a 71 year old female with a fourteen year history of chronic inflammatory demyelinating polyneuropathy (CIDP) and stable monoclonal gammopathy, normally maintained on three–weekly intravenous immunoglobulin (IVIG) therapy. At her best baseline, she has a normal motor examination and reduced vibration sense only to the ankles. She presented with a four week history of progressive numbness and paresthesiae in all four limbs, reduced balance, a decline in mobility with frequent falls and reduced hand function. The deterioration developed after a week of non–bloody diarrhoea with night sweats ...
Source: Journal of Neurology, Neurosurgery and Psychiatry - October 9, 2013 Category: Neurosurgery Authors: Keshavan, A., Gandhi, S., Lunn, M., Reilly, M. Tags: Neurogastroenterology, Immunology (including allergy), Drugs: CNS (not psychiatric), Multiple sclerosis, Neuromuscular disease, Peripheral nerve disease, Stroke, Hypertension Association of British Neurologists (ABN) joint meeting with the Royal College Source Type: research

Mitochondrial metals as a potential therapeutic target in neurodegeneration
Summary Transition metals are critical for enzyme function and protein folding, but in excess can mediate neurotoxic oxidative processes. As mitochondria are particularly vulnerable to oxidative damage due to radicals generated during ATP production, mitochondrial biometal homeostasis must therefore be tightly controlled to safely harness the redox potential of metal enzyme cofactors. Dysregulation of metal functions is evident in numerous neurological disorders including Alzheimer's disease, stroke, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis and Friedrich's Ataxia. This review describes the m...
Source: British Journal of Pharmacology - November 11, 2013 Category: Drugs & Pharmacology Authors: A Grubman, A R White, J R Liddell Tags: Review Article – Mitochondrial Pharmacology: Energy, Injury & Beyond Themed Issue Source Type: research

Serendipitous Recanalization of Basilar Artery Occlusion
Conclusions: Tenecteplase has enhanced biochemical and pharmacokinetic properties that may be ideal for treatment of basilar artery occlusion and should be further investigated in a randomized clinical trial.
Source: Journal of Stroke and Cerebrovascular Diseases - July 8, 2013 Category: Neurology Authors: Jeffrey A. Switzer, Scott E. Forseen, Askiel Bruno, David C. Hess Tags: Case Reports Source Type: research

Dermatomal Sensory Manifestations in Lateral Medullary Infarction
In conclusion, lateral medullary infarction is an important entity to consider in the differential diagnosis of dermatomal sensory manifestations.
Source: Journal of Stroke and Cerebrovascular Diseases - October 24, 2012 Category: Neurology Authors: Hiroki Hongo, Yasutaka Tanaka, Yoshiaki Shimada, Ryota Tanaka, Nobutaka Hattori, Takao Urabe Tags: Case Reports Source Type: research

Ipsilateral Sensorimotor Deficits in Lateral Medullary Infarction: A Case Report
The patient, a 32-year-old man, presented with sudden onset of occipital headache, vertigo, dysarthria, gait ataxia, right Horner syndrome, numbness of the right hand, and mild right hemiparesis. On magnetic resonance imaging, an acute small infarction was located on the right side of the caudal medulla extending dorsomedially. Magnetic resonance angiography showed severe right vertebral artery stenosis. Lateral medullary infarction associated with ipsilateral sensorimotor deficits in the limb is very rare, and the lesion probably involved the ipsilateral dorsal column or decussating lemniscal fibers and corticospinal fibe...
Source: Journal of Stroke and Cerebrovascular Diseases - December 10, 2012 Category: Neurology Authors: Tetsuro Sameshima, Akio Morita, Yumiko Yamaoka, Yasumitsu Ichikawa Tags: Case Reports Source Type: research

Infarct hemisphere and noninfarcted brain volumes affect locomotor performance following stroke
Conclusion: Individuals with chronic lesions within the right MCA territory, as compared to the left MCA territory, exhibit slower, more asymmetrical gait. For these individuals, larger GM volumes within regions outside of the infarcted vascular territory may help preserve locomotor control.
Source: Neurology - March 10, 2014 Category: Neurology Authors: Chen, I.-H., Novak, V., Manor, B. Tags: MRI, Gait disorders/ataxia, Cerebellum, Basal ganglia, Infarction ARTICLE Source Type: research

What Causes Ataxia?
Discussion Coordination and balance problems are caused by various problems affecting the central and peripheral nervous system. Normal development of a child or weakness of a child are commonly mistaken for true ataxia. Ataxia specifically refers to “…impairment of the coordination of movement without loss of muscle strength.” If it is purely due to abnormalities of the cerebellum then there should be no changes in mental status, sensation or weakness. Sometimes it is difficult to determine if there are abnormalities in other areas. For example, Guillian-Barre often presents with difficulty or clumsy wal...
Source: PediatricEducation.org - March 31, 2014 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news

ATAXIN-2 (ATXN2) Regulates RGS8 Expression Via RNA Interaction (P1.045)
CONCLUSIONS: Mutant ATXN2 reduced RGS8 mRNA steady-state levels, and RGS protein levels to an even greater extent. As we showed interaction of ATXN2 and RGS8 mRNA, our data support a role for ATXN2 in mRNA translation. As knockout of the related RGS6 causes severe ataxia in mice, our results implicate RGS family members in the pathogenesis of polyQ-mediated neurodegeneration in SCA2 and point to new therapeutic targets. Study Supported by: Grants RO1NS33123 and RC4NS073009 from the National Institutes of Neurological Disorders and Stroke to SMP.Disclosure: Dr. Paul has nothing to disclose. Dr. Dansithong has nothing to dis...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Paul, S., Dansithong, W., Pulst, S. Tags: Movement Disorders: Basic Mechanisms Source Type: research

Children with Friedreich Ataxia Undergoing Spinal Fusion Are at Risk for Arrhythmias (P2.037)
CONCLUSIONS: Scoliosis surgery is common amongst hospitalized children with FA, but these comprise a small proportion of all children who undergo spinal fusion. Arrhythmias are more common in FA children undergoing spinal fusion; other comorbidities are similar to non-FA children. FA does not affect length of stay, mean hospital charges, and overall mortality in hospitalizations involving spinal fusion. The increased burden of arrhythmias in FA supports cardiac assessment for children with FA and scoliosis.Disclosure: Dr. Lin has nothing to disclose. Dr. Lynch has received personal compensation for activities with Retrotap...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Lin, K., Lynch, D., Flynn, J., Rossano, J. Tags: Movement Disorders: Friedreich ' s and Other Inherited Ataxias Source Type: research

Paroxysmal Dysarthria and Ataxia after Treatment of Brainstem Encephalitis (P6.053)
CONCLUSIONS: Paroxysmal dysarthria and ataxia is most often recognized as a complication of multiple sclerosis, but it can occur in other neurologic diseases with midbrain lesions near or involving the red nucleus. Effective treatments include carbamazepine, acetazolamide, lamotrigine, and phenytoin.Study Supported by: N/A.Disclosure: Dr. Klaas has nothing to disclose. Dr. Boes has nothing to disclose. Dr. Aksamit has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Klaas, J., Boes, C., Aksamit, A. Tags: Movement Disorders: Miscellaneous Ataxias Source Type: research

Selected Health and Life Style Factors and Time to Phenoconversion in Huntington's Disease (S47.003)
CONCLUSIONS: An association between total lifetime caffeinated soda consumption and earlier HD onset was observed, but this was not seen with other caffeinated beverages and may be spurious. Other life style risk factors associated with PD or AD did not modify time to phenoconversion in HD.SUPPORT: NIH (# 2 R01 HG002449-06), National Human Genome Research Institute, National Institute of Neurological Disorders and StrokeDisclosure: Dr. Tanner has received personal compensation for activities with AbbVie and Adamas Pharmaceuticals as a scientific advisor. Dr. Marder has received personal compensation in an editorial capacit...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Tanner, C., Marder, K., Eberly, S., Biglan, K., Oakes, D., Shoulson, I. Tags: Movement Disorders: Huntington ' s Disease, Ataxia, and Wilson Source Type: research

Approach to acute ataxia in childhood: diagnosis and evaluation.
Abstract Ataxia refers to motor incoordination that is usually most prominent during movement or when a child is attempting to maintain a sitting posture. The first part of the review focuses on the anatomic localization of ataxia - both within the nervous system and without - using a combination of historical features and physical findings. The remainder of the review discusses etiological considerations that vary depending on the age group under consideration. In infancy, certain specific diseases, such as opsoclonus myoclonus ataxia syndrome, must receive special mention because the underlying disease process m...
Source: Pediatric Annals - April 1, 2014 Category: Pediatrics Authors: Sivaswamy L Tags: Pediatr Ann Source Type: research

An unusual cause of cerebellar ataxia in an immunocompromised elderly patient
Conclusions: PVB19 CNS infection should be in the differential as a cause of cerebellar ataxia in immunocompromised patients. Recognition is critical to early institution of appropriate therapy. Our patient showed considerable improvement in ataxia after IVIG therapy.Highlights:
Source: Journal of the Neurological Sciences - March 13, 2014 Category: Neurology Authors: Sheetal Shroff, Carlos Kamiya-Matsuoka, Karin Woodman Tags: Short Communications Source Type: research

Piperacillin/tazobactam‐induced neurotoxicity in a hemodialysis patient: A case report
This report presents a case of a hypertensive and diabetic 67‐year‐old woman in regular hemodialysis, which previously had a stroke. She was hospitalized presenting pneumonia, which was initially treated with cefepime. Two days after treatment, she presented dysarthria, left hemiparesis, ataxia, and IX and X cranial nerves paresis. Computed tomography showed no acute lesions and cefepime neurotoxicity was hypothesized, and the antibiotic was replaced by piperacillin/tazobactam. The neurologic signs disappeared; however, 4 days after with piperacillin/tazobactam treatment, the neurological manifestations returned. A new...
Source: Hemodialysis International - July 1, 2014 Category: Hematology Authors: Precil Diego M. M. Neves, Fernanda M. Freitas, Christiane A. Kojima, Beatriz L. Carmello, Rodrigo Bazan, Pasqual Barretti, Luis C. Martin Tags: Case Report Source Type: research

G.P.157: Clinical and pathological features associated with mutations in MICU1
We present the clinical/pathological features in a cohort of 18 patients. Patients presented between birth and 8years with a mild, relatively static, proximal myopathy associated with high Creatinine Kinase (2000–10,000iu/L), learning difficulties and frequent microcephaly. At follow up (5–28yrs), all remained ambulant but variable extrapyramidal symptoms had developed in the majority by the end of the 1st decade. Other features suggestive of mitochondrial dysfunction included peripheral neuropathy, icthyosis, stroke like episodes, episodic weakness, ataxia and cataracts. Cardiomyopathy was not seen. Serum and CSF lact...
Source: Neuromuscular Disorders - September 4, 2014 Category: Neurology Authors: A.M. Childs, K. Pysden, H. Roper, G. Chow, E.H. Niks, M. Kriek, P.F. Chinnery, D. Lewis-Smith, M. Duchen, G. Szabadkai, C. Logan, E. Sheridan, C. Sewry, F. Muntoni Source Type: research