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Condition: Ataxia

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Total 492 results found since Jan 2013.

A retrospective analysis of triptan and dhe use for basilar and hemiplegic migraine.
CONCLUSION: In this retrospective study, triptans and DHE were used with no reported, subsequent acute/subacute ischemic vascular events for the abortive treatment of migraines with basilar and hemiplegic-type features. Although the small sample sizes generated theoretical statistical event rates of 4.5% for BM and 23% for HM, there has been no clear evidence that BM and HM carry an actual elevated risk for vascular events compared with migraine with aura. PMID: 27062528 [PubMed - as supplied by publisher]
Source: Headache - April 7, 2016 Category: Neurology Authors: Mathew PG, Krel R, Buddhdev B, Ansari H, Joshi SG, Spinner WD, Klein BC Tags: Headache Source Type: research

Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.
Abstract Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is caused by deficiency of ornithine translocase leading to predominant tissue accumulation and high urinary excretion of ornithine (Orn), homocitrulline (Hcit) and ammonia. Although affected patients commonly present neurological dysfunction manifested by cognitive deficit, spastic paraplegia, pyramidal and extrapyramidal signs, stroke-like episodes, hypotonia and ataxia, its pathogenesis is still poorly known. Although astrocytes are necessary for neuronal protection. Therefore, in the present study we investigated the effects of Orn and ...
Source: Neurochemical Research - May 8, 2016 Category: Neuroscience Authors: Zanatta Â, Rodrigues MD, Amaral AU, Souza DG, Quincozes-Santos A, Wajner M Tags: Neurochem Res Source Type: research

Genes For Niemann-Pick Type C Disease
Niemann-Pick disease is a class of inherited lipid storage diseases. Niemann-Pick Type C disease is an autosomal recessive neurovisceral lipid storage disorder which leads to systemic and neurological abnormalities including ataxia, seizures, and loss of speech. Patients with the disease typically die as children. The biochemical hallmark of Niemann-Pick Type C cells is the abnormal accumulation of unesterified cholesterol in lysosomes, which results in the delayed homeostatic regulation of both uptake and esterification of low density lipoprotein (LDL) cholesterol. Niemann-Pick Type C is characterized by phenotypic variab...
Source: NIH OTT Licensing Opportunities - February 1, 2006 Category: Research Authors: admin Source Type: research

Less thrombolysis in posterior circulation infarction—a necessary evil?
ConclusionsOur study is, to our knowledge, the largest series reporting comprehensively on PCI verified by diffusion‐weighted imaging. PCI patients are younger than ACI and have better outcome. PCI and ACI are equally investigated in the acute setting, but thrombolysis rates remain 50% lower in PCI.
Source: Acta Neurologica Scandinavica - July 6, 2016 Category: Neurology Authors: K. M. Sand, H. Næss, R. M. Nilsen, L. Thomassen, J. M. Hoff Tags: Original Article Source Type: research

Acute Ataxia in Childhood: 11-Year Experience at a Major Pediatric Neurology Referral Center
We categorized the causes of acute ataxia in the pediatric population—referred to the Division of Neurology—at a large, urban pediatric medical center. Of the 120 cases identified over the past 11 years, post-infectious cerebellar ataxia was the most commonly diagnosed (59%), followed by drug intoxication, opsoclonus-myoclonus ataxia syndrome, episodic ataxia, acute cerebellitis, cerebellar stroke, ADEM, meningitis, cerebral vein thrombosis, Leigh’s disease, Miller-Fisher syndrome, and concussion. Among the patients with post-infectious cerebellar ataxia, 85% were 1–6 years old and all had a history...
Source: Journal of Child Neurology - July 11, 2016 Category: Neurology Authors: Thakkar, K., Maricich, S. M., Alper, G. Tags: Original Articles Source Type: research

Less thrombolysis in posterior circulation infarction —a necessary evil?
ConclusionsOur study is, to our knowledge, the largest series reporting comprehensively on PCI verified by diffusion‐weighted imaging. PCI patients are younger than ACI and have better outcome. PCI and ACI are equally investigated in the acute setting, but thrombolysis rates remain 50% lower in PCI.
Source: Acta Neurologica Scandinavica - July 5, 2016 Category: Neurology Authors: K. M. Sand, H. N æss, R. M. Nilsen, L. Thomassen, J. M. Hoff Tags: Original Article Source Type: research

Skew Deviation: Case Report and Review of the Literature.
CONCLUSION: Skew deviation, a not uncommon clinical condition, should be promptly recognized when binocular vertical diplopia cannot be interpreted by trochlearis and oculomotor nerve lesion, myasthenia gravis, or orbital pathology. Maddox rod, cover test, Parks-Bielschowsky three-step, and other tests should help to establish the diagnosis. The prognosis depends on etiology, but it is commonly favorable; the majority of patients recover spontaneously after less than a year. More invasive management options should be discussed thereafter. PMID: 27471949 [PubMed - as supplied by publisher]
Source: Seminars in Ophthalmology - July 31, 2016 Category: Opthalmology Tags: Semin Ophthalmol Source Type: research

Movement disorders in mitochondrial diseases.
Authors: Tranchant C, Anheim M Abstract Mitochondrial diseases (MIDs) are a large group of heterogeneous disorders due to mutations in either mitochondrial DNA (mtDNA) or nuclear DNA (nDNA) genes, the latter encoding proteins involved in mitochondrial function. A multisystem clinical picture that involves several organs, including both the peripheral and central nervous systems, is a common presentation of MID. Movement disorders, even isolated ones, are not rare. Cerebellar ataxia is common in myoclonic epilepsy with ragged red fibers (MERFF) due to mutations in the mitochondrial transfer RNA (tRNA) lysine gene, i...
Source: Revue Neurologique - August 2, 2016 Category: Neurology Tags: Rev Neurol (Paris) Source Type: research

Recurrent Hemorrhagic Venous Infarctions Caused by Thrombosis of a Pontine Developmental Venous Anomaly and Protein S Mutation
A 34-year-old man presented with an acute onset of upbeat nystagmus, slurred speech, and limb and truncal ataxias. The patient had a history of limb ataxia and gait disturbance previously treated as brainstem encephalitis with corticosteroids 3 years previously. Brain magnetic resonance imaging showed pontine developmental venous anomaly (DVA) and hemorrhagic infarction within the drainage territory of the DVA. Three months later, the patient exhibited recurrent limb ataxia, double vision, and numbness of the left side of the body.
Source: Journal of Stroke and Cerebrovascular Diseases - September 18, 2016 Category: Neurology Authors: Yuri Nakamura, Kei-ichiro Takase, Takuya Matsushita, Satoshi Yoshimura, Ryo Yamasaki, Hiroyuki Murai, Kazufumi Kikuchi, Jun-ichi Kira Tags: Case Studies Source Type: research

Neurological manifestations of autosomal dominant familial Alzheimer ’s disease: a comparison of the published literature with the Dominantly Inherited Alzheimer Network observational study (DIAN-OBS)
Publication date: December 2016 Source:The Lancet Neurology, Volume 15, Issue 13 Author(s): Mengxuan Tang, Davis C Ryman, Eric McDade, Mateusz S Jasielec, Virginia D Buckles, Nigel J Cairns, Anne M Fagan, Alison Goate, Daniel S Marcus, Chengjie Xiong, Ricardo F Allegri, Jasmeer P Chhatwal, Adrian Danek, Martin R Farlow, Nick C Fox, Bernardino Ghetti, Neill R Graff-Radford, Christopher Laske, Ralph N Martins, Colin L Masters, Richard P Mayeux, John M Ringman, Martin N Rossor, Stephen P Salloway, Peter R Schofield, John C Morris, Randall J Bateman Background Autosomal dominant familial Alzheimer’s disease (ADAD) is a rare...
Source: The Lancet Neurology - November 7, 2016 Category: Neurology Source Type: research

Man with Ataxia and Aortic Mass
A 59-year-old man with a history of testicular cancer in remission and dyslipidemia undergoing outpatient evaluation for ataxia was referred to our emergency department after a magnetic resonance imaging scan of the brain led to a diagnosis of a cerebellar stroke and comprehensive transthoracic echocardiogram visualized a mass in the proximal ascending aorta. Upon arrival to the emergency department, point-of-care echocardiography confirmed the presence of a mobile echogenic 2.0-  × 1.9-cm mass distal to the aortic valve.
Source: The Journal of Emergency Medicine - November 13, 2016 Category: Emergency Medicine Authors: Michael R. Ehmann, Geoffrey S. Kelly Tags: Visual Diagnosis in Emergency Medicine Source Type: research

Acute hemifield room tilt illusion in cerebral ischaemia
We present 2 cases in the setting of acute vertebrobasilar territory ischaemia. Case 1: A 56-year old man was admitted with acute dyspnoea and new atrial fibrillation. On day two while lying in bed, he developed vomiting and suddenly perceived the curtain to his left was lying horizontal along the floor and felt he was sliding towards the left. MRI brain revealed acute multiterritory infarction (Figure 1). He was commenced on therapeutic anticoagulation and was well on follow-up. Case 2: A 47-year old man awoke with acute vertigo, vomiting and ataxia, exacerbated by head movement with dysarthria and diplopia. The worl...
Source: Journal of Neurology, Neurosurgery and Psychiatry - November 14, 2016 Category: Neurosurgery Authors: Kinsella, J., Lonergan, R., Killeen, R., McGuigan, C. Tags: Drugs: CNS (not psychiatric), Stroke, Ophthalmology, Ear, nose and throat/otolaryngology ABN Annual Meeting, 17-19 May 2016, The Brighton Centre, Brighton Source Type: research

Genes For Niemann-Pick Type C Disease
Niemann-Pick disease is a class of inherited lipid storage diseases. Niemann-Pick Type C disease is an autosomal recessive neurovisceral lipid storage disorder which leads to systemic and neurological abnormalities including ataxia, seizures, and loss of speech. Patients with the disease typically die as children. The biochemical hallmark of Niemann-Pick Type C cells is the abnormal accumulation of unesterified cholesterol in lysosomes, which results in the delayed homeostatic regulation of both uptake and esterification of low density lipoprotein (LDL) cholesterol. Niemann-Pick Type C is characterized by phenotypic variab...
Source: NIH OTT Licensing Opportunities - February 1, 2006 Category: Research Authors: ajoyprabhu3 Source Type: research

Clinical Study of Seven Patients with Infarction in Territories of the Anterior Inferior Cerebellar Artery
The prominent features of anterior inferior cerebellar artery (AICA) infarction are vertigo, cerebellar ataxia, and impaired hearing. The present study investigated neurological characteristics associated with AICA infarction.
Source: Journal of Stroke and Cerebrovascular Diseases - December 14, 2016 Category: Neurology Authors: Katsuhiko Ogawa, Yutaka Suzuki, Keiko Takahashi, Takayoshi Akimoto, Satoshi Kamei, Masayoshi Soma Source Type: research

Treatment of Steroid-Resistant Hashimoto Encephalopathy With Misidentification Delusions and Catatonia
Though thyroid hormonal imbalance is closely connected to a diverse array of mental illnesses, Hashimoto encephalopathy (HE) is particularly rare and poorly understood. HE is characterized by relapsing encephalopathy in association with Hashimoto disease and high titers of antithyroid antibodies.1 The diagnosis of HE has replaced the previous term of “steroid-responsive encephalopathy associated with antibodies to thyroperoxidase” (SREAT).2 The most frequently observed signs in HE include epileptiform seizures resistant to anticonvulsants, stroke-like episodes, focal neurological deficits, ataxia, confusion, presenile ...
Source: Psychosomatics - October 26, 2016 Category: Psychiatry & Psychology Authors: Yujin Lee, Ellen M. House Tags: Case Reports Source Type: research