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Itemized NIHSS subsets predict positive MRI strokes in patients with mild deficits (P5.138)
Conclusion: NIHSS score subsets predict DWI positivity in mild strokes. The presence of neglect or visual field deficits on the NIHSS subsets is most likely to have an MRI correlate even in patients with low NIHSS.Disclosure: Dr. Yaghi has nothing to disclose. Dr. Herber has nothing to disclose. Dr. Willey has nothing to disclose. Dr. Boehme has nothing to disclose. Dr. Andrews has nothing to disclose. Dr. Marshall has nothing to disclose. Dr. Lazar has received personal compensation for activities with Claret Medical, Inc. as a clinical steering committee participant. Dr. Boden-Albala has received personal compensation fo...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Yaghi, S., Herber, C., Willey, J., Boehme, A., Andrews, H., Marshall, R., Lazar, R., Boden-Albala, B. Tags: Cerebrovascular Disease and Interventional Neurology: Clinical Research Tools, Methods, and Innovations Source Type: research

Interrater Reliability of the National Institutes of Health Stroke Scale: Rating by Emergency Room Physicians and Neurologists in a Population-Based Study (P5.140)
CONCLUSIONS:Overall, there was excellent agreement in total NIHSS between ED physicians and neurologist. The NIHSS components gaze, visual fields, ataxia, sensory, and extinction are areas improvement of assessment and documentation in the ED, and should be specifically evaluated in AIS patients whose scores are considered too mild for rt-PA eligibility. Study Supported by: Disclosure: Dr. Katz has nothing to disclose. Dr. Khoury has nothing to disclose. Dr. Alwell has nothing to disclose. Dr. Moomaw has received research support from the National Institutes of Health. Dr. Kleindorfer has received personal compensation for...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Katz, B., Khoury, J., Alwell, K., Moomaw, C., Kleindorfer, D. Tags: Cerebrovascular Disease and Interventional Neurology: Clinical Research Tools, Methods, and Innovations Source Type: research

Exome Sequencing Improves Clinical Diagnosis of Sporadic or Familial Cerebellar Ataxia (S32.003)
Conclusions: This study demonstrates that clinical exome sequencing in patients with adult-onset and sporadic presentations of ataxia is a high yield test, providing a definitive diagnosis in over one-fifth of patients, and suggesting a potential diagnosis in more than one-third to guide additional phenotyping and diagnostic evaluation. Clinical exome sequencing is therefore an appropriate consideration in the routine genetic evaluation of all patients presenting with chronic progressive cerebellar ataxia. Study Supported By: The National Institute of Mental Health, the National Institute for Neurological Disorders and Str...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Fogel, B., Lee, H., Deignan, J., Strom, S., Kantarci, S., Wang, X., Quintero-Rivera, F., Vilain, E., Grody, W., Perlman, S., Geschwind, D., Nelson, S. Tags: Movement Disorders: Tremor, Ataxia, and More Platform Blitz Source Type: research

Predictors of Poor Outcome in Neuroleptic Malignant Syndrome: Retrospective Analysis of Nationwide Inpatient Sample (S32.006)
CONCLUSIONS: In patients admitted for neuroleptic malignant syndrome, every decade increment in age, acute kidney injury, seizures and respiratory failure were positive predictors of poor outcome. Every calendar year increase was a negative predictor of poor outcome. Study Supported by:Disclosure: Dr. Modi has nothing to disclose. Dr. Dharaiya has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Modi, S., Dharaiya, D. Tags: Movement Disorders: Tremor, Ataxia, and More Platform Blitz Source Type: research

Methotrexate Neurotoxicity Mimicking Stroke (P2.296)
Conclusion: It is important for clinicians to recognize methotrexate-induced neurotoxcity as a possible stroke mimic.Disclosure: Dr. Pham has nothing to disclose. Dr. Ermak has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Pham, Q., Ermak, D. Tags: Cerebrovascular Disease and Interventional Neurology: Inflammation and Immunology Source Type: research

Horizontal Gaze Palsy and Progressive Scoliosis (HGPPS); Case Report (P4.163)
Conclusion: HGPPS is an autosomal recessive condition characterized clinically but lack of voluntary horizontal eye movements and progressive scoliosis, radiologically by the absence of facial colliculi, split sign of the pons, and butterfly appearance of the medulla, and pathologically but the lack of pyramidal tract decussation.Disclosure: Dr. Alzahmi has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Alzahmi, F. Tags: Neurological Consequences of Medical Disease Source Type: research

Neuro-Behcet's Syndrome:Case Reports Emphasizing Challenges of Early Diagnosis (P2.075)
CONCLUSIONS: In both cases, NBS was ultimately diagnosed based on characteristic MRI findings, particularly fluctuating brain stem-diencephalic involvement, superimposed on supportive clinical and CSF profiles. Challenges to early diagnosis are highlighted by our cases and by the literature. The differential diagnosis is often broad at onset. Mucocutaenous symptoms can be a clue but may not be present. Awareness of NBS and its neuroimaging correlates is critical to enable timely diagnosis, particularly given that this condition can favorably respond to steroids and steroid-sparing agents.Disclosure: Dr. Feldman has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Feldman, E. Tags: Neurological Consequences of Autoimmune Disease Source Type: research

Clinical Reasoning: A 28-year-old man with progressive gait disturbance and encephalopathy
A 28-year-old man with sickle cell disease presented with 7 months of difficulty walking. Initial examination 3 months prior to admission to our hospital was thought to be consistent with a polyneuropathy. He was areflexic, was unable to stand on toes or heels with decreased sensation on the left foot to light touch and vibration, had difficulty with heel to shin, and was unable to perform tandem gait. Laboratory tests revealed anemia (hemoglobin 7.2 g/dL) and elevated creatinine (1.49 g/dL). HIV, antinuclear antibodies, antineutrophil cytoplasmic antibodies, hepatitis serologies, rapid plasma reagin, thyroid-stimulating h...
Source: Neurology - December 8, 2014 Category: Neurology Authors: Massaro, A. M., Pruitt, A. Tags: Stroke in young adults, MRI, Gait disorders/ataxia, All Oncology RESIDENT AND FELLOW SECTION Source Type: research

Stroke and nonstroke brain attacks in children
Conclusions: Brain attack etiologies differ from adults, with stroke being the fourth most common diagnosis. These findings will inform development of ED clinical pathways for pediatric brain attacks.
Source: Neurology - April 21, 2014 Category: Neurology Authors: Mackay, M. T., Chua, Z. K., Lee, M., Yock-Corrales, A., Churilov, L., Monagle, P., Donnan, G. A., Babl, F. E. Tags: Childhood stroke, All Cerebrovascular disease/Stroke, All Pediatric, Prevalence studies ARTICLE Source Type: research

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

Brain Herniation Due To Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke Like Events (MELAS) With 3256 Mutation Responsive To Decompressive Craniotomy (P6.257)
CONCLUSIONS:In our MELAS case, decompessive craniotomy is life saving for brain herniation due to SLE. Headache refractory to narcotics and increased lactic acid level are early warning signs of acute SLE since MRI can be normal. Steriod might be indicated for acute treatment of cortical edema.Disclosure: Dr. Cui has nothing to disclose. Dr. Schwendimann has received research support from Lundbeck.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Cui, C., Schwendimann, R. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

Amusia: Unique Presentation of Stroke (P6.261)
CONCLUSIONS:Even though music and speech are similar in principle and execution they are functions of different hemispheres of the brain. Unlike aphasia, anatomical localization of amusia has not been established but it is known that right non dominant hemisphere is concerned with musical ability. Impairment of musical ability in the presence of intact speech can be the only presenting finding in stroke. Their identification is the key to early and appropriate treatment.Study Supported by:NoneDisclosure: Dr. Ramchandani has nothing to disclose. Dr. Alsmaan has nothing to disclose. Dr. Schwarz has nothing to disclose. Dr. A...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Ramchandani, C., Alsmaan, F., Schwarz, H., Alkhoury, Z. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

Recurrent Stroke-like Events Preceding the X-linked Charcot-Marie-Tooth Disease Diagnosis (P6.011)
CONCLUSIONS:The transient encephalopathic syndrome with focal paresis, dysarthria, and ataxia is an under recognized early presentation of CMT1X and it continues to be clinically challenging due to its rarity. Thus, this case emphasizes the need for awareness among practitioners to consider CMT1X in the differential diagnosis of acute encephalopathy with symmetric cerebral white matter signal alterations with restricted diffusion to avoid unnecessary work-up and the associated costs.Study Supported by: noneDisclosure: Dr. Kimbason has nothing to disclose. Dr. Anilkumar has nothing to disclose. Dr. Bronov has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Kimbason, T., Anilkumar, A., Bronov, O. Tags: Child Neurology V 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

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