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Neurology October 2009
Volume 73
Issue 14
| This Week in Neurology(R) |
this week in neurology(r): highlights of the october 6 issue.
Pages: 1083
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| Editorials |
improving stroke prognosis.
- Baird, Alison, FRACP, PhD. Pages: 1084-1085
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autopsy investigation of neurodegenerative disease transplant patients: hindsight is 20/20.
- Morley, James, MD, PhD, Duda, John. Pages: 1086-1087
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| Articles |
prospective validation of the ich score for 12-month functional outcome.
- Hemphill, J, Claude III MD, MAS, Farrant, Mary, RN, MBA, Neill, Terry. Pages: 1088-1094
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Show/Hide Abstract
Background: The ICH Score is a commonly used clinical grading scale for outcome after acute intracerebral hemorrhage (ICH) and has been validated for 30-day mortality, but not long-term functional outcome. The goals of this study were to assess whether the ICH Score accurately stratifies patients with regard to 12-month functional outcome and to further delineate the pace of recovery of patients during the first year post-ICH.Methods: We performed a prospective observational cohort study of all patients with acute ICH admitted to the emergency departments of San Francisco General Hospital and UCSF Medical Center from June 1, 2001, through May 31, 2004. Components of the ICH Score (admission Glasgow Coma Scale score, initial hematoma volume, presence of intraventricular hemorrhage, infratentorial ICH origin, and age) were recorded along with other clinical characteristics. Patients were then assessed with the modified Rankin Scale (mRS) at hospital discharge, 30 days, and 3, 6, and 12 months post-ICH.Results: Of 243 patients, 95 (39%) died during initial acute hospitalization. The ICH Score accurately stratified patients with regard to 12-month functional outcome for various dichotomous cutpoints along the mRS (p < 0.05). Many patients continued to improve across the first year, with a small number of patients becoming disabled or dying due to late events unrelated to the initial ICH.Conclusions: The ICH Score is a valid clinical grading scale for long-term functional outcome after acute intracerebral hemorrhage (ICH). Many ICH patients improve after hospital discharge and this improvement may continue even after 6 months post-ICH.(C)2009AAN Enterprises, Inc.
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pathologic findings in retinal pigment epithelial cell implantation for parkinson disease.
- Farag, Emad, Vinters, Harry, MD, FCAP, Bronstein, Jeff, MD, PhD. Pages: 1095-1102
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Show/Hide Abstract
Background: Attempts at cell-based dopamine replacement therapy in Parkinson disease (PD) have included surgical implantation of adrenal medullary, fetal mesencephalic, and cultured human mesencephalic tissue grafts. Trials involving putamenal implantation of human retinal pigment epithelial (RPE) cells in PD have also been performed. Neuropathologic findings in humans undergoing RPE cell implantation have not heretofore been reported. We describe the brain autopsy findings from a subject enrolled in a clinical trial of RPE cells in gelatin microcarriers for treatment of PD, and suggest factors which may have impacted cell survival.Methods: A 68-year-old man underwent bilateral surgical implantation of 325,000 RPE cells in gelatin microcarriers (Spheramine) but died 6 months after surgery. The left cerebral hemisphere was examined. Routine postmortem formalin fixation was performed and standard, as well as immunohistochemical methods used to highlight senile plaque and Lewy body pathologic changes, iron deposition, cellular inflammation, and reactive astrocytosis in implant regions. Manual cell counts were done of RPE cells.Results: Hematoxylin-eosin and [alpha]-synuclein immunostains confirmed the diagnosis of PD. Needle tracts with matrix material and RPE cells were observed in the context of local inflammatory and astrocytic reactive change. A total of 118 cells were counted (estimated 0.036% survival).Conclusions: Retinal pigment epithelial cells are seen in human brain 6 months postimplantation, but overall survival of implanted cells appeared poor.(C)2009AAN Enterprises, Inc.
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driving under low-contrast visibility conditions in parkinson disease.
- Uc, E, Rizzo, M, Anderson, S, Dastrup, E, Sparks, J, Dawson, J. Pages: 1103-1110
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Show/Hide Abstract
Objective: To assess driving performance in Parkinson disease (PD) under low-contrast visibility conditions.Methods: Licensed, active drivers with mild to moderate PD (n = 67, aged 66.2 +/- 9.0 years, median Hoehn-Yahr stage = 2) and controls (n = 51, aged 64.0 +/- 7.2 years) drove in a driving simulator under high- (clear sky) and low-contrast visibility (fog) conditions, leading up to an intersection where an incurring vehicle posed a crash risk in fog.Results: Drivers with PD had higher SD of lateral position (SDLP) and lane violation counts (LVC) than controls during fog (p < 0.001). Transition from high- to low-contrast visibility condition increased SDLP and LVC more in PD than in controls (p < 0.01). A larger proportion of drivers with PD crashed at the intersection in fog (76.1% vs 37.3%, p < 0.0001). The time to first reaction in response to incursion was longer in drivers with PD compared with controls (median 2.5 vs 2.0 seconds, p < 0.0001). Within the PD group, the strongest predictors of poor driving outcomes under low-contrast visibility conditions were worse scores on measures of visual processing speed and attention, motion perception, contrast sensitivity, visuospatial construction, motor speed, and activities of daily living score.Conclusions: During driving simulation under low-contrast visibility conditions, drivers with Parkinson disease (PD) had poorer vehicle control and were at higher risk for crashes, which were primarily predicted by decreased visual perception and cognition; motor dysfunction also contributed. Our results suggest that drivers with PD may be at risk for unsafe driving in low-contrast visibility conditions such as during fog or twilight.(C)2009AAN Enterprises, Inc.
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spg15 is the second most common cause of hereditary spastic paraplegia with thin corpus callosum.
- Goizet, C, MD, PhD, Boukhris, A, Maltete, D, Guyant-Marechal, L, Truchetto, J, Mundwiller, E, Hanein, S, Jonveaux, P, Roelens, F, Loureiro, J, Godet, E, Forlani, S, Melki, J, MD, PhD, Auer-Grumbach, M, Fernandez, J, Martin-Hardy, P, Sibon, I, MD, PhD, Sole, G, Orignac, I, Mhiri, C, Coutinho, P, MD, PhD, Durr, A, MD, PhD, Brice, A, Stevanin, G. Pages: 1111-1119
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Show/Hide Abstract
Objective: Hereditary spastic paraplegias (HSPs) are very heterogeneous inherited neurodegenerative disorders. Our group recently identified ZFYVE26 as the gene responsible for one of the clinical and genetic entities, SPG15. Our aim was to describe its clinical and mutational spectra.Methods: We analyzed all exons of SPG15/ZFYVE26 gene by direct sequencing in a series of 60 non-SPG11 HSP subjects with associated mental or MRI abnormalities, including 30 isolated cases. The clinical data were collected through the SPATAX network.Results: We identified 13 novel truncating mutations in ZFYVE26, 12 of which segregated at the homozygous or compound heterozygous states in 8 new SPG15 families while 1 was found at the heterozygous state in a single family. Two of 3 splice site mutations were validated on mRNA of 2 patients. The SPG15 phenotype in 11 affected individuals was characterized by early onset HSP, severe progression of the disease, and mental impairment dominated by cognitive decline. Thin corpus callosum and white matter hyperintensities were MRI hallmarks of the disease in this series.Conclusions: The mutations are truncating, private, and distributed along the entire coding sequence of ZFYVE26, which complicates the analysis of this gene in clinical practice. In our series of patients with hereditary spastic paraplegia-thin corpus callosum, the largest analyzed so far, SPG15 was the second most frequent form (11.5%) after SPG11. Both forms share similar clinical and imaging presentations with very few distinctions, which are, however, insufficient to infer the molecular diagnosis when faced with a single patient.(C)2009AAN Enterprises, Inc.
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emergence from minimally conscious state: insights from evaluation of posttraumatic confusion.
- Nakase-Richardson, R, Yablon, S, Sherer, M, Nick, T, Evans, C. Pages: 1120-1126
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Background: Guidelines for defining the minimally conscious state (MCS) specify behaviors that characterize emergence, including "reliable and consistent" functional communication (accurate yes/no responding). Guidelines were developed by consensus because of lack of empirical data.Objective: To evaluate the utility of the operational threshold for emergence from posttraumatic MCS, by determining yes/no accuracy to questions of varied difficulty, including simple orientation questions, using all items from the Yes/No Subscale of the Mississippi Aphasia Screening Test.Method: Prospective observational study of a cohort of responsive patients recovering from traumatic brain injury in an acute inpatient brain injury rehabilitation program.Results: Of the 629 observations from 144 participants, name recognition was the easiest yes/no question, with nonconfused individuals responding with 100% accuracy, whereas only 75% to 78% of confused participants on initial evaluation answered this question correctly. Generalized Estimating Equations analysis revealed that confused participants were more likely to respond inaccurately to all yes/no questions. Nonconfused participants had a reduction in odds of inaccuracy ranging from 45.6% to 99.7% (p = 0.001 to 0.02) depending on the type of yes/no question.Conclusions: Accuracy for simple orientation yes/no questions remains challenging for responsive patients in early recovery from traumatic brain injury. Although name recognition questions are relatively easier than other types of yes/no questions, including situational orientation questions, confused patients still may answer these incorrectly. Results suggest the operational threshold for yes/no response accuracy as a diagnostic criterion for emergence from the minimally conscious state should be revisited, with particular consideration of the type of yes/no questions and the requisite accuracy threshold for responses.(C)2009AAN Enterprises, Inc.
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relative preservation of mmse scores in autopsy-proven dementia with lewy bodies.
- Nelson, P, MD, PhD, Kryscio, R, Jicha, G, MD, PhD, Abner, E, Schmitt, F, Xu, L, Cooper, G, Smith, C, Markesbery, W. Pages: 1127-1133
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Background: Recent studies raised questions about the severity of cognitive impairment associated with dementia with Lewy bodies (DLB). However, there have been few analyses of large, multicenter data registries for clinical-pathologic correlation.Methods: We evaluated data from the National Alzheimer's Coordinating Center registry (n = 5,813 cases meeting initial inclusion criteria) and the University of Kentucky Alzheimer's Disease Center autopsy series (n = 527) to compare quantitatively the severity of cognitive impairment associated with DLB pathology vs Alzheimer disease (AD) and AD+DLB pathologies.Results: Mini-Mental State Examination (MMSE) scores showed that persons with pure DLB had cognitive impairment of relatively moderate severity (final MMSE score 15.6 +/- 8.7) compared to patients with pure AD and AD+DLB (final MMSE score 10.7 +/- 8.6 and 10.6 +/- 8.6). Persons with pure DLB pathology from both data sets had more years of formal education and were more likely to be male. Differences in final MMSE scores were significant (p < 0.01) between pure DLB and both AD+DLB and pure AD even after correction for education level, gender, and MMSE-death interval. Even in cases with extensive neocortical LBs, the degree of cognitive impairment was most strongly related to the amount of concomitant AD-type neurofibrillary pathology.Conclusions: Dementia with Lewy bodies can constitute a debilitating disease with associated psychiatric, motoric, and autonomic dysfunction. However, neocortical Lewy bodies are not a substrate for severe global cognitive impairment as assessed by the Mini-Mental State Examination. Instead, neocortical Lewy bodies appear to constitute or reflect an additive disease process, requiring Alzheimer disease or other concomitant brain diseases to induce severe global cognitive deterioration.(C)2009AAN Enterprises, Inc.
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the video head impulse test: diagnostic accuracy in peripheral vestibulopathy.
- MacDougall, H, Weber, K, McGarvie, L, Halmagyi, G, Curthoys, I. Pages: 1134-1141
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Background: The head impulse test (HIT) is a useful bedside test to identify peripheral vestibular deficits. However, such a deficit of the vestibulo-ocular reflex (VOR) may not be diagnosed because corrective saccades cannot always be detected by simple observation. The scleral search coil technique is the gold standard for HIT measurements, but it is not practical for routine testing or for acute patients, because they are required to wear an uncomfortable contact lens.Objective: To develop an easy-to-use video HIT system (vHIT) as a clinical tool for identifying peripheral vestibular deficits. To validate the diagnostic accuracy of vHIT by simultaneous measures with video and search coil recordings across healthy subjects and patients with a wide range of previously identified peripheral vestibular deficits.Methods: Horizontal HIT was recorded simultaneously with vHIT (250 Hz) and search coils (1,000 Hz) in 8 normal subjects, 6 patients with vestibular neuritis, 1 patient after unilateral intratympanic gentamicin, and 1 patient with bilateral gentamicin vestibulotoxicity.Results: Simultaneous video and search coil recordings of eye movements were closely comparable (average concordance correlation coefficient rc = 0.930). Mean VOR gains measured with search coils and video were not significantly different in normal (p = 0.107) and patients (p = 0.073). With these groups, the sensitivity and specificity of both the reference and index test were 1.0 (95% confidence interval 0.69-1.0). vHIT measures detected both overt and covert saccades as accurately as coils.Conclusions: The video head impulse test is equivalent to search coils in identifying peripheral vestibular deficits but easier to use in clinics, even in patients with acute vestibular neuritis.(C)2009AAN Enterprises, Inc.
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intraepidermal nerve fiber density and its application in sarcoidosis.
- Bakkers, M, Merkies, I, MD, PhD, Lauria, G, Devigili, G, Penza, P, Lombardi, R, Hermans, M, van Nes, S, De Baets, M, MD, PhD, Faber, C, MD, PhD. Pages: 1142-1148
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Background: Intraepidermal nerve fiber density (IENFD) is considered a good diagnostic tool for small fiber neuropathy (SFN).Objectives: To assess stratified normative values for IENFD and determine the reliability and validity of IENFD in sarcoidosis.Methods: IENFD was assessed in 188 healthy volunteers and 72 patients with sarcoidosis (n = 58 with SFN symptoms, n = 14 without SFN symptoms). Healthy controls were stratified (for age and sex), resulting in 6 age groups (20-29, 30-39, [horizontal ellipsis] up to >=70 years) containing at least 15 men and 15 women. A skin biopsy was taken in each participant 10 cm above the lateral malleolus and analyzed in accordance with the international guidelines using bright-field microscopy. Interobserver/intraobserver reliability of IENFD was examined. In the patients, a symptoms inventory questionnaire (SIQ; assessing SFN symptoms) and the Vickrey Peripheral Neuropathy Quality-of-Life Instrument-97 (PNQoL-97) were assessed to examine the discriminative ability of normative IENFD values.Results: There was a significant age-dependent decrease of IENFD values in healthy controls, with lower densities in men compared with women. Good interobserver/intraobserver reliability scores were obtained ([kappa] values >=0.90). A total of 21 patients with sarcoidosis had a reduced IENFD score (<5th percentile; 19 [32.8%] in patients with SFN symptoms, 2 [14.3%] in patients without SFN symptoms). The validity of the normative IENFD values was demonstrated by distinguishing between the SIQ scores and various PNQoL-97 values for the different patient groups.Conclusion: This study provides clinically applicable distal intraepidermal nerve fiber density normative values, showing age- and sex-related differences.(C)2009AAN Enterprises, Inc.
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gamma knife radiosurgery for multiple sclerosis-related trigeminal neuralgia.
- Zorro, O, Lobato-Polo, J, Kano, H, MD, PhD, Flickinger, J, Lunsford, L, Kondziolka, D, MD, MSc. Pages: 1149-1154
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Background: Surgical options for multiple sclerosis (MS) related to trigeminal neuralgia (TN), a severe and disabling pain disorder, include percutaneous rhizotomy, stereotactic radiosurgery, or microsurgical nerve section. Our goal was to evaluate clinical outcomes after gamma knife radiosurgery (GKRS) in patients with MS with TN.Methods: We evaluated clinical outcomes in 37 patients with TN managed over a 12-year period. The maximum TN target dose varied between 70 and 90 Gy. Seventy-eight percent of patients had failed prior surgery. In 9, GKRS was the first procedure. Median follow-up was 56.7 months (range, 6-174). Pain relief was assessed in each patient by physicians who did not participate in the surgery.Results: Eventual complete pain relief (BNI grade I) after GKRS and reasonable pain control (BNI grade I-IIIb) after GKRS were noted in 23 patients (62.1%) and 36 patients (97.3%) at some point in their course. Reasonable pain control (BNI grade I-IIIb) after GKRS was maintained in 82.6%, 73.9%, and 54.0% of patients after 1, 3, and 5 years. Fourteen patients (37.8%) underwent a second or a subsequent procedure for residual or recurrent pain. Eight patients underwent a second GKRS, 5 underwent percutaneous glycerol rhizotomy, and 1 underwent balloon microcompression. The complication rate after GKRS was 5.4% (new onset of nondisabling paresthesias). No patient developed dysesthesias.Conclusions: Gamma knife radiosurgery is the most minimally invasive surgical technique for multiple sclerosis-related trigeminal neuralgia and has low morbidity. For this reason, gamma knife radiosurgery proved to be a satisfactory management strategy for multiple sclerosis-related trigeminal neuralgia.(C)2009AAN Enterprises, Inc.
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| Historical Neurology |
hughlings jackson's suggestion for the treatment of epilepsy.
- York, George, Steinberg, David. Pages: 1155-1158
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John Hughlings Jackson articulated a neurologic method of systematically evaluating the anatomy, physiology, and pathology of every patient with neurologic disease. He used this mode of analysis to develop a theory of the physiology of epilepsy. We examined an example of his method in a newly discovered, unpublished manuscript containing his suggestions for the treatment of epilepsy based on his physiologic ideas. He had his private papers destroyed at the time of his death, but the Rockefeller Library of the University College London Institute of Neurology, Queen Square, contains a collection of his papers probably saved from destruction by his collaborator James Taylor. Among these articles is an 1899 memorandum, labeled "For Private Circulation" and entitled "A Suggestion for the Treatment of Epilepsy." In it, Hughlings Jackson claimed that focal discharging lesions cause both focal and generalized epilepsy, and that the cells in the lesion discharge their energy more easily than normal tissue. Citing microscopic evidence that such lesions are congested and inflamed, and that tuberculin destroys such tissue in the lung, he reasoned that destroying these unstable neurons with tuberculin would improve epilepsy. In this private manuscript, Hughlings Jackson uses an unusually detailed analysis of the pathology, anatomy, and physiology of epilepsy to predict a scientific approach to its treatment.(C)2009AAN Enterprises, Inc.
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| Clinical/Scientific Notes |
core-rod myopathy caused by mutations in the nebulin gene.
- Romero, N, MD, PhD, Lehtokari, V, Quijano-Roy, S, MD, PhD, Monnier, N, Claeys, K, MD, PhD, Carlier, R, Pellegrini, N, Orlikowski, D, Barois, A, Laing, N, Lunardi, J, Fardeau, M, Pelin, K, Wallgren-Pettersson, C, MD, PhD. Pages: 1159-1161
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lingo1 variant increases risk of familial essential tremor.
- Tan, E, Teo, Y, Prakash, K, Li, R, Lim, H, Angeles, D, Tan, L, Au, W, Yih, Y, Zhao, Y. Pages: 1161-1162
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| Video NeuroImages |
ageotropic central positional nystagmus in nodular infarction.
- Nam, Jungmoo, Kim, Seonhye, Huh, Youngeun, Kim, Ji, Soo MD, PhD. Pages: 1163
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| Resident & Fellow Section |
clinical reasoning: a 54-year-old woman with hand dysesthesia: many dimensions to a common problem.
- Vijayan, J, MD, DM, Therimadasamy, A, Lau, T, Wilder-Smith, E, MD, DTM, H, FAMS. Pages: e68-e72
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teaching video neuroimages: excessive grinning in wilson disease.
- Cetlin, R, Rodrigues, G, Pena-Pereira, M, Oliveira, D, Souza, C, Tumas, V, MD, PhD. Pages: e73
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| Correspondence |
cobblestone-like brain dysgenesis and altered glycosylation in congenital cutis laxa, debre type.
- Morava, E, MD, PhD, Wevers, R, Willemsen, M, MD, PhD, Lefeber, D. Pages: 1164-1165
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neurologic improvement after peripheral blood stem cell transplantation in poems.
- Sabatelli, M, Luigetti, M, Laurenti, L, Conte, A, Madia, F, De Matteis, S, Chiusolo, P, Tarnani, M, Sica, S. Pages: 1165-1166
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| Departments: Calendar |
calendar.
Pages: 1167
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| Departments: Changes * People * Comments |
changes * people * comments.
- Joynt, Robert, MD, PhD. Pages: 1168-1170
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| Future Issues |
in the next issue of neurology(r): volume 73, number 15, october 13, 2009.
Pages: A72
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