An unusual stroke-like clinical presentation of Creutzfeldt-Jakob disease: acute vestibular syndrome

INTRODUCTION Vertigo and dizziness are common neurological symptoms in general practice. Most patients have benign peripheral vestibular disorders, but some have dangerous central causes. Recent research has shown that bedside oculomotor examinations accurately discriminate central from peripheral lesions in those with new, acute, continuous vertigo/dizziness with nausea/vomiting, gait unsteadiness, and nystagmus, known as the acute vestibular syndrome. CASE REPORT A 56-year-old man presented to the emergency department with acute vestibular syndrome for 1 week. The patient had no focal neurological symptoms or signs. The presence of direction-fixed, horizontal nystagmus suppressed by visual fixation without vertical ocular misalignment (skew deviation) was consistent with an acute peripheral vestibulopathy, but bilaterally normal vestibuloocular reflexes, confirmed by quantitative horizontal head impulse testing, strongly indicated a central localization. Because of a long delay in care, the patient left the emergency department without treatment. He returned 1 week later with progressive gait disturbance, limb ataxia, myoclonus, and new cognitive deficits. His subsequent course included a rapid neurological decline culminating in home hospice placement and death within 1 month. Magnetic resonance imaging revealed restricted diffusion involving the basal ganglia and cerebral cortex. Spinal fluid 14-3-3 protein was elevated. The rapidly progressive clinical course with dementia, ataxia, and myoclonus plus corroborative neuroimaging and spinal fluid findings confirmed a clinicoradiographic diagnosis of Creutzfeldt-Jacob disease. CONCLUSIONS To our knowledge, this is the first report of an initial presentation of Creutzfeldt-Jacob disease closely mimicking vestibular neuritis, expanding the known clinical spectrum of prion disease presentations. Despite the initial absence of neurological signs, the central lesion location was differentiated from a benign peripheral vestibulopathy at the first visit using simple bedside vestibular tests. Familiarity with these tests could help providers prevent initial misdiagnosis of important central disorders in patients presenting vertigo or dizziness

Nystagmus Assessments Documented by Emergency Physicians in Acute Dizziness Presentations: A Target for Decision Support?

Objectives:  Dizziness is a common presenting complaint to the emergency department (ED), and emergency physicians (EPs) consider these presentations a priority for decision support. Assessing for nystagmus and defining its features are important steps for any acute dizziness decision algorithm. The authors sought to describe nystagmus documentation in routine ED care to determine if nystagmus assessments might be an important target in decision support efforts. Methods:  Medical records from ED visits for dizziness were captured as part of a surveillance study embedded within an ongoing population‐based cohort study. Visits with documentation of a nystagmus assessment were reviewed and coded for presence or absence of nystagmus, ability to draw a meaningful inference from the description, and coherence with the final EP diagnosis when a peripheral vestibular diagnosis was made. Results:  Of 1,091 visits for dizziness, 887 (81.3%) documented a nystagmus assessment. Nystagmus was present in 185 of 887 (20.9%) visits. When nystagmus was present, no further characteristics were recorded in 48 of the 185 visits (26%). The documentation of nystagmus (including all descriptors recorded) enabled a meaningful inference about the localization or cause in only 10 of the 185 (5.4%) visits. The nystagmus description conflicted with the EP diagnosis in 113 (80.7%) of the 140 visits that received a peripheral vestibular diagnosis. Conclusions:  Nystagmus assessments are frequently documented in acute dizziness presentations, but details do not generally enable a meaningful inference. Recorded descriptions usually conflict with the diagnosis when a peripheral vestibular diagnosis is rendered. Nystagmus assessments might be an important target in developing decision support for dizziness presentations.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/86927/1/j.1553-2712.2011.01093.x.pd

Rising Annual Costs of Dizziness Presentations to U.S. Emergency Departments

Objectives Dizziness and vertigo account for roughly 4% of chief symptoms in the emergency department ( ED ). Little is known about the aggregate costs of ED evaluations for these patients. The authors sought to estimate the annual national costs associated with ED visits for dizziness. Methods This cost study of adult U.S. ED visits presenting with dizziness or vertigo combined public‐use ED visit data (1995 to 2009) from the National Hospital Ambulatory Medical Care Survey ( NHAMCS ) and cost data (2003 to 2008) from the Medical Expenditure Panel Survey ( MEPS ). We calculated total visits, test utilization, and ED diagnoses from NHAMCS . Diagnosis groups were defined using the Healthcare Cost and Utilization Project's Clinical Classifications Software ( HCUP ‐ CCS ). Total visits and the proportion undergoing neuroimaging for future years were extrapolated using an autoregressive forecasting model. The average ED visit cost‐per‐diagnosis‐group from MEPS were calculated, adjusting to 2011 dollars using the Hospital Personal Health Care Expenditures price index. An overall weighted mean across the diagnostic groups was used to estimate total national costs. Year 2011 data are reported in 2011 dollars. Results The estimated number of 2011 US ED visits for dizziness or vertigo was 3.9 million (95% confidence interval [ CI ] = 3.6 to 4.2 million). The proportion undergoing diagnostic imaging by computed tomography ( CT ), magnetic resonance imaging ( MRI ), or both in 2011 was estimated to be 39.9% (39.4% CT , 2.3% MRI ). The mean per‐ ED ‐dizziness‐visit cost was $1,004 in 2011 dollars. The total extrapolated 2011 national costs were$3.9 billion. HCUP ‐ CCS key diagnostic groups for those presenting with dizziness and vertigo included the following (fraction of dizziness visits, cost‐per‐ ED ‐visit, attributable annual national costs): otologic/vestibular (25.7%; $768;$757 million), cardiovascular (16.5%, $1,489;$941 million), and cerebrovascular (3.1%; $1059;$127 million). Neuroimaging was estimated to account for about 12% of the total costs for dizziness visits in 2011 ( CT scans $360 million, MRI scans$110 million). Conclusions Total U.S. national costs for patients presenting with dizziness to the ED are substantial and are estimated to now exceed $4 billion per year (about 4% of total ED costs). Rising costs over time appear to reflect the rising prevalence of ED visits for dizziness and increased rates of imaging use. Future economic studies should focus on the specific breakdown of total costs, emphasizing areas of high cost and use that might be safely reduced. Resumen Incremento Anual de los Costes de las Atenciones por Mareo en los Servicios de Urgencias de Estados Unidos Objectivos El mareo y el vértigo suman aproximadamente el 4% de los motivos de consulta en el servicio de urgencias ( SU ). Se conoce poco sobre los costes globales de las evaluaciones del SU en estos pacientes. Se buscó estimar los costes anuales nacionales asociados con las visitas al SU por mareo. Metodología Este estudio de costes de visitas al SU de adultos norteamericanos que acudieron con mareo o vértigo combinó los datos públicos de las visitas a los SU (1995 a 2009) recogidos por el National Hospital Ambulatory Medical Care Survey ( NHAMCS ) y los costes (2003 a 2008) recogidos por el Medical Expenditure Panel Survey ( MEPS ). Se calcularon el total de visitas, el uso de pruebas diagnósticas y los diagnósticos del SU del NHAMCS . Los grupos diagnósticos se definieron según el Healthcare Cost and Utilization Project's Clinical Classifications Software ( HCUP ‐ CCS ). Los datos del año 2011 se documentaron en dólares de 2011. El total de visitas y la proporción de neuroimagen llevada a cabo en los futuros años se extrapoló usando un modelo predictivo autorregresivo. La media del coste por visita al SU por grupo diagnóstico del MEPS se calculó, ajustándose a dólares de 2011, mediante el índice de precios de los Hospital Personal Health Care Expenditures. Se utilizó una media ponderada global entre los grupos diagnósticos para estimar los costes totales nacionales. Resultados El número de visitas al SU en Estados Unidos en 2011 por mareo o vértigo fue de 3,9 millones ( IC 95% = 3,6 a 4,2 millones). El porcentaje de pruebas diagnósticas de imagen llevadas a cabo por tomografía computarizada ( TC ), resonancia magnética ( RM ) o ambas en 2011 se estimó en un 39,9% (39,4% TC , 2,3% RM ). La media de coste por visita al SU por mareo fue de 1.004 dólares de 2011. Los costes totales, extrapolados para todo el país, fueron de 3.900 millones de dólares. Los grupos diagnósticos HCUP ‐ CCS para aquéllos que presentaron mareo o vértigo incluyeron los siguientes (proporción de visitas por mareo; coste por visita al SU ; costes anuales nacionales atribuibles): otológico/vestibular (25,7%; 768 dólares; 757 millones de dólares), cardiovascular (16,5%, 1.489 dólares; 941 millones de dólares) y cerebrovascular (3,1%; 1.059 dólares; 127 millones de dólares). Se estimó una suma en la neuroimagen del 12% del total de costes para las visitas por mareo en 2011 (360 millones de dólares para la TC y 110 millones de dólares para la RM ). Conclusiones Los costes totales en Estados Unidos para los pacientes que acuden por mareo al SU son sustanciales, y se estima que sobrepasan en estos momentos los 4.000 millones de dólares por año (aproximadamente un 4% de los costes totales del SU ). El incremento de los costes con el paso del tiempo parece reflejar el crecimiento de la prevalencia de las visitas al SU por mareo y el aumento de porcentajes de utilización de la neuroimagen. Futuros estudios económicos deberían centrarse en el desglose de los costes totales, y hacer énfasis en las áreas de alto uso y coste que pueden ser reducidas sin riesgo.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/99059/1/acem12168.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/99059/2/acem12168-sup-0001-DataSupplementS1.pd

Transient Vestibulopathy in Wallenberg's Syndrome: Pathologic Analysis

Objective: To report an unusual lateral medullary stroke (LMS) associated with transient unidirectional horizontal, nystagmus, and decreased horizontal vestibulo-ocular reflex (h-VOR) gain that mimicked a peripheral vestibulopathy. MRI suggested involvement of caudal medial vestibular nucleus (MVN);however, the rapid resolution of the nystagmus and improved h-VOR gain favored transient ischemia without infarction. Decreased h-VOR gain is expected with peripheral vestibular lesions within the labyrinth or superior vestibular nerve;less frequently lateral pontine strokes involving the vestibular root entry, the vestibular fascicle, or neurons within the MVN may be responsible. The h-VOR is typically normal in LMS. Methods: Clinicopathologic examination of a 61-year-old man with an acute vestibular syndrome (AVS) and left LMS who died 3 weeks after the stroke. Postmortem brainstem analysis was performed. Results: The stroke involved the lateral medulla and pontomedullary junction, near the MVN, sparing the cerebellum and pons. To explain transient vestibular findings there are two possible hypotheses;the first would be that the MVN survived the ischemic process and would be histologically intact, and the second that vestibular afferents in the horizontal semicircular canal were ischemic and recovered after the ischemic process. Neuropathological examination showed a left LMS whose extent matched that seen by imaging. Non-ocular motor signs correlated well with structures affected by the infarction. Neurons and glia within nearby MVN were spared, as predicted by the rapid normalization of the ocular motor signs. Although unlikely, the possibility of transient intralabyrinthine arteriolar ischemia cannot be excluded. Additionally, truncal lateropulsion was due to combined lateral vestibulospinal tract and lateral reticular nucleus infarction. Conclusion: LMS may rarely be associated with an AVS that either represents or mimics a peripheral vestibulopathy. To our knowledge, this is the first neuropathologic examination of the brainstem of an LMS associated with transient vestibular findings occurring in the context of an anterior/posterior (AICA/PICA) cerebellar arterial variant stroke

Small Posterior Fossa Strokes Causing Severe Vertigo: Anatomic Distribution and Clinical Features of the Lacunar Acute Vestibular Syndrome

Small brainstem and cerebellar strokes affecting central vestibular pathways may present clinically with acute vestibular syndrome (AVS). Careful study of acute sub-centimeter lesion locations may help elucidate structure-function relationships. We sought to describe the frequency, anatomic distribution, and clinical manifestations of small posterior fossa strokes in patients with AVS

The Value of Supplemental Maddox Rod Testing in Patients with Diplopia and Strabismus

Binocular diplopia results from ocular misalignment. Anatomic and, ultimately, etiologic diagnosis rests on defining a pattern of ocular separation identified by bedside techniques such as alternate cover or Maddox rod testing. These techniques have different test characteristics but, to our knowledge, have not previously been directly compared

Taking the History From a Dizzy Patient: Why "What Do You Mean By Dizzy?" Should Not Be the First Question You Ask

Traditional teaching instructs clinicians to classify dizziness as vestibular if the patient reports vertigo, cardiovascular if the patient reports presyncope, neurologic if the patient reports disequilibrium, and psychiatric or metabolic if the patient reports non-specific symptoms. It is unknown whether patients can describe their symptom quality well enough to be classified as one of the four "types" of dizziness