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Mark Sherer - One of the best experts on this subject based on the ideXlab platform.
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prospective comparison of Acute Confusion severity with duration of post traumatic amnesia in predicting employment outcome after traumatic brain injury
Journal of Neurology Neurosurgery and Psychiatry, 2007Co-Authors: Risa Nakaserichardson, Stuart A. Yablon, Mark ShererAbstract:Background: Measurement of the duration of post-traumatic amnesia (PTA) is common practice, serving as an important index of the severity of traumatic brain injury (TBI) and a predictor of functional outcome. However, controversy exists regarding the nature of PTA; some studies indicate that it is a Confusional state with symptoms that extend beyond disorientation and amnesia. Objective: To evaluate the contribution of the severity of Acute Confusion 1 month after TBI to prediction of employment at 1 year after injury, comparing it with PTA duration. Methods: Prospective study involving 171 participants with complete data, who met the study criteria, from 228 consecutive TBI Model System admissions. Outcome measures included weekly administration of the Delirium Rating Scale-Revised-98 (DelRS-R98) to measure the severity of Acute Confusion. Evaluations closest to 1 month after injury were used for study purposes. Duration of PTA was defined as the interval from injury until two consecutive Galveston Orientation and Amnesia Test scores of ⩾76 were obtained within a period of 24–72 h. Univariable and multivariable logistic regression were used to predict employment status at 1 year after injury. Results: Age, education and DelRS-R98 were significant predictors accounting for 34% of outcome variance. Individuals with greater Confusion severity at 1 month after injury, older age and lower levels of education were less likely to be employed at 1 year after injury. Severity of Confusion was more strongly associated with employment outcome (r s = −0.39) than was PTA duration (r s = −0.34). Conclusions: In addition to demographic indices, severity of Acute Confusion makes a unique contribution to predicting late outcome after TBI.
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multidimensional assessment of Acute Confusion after traumatic brain injury
Archives of Physical Medicine and Rehabilitation, 2005Co-Authors: Mark Sherer, Stuart A. Yablon, Risa Nakasethompson, Samuel T GontkovskyAbstract:Abstract Sherer M, Nakase-Thompson R, Yablon SA, Gontkovsky ST. Multidimensional assessment of Acute Confusion after traumatic brain injury. Objectives To describe the phenomenology of posttraumatic Confusional state (PTCS) and to provide preliminary validation of a new procedure, the Confusion Assessment Protocol (CAP), for assessing PTCS. Design Criterion standard investigation. Setting Inpatient traumatic brain injury (TBI) rehabilitation program. Participants Two consecutive series of patients (n=62, n=93) with TBI admitted for inpatient rehabilitation. Interventions Not applicable. Main outcome measures Clinical diagnosis of delirium based on Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) criteria, classification of posttraumatic amnesia (PTA) based on the Galveston Orientation and Amnesia Test (GOAT), and Disability Rating Scale score at time of rehabilitation hospital discharge. Results Agreement between the diagnosis of PTCS with the CAP and DSM-IV classification of delirium was 87%, and agreement between PTCS and PTA using GOAT criteria was 90%. Patients classified as in PTCS sustained more severe injuries and required longer rehabilitation stays. Confusion status was associated with poorer functional status at rehabilitation discharge. Conclusions The CAP is a brief, structured, repeatable measure of multiple neurobehavioral aspects of PTCS. Confusion status as determined by CAP assessment contributed to prediction of outcome at rehabilitation discharge after adjustment for other potential predictors.
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Acute Confusion following traumatic brain injury
Brain injury, 2004Co-Authors: Risa Nakase-thompson, Mark Sherer, Stuart A. Yablon, Todd G. Nick, Paula T. TrzepaczAbstract:Primary objective: To determine the incidence, duration and symptoms associated with Acute Confusion/delirium among traumatic brain injury (TBI) neuro-rehabilitation admissions.Research design: Prospective evaluation of neurobehavioural impairments following TBI among inpatient neurorehabilitation admissions.Methods and procedures: Eighty-five consecutive TBI model system patients were evaluated using measures of orientation, cognition, motor restlessness and delirium.Main outcomes and results: Fifty-nine individuals met Diagnostic and Statistical Manual of Mental Disorders—Fourth Edition Delirium Diagnostic Criteria (DDC) on initial evaluation and 42 of these resolved delirium during inpatient rehabilitation. Multivariable logistic regression analyses revealed significant unique associations of the Galveston Orientation and Amnesia Test (GOAT), Delirium Rating Scale, Cognitive Test for Delirium and time elapsed since injury with DDC status.Conclusions: Findings indicate that delirium is common among neur...
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article 5 a multidimensional measure of Acute Confusion after traumatic brain injury the Confusion assessment protocol 1
Archives of Physical Medicine and Rehabilitation, 2003Co-Authors: Mark Sherer, Risa Nakasethompson, Stuart A. YablonAbstract:Abstract Objective: To provide preliminary validation of a new measure of posttraumatic Confusional state (PTCS), the Confusion Assessment Protocol (CAP), that assesses 7 key symptoms of PTCS (disorientation, cognitive impairment, restlessness, fluctuation in presentation, nighttime sleep disturbance, decreased daytime arousal, psychotic-type symptoms). Design: Criterion standard investigation. Setting: Inpatient traumatic brain injury (TBI) rehabilitation program. Participants: 62 consecutive patients with moderate or severe TBI admitted for inpatient rehabilitation. Interventions: Not applicable. Main Outcome Measure: Clinical diagnosis of delirium based on Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) criteria. Results: 38 of 44 (86%) patients who were classified as confused on the CAP met the DSM-IV criteria for delirium while only 2 of 18 (11%) patients classified as nonconfused on the CAP met the DSM-IV criteria. Overall agreement between the CAP and the DSM-IV classification was 87%. Conclusions: The CAP is a brief, structured, repeatable measure of multiple aspects of PTCS. Classification of patients as confused versus nonconfused with the CAP showed excellent agreement with clinical diagnosis of delirium using DSM-IV criteria.
Stuart A. Yablon - One of the best experts on this subject based on the ideXlab platform.
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prospective comparison of Acute Confusion severity with duration of post traumatic amnesia in predicting employment outcome after traumatic brain injury
Journal of Neurology Neurosurgery and Psychiatry, 2007Co-Authors: Risa Nakaserichardson, Stuart A. Yablon, Mark ShererAbstract:Background: Measurement of the duration of post-traumatic amnesia (PTA) is common practice, serving as an important index of the severity of traumatic brain injury (TBI) and a predictor of functional outcome. However, controversy exists regarding the nature of PTA; some studies indicate that it is a Confusional state with symptoms that extend beyond disorientation and amnesia. Objective: To evaluate the contribution of the severity of Acute Confusion 1 month after TBI to prediction of employment at 1 year after injury, comparing it with PTA duration. Methods: Prospective study involving 171 participants with complete data, who met the study criteria, from 228 consecutive TBI Model System admissions. Outcome measures included weekly administration of the Delirium Rating Scale-Revised-98 (DelRS-R98) to measure the severity of Acute Confusion. Evaluations closest to 1 month after injury were used for study purposes. Duration of PTA was defined as the interval from injury until two consecutive Galveston Orientation and Amnesia Test scores of ⩾76 were obtained within a period of 24–72 h. Univariable and multivariable logistic regression were used to predict employment status at 1 year after injury. Results: Age, education and DelRS-R98 were significant predictors accounting for 34% of outcome variance. Individuals with greater Confusion severity at 1 month after injury, older age and lower levels of education were less likely to be employed at 1 year after injury. Severity of Confusion was more strongly associated with employment outcome (r s = −0.39) than was PTA duration (r s = −0.34). Conclusions: In addition to demographic indices, severity of Acute Confusion makes a unique contribution to predicting late outcome after TBI.
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multidimensional assessment of Acute Confusion after traumatic brain injury
Archives of Physical Medicine and Rehabilitation, 2005Co-Authors: Mark Sherer, Stuart A. Yablon, Risa Nakasethompson, Samuel T GontkovskyAbstract:Abstract Sherer M, Nakase-Thompson R, Yablon SA, Gontkovsky ST. Multidimensional assessment of Acute Confusion after traumatic brain injury. Objectives To describe the phenomenology of posttraumatic Confusional state (PTCS) and to provide preliminary validation of a new procedure, the Confusion Assessment Protocol (CAP), for assessing PTCS. Design Criterion standard investigation. Setting Inpatient traumatic brain injury (TBI) rehabilitation program. Participants Two consecutive series of patients (n=62, n=93) with TBI admitted for inpatient rehabilitation. Interventions Not applicable. Main outcome measures Clinical diagnosis of delirium based on Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) criteria, classification of posttraumatic amnesia (PTA) based on the Galveston Orientation and Amnesia Test (GOAT), and Disability Rating Scale score at time of rehabilitation hospital discharge. Results Agreement between the diagnosis of PTCS with the CAP and DSM-IV classification of delirium was 87%, and agreement between PTCS and PTA using GOAT criteria was 90%. Patients classified as in PTCS sustained more severe injuries and required longer rehabilitation stays. Confusion status was associated with poorer functional status at rehabilitation discharge. Conclusions The CAP is a brief, structured, repeatable measure of multiple neurobehavioral aspects of PTCS. Confusion status as determined by CAP assessment contributed to prediction of outcome at rehabilitation discharge after adjustment for other potential predictors.
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Acute Confusion following traumatic brain injury
Brain injury, 2004Co-Authors: Risa Nakase-thompson, Mark Sherer, Stuart A. Yablon, Todd G. Nick, Paula T. TrzepaczAbstract:Primary objective: To determine the incidence, duration and symptoms associated with Acute Confusion/delirium among traumatic brain injury (TBI) neuro-rehabilitation admissions.Research design: Prospective evaluation of neurobehavioural impairments following TBI among inpatient neurorehabilitation admissions.Methods and procedures: Eighty-five consecutive TBI model system patients were evaluated using measures of orientation, cognition, motor restlessness and delirium.Main outcomes and results: Fifty-nine individuals met Diagnostic and Statistical Manual of Mental Disorders—Fourth Edition Delirium Diagnostic Criteria (DDC) on initial evaluation and 42 of these resolved delirium during inpatient rehabilitation. Multivariable logistic regression analyses revealed significant unique associations of the Galveston Orientation and Amnesia Test (GOAT), Delirium Rating Scale, Cognitive Test for Delirium and time elapsed since injury with DDC status.Conclusions: Findings indicate that delirium is common among neur...
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article 5 a multidimensional measure of Acute Confusion after traumatic brain injury the Confusion assessment protocol 1
Archives of Physical Medicine and Rehabilitation, 2003Co-Authors: Mark Sherer, Risa Nakasethompson, Stuart A. YablonAbstract:Abstract Objective: To provide preliminary validation of a new measure of posttraumatic Confusional state (PTCS), the Confusion Assessment Protocol (CAP), that assesses 7 key symptoms of PTCS (disorientation, cognitive impairment, restlessness, fluctuation in presentation, nighttime sleep disturbance, decreased daytime arousal, psychotic-type symptoms). Design: Criterion standard investigation. Setting: Inpatient traumatic brain injury (TBI) rehabilitation program. Participants: 62 consecutive patients with moderate or severe TBI admitted for inpatient rehabilitation. Interventions: Not applicable. Main Outcome Measure: Clinical diagnosis of delirium based on Diagnostic and Statistical Manual of Mental Disorders, 4th Edition (DSM-IV) criteria. Results: 38 of 44 (86%) patients who were classified as confused on the CAP met the DSM-IV criteria for delirium while only 2 of 18 (11%) patients classified as nonconfused on the CAP met the DSM-IV criteria. Overall agreement between the CAP and the DSM-IV classification was 87%. Conclusions: The CAP is a brief, structured, repeatable measure of multiple aspects of PTCS. Classification of patients as confused versus nonconfused with the CAP showed excellent agreement with clinical diagnosis of delirium using DSM-IV criteria.
Jose Biller - One of the best experts on this subject based on the ideXlab platform.
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do not forget susac syndrome in patients with unexplained Acute Confusion
Journal of Stroke & Cerebrovascular Diseases, 2015Co-Authors: Michael Star, Rick Gill, Maria Bruzzone, Felipe De Alba, Michael J Schneck, Jose BillerAbstract:Background To report 2 patients with Susac syndrome presenting with Acute Confusion and abnormal neuroimaging and cerebrospinal fluid (CSF) findings, both of whom were initially misdiagnosed. Susac syndrome is a rare, though likely underdiagnosed, endotheliopathy defined clinically by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss (SNHL). Its pathogenesis remains poorly understood. The classic clinical triad may not be fully developed at initial presentation, even at the time magnetic resonance imaging (MRI) demonstrates characteristic central corpus callosum lesions. Methods We describe the patient's clinical course, investigations, management, and follow-up. Results A 57-year-old woman had Acute onset of Confusion and was initially thought to have multiple strokes. MRI demonstrated restricted diffusion involving the splenium of the corpus callosum and CSF showed elevated protein levels. Audiometry showed asymmetric bilateral SNHL. Fluorescein angiography showed multiple bilateral BRAOs. Patient received corticosteroids and intravenous immunoglobulin (IVIG). At 1-month follow-up, patient's Confusion had resolved. The second patient was a 32-year-old man who had Acute onset of Confusion. CSF demonstrated pleocytosis and elevated protein. MRI demonstrated restricted diffusion involving the corpus callosum. Patient was discharged and rehospitalized 20 days later where he was diagnosed with Acute disseminated encephalomyelitis. Patient presented a third time with vision loss. Fluorescein angiography demonstrated bilateral BRAOs. Audiometry noted bilateral SNHL. He received IVIG and discharged on a prednisone taper and mycophenolate mofetil. At 1-month follow-up, patient's Confusion had resolved. Conclusions We report 2 patients evaluated within 1 month of each other who presented with Acute Confusion and were eventually diagnosed with Susac syndrome. Susac syndrome should be considered in young patients with otherwise unexplained Acute onset of Confusion with MRI and CSF changes as described previously.
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do not forget susac syndrome in patients with unexplained Acute Confusion p6 303
Neurology, 2014Co-Authors: Michael Star, Rick Gill, Maria Bruzzone, Felipe De Alba, Michael J Schneck, Jose BillerAbstract:OBJECTIVE: To report two patients with Susac syndrome presenting with Acute Confusion and abnormal neuroimaging and cerebrospinal fluid (CSF) findings, both of whom were initially misdiagnosed. BACKGROUND: Susac syndrome is an unusual autoimmune endotheliopathy clinically characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss (SNHL). Its pathogenesis remains poorly understood. The classic clinical triad may not be fully developed at initial presentation, even at the time MRI demonstrates characteristic central corpus callosum lesions. DESIGN/METHODS: We describe their clinical course, investigations, management, and follow-up. RESULTS: A 57-year-old woman had Acute onset of Confusion and was initially thought to have multiple strokes. MRI demonstrated restricted diffusion involving the splenium of the corpus callosum. CSF showed a protein level of 164mg/dl and 8 white blood cells (79% lymphocytes). Audiology showed asymmetric bilateral SNHL. Fluorescein angiography (FA) showed multiple bilateral BRAOs. Patient received corticosteroids and IVIG. At one month follow up, patient’s Confusion had resolved. The second patient was a 32-year-old man who had Acute onset of Confusion. CSF demonstrated 24 white blood cells (87% lymphocytes) and a protein level of 234mg/dl. MRI demonstrated restricted diffusion involving the corpus callosum. Patient was discharged and re-hospitalized twenty days later where he was diagnosed with Acute disseminated encephalomyelitis. Patient presented a third time with vision loss. FA demonstrated bilateral BRAOs. Audiology noted bilateral SNHL. He received IVIG and discharged on a prednisone taper and mycophenolate mofetil. At one month follow-up, patient’s Confusion had resolved. CONCLUSIONS: We report two patients evaluated within one month of each other who presented with Acute Confusion associated with MRI and CSF abnormalities and were eventually diagnosed with Susac syndrome. Susac syndrome remains under-diagnosed and should be included in the differential diagnosis of patients with unexplained Confusional state associated with CSF pleocytosis/proteinorrachia and MRI callosal abnormalities. Disclosure: Dr. Star has nothing to disclose. Dr. Bruzzone has nothing to disclose. Dr. De Alba has nothing to disclose. Dr. Gill has nothing to disclose. Dr. Schneck has received personal compensation in an editorial capacity for eMedicine. Dr. Schneck has received research support from NIH and Lundbeck. Dr. Biller has received personal compensation in an editorial capacity for the Journal of Stroke and Cerebrovascular Diseases and Frontiers in Neurology.
Kennith Culp - One of the best experts on this subject based on the ideXlab platform.
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clinical profile of Acute Confusion in the long term care setting
Clinical Nursing Research, 2003Co-Authors: Pamela Z. Cacchione, Kennith Culp, Joan Laing, Toni TrippreimerAbstract:Aspects of Acute Confusion (AC) including risk factors, behavior patterns, and outcomes are not well documented in long-term care (LTC) residents. The purpose of this prospective study was to describe the clinical profile of AC in LTC including risk factors, behavior patterns, etiologies, and 3-month outcomes. Seventy-four elderly LTC residents were assessed for AC, depression, and global cognitive impairment. Risk factors associated with AC included hearing deficits, depression, pulmonary disorders, and abnormal serum sodium or potassium levels. Behavior patterns of Acutely confused residents included hyperactive (n = 9, 31%), hypoactive (n = 8, 28%), and mixed (n = 7, 24%). In the majority of the AC cases, the etiology was multifactorial: Infections and dehydration were the most common causes. Residents with AC had very poor 3-month outcomes. Thirty-four percent (n = 10) of the residents with AC died within 3 months of the evaluation. This study highlights the complexity and serious nature of AC in this...
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hydration and Acute Confusion in long term care residents
Western Journal of Nursing Research, 2003Co-Authors: Kennith Culp, Janet C. Mentes, Bonnie J. WakefieldAbstract:Although it is generally appropriate for a healthy adult to consume 2000 to 2500 ml per day, the literature does not address evaluating any standard. The objective here was to develop a weight-based hydration management intervention and evaluate the impact of this on the incidence of Acute Confusion (AC) using an N = 98. The intervention consisted of a fluid intake goal based on 100 ml per kg for the first 10 kg, 50ml/kg for the next 10 kg, and 15 ml for the remaining body weight. The treatment group received instruction and assistance on the fluid goal and the control group received routine care. Measurements included serum electrolytes, bioimpedance analysis, urinalysis, Mini-Mental State Exam, and the NEECHAM. There was no difference in the incidence of AC between treatment and controls, but those individuals with ≥ 90% compliance demonstrated higher ECF volumes and also lower urine leukocyte counts.
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Risk for Acute Confusion in Sensory-Impaired, Rural, Long-Term-Care Elders
Clinical nursing research, 2003Co-Authors: Pamela Z. Cacchione, Kennith Culp, Mary J. Dyck, Joan LaingAbstract:Acute Confusion is a common geriatric syndrome in long-term care (LTC) elders with prevalence rates of 10% to 39%. Sensory impairment, specifically vision and hearing impairment, is even more common in LTC, with prevalence rates of 40% to 90%. The purpose of this study was to investigate the risk relationship between sensory impairment and the development of Acute Confusion in LTC elders. Each resident (N = 114) underwent sensory screening and then was followed for 28 days to monitor for the onset of Acute Confusion. Twenty residents (17.5%) developed Acute Confusion, 60 residents (52.6%) were found to be visually impaired, 49 (44.1%) were hearing impaired, and 28 (24.6%) were found to be dually impaired. Significant relationships between vision impairment, odds ratio (OR) = 3.67, confidence interval (CI) (1.13, 11.92), and dual sensory impairment, OR = 2.88, CI (1.04, 8.26), with the development of Acute Confusion were identified.
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Studying Acute Confusion in long-term care: clinical investigation or secondary data analysis using the minimum data set?
Journal of gerontological nursing, 2001Co-Authors: Kennith Culp, Janet C. Mentes, Eleanor S. McconnellAbstract:Clinical investigations of Acute Confusion have largely been initiated in the Acute care setting, where no uniform patient assessment exists. No reliable estimates of the prevalence of Acute Confusion in long-term care (LTC) residents have been reported. Delirium indicators are present in the nursing home Minimum Data Set (MDS), suggesting that MDS assessments could be used to facilitate studies of Acute Confusion in LTC. Methods to study Acute Confusion in LTC are discussed, with an emphasis on the advantages and disadvantages of using secondary analysis of MDS assessments as one research strategy.
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training of Acute Confusion resource nurses knowledge perceived confidence and role
Journal of Gerontological Nursing, 2001Co-Authors: Carla Gene Rapp, Janet C. Mentes, Bonnie J. Wakefield, Lisa L. Onega, Paula R. Mobily, Mary Kundrat, Jackie Akins, Karen Wadle, Toni Trippreimer, Kennith CulpAbstract:Most nurses function as generalists; however, some function in "expert" roles based on informal training as Resource Nurses. Training usually focuses on assessment and management of a specific problem, with the goal of creating a readily available "expert" for every unit. The primary activity of the Resource Nurse is to provide expert care, education, and consultation for patients, families, and staff. The Iowa-Veterans Affairs Nursing Research Consortium (IVANRC) addressed the need to manage Acutely confused/delirious clients by training staff nurse volunteers (N = 129) from all units of the four Iowa Veterans Affairs facilities to act as unit-based Acute Confusion Resource Nurses (ACRNs). A day-long workshop included didactic content addressing etiology and presentation of Acute Confusion (AC), use of the IVANRC protocol to assess for AC, and basic information on treatment and management of AC. The nurses also participated in an efficacy-based experiential learning program on AC assessment that involved demonstrating assessment of AC and role enactment practice exercises in which ACRNs practiced the assessment. A test of knowledge of AC and perceived level of confidence in assessing Acutely confused patients was administered before and after completion of the program. Paired t tests comparing pre- and posttest scores showed that knowledge and confidence significantly increased for the nurses as a result of their participation in the educational program. Eighteen months later, a second program was conducted to update current ACRNs and train additional RNs to enact this role. Pre- and posttest scores were obtained, with paired t tests showing a significant increase in knowledge for the participants. Twenty-four (49%) of the second program attendees had attended the first program. These participants had significantly higher scores on the second program pretest than those participants who had not attended the previous program, indicating a retention of knowledge from the first program.
Michael Star - One of the best experts on this subject based on the ideXlab platform.
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do not forget susac syndrome in patients with unexplained Acute Confusion
Journal of Stroke & Cerebrovascular Diseases, 2015Co-Authors: Michael Star, Rick Gill, Maria Bruzzone, Felipe De Alba, Michael J Schneck, Jose BillerAbstract:Background To report 2 patients with Susac syndrome presenting with Acute Confusion and abnormal neuroimaging and cerebrospinal fluid (CSF) findings, both of whom were initially misdiagnosed. Susac syndrome is a rare, though likely underdiagnosed, endotheliopathy defined clinically by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss (SNHL). Its pathogenesis remains poorly understood. The classic clinical triad may not be fully developed at initial presentation, even at the time magnetic resonance imaging (MRI) demonstrates characteristic central corpus callosum lesions. Methods We describe the patient's clinical course, investigations, management, and follow-up. Results A 57-year-old woman had Acute onset of Confusion and was initially thought to have multiple strokes. MRI demonstrated restricted diffusion involving the splenium of the corpus callosum and CSF showed elevated protein levels. Audiometry showed asymmetric bilateral SNHL. Fluorescein angiography showed multiple bilateral BRAOs. Patient received corticosteroids and intravenous immunoglobulin (IVIG). At 1-month follow-up, patient's Confusion had resolved. The second patient was a 32-year-old man who had Acute onset of Confusion. CSF demonstrated pleocytosis and elevated protein. MRI demonstrated restricted diffusion involving the corpus callosum. Patient was discharged and rehospitalized 20 days later where he was diagnosed with Acute disseminated encephalomyelitis. Patient presented a third time with vision loss. Fluorescein angiography demonstrated bilateral BRAOs. Audiometry noted bilateral SNHL. He received IVIG and discharged on a prednisone taper and mycophenolate mofetil. At 1-month follow-up, patient's Confusion had resolved. Conclusions We report 2 patients evaluated within 1 month of each other who presented with Acute Confusion and were eventually diagnosed with Susac syndrome. Susac syndrome should be considered in young patients with otherwise unexplained Acute onset of Confusion with MRI and CSF changes as described previously.
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do not forget susac syndrome in patients with unexplained Acute Confusion p6 303
Neurology, 2014Co-Authors: Michael Star, Rick Gill, Maria Bruzzone, Felipe De Alba, Michael J Schneck, Jose BillerAbstract:OBJECTIVE: To report two patients with Susac syndrome presenting with Acute Confusion and abnormal neuroimaging and cerebrospinal fluid (CSF) findings, both of whom were initially misdiagnosed. BACKGROUND: Susac syndrome is an unusual autoimmune endotheliopathy clinically characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAOs), and sensorineural hearing loss (SNHL). Its pathogenesis remains poorly understood. The classic clinical triad may not be fully developed at initial presentation, even at the time MRI demonstrates characteristic central corpus callosum lesions. DESIGN/METHODS: We describe their clinical course, investigations, management, and follow-up. RESULTS: A 57-year-old woman had Acute onset of Confusion and was initially thought to have multiple strokes. MRI demonstrated restricted diffusion involving the splenium of the corpus callosum. CSF showed a protein level of 164mg/dl and 8 white blood cells (79% lymphocytes). Audiology showed asymmetric bilateral SNHL. Fluorescein angiography (FA) showed multiple bilateral BRAOs. Patient received corticosteroids and IVIG. At one month follow up, patient’s Confusion had resolved. The second patient was a 32-year-old man who had Acute onset of Confusion. CSF demonstrated 24 white blood cells (87% lymphocytes) and a protein level of 234mg/dl. MRI demonstrated restricted diffusion involving the corpus callosum. Patient was discharged and re-hospitalized twenty days later where he was diagnosed with Acute disseminated encephalomyelitis. Patient presented a third time with vision loss. FA demonstrated bilateral BRAOs. Audiology noted bilateral SNHL. He received IVIG and discharged on a prednisone taper and mycophenolate mofetil. At one month follow-up, patient’s Confusion had resolved. CONCLUSIONS: We report two patients evaluated within one month of each other who presented with Acute Confusion associated with MRI and CSF abnormalities and were eventually diagnosed with Susac syndrome. Susac syndrome remains under-diagnosed and should be included in the differential diagnosis of patients with unexplained Confusional state associated with CSF pleocytosis/proteinorrachia and MRI callosal abnormalities. Disclosure: Dr. Star has nothing to disclose. Dr. Bruzzone has nothing to disclose. Dr. De Alba has nothing to disclose. Dr. Gill has nothing to disclose. Dr. Schneck has received personal compensation in an editorial capacity for eMedicine. Dr. Schneck has received research support from NIH and Lundbeck. Dr. Biller has received personal compensation in an editorial capacity for the Journal of Stroke and Cerebrovascular Diseases and Frontiers in Neurology.
