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BACKGROUND: Neurolymphomatosis refers to infiltration of the peripheral nervous system (PNS) by non-Hodgkin lymphoma (NHL). Diagnostic intervals in neurolymphomatosis and factors delaying diagnosis have not been evaluated. We therefore aimed to analyze diagnostic intervals in a large cohort. METHODS: The quality control database at Yale Cancer Center, Section of Neuro-Oncology, was searched for neurolymphomatosis cases diagnosed between 2001 and 2021. Univariate analyses were performed to identify parameters influencing diagnostic intervals. RESULTS: We identified 22 neurolymphomatosis cases including 7 with primary and 15 with secondary disease, which occurred a median (range: 4-144) of 16 months after initial NHL diagnosis. Patients typically presented with painful polyneuropathy (73%), that was asymmetrical and rapidly progressive. Diagnosis was based on PNS biopsy (50%) or integration of neuroimaging findings (50%) with NHL history and diagnostic cerebrospinal fluid examinations. Median interval from symptom onset to diagnosis was 3 months (range: 1-12). Secondary neurolymphomatosis compared to primary disease (median 2 vs. 6 months, p = 0.02), and cases with rapidly-progressive asymmetrical neuropathy as opposed to other presentations (median 2 vs. 6 months; p < 0.001) were diagnosed earlier. Upfront conventional CT compared to other modalities (median 2 vs. 5 months p = 0.04) and nerve root localization as opposed to other disease sites (median 1.5 vs. 4 months; p = 0.04) delayed diagnosis. CONCLUSIONS: NL type and localization, neuropathy course and distribution, and imaging modality selected for initial evaluation influence diagnostic intervals in neurolymphomatosis. Knowledge of this rare entity is critical for early suspicion, and diagnosis.
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OBJECTIVE: The study aimed to measure the validity of International Classification of Diseases, 10th Edition (ICD-10) code F44.5 for functional seizure disorder (FSD) in the Veterans Affairs Connecticut Healthcare System electronic health record (VA EHR). METHODS: The study used an informatics search tool, a natural language processing algorithm and a chart review to validate FSD coding. RESULTS: The positive predictive value (PPV) for code F44.5 was calculated to be 44%. DISCUSSION: ICD-10 introduced a specific code for FSD to improve coding validity. However, results revealed a meager (44%) PPV for code F44.5. Evaluation of the low diagnostic precision of FSD identified inconsistencies in the ICD-10 and VA EHR systems. CONCLUSION: Information system improvements may increase the precision of diagnostic coding by clinicians. Specifically, the EHR problem list should include commonly used diagnostic codes and an appropriately curated ICD-10 term list for 'seizure disorder,' and a single ICD code for FSD should be classified under neurology and psychiatry.
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Epilepsia , Classificação Internacional de Doenças , Humanos , Algoritmos , Registros Eletrônicos de Saúde , Epilepsia/diagnóstico , Processamento de Linguagem NaturalRESUMO
OBJECTIVE: Patients with developmental disabilities (DD) are frequently excluded from acute ischemic stroke (AIS) randomized control trials. We sought to evaluate the impact of having DD on this patient cohort. METHODS: The National Inpatient Sample was analyzed to explore the impact of AIS and treatment on discharge dispositions in patients with DD. Clinical characteristics, treatments, and outcomes were compared to fully-abled patients with AIS. RESULTS: 1,605,723 patients with AIS were identified from 2010-2019, of whom 4094 (0.30%) had a DD. AIS patients with DD were younger (60.31 vs 70.93 years, p < 0.01), less likely to be Caucasian (66.37%vs 68.09%, p = 0.01), and had higher AIS severity (0.63 vs 0.58, p < 0.01). Tissue plasminogen activator (tPA) was administered in 99,739 (6.2%) fully-abled patients and 196 (4.79%) of patients with DD (p < 0.01). Endovascular thrombectomy (EVT) was performed in 21,066 (1.31%) of fully-abled patients and 35 (0.85%) of patients with DD (p < 0.01). The presence of developmental disabilities were predictive of lower rates of tPA (OR:0.71,CI:0.56-0.87,p < 0.01) and EVT (OR:0.24,CI:0.16-0.36,p < 0.01). In a propensity score-matched cohort of all AIS patients who underwent EVT, there was no difference in functional outcome (p = 0.41), in-hospital mortality (0.10), and LOS (p = 0.79). CONCLUSION: AIS patients with DD were less likely to receive tPA and EVT compared to fully-abled patients. Individuals with DD had higher mortality and worse discharge disposition. There was no significant difference in post-EVT outcomes between fully-abled patients and patients with developmental disabilities. In the absence of prospective clinical trials, population based cross-sectional analyses such as the present study provide valuable clinical insight.
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Isquemia Encefálica , Procedimentos Endovasculares , AVC Isquêmico , Acidente Vascular Cerebral , Humanos , Criança , Ativador de Plasminogênio Tecidual/uso terapêutico , Acidente Vascular Cerebral/terapia , Estudos Transversais , AVC Isquêmico/etiologia , Terapia Trombolítica/métodos , Estudos Prospectivos , Deficiências do Desenvolvimento/induzido quimicamente , Deficiências do Desenvolvimento/tratamento farmacológico , Resultado do Tratamento , Trombectomia/métodos , Isquemia Encefálica/cirurgia , Procedimentos Endovasculares/métodosRESUMO
We report the first quantitative systematic review of cerebrovascular disease in coronavirus disease 2019 (COVID-19) to provide occurrence rates and associated mortality. Through a comprehensive search of PubMed we identified 8 cohort studies, 5 case series, and 2 case reports of acute cerebrovascular disease in patients with confirmed COVID-19 diagnosis. Our first meta-analysis utilizing the identified publications focused on comorbid cerebrovascular disease in recovered and deceased patients with COVID-19. We performed 3 additional meta-analyses of proportions to produce point estimates of the mortality and incidence of acute cerebrovascular disease in COVID-19 patients. Patient's with COVID-19 who died were 12.6 times more likely to have a history of cerebrovascular disease. We estimated an occurrence rate of 2.6% (95% confidence interval, 1.2-5.4%) for acute cerebrovascular disease among consecutively admitted patients with COVID-19. While for those with severe COVID-19' we estimated an occurrence rate of 6.5% (95% confidence interval, 4.4-9.6%). Our analysis estimated a rate of 35.5% for in-hospital mortality among COVID-19 patients with concomitant acute cerebrovascular disease. This was consistent with a mortality rate of 34.0% which we obtained through an individual patient analysis of 47 patients derived from all available case reports and case series. COVID-19 patients with either acute or chronic cerebrovascular disease have a high mortality rate with higher occurrence of cerebrovascular disease in patients with severe COVID-19.
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COVID-19 , Transtornos Cerebrovasculares , Humanos , Transtornos Cerebrovasculares/epidemiologia , Transtornos Cerebrovasculares/diagnóstico , COVID-19/complicações , COVID-19/epidemiologia , Teste para COVID-19 , Fatores de Risco , SARS-CoV-2RESUMO
OBJECTIVE: The increased rate of suicide associated with epilepsy has been described, but no studies have reported the rates of suicide and suicide-related behavior (SRB) associated with psychogenic nonepileptic seizures (PNESs). METHODS: This retrospective cohort study analyzed data from October 2002 to October 2017 within Veterans Health Administration services. Of 801,734 veterans, 0.09% had PNES, 1.37% had epilepsy, and 98.5% had no documented seizures. Veterans coded for completed suicide, suicide attempts, and suicidal ideation were identified from electronic health records. The primary measure was the suicide-specific standardized mortality ratio (SMR) based on the number of suicide deaths and CDC national suicide mortality database. A Poisson regression was used to calculate the relative risk (RR) of suicide across groups. RESULTS: A total of 1,870 veterans (mean age [SD] 33.76 [7.81] years) completed suicide. Veterans with PNES (RR = 1.75, 95% confidence interval [CI] 0.84-4.24) and veterans with epilepsy (RR = 2.19, 95% CI 2.10-2.28) had a higher risk of suicide compared with the general veteran population. Veterans with PNES or epilepsy had a higher risk of suicide and SRB if they had comorbid alcohol abuse, illicit drug abuse, major depression, posttraumatic stress disorder, and use of psychotropic medications. Conversely, those who were married or attained higher education were at a decreased risk. The SMR for completed suicide for PNES, epilepsy, and the comparison group was 2.65 (95% CI 1.95-5.52), 2.04 (95% CI 1.60-2.55), and 0.70 (95% CI 0.67-0.74), respectively. CONCLUSIONS: Veterans with seizures (both psychogenic and epileptic) are at a greater risk of death by suicide and SRB than the comparison group. These findings suggest that although the pathophysiology of PNES and epilepsy is different, the negative impact of seizures is evident in the psychosocial outcomes in both groups.
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OBJECTIVE: Neuroprognostication guidelines suggest that early head computed tomography (HCT) might be useful in the evaluation of cardiac arrest (CA) patients following return of spontaneous circulation. We aimed to determine the impact of early HCT, performed within the first 6 h following CA, on decision-making following resuscitation. METHODS: We identified a cohort of initially unconscious post-CA patients at a tertiary care academic medical center from 2012 to 2017. Variables pertaining to demographics, CA details, post-CA care, including neuroimaging and neurophysiologic testing, were abstracted retrospectively from the electronic medical records. Changes in management resulting from HCT findings were recorded. Blinded board-certified neurointensivists adjudicated HCT findings related to hypoxic-ischemic brain injury (HIBI) burden. The gray-white matter ratio (GWR) was also calculated. RESULTS: Of 302 patients, 182 (60.2%) underwent HCT within six hours of CA (early HCT group). Approximately 1 in 4 early HCTs were abnormal (most commonly HIBI changes; 78.7%, n = 37), which resulted in a change in management in nearly half of cases (46.8%, n = 22). The most common changes in management were de-escalation in care [including transition to do not resuscitate status), withholding targeted temperature management, and withdrawal of life sustaining therapy (WLST)]. In cases with radiographic HIBI, mean [standard deviation] GWR was lower (1.20 [0.10] vs 1.30 [0.09], P < 0.001) and progression to brain death was higher (44.4% vs 2.9%; P < 0.001). The inter-rater reliability (IRR) of early HCT to determine presence of HIBI between radiology and three neurointensivists had a wide range (κ 0.13-0.66). CONCLUSION: Early HCT identified abnormalities in 25% of cases and frequently influenced therapeutic decisions. Neuroimaging interpretation discrepancies between radiology and neurointensivists are common and agreement on severity of HIBI on early HCT is poor (k 0.11).
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Parada Cardíaca , Substância Cinzenta , Parada Cardíaca/terapia , Humanos , Prognóstico , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: Immunodeficiency is a major risk factor for primary central nervous system lymphoma (PCNSL), but data on the disease in immunocompromised hosts are scarce. We aimed to define clinical and imaging features and determine prognostic factors for immunodeficiency-associated PCNSL. METHODS: All PCNSL cases seen at Yale-New Haven Hospital between 2002 and 2017 were retrospectively screened for immunodeficiency. For patients with immunosuppression, biopsies were evaluated and clinical data were collected. Predictors of survival were identified using Kaplan-Meier survival analysis and log-rank test. p values < 0.05 were considered significant. RESULTS: 23 patients with immunodeficiencies were identified: eleven on immunosuppressants after solid organ transplantation, seven with human immunodeficiency virus infection, and five on immunosuppressive treatment due to various autoimmune disorders. PCNSL cases were largely Epstein-Barr-Virus positive (78%), histologically classified as diffuse large B cell lymphomas (87%), and showed peripheral contrast enhancement (81%) and corresponding heterogeneous diffusion-weighted imaging patterns (DWI) on magnetic resonance imaging (MRI) (71%). Median overall survival was 31 months. Age > 60 years at diagnosis (p < 0.01), peripheral enhancement of the mass on MRI (p = 0.04), heterogeneous DWI patterns (p = 0.04), and clonal immunoglobulin heavy chain gene rearrangement (IgHR) (p = 0.03) were found to be negative prognostic markers. CONCLUSIONS: Immunodeficiency-associated PCNSL presents with similar clinical, pathological and imaging features. Age > 60 years, clonal IgHR, heterogeneous DWI pattern and peripheral enhancement on MRI may serve as predictors of less favorable outcome.
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Biomarcadores Tumorais/análise , Neoplasias do Sistema Nervoso Central/diagnóstico , Síndromes de Imunodeficiência/complicações , Linfoma não Hodgkin/diagnóstico , Adolescente , Adulto , Idoso , Neoplasias do Sistema Nervoso Central/etiologia , Neoplasias do Sistema Nervoso Central/metabolismo , Criança , Feminino , Seguimentos , Humanos , Processamento de Imagem Assistida por Computador/métodos , Linfoma não Hodgkin/etiologia , Linfoma não Hodgkin/metabolismo , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
BACKGROUND: Functional neurological disorders (FNDs) are neurological symptoms that cannot be explained by an underlying neurological lesion or other medical illness and that do not have clear neuropathological correlates. Psychogenic non-epileptic seizures (PNES) are a common and highly disabling form of FND, characterized by paroxysmal episodes of involuntary movements and altered consciousness that can appear clinically similar to epileptic seizures. PNES are unique among FNDs in that they are diagnosed by video electroencephalographic (VEEG), a well-established biomarker for the disorder. The course of illness and response to treatment of PNES remain controversial. This study aims to describe the epidemiology of PNES in the Department of Veterans Affairs Healthcare System (VA), evaluate outcomes of veterans offered different treatments, and compare models of care for PNES. METHODS: This electronic health record (EHR) cohort study utilizes an informatics search tool and a natural language processing algorithm to identify cases of PNES nationally. We will use VA inpatient, outpatient, pharmacy, and chart abstraction data across all 170 medical centers to identify cases in fiscal years 2002-2018. Outcome measurements such as seizure frequency, emergency room visits, hospital admissions, suicide-related behavior, and the utilization of psychotherapy prior to and after PNES diagnosis will be used to assess the effectiveness of models of care. DISCUSSION: This study will describe the risk factors and course of treatment of a large cohort of people with PNES. Since PNES are cared for by a variety of different modalities, treatment orientations, and models of care, effectiveness outcomes such as seizure outcomes and utilization of emergency visits for seizures will be assessed. Outcome measurements such as seizure frequency, emergency room visits, hospital admissions, suicide-related behavior, and psychotherapy prior to and after PNES diagnosis will be used to assess the effectiveness of models of care.