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Gomez-Lopez-Hernandez syndrome (GLHS), also known as cerebello-trigeminal-dermal dysplasia, is a neurocutaneous disorder typically presenting in childhood. GLHS is characterized by rhombencephalosynapsis (RES) and partial alopecia, with or without trigeminal anesthesia. We describe a rare case of GLHS in a paucisymptomatic adult who presented with new-onset seizure-like activity. Magnetic resonance imaging revealed partial midline fusion of the cerebellar hemispheres, incomplete development of vermis, and slight medialization of the dentate nuclei: all consistent with the diagnosis of RES. Radiographic evidence combined with partial alopecia, truncal ataxia, and muscular hypotonia are suggestive GLHS diagnosis. Our report not only highlights the importance of maintaining GLHS on the differential for new-onset seizure-like activity, but also demonstrates how patients with GLHS may be minimally symptomatic and diagnosed in adulthood. Lay Summary: The Gomez-Lopez-Hernandez syndrome (GLHS), or cerebellotrigeminal-dermal dysplasia, is a rare condition that affects both the nervous system and the skin. It involves abnormal development of the brain, partial alopecia [thinning of hair], and loss of sensation in the face. One specific brain malformation, called rhombencephalosynapsis (RES), results from abnormal formation of the cerebellum and is seen in GHLS.Both RES and GLHS present early in childhood, and cases presenting later in life are exceptionally rare. Here we describe a young adult with RES and GLHS whose normal development and mild clumsiness eluded recognition by doctors until early adulthood when she presented with a single seizure.
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OBJECTIVE: Headache relief after anterior cervical spine surgery has been reported. No study, however, has followed patients out to 10 years to assess the durability of headache improvement. The authors analyzed a group of patients with a 10-year follow-up after one- or two-level cervical disc arthroplasty (CDA) from an FDA investigational device exemption (IDE) study. METHODS: The authors performed a post hoc analysis of 189 patients treated with CDA from the 9 highest enrolling sites in a prospective multicenter randomized US FDA IDE clinical trial. Patients had one- or two-level CDA at contiguous levels from C3 to C7 using the Mobi-C device. The authors evaluated headache scores from the headache section of the Neck Disability Index (NDI), along with associated demographic variables (age, sex, race, ethnicity, and BMI). Preoperative and 10-year postoperative headache scores were analyzed. Primary analysis was conducted via the Wilcoxon rank-sum test, followed by univariate and multivariable logistic regression. RESULTS: After accounting for age, BMI, race, ethnicity, and sex, there was sustained headache improvement 10 years after CDA (p = 0.04). Preoperatively, the median NDI score was 3.00 (IQR 1.00-4.00) and after 10 years it was 1.00 (IQR 0.00-2.00), with a decrease in the NDI score by 1.00 point (95% CI 0.00-2.00, p = 0.04). For one-level CDA, the median NDI score was 3.00 (IQR 1.00-4.00) preoperatively but 1.00 (IQR 0.00-2.00) at 10 years, with an estimated reduction in the NDI score of 1 point (95% CI 1.00-2.00, p < 0.0001). For two-level CDA, the median NDI score was 3.00 (IQR 1.75-4.00) preoperatively and 1.00 (IQR 0.00-2.00) at 10 years, with an estimated reduction in the NDI score of 1 point (95% CI 1.00-2.00, p < 0.0001). CONCLUSIONS: Headache relief provided by cervical CDA, for symptomatic C3-7 cervical spondylosis, was sustained even 10 years after surgery. There was no difference in headache improvement between the one- and two-level CDA groups, or among BMI, sex, race, and ethnicity strata.
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Degeneração do Disco Intervertebral , Fusão Vertebral , Substituição Total de Disco , Humanos , Degeneração do Disco Intervertebral/cirurgia , Resultado do Tratamento , Estudos Prospectivos , Vértebras Cervicais/cirurgia , Discotomia/efeitos adversos , Substituição Total de Disco/efeitos adversos , Artroplastia , Cefaleia/etiologia , Cefaleia/cirurgia , Fusão Vertebral/efeitos adversosRESUMO
OBJECTIVES AND BACKGROUND: Hemifacial spasm (HFS) is a disabling condition that imposes significant burden upon patients. Microvascular decompression (MVD) surgery is the most effective and long-lasting treatment for HFS, but outcomes following this surgery may vary based on a variety of clinical and operative factors. A more thorough understanding of the variables that impact patient outcome after MVD surgery is needed. METHODS: A systematic review and meta-analysis of Medline, Embase, and Central was conducted (n = 2108 screened; n = 86 included) with the goal of determining the impact of the following variables on outcome: duration of disease, geographic location, intraoperative use of an endoscope, and intraoperative finding of single versus multi-vessel neurovascular compression. RESULTS: Most cases of hemifacial spasm occur on the left side (53.9%, p < 0.001) and are more common in women than men (66.5% versus 33.5%, p < 0.0001). The offending vessel frequencies were: 40.8% anterior inferior cerebellar artery [AICA], 24.9% posterior inferior cerebellar artery [PICA], 17.2% multiple vessels, and 4.7% vertebral artery [VA]. Multiple vessel combinations involved: 26.5% PICA + AICA, 24.6% PICA + VA, 23.1% AICA + VA, and 4.7% AICA + PICA + VA. Relative to the Americas, AICA was less frequent in Europe (p = 0.005), while PICA more frequent in Europe (p = 0.009) and Asia (p < 0.0001). With endoscope assistance, frequency of multiple vessels identified was 31.7% (versus 14.7% with non-endoscopic, p = 0.005), and 27.4% for AICA (43.5% with non-endoscopic, p = 0.003). Spasm improvement was 94.1% near discharge and 96.0% at maximum follow-up. Complications occurred in 16.5% of cases, with spasm recurrence in 2.4%. Greatest frequency of spasm improvement (p < 0.0001) and lowest spasm recurrence rates (p = 0.0005) were reported in series from Asia. For every additional month of pre-operative spasm, the effect size of post-operative improvement decreased (p = 0.04). With every subsequent postoperative month, the effect size of spasm improvement increased (p = 0.0497). The frequency of spasm improvement was significantly higher in series published after 2005 (94.4% versus 97.4%, p = 0.005). CONCLUSION: Clinical outcomes following MVD for HFS have improved since 2005. Consideration should be given to earlier operation (shorter disease duration) and use of an endoscope may increase detection of multiple offending vessels. Further studies are needed to understand regional differences in culprit vessel incidence and surgical outcomes in the Americas, Europe, and Asia.
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Espasmo Hemifacial , Cirurgia de Descompressão Microvascular , Doenças Vasculares , Masculino , Humanos , Feminino , Espasmo Hemifacial/cirurgia , Espasmo Hemifacial/etiologia , Resultado do Tratamento , Estudos Retrospectivos , Artéria Vertebral/diagnóstico por imagem , Artéria Vertebral/cirurgia , Doenças Vasculares/cirurgiaRESUMO
Pressure control cam dislocation is a rare finding in patients with a programmable shunt valve that should be considered when evaluating patients with signs and symptoms of shunt malfunction. The objective of this paper is to review the mechanism, clinical presentation, and radiographic findings associated with pressure control cam (PCC) dislocation, in addition to presenting a novel case to add to the scarce literature on this topic. A systematic review of the literature were performed using PubMed, Embase, and Cochrane from database inception. PCC dislocation is exceedingly rare and can present with no symptoms, positional headache, neck pain, nausea, or vomiting. Skull x-ray findings demonstrate a clear black "X" at the distal end of the valve, due to the PCC disarticulation from atop the base plate of the plastic valve housing. Intraoperatively, a "Y"-shaped crack atop the plastic valve housing may be present and the PCC may be fully separated from the shunt or found at the distal end of the plastic valve housing. Prior reports of dislocation of the PCC have occurred 7-9 years after implantation, with inciting events including direct trauma, programmable valve adjustment, and utilization 3-Tesla magnetic resonance image scan.
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To promote health equity within the United States (US), randomized clinical trials should strive for unbiased representation. Thus, there is impetus to identify demographic disparities overall and by disease category in US clinical trial recruitment, by trial phase, level of masking, and multi-center status, relative to national demographics. A systematic review and meta-analysis were conducted using MEDLINE, Embase, CENTRAL, and ClinicalTrials.gov, between 01/01/2008 to 12/30/2019. Clinical trials (N = 5,388) were identified based on the following inclusion criteria: study type, location, phase, and participant age. Each clinical trial was independently screened by two researchers. Data was pooled using a random-effects model. Median proportions for gender, race, and ethnicity of each trial were compared to the 2010 US Census proportions, matched by age. A second analysis was performed comparing gender, race, and ethnicity proportions by trial phase, multi-institutional status, quality, masking, and study start year. 2977 trials met inclusion criteria (participants, n = 607,181) for data extraction. 36% of trials reported ethnicity and 53% reported race. Three trials (0.10%) included transgender participants (n = 5). Compared with 2010 US Census data, females (48.3%, 95% CI 47.2-49.3, p < 0.0001), Hispanics (11.6%, 95% CI 10.8-12.4, p < 0.0001), American Indians and Alaskan Natives (AIAN, 0.19%, 95% CI 0.15-0.23, p < 0.0001), Asians (1.27%, 95% CI 1.13-1.42, p < 0.0001), Whites (77.6%, 95% CI 76.4-78.8, p < 0.0001), and multiracial participants (0.25%, 95% CI 0.21-0.31, p < 0.0001) were under-represented, while Native Hawaiians and Pacific Islanders (0.76%, 95% CI 0.71-0.82, p < 0.0001) and Blacks (17.0%, 95% CI 15.9-18.1, p < 0.0001) were over-represented. Inequitable representation was mirrored in analysis by phase, institutional status, quality assessment, and level of masking. Between 2008 to 2019 representation improved for only females and Hispanics. Analysis stratified by 44 disease categories (i.e., psychiatric, obstetric, neurological, etc.) exhibited significant yet varied disparities, with Asians, AIAN, and multiracial individuals the most under-represented. These results demonstrate disparities in US randomized clinical trial recruitment between 2008 to 2019, with the reporting of demographic data and representation of most minorities not having improved over time.
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Etnicidade , Promoção da Saúde , Feminino , Humanos , Estados Unidos , Adulto , Ensaios Clínicos Controlados Aleatórios como Assunto , População Branca , HavaíRESUMO
Introduction: Better characterizing moyamoya disease (MMD) from ischemic strokes of other etiologies may facilitate earlier diagnosis by raising suspicion for a diagnostic work-up. Methods: To identify associated variables, MMD cases (n = 12) were compared against three sets of controls: age-, sex-, and race-matched controls of patients with general neurological disorders (n = 48), unmatched general controls (n = 48), and unmatched non-MMD ischemic stroke controls (n = 48). Results: MMD patients were 32 years (p < 0.0001) younger than ischemic stroke controls. Relative to non-MMD ischemic strokes, MMD patients had greater odds of presenting with visual field defects (OR: 9.13, p = 0.09) or dizziness (OR: 9.13, p = 0.09), as well as being female (OR: 8.04, p = 0.008), Asian (OR: 3.68, p = 0.087), employed (OR: 6.96, p = 0.02), having migraines (OR: 21.61, p = 0.005), epilepsy (OR: 6.69, p = 0.01), insomnia (OR: 8.90, p = 0.099), and a lower Charlson Comorbidity Index (CCI; p = 0.002). Patients with MMD, compared to non-MMD ischemic strokes, also had a 4.67 kg/ m 2 greater body mass index (BMI) and larger odds (OR relative to normal BMI: 21.00, p = 0.03) of being from obesity class III (>40 kg/ m 2 ), yet reduced odds of coronary artery disease (OR: 0.13, p = 0.02). Relative to general controls, MMD patients had greater odds of diabetes mellitus type 2 (OR: 10.07, p = 0.006) and hypertension (OR: 7.28, p = 0.004). Conclusion: MMD not only has a unique clinical presentation from other ischemic strokes, but also unique comorbidities, which may facilitate earlier work-up and treatment.
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INTRODUCTION: Patients with psychogenic non-epileptic seizures (PNES) experience significant morbidity and early mortality, secondary to delayed diagnosis. Better characterizing risk factors and exploring how PNES differentially affects sex and racial strata may facilitate earlier diagnosis. METHODS: From a Hawai'i neuroscience institution, 101 PNES patients were investigated in relation to sociodemographic and medical comorbidities. Cases were compared to 202 sex-, age-, and race-matched controls-representing patients with neurological disorders (general controls)-, as well as 404 unmatched epilepsy controls. RESULTS: Relative to general controls, PNES patients had increased odds (p < 0.05) of being: female, younger age, Native Hawaiian or other Pacific Islander (NHPI), suburban origin, from the lowest income quartile, Medicaid beneficiaries, homeless, current/former smoker, illicit drug users (marijuana, opioids/narcotics, polysubstance abuse), have anxiety, depression, post-traumatic stress disorder, bipolar disorder, traumatic history, World Health Organization obesity class 3, traumatic brain injury, epilepsy, and somatoform disorder. In relation to epilepsy controls, PNES patients exhibited increased odds of being: employed, having attention-deficit/hyperactivity disorder, asthma, migraines, and chronic pain. Relative to females, male PNES patients exhibited increased odds of military insurance, diabetes mellitus type 2, and hypertension. Relative to Whites, the NHPI and Asian PNES patients presented increased odds of asthma, migraines, chronic pain, gastroesophageal reflux disease, and thyroid disease. Per multivariable logistic regression, anxiety was the only consistent predictor of PNES across all sex and race strata. CONCLUSION: Predictors of PNES's vary amongst the strata of race and sex. Lower socioeconomic status, along with several psychiatric and medical comorbidities, could increase a clinician's suspicion for earlier medical workup and diagnosis of PNES.
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Asma , Dor Crônica , Epilepsia , Transtornos de Enxaqueca , Estudos de Casos e Controles , Eletroencefalografia , Epilepsia/diagnóstico , Epilepsia/epidemiologia , Feminino , Humanos , Masculino , Convulsões Psicogênicas não Epilépticas , Estudos Retrospectivos , Fatores de Risco , Convulsões/diagnóstico , Convulsões/epidemiologiaRESUMO
INTRODUCTION: Growth of some pituitary tumors is driven by hormones which vary in concentration along the lines of patient socioeconomic status. Thus, pituitary tumors may exhibit disparities in incidence upon stratification by socioeconomic variables. Exploring for these disparities could provide direction in tumor etiology elucidation and identification of healthcare inequalities. METHODS: To investigate pituitary adenoma and carcinoma incidence (per 100,000) with respect to sex, age, income, residence, and race/ethnicity, we searched the largest American administrative dataset (1997-2016), the National (Nationwide) Inpatient Sample (NIS), which surveys 20% of United States (US) discharges. RESULTS: Annual national incidence was 2.80 for adenomas and 0.046 for carcinomas. For adenomas, males had an incidence of 2.63, similar (p = 0.17) to females at 2.78; likewise, for carcinomas, males had a statistically equivalent (p = 0.24) incidence at 0.051 to females at 0.041. Amongst age groups, for adenomas incidence progressively rose, peaking 65-84 years old (6.12), before declining. For adenomas and carcinomas respectively, patients with low income had an incidence of 2.66 and 0.044, similar (p = 0.11; p = 0.72) to the 3.01 and 0.041 of middle/high income patients. Incidence was greatest for adenomas amongst urban centers (3.47), followed by rural (3.16) and suburban (3.01) communities. Examining race/ethnicity (p = 0.0000016), for adenomas, incidences amongst Blacks, Asian/Pacific Islanders, Hispanics, and Whites were as follows, respectively: 3.64, 2.57, 2.54, 2.44. Annually, incidence for adenomas was increasing (τ = 0.63, p = 0.00021), but decreasing (τ = -0.60, p = 0.00085) for carcinomas. Specifically, for carcinomas incidence was only decreasing for females and the middle/high income. CONCLUSION: In the US, time-enduring healthcare disparities were identified for pituitary adenomas and carcinomas, against the background of sociodemographic strata. For carcinomas, annual incidence was declining only for middle/high income patients and females, which supporting prior investigations that low income patients and males are experiencing barriers to definitive treatment for pituitary adenomas. Incidence was also found to be greatest Blacks and urban residents.
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Adenoma , Carcinoma , Neoplasias Hipofisárias , Adenoma/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Etnicidade , Feminino , Humanos , Masculino , Neoplasias Hipofisárias/epidemiologia , Classe Social , Estados Unidos/epidemiologiaRESUMO
Mammalian motor systems adapt to the demands of their environment. For example, muscle fibre types change in response to increased load or endurance demands. However, for adaptations to be effective, motoneurons must adapt such that their properties match those of the innervated muscle fibres. We used a rat model of chronic functional overload to assess adaptations to both motoneuron size and a key modulatory synapse responsible for amplification of motor output, C-boutons. Overload of extensor digitorum longus (EDL) muscles was induced by removal of their synergists, tibialis anterior muscles. Following 21 days survival, EDL muscles showed an increase in fatigue resistance and a decrease in force output, indicating a shift to a slower phenotype. These changes were reflected by a decrease in motoneuron size. However, C-bouton complexes remained largely unaffected by overload. The C-boutons themselves, quantified by expression of vesicular acetylcholine transporter, were similar in size and density in the control and overload conditions. Expression of the post-synaptic voltage-gated potassium channel (KV 2.1) was also unchanged. Small conductance calcium-activated potassium channels (SK3) were expressed in most EDL motoneurons, despite this being an almost exclusively fast motor pool. Overload induced a decrease in the proportion of SK3+ cells, however, there was no change in density or size of clusters. We propose that reductions in motoneuron size may promote early recruitment of EDL motoneurons, but that C-bouton plasticity is not necessary to increase the force output required in response to muscle overload.
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Canais de Potássio Cálcio-Ativados , Canais de Potássio de Abertura Dependente da Tensão da Membrana , Animais , Mamíferos , Neurônios Motores/fisiologia , Músculo Esquelético/inervação , Ratos , Proteínas Vesiculares de Transporte de AcetilcolinaRESUMO
The clinical indications and added value of obtaining MRI in the acute phase of spinal cord injury (SCI) remain controversial. This review aims to critically evaluate evidence regarding the role of MRI to influence decision-making and outcomes in acute SCI. A systematic review and meta-analysis were performed according to PRISMA methodology to identify studies that address six key questions (KQs) regarding diagnostic accuracy, frequency of abnormal findings, frequency of altered decision-making, optimal timing, and differences in outcomes related to obtaining an MRI in acute SCI. A total of 32 studies were identified that addressed one or more KQs. MRI showed no adverse events in 156 patients (five studies) and frequently identified cord compression (70%, 12 studies), disc herniation (43%, 16 studies), ligamentous injury (39%, 13 studies), and epidural hematoma (10%, two studies), with good diagnostic accuracy (seven comparative studies) except for fracture detection. MRI findings often altered management, including timing of surgery (78%, three studies), decision to operate (36%, 15 studies), and surgical approach (29%, nine studies). MRI may also be useful to determine the need for instrumentation (100%, one study), which levels to decompress (100%, one study), and if reoperation is needed (34%, two studies). The available literature consistently concluded that MRI was useful prior to surgical treatment (13 studies) and after surgery to assess decompression (two studies), but utility before/after closed reduction of cervical dislocations was unclear (three studies). One study showed improved outcomes with an MRI-based protocol but had a high risk of bias. Heterogeneity was high for most findings (I2 > 0.75). MRI is safe and frequently identifies findings alter clinical management in acute SCI, although direct evidence of its impact on outcomes is lacking. MRI should be performed before and after surgery, when feasible, to facilitate improved clinical decision-making. However, further research is needed to determine its optimal timing, effect on outcomes, cost-effectiveness, and utility before and after closed reduction.
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Spinal cord injury (SCI) is a devasting condition with no reliable treatment. Spina bifida is the most common cause of congenital SCI. Cell-based therapies using mesenchymal stem/stromal cells (MSCS) have been largely utilized in SCI. Several clinical trials for acquired SCI use adult tissue-derived MSC sources, including bone-marrow, adipose, and umbilical cord tissues. The first stem/stromal cell clinical trial for spina bifida is currently underway (NCT04652908). The trial uses early gestational placental-derived mesenchymal stem/stromal cells (PMSCs) during the fetal repair of myelomeningocele. PMSCs have been shown to exhibit unique neuroprotective, angiogenic, and antioxidant properties, all which are promising applications for SCI. This review will summarize the unique properties and current applications of PMSCs and discuss their therapeutic role for acquired SCI.
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Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais/citologia , Placenta/citologia , Traumatismos da Medula Espinal/congênito , Traumatismos da Medula Espinal/terapia , Bioengenharia , Ensaios Clínicos como Assunto , Feminino , Humanos , GravidezRESUMO
INTRODUCTION: Given that the success of vaccines against coronavirus disease 2019 (COVID-19) relies on herd immunity, identifying patients at risk for vaccine hesitancy is imperative-particularly for those at high risk for severe COVID-19 (i.e., minorities and patients with neurological disorders). METHODS: Among patients from a large neuroscience institute in Hawaii, vaccine hesitancy was investigated in relation to over 30 sociodemographic variables and medical comorbidities, via a telephone quality improvement survey conducted between 23 January 2021 and 13 February 2021. RESULTS: Vaccine willingness (n = 363) was 81.3%. Univariate analysis identified that the odds of vaccine acceptance reduced for patients who do not regard COVID-19 as a severe illness, are of younger age, have a lower Charlson Comorbidity Index, use illicit drugs, or carry Medicaid insurance. Multivariable logistic regression identified the best predictors of vaccine hesitancy to be: social media use to obtain COVID-19 information, concerns regarding vaccine safety, self-perception of a preexisting medical condition contraindicated with vaccination, not having received the annual influenza vaccine, having some high school education only, being a current smoker, and not having a prior cerebrovascular accident. Unique amongst males, a conservative political view strongly predicted vaccine hesitancy. Specifically for Asians, a higher body mass index, while for Native Hawaiians and other Pacific Islanders (NHPI), a positive depression screen, both reduced the odds of vaccine acceptance. CONCLUSION: Upon identifying the variables associated with vaccine hesitancy amongst patients with neurological disorders, our clinic is now able to efficiently provide ancillary COVID-19 education to sub-populations at risk for vaccine hesitancy. While our results may be limited to the sub-population of patients with neurological disorders, the findings nonetheless provide valuable insight to understanding vaccine hesitancy.
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INTRODUCTION: Against the backdrop of the diverse minority-majority state of Hawaii, this study seeks to better characterize associations between idiopathic intracranial hypertension (IIH) with sociodemographic variables and medical comorbidities. METHODS: A retrospective case-control study was conducted by utilizing 54 IIH patients and 216 age-, sex-, and race-matched controls, 216 unmatched controls, and 63 age-, sex-, and race-matched migraine patients. RESULTS: Relative to controls, IIH were 25 years younger (p < 0.0001) and 10.18 kg/m2 heavier (p < 0.0001), as well as exhibited greater odds of the following variables (p < 0.05): female (odds ratio [OR]: 8.87), the lowest income quartile (OR: 2.33), Native Hawaiian or other Pacific Islander (NHPI; OR: 2.23), Native American or Alaskan Native (OR: 16.50), obesity class 2 (35.0-39.9 kg/m2; OR: 4.10), obesity class 3 (>40 kg/m2; OR: 6.10), recent weight gain (OR: 11.66), current smoker (OR: 2.48), hypertensive (OR: 3.08), and peripheral vascular disease (OR: 16.42). Odds of IIH were reduced (p < 0.05) for patients who were Asian (OR: 0.27) or students (OR: 0.30;). Unique from Whites, NHPI IIH patients exhibited greater odds (p < 0.05) for being from lower socioeconomic status and currently smoking, as well as potential association with seizures (p = 0.08). Compared to migraines, IIH headaches were at increased odds of occurring (p < 0.05) occipitally, for greater than 15 days per month, aggravated by postural changes, and comorbid with dizziness and tinnitus. CONCLUSIONS: These results not only better characterize IIH, but also highlight socioeconomic and racial disparities in diagnosis.
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Pseudotumor Cerebral/diagnóstico , Adulto , Estudos de Casos e Controles , Feminino , Disparidades nos Níveis de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Fatores SocioeconômicosRESUMO
Although frequently prescribed, certain antibiotics such as trimethoprim-sulfamethoxazole carry the risk of a rare yet life-threatening adverse effect, termed drug-induced aseptic meningitis. Morbidity can be avoided if the medication is identified and discontinued. Patients in reported cases tend to be female and have an autoimmune disease or prior adverse reaction to the offending agent. As a rare and poorly characterized condition, the subset of patients using antibiotics at risk for aseptic meningitis remains unclear; hence, cataloging these adverse events remains critical for better elucidating the disease. Here, we report a 62-year-old man with psoriasis and no prior history of sulfa allergy, who presented with a sudden onset of fever, chills, vomiting, and muscle aches 5 hours after taking single doses of trimethoprim-sulfamethoxazole and ciprofloxacin. Common infectious causes were ruled out, and his medications were discontinued. Despite initial symptom resolution with discontinuation, the patient neurologically deteriorated over the next two days before eventually recovering with supportive care. This case highlights the variable presentation of drug-induced aseptic meningitis. In contrast to previous reports of drug-induced aseptic meningitis, our patient was male, older than the median age of 40 years, and did not have a prior adverse reaction to the antibiotic. Furthermore, to the best of our knowledge, we report a possible case of antibiotic-induced aseptic meningitis in a patient with psoriasis. Lastly, the case emphasizes not only the value of a thorough medication history but also the importance of recognizing that patients may deteriorate in the first 48 hours before resolution.
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Anti-Infecciosos , Meningite Asséptica , Psoríase , Adulto , Antibacterianos/efeitos adversos , Anti-Infecciosos/efeitos adversos , Feminino , Humanos , Masculino , Meningite Asséptica/induzido quimicamente , Meningite Asséptica/diagnóstico , Pessoa de Meia-Idade , Psoríase/induzido quimicamente , Psoríase/tratamento farmacológico , Combinação Trimetoprima e Sulfametoxazol/efeitos adversosRESUMO
RATIONALE: Malignant infiltration accounts for 0.5% of acute liver failure cases, with non-Hodgkin's lymphoma the predominant cause. Adult T-cell lymphoma/leukemia (ATLL) is a rarer source of acute hepatitis, with only 3 cases reported and all resulting in immediate deterioration with death. ATLL rises from human T-lymphocytic virus-1 (HTLV-1), commonly found in Japan (southern and northern islands), the Caribbean, Central and South America, intertropical Africa, Romania, and northern Iran. In Micronesia, HTLV-1 infection amongst native-born is absent or exceedingly rare. PATIENT CONCERNS: A 77-year-old Marshallese man presented to the emergency department with a 1-week history of generalized weakness, fatigue, and nausea. The physical exam revealed a cervical papulonodular exanthem and scleral icterus. DIAGNOSIS: Laboratory studies were remarkable for aspartate-aminotransferase of 230âIU/L (reference range [RR]: 0-40), alanine-aminotransferase of 227âIU/L (RR: 0-41), alkaline phosphatase of 133âIU/L (RR: 35-129), and total bilirubin of 4.7âmg/dL (RR: 0-1.2), supporting acute liver injury. Platelet count was 11.6x104/µL (RR: 15.1-42.4â×â104), hemoglobin was 13.8âg/dL (RR: 13.7-17.5), and white blood cell count was 7570/µL (RR: 3800-10,800) with 81.8% neutrophils (RR: 34.0-72.0) and 10.4% lymphocytes (RR: 12.0-44.0). The peripheral blood smear demonstrated abnormal lymphocytes with occasional flower cell morphology. HTLV-1/2 antibody tested positive. The skin and liver biopsies confirmed atypical T-cell infiltrate. The diagnosis of ATLL was established. INTERVENTIONS: The patient elected for palliative chemotherapy with cyclophosphamide, vincristine, and prednisone (CVP). He began antiviral treatment with zidovudine 250âmg bis in die (BID) indefinitely. Ursodiol and cholestyramine were added for his hyperbilirubinemia. OUTCOMES: Four weeks from admission, the patient returned to near baseline functional status and was discharged home. LESSONS: This case highlights that ATLL can initially present as isolated acute hepatitis, and how careful examination of peripheral blood-smear may elucidate hepatitis etiology. We also present support for utilizing ursodiol with cholestyramine for treating a hyperbilirubinemia. Moreover, unlike prior reports of ATLL presenting as liver dysfunction, combined antiviral and CVP chemotherapy was effective in this case. Lastly, there are seldom demographic reports of HTLV-1 infection from the Micronesian area, and our case represents the first indexed case of HTLV-1-associated-ATLL presenting as acute liver failure in a Marshallese patient.
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Infecções por HTLV-I/complicações , Infecções por HTLV-I/diagnóstico , Falência Hepática Aguda/complicações , Linfoma de Células T/complicações , Linfoma de Células T/diagnóstico , Idoso , Diagnóstico Diferencial , Vírus Linfotrópico T Tipo 1 Humano , Humanos , Falência Hepática Aguda/diagnóstico , Masculino , Micronésia , Cuidados PaliativosRESUMO
OBJECTIVE/INTRODUCTION: Although a critical chemotherapeutic, temozolomide's optimal regimen for 2016 World Health Organization (WHO) Grade II gliomas remains elusive, hence there is utility in not only cataloging survival outcomes of Grade II glioma subtypes against the background of temozolomide regimens, but also quantifying differences in progression-free survival (PFS) and overall survival (OS). MATERIALS AND METHODS: A systematic review of MEDLINE, Embase, and Cochrane Central Register of Controlled Trails was conducted by using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis and the Cochrane Handbook of Systemic Reviews of Interventions. RESULTS: Each molecular subtype of WHO Grade II glioma had a different temozolomide regimen identified as optimal in prolonging PFS and OS. For PFS, with temozolomide, the 25th, 50th, and 75th percentiles, were as follows (in months), respectively-A-wt II: 6.90, 12.95, and 19.95; A-mt II: 34.45, 36.01, and 39.60; OD II: 37.90, 46.00, and 55.03 (P = 0.016). For OS, the first quartile (25%), median (50%), third quartile (75%), were respectively identified (in months-A-wt II: 21.6 (median; n = 1); A-mt II: 60.6, 85.2, and 109.8; OD II: 86.1, 96.2, and 106.3 (P = 0.37). CONCLUSION: For each tumor molecular subtype, a different temozolomide regimen was identified as optimal for prolonging PFS and OS. Furthermore, regardless of temozolomide regimen, A-wt II had a significantly shorter PFS than A-mt II and OD-II. Overall, the data can provide useful prognostic insight to patients when making critical treatment decisions. Moreover, by cataloging and assessing survival outcomes per temozolomide regimen, such may facilitate future clinical trial design.
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Epidemiology provides an avenue for deciphering disease pathogenesis. By determining incidence across socioeconomic and demographic variables in the context of benign cerebral meningiomas (BCM), epidemiologic data may aid in elucidating and addressing healthcare inequalities. To investigate BCM incidence (per 100,000) with respect to sex, age, income, residence, and race/ethnicity, we queried the largest United States (US) administrative dataset (1997-2016), the National (Nationwide) Inpatient Sample (NIS), which surveys 20% of US discharges. Annual national BCM incidence was 5.01. Females had an incidence of 6.78, higher (p = 0.0000038) than males at 3.14. Amongst age groups incidence varied (p = 1.65 × 10-11) and was highest amongst those 65-84 (16.71) and 85+ (18.32). Individuals with middle/high income had an incidence of 5.27, higher (p = 0.024) than the 4.91 of low income patients. Depending on whether patients lived in urban, suburban, or rural communities, incidence varied (χ2 = 8.22, p = 0.016) as follows, respectively: 5.23; 4.96; 5.51. Amongst race/ethnicity (p = 8.15 × 10-14), incidence for Whites, Blacks, Asian/Pacific Islanders, Hispanics, and Native Americans were as follows, respectively: 5.05; 4.59; 4.22; 2.99; 0.55. In the US, BCM annual incidence exhibited disparities amongst socioeconomic and demographic subsets. Disproportionately, incidence was greatest for patients who were White, Black, female, 65 and older, and middle/high income.
Assuntos
Neoplasias Encefálicas/economia , Etnicidade , Disparidades em Assistência à Saúde/economia , Renda , Neoplasias Meníngeas/economia , Meningioma/economia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Encefálicas/etnologia , Neoplasias Encefálicas/terapia , Feminino , Humanos , Masculino , Neoplasias Meníngeas/etnologia , Neoplasias Meníngeas/terapia , Meningioma/etnologia , Meningioma/terapia , Pessoa de Meia-Idade , Estudos Retrospectivos , Estados Unidos/etnologia , Adulto JovemRESUMO
INTRODUCTION: Spinal meningiomas constitute the majority of primary spinal neoplasms, yet their pathogenesis remains elusive. By investigating the distribution of these tumors across sociodemographic variables can provide direction in etiology elucidation and healthcare disparity identification. METHODS: To investigate benign and malignant spinal meningioma incidences (per 100,000) with respect to sex, age, income, residence, and race/ethnicity, we queried the largest American administrative dataset (1997-2016), the National (Nationwide) Inpatient Sample (NIS), which surveys 20% of United States (US) discharges. RESULTS: Annual national incidence was 0.62 for benign tumors and 0.056 for malignant. For benign meningiomas, females had an incidence of 0.81, larger (P=0.000004) than males at 0.40; yet for malignant meningiomas, males had a larger (P=0.006) incidence at 0.062 than females at 0.053. Amongst age groups, peak incidence was largest for those 65-84 years old (2.03) in the benign group, but 45-64 years old (0.083) for the malignant group. For benign and malignant meningiomas respectively, individuals with middle/high income had an incidence of 0.67 and 0.060, larger (P=0.000008; P=0.04) than the 0.48 and 0.046 of low income patients. Incidences were statistically similar (P=0.2) across patient residence communities. Examining race/ethnicity (P=0.000003) for benign meningiomas, incidences for Whites, Asian/Pacific Islanders, Hispanics, and Blacks were as follows, respectively: 0.83, 0.42, 0.28, 0.15. CONCLUSIONS: Across sociodemographic strata, healthcare inequalities were identified with regards to spinal meningiomas. For benign spinal meningiomas, incidence was greatest for patients who were female, 65-84 years old, middle/high income, living in rural communities, White, and Asian/Pacific Islander. Meanwhile, for malignant spinal meningiomas incidence was greatest for males, those 45-65 years old, and middle/high income.
Assuntos
Disparidades em Assistência à Saúde/economia , Neoplasias Meníngeas/economia , Neoplasias Meníngeas/epidemiologia , Meningioma/economia , Meningioma/epidemiologia , Fatores Socioeconômicos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Bases de Dados Factuais/economia , Bases de Dados Factuais/tendências , Feminino , Disparidades em Assistência à Saúde/tendências , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Neoplasias da Medula Espinal/economia , Neoplasias da Medula Espinal/epidemiologia , Estados Unidos/epidemiologia , Adulto JovemRESUMO
Background: Obesity's risk increases for low-income, female, young, and Black patients. By extrapolation, idiopathic intracranial hypertension (IIH)-a disease associated with body mass index-would potentially display socioeconomic and demographic disparities. Methods: IIH incidence (per 100,000) was investigated with respect to sex, age, income, residence, and race/ethnicity, by querying the largest United States (US) healthcare administrative dataset (1997-2016), the National (Nationwide) Inpatient Sample. Results: Annual national incidence (with 25th and 75th quartiles) for IIH was 1.15 (0.91, 1.44). Females had an incidence of 1.97 (1.48, 2.48), larger (p = 0.0000038) than males at 0.36 (0.26, 0.38). Regarding age, largest incidence was among those 18-44 years old at 2.47 (1.84, 2.73). Low-income patients had an incidence of 1.56 (1.47, 1.82), larger (p = 0.00024) than the 1.21 (1.01, 1.36) of the middle/high. No differences (χ2 = 4.67, p = 0.097) were appreciated between urban (1.44; 1.40, 1.61), suburban (1.30; 1.09, 1.40), or rural (1.46; 1.40, 1.48) communities. For race/ethnicity (χ2 = 57, p = 2.57 × 10-12), incidence was largest for Blacks (2.05; 1.76, 2.74), followed by Whites (1.04; 0.79, 1.41), Hispanics (0.67; 0.57, 0.94), and Asian/Pacific Islanders (0.16; 0.11, 0.19). Year-to-year, incidence rose for all strata subsets except Asian/Pacific Islanders (τ = -0.84, p = 0.00000068). Conclusion: IIH demonstrates several sociodemographic disparities. Specifically, incidences are larger for those low-income, Black, 18-44 years old, or female, while annually increasing for all subsets, except Asian/Pacific Islanders. Hence, IIH differentially afflicts the US population, yielding in healthcare inequalities.