Neurologist最新文献

Objective: We mainly explore the predictive value of Barthel Index (BI), SPAN-100, and National Institute of Health stroke scale (NIHSS) scores on clinical prognosis and functional outcomes in thrombolytic patients and compare the differences in the predictive values of the above 3 scales so as to provide an effective basis to evaluate the prognosis of thrombolytic patients.

Methods: Data were collected from 212 patients with the first-onset AIS (acute ischemic stroke). The enrolled patients were treated with recombinant tissue plasminogen activator thrombolytic therapy and were divided into 2 groups according to the modified Rankin Scale (mRS) score at discharge: the prognosis group (mRS≤2 points) and the poor prognosis group (mRS≥3 points). Logistic multivariate analysis was used to analyze the predictors of poor prognosis in patients with thrombolysis. MedCalc software was used to plot receiver operating characteristic (ROC) curves, calculate the area under the ROC curve (AUC), and compare the prediction performance of the 3 scales by the Delong and colleagues' method, and the difference of P <0.05 was statistically significant.

Results: Logistic binary regression multivariate analysis suggested that BI was a predictor of poor prognosis for thrombolytic therapy in patients with AIS. The lower the BI score, the poorer the prognosis. The AUC for BI score was 0.862, 95% CI (0.808-0.906), NIHSS score AUC was 0.665, 95% CI (0.597-0.728), and SPAN-100 score AUC was 0.640, 95% CI (0.572-0.705). AUC comparison of 3 scoring ROC curves suggested statistically significant differences between BI and NIHSS ( PC =0.0000), BI and SPAN-100 ( PC =0.0000); no significant difference was observed between SPAN-100 and NIHSS ( PC =1.7997).

Conclusions: Simple BI scores have a high prognostic value for thrombolytic therapy in AIS.

Background: The role of Chiari network (CN) and Eustachian valves (EVs) in cardioembolic strokes is still unclear. There is inconsistency in the literature regarding clinical approach to these lesions to reduce stroke risk. We aimed to describe clinical presentation, neuroimaging and cardioimaging features, as well as management approaches for CN and EV in stroke context.

Review summary: A systemic search was carried out using PubMed and Web of Science following PRISMA guidelines, Supplemental Digital Content 1 ( http://links.lww.com/NRL/A123 ). We retrieved 4 case-control studies, 2 cross sectional studies as well 8 case reports, with a total of 883 patients with a mean age of 44.6 years (±13.8). The combined prevalence of EV/CN in stroke-related patent foramen ovale (PFO) patients was 50% (95% CI: 31-68). With isolated prevalence for EV and CN of 43% (95% CI: 25-63), 18% (95% CI: 12-25), respectively. Patients with history of stroke had higher prevalence of EV/CN compared with controls odds ratio=2.45 (95% CI: 1.2-5, P <0.01). All case-control and cross-sectional studies defined EV/CN by transesophageal echocardiography or intracardiac cardiography. In the 8 case reports, 7 cases were diagnosed by transesophageal echocardiography, while only 1 case was diagnosed postmortem.

Conclusion: EV/CN are relatively common findings in stroke patients with PFO. While it appears that presence of EV/CN with a PFO increases the risk of cardioembolic stroke, they remain underrecognized. EV/CN should be considered as high-risk PFO features. There is a scarcity of research emphasizing their role in clinical decision making, especially PFO closure and antithrombotic therapy choice.

Objectives: Elevation of the systemic immune inflammation (SII) index and system inflammation response index (SIRI) is known to be associated with higher risk of stroke and all-cause death. However, no study has reported their correlation with early neurological deterioration (END) following recombinant tissue-type plasminogen activator (IV-rtPA) in acute ischemic stroke patients. The aim of this study was to explore the correlation of SII and SIRI with the risk of END after IV-rtPA.

Methods: Included in this study were 466 consecutive patients treated with IV-rtPA. SII and SIRI were calculated according to blood cell counts before IV-rtPA. Patients were divided into 3 groups based on trisectional quantiles according to SII and SIRI values. The risk of END was assessed by multivariate regression. The overall discriminative ability of SII and SIRI in predicting END was assessed by receiver operating characteristic curve analysis.

Results: Of the 466 included patients, 62 (13.3%) were identified as having END. Compared with the first tertile of SII, multivariable regression analysis demonstrated that patients were more likely to have END (odds ratio 2.54; 95% CI: 1.23-5.23) and poor outcome at 90 days (odds ratio 2.02; 95% CI: 1.06-3.86) in third tertile after adjustment for potential confounders. In addition, a cutoff value of 591.63 for SII was detected in predicting post-thrombolysis END with a sensitivity of 58.1% and a specificity of 64.6% (area under the curve 0.61; 95% CI: 0.54-0.69).

Conclusions: Higher SII but not SIRI may prove to be a predictor for high risk of END and a poor functional outcome at 90 days after IV-rtPA.

Introduction: BRCA1-associated ataxia-telangiectasia-mutated activator-1 (BRAT1) is responsible for cell cycle surveillance and mitochondrial function. The implications of adult-onset BRAT1-variant and the resulting phenotypic neurocognitive and imaging features have not been previously described.

Case report: A 66-year-old man with a recent diagnosis of classic Hodgkin lymphoma was referred to neuro-oncology for cognitive and motor decline, and progressive cerebral white matter changes noted on magnetic resonance imaging (MRI). A neurological examination revealed global weakness, broad-based gait, and bilateral extensor plantar responses. Brain MRI demonstrated periventricular, deep, and subcortical white matter T2/FLAIR hyperintensities without contrast enhancement. Cerebral spinal fluid studies were unremarkable. A GeneDX genetic leukodystrophy panel conduction revealed a pathogenic variant (c.294dupA; p.L99TfsX92) resulting in a truncated protein of BRAT1, along with a variant of uncertain significance (c.746A>G;p.E249G). A presumptive diagnosis of late-onset leukoencephalopathy secondary to the BRAT1 variant was made. In an attempt to combat his mitochondrial dysfunction, he was initiated on a mitochondrial cocktail, including B-100 complex and coenzyme Q10. He began lymphoma-directed combination chemotherapy and developed precipitous functional decline after 2 cycles of therapy. Compared with prechemotherapy imaging, repeat positron emission tomography/computed tomography metabolic imaging showed a response after 3 cycles of chemotherapy; however, repeat brain MRI showed worsening diffuse white matter hyperintensities and cerebral atrophy.

Conclusion: Given the variability in phenotypes and clinical onset, leukodystrophies can be a diagnostic challenge. This case demonstrated progressive BRAT1-associated leukodystrophy exacerbated by chemotherapy-induced toxic leukoencephalopathy. Mitochondrial energy deficiency in the context of multiple metabolic insults was likely underlying the progressive neurological decline observed in this case of genetic leukodystrophy.

Introduction: The differential diagnosis of a spinal intradural extramedullary mass lesion is broad and includes meningioma, schwannoma, neurofibroma, leptomeningeal metastasis, and myxopapillary ependymoma. Though rare, lymphoma should be included in the differential diagnosis of a dural mass lesion.

Case report: A 38-year-old man presented with back pain that progressed over 1 month with associated focal tenderness over his mid to lower thoracic spine. He developed intermittent numbness of the bilateral lower extremities, nuchal rigidity, difficulty sleeping, and night sweats. A magnetic resonance imaging of the thoracic spine demonstrated a dorsal intradural extramedullary enhancing lesion from T7 to T10 extending outside the spinal canal. Dural thickening across the entire circumference of the spinal cord was noted. Computed tomography (CT)-guided biopsy of the thoracic lesion was performed, and pathology was consistent with follicular lymphoma. Fluorodeoxyglucose positron emission tomography:CT demonstrated no systemic disease. Bone marrow biopsy was negative for malignancy. Symptoms resolved with dexamethasone therapy. He was treated with bendamustine and rituximab with follow-up positron emission tomography:CT 2 months later demonstrating a complete response.

Conclusions: Lymphoma can rarely present as an isolated dural lesion and should be considered in the differential diagnosis of intradural extramedullary spinal mass lesions. Prompt diagnosis and initiation of treatment can lead to complete response and resolution of symptoms.

Background: Limb-shaking is one of the transient ischemic attacks (TIA) 'chameleons.' This literature review aims to evaluate the clinical, epidemiological profile, pathologic mechanisms, and management of limb-shaking TIA.

Review summary: Relevant reports in Medline's (PubMed) database were identified and assessed by 2 reviewers without language restriction from 1985 to 2022. A total of 82 reports containing 161 cases that developed limb-shaking TIA were reported. The mean and median age were 61.36 (SD: 15.29) and 62 years (range: 4-93 y). Most of the individuals affected were males (64.34%). Limb-shaking was reported as unilateral in 83.33% of the patients. Limb-shaking presented with other neurological deficits in 44.33% of the individuals, in which the most common concurrent neurological deficit was the weakness of at least 1 limb. A recurrence of the "shaking" phenomenon was observed in 83 individuals. A trigger of limb-shaking was reported in 69 cases, and the most common was changing body position. The internal carotid artery was the most frequent vessel involved in limb-shaking. A chronically occluded internal carotid artery was observed in 42 individuals. Hypertension was the most common comorbidity. The management was conservative in 42.30% of the cases. The most frequent misdiagnoses were seizures. A full recovery was achieved in 56.60% of the individuals.

Conclusions: Limb-shaking TIA could be defined as involuntary, rhythmic, brief (<5 min), recurrent, jerky movement usually precipitated by activities that may reduce cerebral blood flow. The "shaking" phenomenon was primarily described as a manifestation of symptomatic complete internal carotid artery obstruction.

Introduction: Acute kidney injury is a well-known complication of generalized tonic-clonic seizures, most commonly due to rhabdomyolysis. Elevated serum uric acid resulting in uric acid nephropathy is an overlooked cause of acute kidney injury in these patients, with only a few published case reports.

Case report: In the first case, a 23-year-old male was admitted with status epilepticus. His kidney function worsened and he developed anuria. He had a serum uric acid level of 20.7 mg/dL and required multiple sessions of hemodialysis. In the second case, a 32-year-old male was admitted with acute kidney injury after experiencing a breakthrough seizure. He had a serum uric acid level of 20.4 mg/dL and was treated with rasburicase with recovery of renal function. In the third case, a 29-year-old male was admitted with status epilepticus. His renal function deteriorated. His serum uric acid level was 19.5 mg/dL. He required hemodialysis and rasburicase.

Conclusion: Uric acid nephropathy is a rare complication of generalized tonic-clonic seizures, which is poorly recognized by healthcare providers. We advocate for Nephrology consultation early in a patient's hospitalization to discuss the use of rasburicase to avoid the associated morbidity of renal replacement therapies.

Objectives: Spinal cord injury (SCI) is any spinal cord injury or affliction that results in temporary or permanent impairment of motor or sensory function. This study determined the prevalence of frailty and its impact on in-hospital outcomes of patients admitted with acute traumatic SCI (TSCI).

Methods: This retrospective study extracted data of adults 18 to 85 years with acute TSCI from the US Nationwide Inpatient Sample (NIS) 2016 to 2018. Frailty status were assessed by the 11-factor modified Frailty Index (mFI-11) through claim codes. Patients with an mFI ≥3 were classified as frail. Associations between study variables and in-hospital mortality, discharge status, prolonged length of stay, severe infection, and hospital costs were determined by univariate and multivariable regression analyses.

Results: A total of 52,263 TSCI patients were identified, where 12,203 (23.3%) patients were frail. After adjusting for relevant confounders, frailty was independently associated with increased risk for in-hospital mortality [adjusted odds ratio (aOR) = 1.25, 95% CI:1.04-1.49], unfavorable discharge (aOR =1.15, 95% CI: 1.09-1.22), prolonged length of stay (aOR =1.32, 95% CI: 1.24-1.40), and severe infection (aOR =2.52, 95% CI: 2.24-2.83), but not hospital cost. Stratified analyses revealed frailty was associated with higher unfavorable discharge and severe infection regardless of age, Charlson Comorbidity Index, and injury level.

Conclusions: In acute TSCI, frailty is independently associated with increased risk for adverse inpatient outcomes in terms of in-hospital mortality, prolonged hospital stays, unfavorable discharge, and particularly severe infection.

Introduction: C9orf72 expansion is the most common genetic abnormality in behavioral variant frontotemporal dementia (bvFTD) and amyotrophic lateral sclerosis. Although psychiatric prodromes are common in C9orf72 expansion carriers, there are only scattered reported cases of primary psychiatric disorders, such as bipolar disorder, diagnosed at disease onset. Moreover, C9orf72 carrier status is rarely identified in bipolar disorder genetic studies.

Case report: A 51-year-old, right-handed woman with 16 years of education presented for evaluation of long-standing cognitive and behavioral change. She initially displayed symptoms of mania and florid, multimodal psychotic symptoms at age 39. Her bipolar disorder symptoms were initially responsive to medication; however, she later developed executive dysfunction and behavioral symptoms consistent with bvFTD. She became progressively nonverbal, and her limited speech was notable for speech apraxia. At the time of presentation, she demonstrated cortical sensory deficit, ideomotor and oral-buccal apraxia, and unstable gait. Neuroimaging revealed diffuse brain atrophy. Postmortem histopathological evaluation revealed frontotemporal lobar degeneration with TDP-43 inclusions, type B, and genetic study identified C9orf72 expansion. A detailed review of family history found a strong paternal history of bipolar disorder and substance use disorder.

Conclusions: We describe a rare case of C9orf72 expansion initially characterized by late-onset bipolar disorder and florid, multimodal psychotic symptoms, followed years later by bvFTD diagnosis. This report emphasizes the importance of completing a neurological examination, obtaining a detailed family history, and pursuing genetic screening to distinguish between primary psychiatric disorder and bvFTD in individuals who meet the criteria for late-onset bipolar disorder.

Background: Powassan virus (POWV) encephalitis is an arbovirus infection and the only tick-borne encephalitis serogroup which is present in mainland North America. The magnetic resonance (MR) imaging described with POWV encephalitis is varied, nonspecific, and limited in number, and as such, imaging patterns and outcomes with this arbovirus infection are not well appreciated.

Methods: A case report and literature review of the MR imaging associated with POWV encephalitis and correlate of the MR pattern with outcome is considered.

Results: The cerebellar dominant MR imaging pattern was identified in 50% of POWV encephalitis cases and was associated with a 60% fatality rate. POWV encephalitis with prominent cerebellar involvement on MR imaging responded to intravenous steroid.

Conclusions: A cerebellar dominant MR pattern in POWV encephalitis was common, associated with a poor prognosis, and recognition could change management from supportive to life-saving.