Levodopa Responsive Parkinsonism in Patients with Hemochromatosis: Case Presentation and Literature Review

Hemochromatosis is an autosomal recessive disorder which leads to abnormal iron deposition in the parenchyma of multiple organs causing tissue damage. Accumulation of iron in the brain has been postulated to be associated with several neurodegenerative diseases including Parkinson\u27s disease. The excess iron promotes Parkin and α-synuclein aggregation in the neurons. Excess iron has also been noted in substantia nigra on MRI especially using susceptibility weighted imaging in patients with Parkinson\u27s disease. We present a case of a young male with alleles for both C282Y and H63D who presented with signs of Parkinsonism and demonstrated significant improvement with levodopa treatment

“What are the odds?”: A rare clinical syndrome from a rare vascular condition caused by a commonly used medication

Objective: Describe a case of a patient developing Balint\u27s syndrome from bilateral parieto-occiptal ischemic infarcts secondary to reversible cerebral vasoconstriction syndrome (RCVS) after sumatriptan use. Background: Sumatriptan is a commonly prescribed anti-migraine medication which has 5HT 1B & 1D agonistic properties. Injudicious use of sumatriptan can lead to intracranial vasoconstriction with disastrous outcomes as exemplified in our patient. Design/Methods: Case-study Results: 29-years old female with a remote history of headaches presented with severe headaches and acute encephalopathy. MRI revealed bilateral parieto-occipital infarcts. Autoimmune workup and cerebrospinal fluid analysis were unremarkable. Catheter angiography revealed moderate diffuse spasm of the basilar, bilateral intracranial internal carotid, and proximal middle cerebral arteries (MCA). Use of intra-arterial nicardipine during the angiography procedure was limited due to patient\u27s baseline hypotension. Verapamil was started along with fludrocortisone. Her progress was followed by serial transcranial-doppler which revealed resolution of MCA vasospasm. After clinical improvement with verapamil, she admitted taking 300-mg sumatriptan over a 5-day period prior to the onset of her encephalopathy. In addition, her neurological exam demonstrated clinical signs of optic ataxia and simultagnosia, consistent with Balint\u27s syndrome due to the location of the stroke Conclusions: On reviewing the literature, only two cases of RCVS have been reported with sumatriptan use. Our patient possesses a high educational value due to the presence of a rare clinical syndrome of Balint\u27s, from an unusual vascular pathology of RCVS, which was likely secondary from a medication with vasoconstrictive properties. RCVS commonly causes a “thunderclap” headache but in severe cases, can also be associated with ischemic or hemorrhagic infarction, encephalopathy, and seizures. Location of the ischemic infarcts in the bilateral parietal region enabled our patient to possess a mysterious clinical syndrome of simultagnosia and optic ataxia which was first described in 1909. The only sign missing in our patient from the classical Balint syndrome was ocular apraxia

Factors affecting distal hyperintense vessel sign, a postulated marker for intracranial collateral circulation

Objective: To investigate factors affecting the distal hyperintense vessel sign (DHVS) Background: DHVS on FLAIR sequence is considered to originate from the leptomeningeal collateral flow. Older studies have analyzed factors affecting collateral circulation using CT and conventional angiographies but so far, no study has investigated the association between DHVS and said factors to strengthen the hypothesis of the origin of DHVS. Design/Methods: Two-hundred charts were reviewed based on ICD-9 codes. Thirty-eight were selected based on the presence of acute ischemic stroke due to symptomatic internal carotid or middle cerebral artery disease. A neuroradiologist blindly quantified DHVS using the number of cortical-MCA regions (defined by ASPECTS) positive for DHVS (range 0-7). The patients were dichotomized into groups of \u3c3 and ≥3 score. Demographics, coexisting risk factors, clinical measures of stroke severity (NIHSS and mRS at discharge and follow-up) and stroke volume were compared between two groups. Fischer\u27s exact test was used for binary variables. Two-sample t-test and Wilcoxon- test was used for continuous variables. Results: Seven (18%) patients had DHVS-score ≥3. These patients were significantly younger (53.6 vs 66.5 yr, p=0.036), less likely to have hypertension (29% v 87%, p=0.004), diabetes (0% v 47%, p=0.016), and have lower measures of glucose intolerance (mean A1c 5.6 v 6.2, p=0.006). No statistically significant difference was noted with other variables. Conclusions: We note consistencies between prior studies and our study showing that younger patients without hypertension have better collaterals. We also found an association of diabetes with less DHVS whereas prior studies have yielded an inconsistent association. While a correlation between stroke severity and stroke volume could not be confirmed, low power and wide stroke-to-MRI gap (range 1-8 d) were important limitations here. Further study correcting these shortcomings is warranted. In conclusion, our findings support the origins of DHVS from collateral circulation and are affected by traditional cerebrovascular risk factors

Factors affecting distal hyperintense vessel sign, a postulated marker for intracranial collateral circulation.

Objective: To investigate factors affecting the distal hyperintense vessel sign (DHVS) Background: DHVS on FLAIR sequence is considered to originate from the leptomeningeal collateral flow. Older studies have analyzed factors affecting collateral circulation using CT and conventional angiographies but so far, no study has investigated the association between DHVS and said factors to strengthen the hypothesis of the origin of DHVS. Design/Methods: Two-hundred charts were reviewed based on ICD-9 codes. Thirty-eight were selected based on the presence of acute ischemic stroke due to symptomatic internal carotid or middle cerebral artery disease. A neuroradiologist blindly quantified DHVS using the number of cortical-MCA regions (defined by ASPECTS) positive for DHVS (range 0-7). The patients were dichotomized into groups of \u3c3 and ≥3 score. Demographics, coexisting risk factors, clinical measures of stroke severity (NIHSS and mRS at discharge and follow-up) and stroke volume were compared between two groups. Fischer\u27s exact test was used for binary variables. Two-sample t-test and Wilcoxon- test was used for continuous variables. Results: Seven (18%) patients had DHVS-score ≥3. These patients were significantly younger (53.6 vs 66.5 yr, p=0.036), less likely to have hypertension (29% v 87%, p=0.004), diabetes (0% v 47%, p=0.016), and have lower measures of glucose intolerance (mean A1c 5.6 v 6.2, p=0.006). No statistically significant difference was noted with other variables. Conclusions: We note consistencies between prior studies and our study showing that younger patients without hypertension have better collaterals. We also found an association of diabetes with less DHVS whereas prior studies have yielded an inconsistent association. While a correlation between stroke severity and stroke volume could not be confirmed, low power and wide stroke-to-MRI gap (range 1-8 d) were important limitations here. Further study correcting these shortcomings is warranted. In conclusion, our findings support the origins of DHVS from collateral circulation and are affected by traditional cerebrovascular risk factors

Improving stroke care delivery for in-hospital stroke alerts by addition of overhead “code stroke” announcements: Single comprehensive stroke center\u27s experience

Introduction: In-hospital strokes account for 2-17% of all stroke patients. Mobilization of resources for in-hospital strokes is challenging and it takes longer to complete the necessary evaluations as compared to those presenting to the emergency room. Dedicated “stroke code teams” for in-hospital stroke patients have been shown to improve the adherence to the AHA\u27s get with the guidelines (GWTG) initiative but there is a paucity of research analyzing this process. Aim: To assess whether the addition of an overhead “code stroke” announcement to an existing group paging system for inhospital strokes decreases the time required for obtaining the necessary evaluations. Methods: An overhead “code stroke” announcement and process education was introduced at our hospital in addition to the existing stroke group paging system. We analyzed prospectively collected time based measures for in-hospital stroke code activations 4 months before and after implementation. Wilcoxon two-sample test was used to analyze the relevant time variables between the two groups. Results: The post-stroke group had significantly shorter times from stroke alert to CT and CTA interpretation, time from symptom onset to CT and CTA interpretation, and time for CT and CTA interpretation when compared to the pre-stroke alert group (see table). The post-stroke alert period captured significantly more acute strokes than the pre-stroke alert period (6.25 vs 1.75 per month, p=0.001 from binomial test). Conclusion: The addition of an overhead “code stroke” along with process education can drastically reduce important GWTG\u27s acute stroke evaluation times at a comprehensive stroke center. We suggest that improved coordination between the services, along with the urgency associated with an overhead “code stroke”, led to improvement in the evaluation times. We also noted an increase in the number of stroke-code activations, probably due to increased perception and awareness of stroke as a true emergency. (Table Presented)

Decreasing “door to admission” times for intravenous tPA treated patients: A single comprehensive stroke center experience

Introduction: AHA\u27s Get with The Guidelines (GTWG) initiative has recommended “door to admission” time for patients receiving IV tPA to beours, complicated administrative steps and coordination required to admit patients often leads to delays. There has been a paucity of studies aiming to streamline this process for patients treated with IV tPA. Aim: To assess whether having a dedicated rapid response team (RRT) RN within the ED to coordinate care of patients receiving IV tPA reduces “door to admission” times. Methods: A policy of notifying RRT of all patients eligible for IV tPA was implemented in the ED. The responsibility of RRT was to coordinate care between neurology resident, ED nurse, acute stroke unit (ASU) charge nurse and admissions office to ensure timely completion of steps required for admission. “Door to admission” times were collected prospectively for 3 months before and after intervention. The compliance of recording neuro checks and vital signs per AHA guidelines for post tPA care were also measured. Wilcoxon two sample test was used to analyze time variables and compliance rates. Results: The post intervention group had significantly decreased “door to admission” and “bolus to admission” times (see table). Also, significantly fewer patients had \u3e1 vital signs checks miss in post-intervention group (6% vs 38%; p=0.033). There was no significant increase in the compliance rate of neuro checks. Discussion: By adding RRT in coordination of care for IV tPA patients in the ED, we were able to drastically reduce our door to admission times. The improvement was because of faster placement and prioritized processing of the admission orders. RRT acted as a “control center” relaying vital information to admission office and ASU charge nurse. This also freed ED RN to improve compliance rate of recording vital signs in IV tPA patients. Similar interventions can be used by other centers to decrease their “door to admission” times. (Table Presented)

Stroke Care Trends During COVID-19 Pandemic in Zanjan Province, Iran. From the CASCADE Initiative: Statistical Analysis Plan and Preliminary Results

Background: The emergence of the COVID-19 pandemic has significantly impacted global healthcare systems and this may affect stroke care and outcomes. This study examines the changes in stroke epidemiology and care during the COVID-19 pandemic in Zanjan Province, Iran. Methods: This study is part of the CASCADE international initiative. From February 18, 2019, to July 18, 2020, we followed ischemic and hemorrhagic stroke hospitalization rates and outcomes in Valiasr Hospital, Zanjan, Iran. We used a Bayesian hierarchical model and an interrupted time series analysis (ITS) to identify changes in stroke hospitalization rate, baseline stroke severity [measured by the National Institutes of Health Stroke Scale (NIHSS)], disability [measured by the modified Rankin Scale (mRS)], presentation time (last seen normal to hospital presentation), thrombolytic therapy rate, median door-to-needle time, length of hospital stay, and in-hospital mortality. We compared in-hospital mortality between study periods using Cox-regression model. Results: During the study period, 1,026 stroke patients were hospitalized. Stroke hospitalization rates per 100,000 population decreased from 68.09 before the pandemic to 44.50 during the pandemic, with a significant decline in both Bayesian [Beta: -1.034; Standard Error (SE): 0.22, 95% CrI: -1.48, -0.59] and ITS analysis (estimate: -1.03, SE = 0.24, p \u3c 0.0001). Furthermore, we observed lower admission rates for patients with mild (NIHSS \u3c 5) ischemic stroke (p \u3c 0.0001). Although, the presentation time and door-to-needle time did not change during the pandemic, a lower proportion of patients received thrombolysis (-10.1%; p = 0.004). We did not see significant changes in admission rate to the stroke unit and in-hospital mortality rate; however, disability at discharge increased (p \u3c 0.0001). Conclusion: In Zanjan, Iran, the COVID-19 pandemic has significantly impacted stroke outcomes and altered the delivery of stroke care. Observed lower admission rates for milder stroke may possibly be due to fear of exposure related to COVID-19. The decrease in patients treated with thrombolysis and the increased disability at discharge may indicate changes in the delivery of stroke care and increased pressure on existing stroke acute and subacute services. The results of this research will contribute to a similar analysis of the larger CASCADE dataset in order to confirm findings at a global scale and improve measures to ensure the best quality of care for stroke patients during the COVID-19 pandemic

Stroke Care Trends During COVID-19 Pandemic in Zanjan Province, Iran. From the CASCADE Initiative: Statistical Analysis Plan and Preliminary Results.

BackgroundThe emergence of the COVID-19 pandemic has significantly impacted global healthcare systems and this may affect stroke care and outcomes. This study examines the changes in stroke epidemiology and care during the COVID-19 pandemic in Zanjan Province, Iran.MethodsThis study is part of the CASCADE international initiative. From February 18, 2019, to July 18, 2020, we followed ischemic and hemorrhagic stroke hospitalization rates and outcomes in Valiasr Hospital, Zanjan, Iran. We used a Bayesian hierarchical model and an interrupted time series analysis (ITS) to identify changes in stroke hospitalization rate, baseline stroke severity [measured by the National Institutes of Health Stroke Scale (NIHSS)], disability [measured by the modified Rankin Scale (mRS)], presentation time (last seen normal to hospital presentation), thrombolytic therapy rate, median door-to-needle time, length of hospital stay, and in-hospital mortality. We compared in-hospital mortality between study periods using Cox-regression model.ResultsDuring the study period, 1,026 stroke patients were hospitalized. Stroke hospitalization rates per 100,000 population decreased from 68.09 before the pandemic to 44.50 during the pandemic, with a significant decline in both Bayesian [Beta: -1.034; Standard Error (SE): 0.22, 95% CrI: -1.48, -0.59] and ITS analysis (estimate: -1.03, SE = 0.24, p < 0.0001). Furthermore, we observed lower admission rates for patients with mild (NIHSS < 5) ischemic stroke (p < 0.0001). Although, the presentation time and door-to-needle time did not change during the pandemic, a lower proportion of patients received thrombolysis (-10.1%; p = 0.004). We did not see significant changes in admission rate to the stroke unit and in-hospital mortality rate; however, disability at discharge increased (p < 0.0001).ConclusionIn Zanjan, Iran, the COVID-19 pandemic has significantly impacted stroke outcomes and altered the delivery of stroke care. Observed lower admission rates for milder stroke may possibly be due to fear of exposure related to COVID-19. The decrease in patients treated with thrombolysis and the increased disability at discharge may indicate changes in the delivery of stroke care and increased pressure on existing stroke acute and subacute services. The results of this research will contribute to a similar analysis of the larger CASCADE dataset in order to confirm findings at a global scale and improve measures to ensure the best quality of care for stroke patients during the COVID-19 pandemic