Managing Alkaptonuria in Absence of Appropriate Medication: A Case Report and Review of Literature.

Alkaptonuria is an inborn error of metabolism inherited as an autosomal recessive disorder due to a mutation in the homogentisic acid dioxygenase gene. It occurs rarely (global prevalence of alkaptonuria is 1 in 100,000 to 250,000), and mainly affects the joints and connective tissue of the body due to deposition of homogentisic acid giving affected areas a blue-black discoloration (ochronosis).In this case report, we present a male patient, aged 47 years, with joint and scleral involvement. He had been diagnosed many years ago with the disease by gas chromatography. His symptoms kept progressively worsening since he was recently prescribed physiotherapy and vitamin C for his disease, which has not been shown to be an effective treatment. A main reason for his disease deterioration was also the lack of nitisinone availability in his home country, as well as in the subcontinent region generally. We also presen a summary of some previously reported cases and treatment regimens to compare our case and present the comparison as a learning source for future physicians.

Evaluating the strength and quality of evidence in American heart association/American stroke association's guidelines for aneurysmal subarachnoid hemorrhage and spontaneous intracerebral hemorrhage.

BACKGROUND: Clinical practice recommendations guide healthcare decisions. This study aims to evaluate the strength and quality of evidence supporting the American Heart Association (AHA)/American Stroke Association (ASA) guidelines for aneurysmal subarachnoid hemorrhage (aSAH) and spontaneous intracerebral hemorrhage (ICH). METHODS: We reviewed the current AHA/ASA guidelines for aSAH and spontaneous ICH and compared with previous guidelines. Guidelines were classified based on the Class of recommendation (COR) and Level of evidence (LOE). COR signifies recommendation strength (COR 1: Strong; COR 2a: Moderate; COR 2b: Weak; COR 3: No Benefit/Harm), while LOE denotes evidence quality (LOE A: High-Quality; LOE B-NR: Moderate-Quality, Not Randomized; LOE B-R: Moderate-Quality, Randomized; LOE C-EO: Expert Opinion; LOE C-LD: Limited Data). RESULTS: For aSAH, we identified 84 recommendations across 15 guideline categories. Of these, 31% were classified as COR I, 30% as COR 2a, 17% as COR 2b, and 18% as COR 3. In terms of LOE, 7% were based on LOE A, 10% on LOE B-R, 65% on LOE B-NR, 14% on LOE C-LD, and 5% on LOE C-EO. Compared to previous guidelines, there was a 46% decrease in LOE A, a 45% increase in LOE B, and an 11% decrease in LOE C. For spontaneous ICH, 124 guidelines were identified across 31 guideline categories. Of these, 28% were COR I, 32% COR 2b, and 9% COR 3. For LOE, 4% were based on LOE A, 35% on LOE B-NR, and 42% on LOE C-LD. Compared to previous guidelines, there was a 78% decrease in LOE A, an 82% increase in LOE B, and a 14% increase in LOE C. This analysis highlights that less than a third of AHA/ASA guidelines are classified as the highest class of recommendation, with less than 10% based on the highest LOE. CONCLUSION: Less than a third of AHA/ASA guidelines on aSAH and spontaneous ICH are classified as the highest class of recommendation with less than 10% based on highest LOE. There appears to be a decrease in proportion of guidelines based on highest LOE in most recent guidelines.

Trends in performance of thrombectomy for acute ischemic stroke patients in teaching and non-teaching hospitals.

OBJECTIVES: The value of thrombectomy in patients with acute ischemic stroke cannot be understated. As such, whether these patients get access to this treatment can significantly impact their disease outcomes. We analyzed the trends in thrombectomy adoption between teaching and non-teaching hospitals in the United States, and their impact on overall patient care. MATERIALS AND METHODS: We conducted a retrospective analysis of hospital admissions in the Nationwide Inpatient Sample with a diagnosis of acute ischemic stroke between 2012 and 2020. We compared the annual total number and proportion of patients undergoing thrombectomy between teaching and non-teaching hospitals, and their corresponding outcomes. RESULTS: A total of 3,823,490 and 1,875,705 patients were admitted to teaching and non-teaching hospitals during the study duration, respectively. The proportion of patients who underwent thrombectomy increased from 1.60 % to 7.02 % (p-value for trend p < 0.001) in teaching hospitals and from 0.32 % to 2.20 % (p-value trend p < 0.001) in non-teaching hospitals. The absolute increase in the number of acute ischemic stroke patients undergoing thrombectomy was highest in teaching hospitals particularly those with large bed size, an increase from 3635 patients in 2012 to 24,730 patients in 2020. Higher rates of intravenous thrombolysis and patient transfer prior to thrombectomy were seen in teaching hospitals compared with non-teaching hospitals. CONCLUSIONS: The study highlights disparities between teaching and non-teaching hospitals, with teaching hospitals showing a disproportionately higher rate of thrombectomy adoption in acute ischemic stroke patients. Further studies are needed to understand the barriers to the adoption of thrombectomy in non-teaching hospitals.

Effect of Cilostazol in Animal Models of Cerebral Ischemia and Subarachnoid Hemorrhage: A Systematic Review and Meta-Analysis.

BACKGROUND: Cilostazol, a phosphodiesterase III inhibitor, appears to be a promising agent for preventing cerebral ischemia in patients with aneurysmal subarachnoid hemorrhage. Here, the authors perform a systematic review and meta-analysis to quantitatively assess the effects of cilostazol on brain structural and functional outcomes in animal models of cerebral ischemia and subarachnoid hemorrhage-induced cerebral vasospasm. METHODS: By using the PRISMA guidelines, a search of the PubMed, Scopus, and Web of Science was conducted to identify relevant studies. Study quality of each included study for both systematic reviews were scored by using an adapted 15-item checklist from the Collaborative Approach to Meta-Analysis of Animal Data from Experimental Studies. We calculated a standardized mean difference as effect size for each comparison. For each outcome, comparisons were combined by using random-effects modeling to account for heterogeneity, with a restricted maximum likelihood estimate of between-study variance. RESULTS: A total of 22 (median [Q1, Q3] quality score of 7 [5, 8]) and 6 (median [Q1, Q3] quality score of 6 [6, 6]) studies were identified for cerebral ischemia and subarachnoid hemorrhage-induced cerebral vasospasm, respectively. Cilostazol significantly reduced the infarct volume in cerebral ischemia models with a pooled standardized mean difference estimate of - 0.88 (95% confidence interval [CI] [- 1.07 to - 0.70], p < 0.0001). Cilostazol significantly reduced neurofunctional deficits in cerebral ischemia models with a pooled standardized mean difference estimate of - 0.66 (95% CI [- 1.06 to - 0.28], p < 0.0001). Cilostazol significantly improved the basilar artery diameter in subarachnoid hemorrhage-induced cerebral vasospasm with a pooled standardized mean difference estimate of 2.30 (95% CI [0.94 to 3.67], p = 0.001). Cilostazol also significantly improved the basilar artery cross-section area with a pooled standardized mean estimate of 1.88 (95% CI [0.33 to 3.43], p < 0.05). Overall, there was between-study heterogeneity and asymmetry in the funnel plot observed in all comparisons. CONCLUSIONS: Published animal data support the overall efficacy of cilostazol in reducing infarct volume and neurofunctional deficits in cerebral ischemia models and cerebral vasospasm in subarachnoid hemorrhage models.

Knowledge and perception of medical students towards the use of artificial intelligence in healthcare.

OBJECTIVE: To assess the knowledge and perception of medical students regarding the utility and applications of artificial intelligence in medicine. METHODS: The cross-sectional study was conducted at the Shifa College of Medicine, Islamabad, Pakistan, from February to August 2021, and comprised medical students regardless of gender or year of studies. Data was collected using a pretested questionnaire. Differences in perceptions were explored relative to gender and the year of studies. Data was analysed using SPSS 23. RESULTS: Of the 390 participants, 168(43.1%) were males and 222(56.9%) were females. The overall mean age was 20±1.65 years. There were 121(31%) students from the first year of studies, 122(31.3%) second year, 30(7.7%) from third year, 73(18.7%) from fourth year, and 44(11.3%) from the fifth year. Most participants 221(56.7%) had a good familiarity with artificial intelligence, and 226(57.9%) agreed that the biggest advantage of using artificial intelligence in healthcare was its ability to speed up the processes. In terms of gender of year of studies, there were no significant differences on both counts (p>0.05). CONCLUSIONS: Medical students, regardless of age and year of studies, were found to have a good understanding of the usage and application of artificial intelligence in medicine.

Failure Rates of Conservative Management of Minimally Symptomatic Chronic Subdural Hematoma: A Systematic Review and Meta-Analysis.

BACKGROUND: Conservative treatments for minimally symptomatic chronic subdural hematoma (cSDH) are debated, with surgery as the primary option. OBJECTIVE: To assess failure rates of a conservative approach for management of cSDH. METHODS: We searched PubMed, SCOPUS, Web of Science, and ClinicalTrials.gov for studies on conservative management of cSDH and analyzed the data using R (version 4.1.2). RESULTS: A total of 35 studies including 2095 patients were analyzed: 950 (45%) of the patients were in the observation group, 671 (32%) in the corticosteroid group, 355 (17%) in the atorvastatin group, 43 (2%) in the mannitol group, 52 (2.5%) in the tranexamic acid group, and 24 (1.1%) in the etizolam group. Our pooled analysis showed that 19.82% of patients required rescue surgery (95% confidence interval [CI]: 12.98% to 26.66%, P < 0.0001). The overall pooled risk ratio (RR) for the effect of interventions on the need for rescue surgery was 0.2424 (95% CI: 0.1577 to 0.3725, Iˆ2 = 90.5%, P < 0.0001). Subgroup analysis showed varied effects: observation group (RR = 0.3482, 95% CI: 0.1045 to 1.1609, Iˆ2 = 94.0%), corticosteroids (RR = 0.2988, 95% CI: 0.1671 to 0.5344, Iˆ2 = 90.8%), atorvastatin (RR = 0.1609, 95% CI: 0.0985 to 0.2627, Iˆ2 = 53.2%), mannitol (RR = 0.0370, 95% CI: 0.0009 to 1.5244), and tranexamic acid (RR = 0.0585, 95% CI: 0.0026 to 1.2924). CONCLUSIONS: The rate of rescue surgery in conservatively managed cSDH patients remains high. Corticosteroids or atorvastatin demonstrates some potential benefit in reducing the failure rate but collective effectiveness is unknown.

Prevalence, trends, and outcomes of cerebral infarction in patients with aneurysmal subarachnoid hemorrhage in the USA.

BACKGROUND AND PURPOSE: Cerebral infarction remains an important cause of death or disability in patients with aneurysmal subarachnoid hemorrhage (SAH). The prevalence, trends, and outcomes of cerebral infarction in patients with aneurysmal SAH at a national level are not known. METHODS: We identified the proportion of patients who develop cerebral infarction (ascertained using validated methodology) among patients with aneurysmal SAH and annual trends using the Nationwide Inpatient Sample (NIS) from 2016 to 2021. We analyzed the effect of cerebral infarction on in-hospital mortality, routine discharge without palliative care (based on discharge disposition), poor outcome defined by the NIS SAH outcome measure, and length and costs of hospitalization after adjusting for potential confounders. RESULTS: A total of 35,305 (53.6%) patients developed cerebral infarction among 65,840 patients with aneurysmal SAH over a 6-year period. There was a trend toward an increase in the proportion of patients who developed cerebral infarction from 51.5% in 2016 to 56.1% in 2021 (p trend p<.001). Routine discharge was significantly lower (30.5% vs. 37.8%, odds ratio [OR] 0.82, 95% confidence interval [CI] 0.75-0.89, p<.001), and poor outcome defined by NIS-SAH outcome measure was significantly higher among patients with cerebral infarction compared with those without cerebral infarction (67.4% vs. 59.3%, OR 1.29, 95% CI 1.18-1.40, p<.001). There was no difference in in-hospital mortality (13.0% vs. 13.6%, OR 0.94, 95% CI 0.85-1.05, p = .30). The length of stay (median 18 days [interquartile range [IQR] 13-25] vs. 14 days [IQR 9-20]), coefficient 3.04, 95% CI 2.44-3.52 and hospitalization cost (median $96,823 vs. $71,311, coefficient 22,320, 95% CI 20,053-24,587) were significantly higher among patients who developed cerebral infarction compared with those who did not develop cerebral infarction. CONCLUSIONS: Cerebral infarction was seen in 54% of the patients with a trend toward an increase in the affected proportion of patients with aneurysmal SAH. Patients with cerebral infarction had higher rates of adverse outcomes and required higher resources during hospitalization.

1-Year outcomes of Resolute Onyx Zotarolimus-Eluting Stent for symptomatic intracranial atherosclerotic disease: A multicenter propensity score-matched comparison with stenting versus aggressive medical management for preventing recurrent stroke in intracranial stenosis trial.

BACKGROUND: Intracranial atherosclerotic disease (ICAD) is one of the most prevalent causes of stroke across the world. Endovascular treatment has gained prominence but remains a challenge with unfavorable results. Recent literature has demonstrated that the Resolute Onyx Zotarolimus-Eluting Stent (RO-ZES) is a technically safe option with low complication rates along with 30-day outcomes associated with intracranial stent placement for ICAD with RO-ZES compared to results from the Stenting Versus Aggressive Medical Management for Preventing Recurrent Stroke in Intracranial Stenosis (SAMMPRIS) trial. Here, we aim to compare outcomes at one year with the SAMMPRIS trial with a multicenter longitudinal study. MATERIALS AND METHODS: Prospectively maintained databases across seven stroke centers were analyzed for adult patients undergoing RO-ZES placement for ICAD between January 2019 and May 2023. The primary endpoint was composite of one-year stroke, ICH, and/or death. These data were propensity score matched using age, sex, hypertension, diabetes mellitus, smoking status, and impacted vessel for comparison between RO-ZES and the SAMMPRIS percutaneous angioplasty and stenting groups (S-PTAS). RESULTS: A total of 104 patients were included, mean age ± SD: 64.9 ± 10.9 years, 25.5% female. Propensity score match analysis of the 104 patients with S-PTAS demonstrated one-year stroke, ICH, and/or death rate of 11.5% in the RO-ZES group and 28.1% in the S-PTAS group (odds ratio 4.17, 95% CI 2.06-8.96, p = 0.001). CONCLUSION: The RO-ZES system demonstrates strong potential to reduce long-term complications at one year compared with the S-PTAS group. Further prospective multicenter studies are needed to corroborate and build upon these findings.

Intracerebral hemorrhage risk after adjunct intraarterial thrombolysis in thrombectomy-treated acute ischemic stroke.

BACKGROUND AND PURPOSE: Intraarterial thrombolysis as an adjunct to mechanical thrombectomy is increasingly being considered to enhance reperfusion in acute ischemic stroke patients. Intraarterial thrombolysis may increase the risk of post-thrombectomy intracerebral hemorrhage (ICH) in certain patient subgroups. METHODS: We analyzed acute ischemic stroke patients treated with mechanical thrombectomy in a multicenter registry. The occurrence of any (asymptomatic and symptomatic) post-thrombectomy ICH was ascertained using standard definition requiring serial neurological examinations and computed tomographic scans acquired within 48 hours of the thrombectomy. We determined the risk of ICH in subgroups defined by clinical characteristics and the use of intravenous (IV) thrombolysis. RESULTS: A total of 146 (7.5%) patients received intraarterial thrombolysis among 1953 acute ischemic stroke patients who underwent mechanical thrombectomy. The proportion of patients who developed any ICH was 26 (17.8%) and 510 (28.2%) among patients who were and were not treated with intraarterial thrombolysis, respectively (p = .006). The proportion of patients who developed symptomatic ICH was 4 (2.7%) and 30 (1.7%) among patients who were and were not treated with intraarterial thrombolysis, respectively (p = .34). Among patients who received IV thrombolysis (n = 1042), the proportion of patients who developed any ICH was 9 (16.7%) and 294 (30.7%) among patients who were and were not treated with intraarterial thrombolysis, respectively (p = .028). The risk was not different in strata defined by age, gender, location of occlusion, preprocedure National Institutes of Health Stroke Scale score, time interval between symptom onset and thrombectomy, Alberta Stroke Program Early CT Score, systolic blood pressure, and serum glucose concentrations. CONCLUSIONS: In patients undergoing mechanical thrombectomy, the risk of any ICH and symptomatic ICH was not increased with intraarterial thrombolysis, including in those who had already received IV thrombolytics.

Low pressure headache and cerebral fat embolism from a sacral fracture through a Tarlov cyst: a case report.

BACKGROUND: Here we report the only formally documented case in the United Kingdom, to our knowledge, of a cerebral fat embolism secondary to non-iatrogenic trauma through a Tarlov cyst. This case demonstrates the pathology clearly giving an excellent opportunity to demonstrate a rarely seen pathology as well as illustrating the importance of the patient history to guiding further management. CASE PRESENTATION: A middle-aged patient was admitted on the acute medical take complaining of severe headache with photophobia, having just returned after a skiing holiday. Computerised tomography scan of the head showed fat within the anterior horn of both lateral ventricles, and within the subarachnoid space. Re-discussion with the patient and subsequent MRI (Magnetic Resonance Imaging) of the spine identified the pathogenesis of her symptoms: a sacral insufficiency fracture through a Tarlov cyst, causing subarachnoid fat embolism and symptoms of a low-pressure headaches due to a dural leak. Patient was medically managed and discharged with planned follow-up. Due to the Coronavirus pandemic and resolution of the patient's symptoms, they declined further follow up imaging. CONCLUSIONS: The case demonstrates a rarely seen pathology as cause of a common presenting problem, headache. Emphasizing the importance of history taking and appropriate investigations in medical cases that do not conform to the usual diagnosis.

Balloon-Assisted Catheterization of Occluded Carotid Artery (BOCA) Technique in Acute Stroke.

BACKGROUND: Acute ischemic stroke from tandem occlusion of internal carotid artery (ICA) poses a technical challenge to neurointerventionalists. OBJECTIVE: To present a novel balloon-assisted catheterization of occluded carotid artery (BOCA) technique used for rapid and effective catheterization of occluded/critically stenosed ICA in tandem occlusion. METHODS: A retrospective review of 10 patients with tandem carotid occlusion, treated with BOCA technique for revascularization between July 2020 and June 2021, was performed. Clinical, radiographic, and procedural data; details of BOCA technique; complications; and outcomes were reviewed. RESULTS: Of the 10 patients, 8 (80%) had complete occlusion of the cervical ICA and the remaining 2 had high-grade stenosis with poor intracranial flow. The mean age was 63.2 years. The mean presenting NIH Stroke Scale was 13.4. The BOCA technique resulted in recanalization of ICA in all patients and allowed mechanical thrombectomy of middle cerebral artery. Thrombolysis in cerebral infarction grade 2b/3 was achieved in all 10 patients. The mean groin-to-reperfusion time was 41.4 minutes. The mean internal carotid artery stenosis was 99.7% preoperatively and 41.1% postoperatively. Only one patient needed stent at the end of the procedure because of dissection. CONCLUSION: The BOCA technique can be used in distal first approach for acute stroke from tandem ICA occlusion. This technique allows direct guide catheterization of occluded ICA by tracking over a partially inflated balloon.

Loin to groin pain: The importance of a differential diagnosis.

INTRODUCTION: Ureteric colic frequently presents as loin to groin pain and accounts for a significant proportion of emergency urological admissions. However, a number of differential diagnoses should be considered in a systematic approach when assessing patients. PRESENTATION OF CASE: We report a case of a 30 year old man admitted with severe unilateral loin to groin pain following lumbar specific weightlifting exercises. After a significant delay due to initial mis-diagnosis he was diagnosed with acute paravertebral lumbar compartment syndrome (PVCS) and managed conservatively. DISCUSSION: Exertional PVCS is a rare and potentially life threatening condition arising following lumbar specific exercise that has only been recorded a handful of times previously. Patients typically present with intractable lumbar pain and rhabdomyolysis 6-12h following exercise. Due to initial diagnostic delay our case was managed conservatively with fluid resuscitation and monitoring of renal function. CONCLUSION: Assessment of patients with loin pain requires a systematic approach. PVCS is a rare cause of lumbar back and loin pain but one that should be considered, particularly in active young males. Early diagnosis is key to prevent the potential sequalae of untreated rhabdomyolysis. There is currently no consensus on management option for PVCS with only a few cases being reported in the literature. We describe successful management with supportive non operative treatment.