Normal preoperative levels of gamma-glutamyltransferase predict the absence of common bile duct stones in patients scheduled for laparoscopic cholecystectomy: a retrospective cohort study.

Laparoscopic cholecystectomy, nowadays, is considered the gold standard option for management in patients diagnosed with symptomatic cholelithiasis. Nevertheless, some patients may have coexisting choledocholithiasis, which manifests later in life with grave complications such as cholangitis and pancreatitis. The objective of this study is to evaluate the role of preoperative gamma-glutamyltransferase (GGT) in predicting choledocholithiasis in patients undergoing laparoscopic cholecystectomy. Method: A total of 360 patients with symptomatic cholelithiasis based on diagnosis aided with abdominal ultrasound were included in the study. The study design was a retrospective cohort. Patients were evaluated based on a comparison between findings of per-operative cholangiogram and laboratory measure of GGT. Result: The mean age of study participants was 47.22 (±28.41) years. Mean GGT levels were 121.54 (±87.91) U/l. One hundred (27.7%) participants had raised GGT. But only 19.4% had been diagnosed with filling defect positive on cholangiogram. The predictability of GGT for positive cholangiogram is statistically significant at less than 0.001 with an area under the curve of 0.922 (0.887-0.957), sensitivity of 95.7%, specificity of 88.6%, and accuracy of 90%. The standard error reported (0.018) was found to be relatively low. Conclusion: Based on the provided information, it is concluded that GGT plays an important role in predicting the coexistence of choledocholithiasis in symptomatic cholelithiasis and can be used in the setting where the facility of per-operative cholangiogram is not available.

Clinicopathological correlation of aspartate aminotransferase-to-platelet ratio (APRI) and aspartate aminotransferase-to-alanine aminotransferase ratio (AAR) with survival in esophageal carcinoma patients: a retrospective cohort analysis of 951 patients.

Esophageal cancer is the eighth most prevalent cancer globally. Previously, several biomarkers have been used to predict the prognosis, although with variable reliability. Interestingly, it is noted that changes in liver function tests levels before and after neoadjuvant treatment are predictive in terms of cancer recurrence. Objectives: The objectives of the current study were to associate novel markers, including aspartate aminotransferase-to-platelet ratio (APRI) and aspartate aminotransferase-to-alanine aminotransferase ratio (AAR) with survival in esophageal malignancy. Materials and Methods: A retrospective study in a tertiary care hospital (single-center) included 951 patients having diagnosed esophageal carcinoma of any age group. Results: The median (interquartile range) age of study participants were 50 (38-60) years, including 43% males and 57% female patients, while the median (interquartile range) levels of AAR and APRI were 0.97 (0.81-1.25) and 0.19 (0.13-0.29), respectively. AAR was found to be higher in dysphagia for solids only and dysphagia for both liquids and solids rather than liquids only (P=0.002), while other associations included well-differentiated tumor grade (P=0.011), finding of esophageal stricture on esophagogastroduodenoscopy (P=0.015), and characteristic of mass on computerized tomography scan being both circumferential and mural (P=0.005). APRI was found to be higher in adenocarcinoma (P=0.038), and finding of circumferential±ulcerated mass on esophagogastroduodenoscopy (P<0.001). On survival analysis, adenocarcinoma (P<0.001), luminal narrowing (P=0.002), AAR greater than 1.0 (P=0.006), and APRI greater than 0.2 (P=0.007) were found to be poor survival predictors. On Cox proportional hazards regression, APRI was found to be more associated with poor survival than AAR (Hazard ratio: 1.682, 1.208-2.340, P=0.002). Conclusion: This study correlated clinical and pathological features of esophageal malignancy with noninvasive markers of hepatic function.

Role of serum C-reactive protein (CRP)/Albumin ratio in predicting the severity of acute pancreatitis: A retrospective cohort.

Acute pancreatitis is a disease with a wide spectrum of severity, complications, and outcome with severe life-threatening complications develop in patients leading to high mortality in severe acute pancreatitis. The rationale of this study is to diagnose the severity of acute pancreatitis using a single test ratio, i.e., CRP/albumin ratio which is a combination of markers for systemic inflammation and nutritional status. All those patients with age group 16-80 years who were diagnosed with acute pancreatitis and admitted subsequently to ICU were included. Severe pancreatitis was determined as CT severity score above 7. About 41% patients out of total 225 had severe pancreatitis. CRP/albumin ratio >4.35 had a sensitivity of 87% and accuracy of 76% to predict acute severe pancreatitis. Elevated CRP/albumin ratio was also associated with complications like multi-organ failure OR: 2.31 [1.3-4.2], duodenal thickening OR: 2.25 [1.2-4.2], and ascites OR: 2.90 [1.5-5.6]. Although, the severity of this elevation varied with different age groups, such non-invasive and readily available parameters should be relied upon admission to risk stratify the patients suffering from pancreatitis. CRP/albumin ratio has higher sensitivity and negative predictive value to predict severe pancreatitis than CRP alone and hence give additional advantage as a prognostic marker, although Delong's test to compare AUROC was indifferent (P-value: 0.22).

A Rare Case of Extensive Cerebral Venous Sinus Thrombosis Complicated by Heparin-Induced Thrombocytopenia.

Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening cause of stroke. Several risk factors have been identified including hypercoagulable state, malignancy, use of oral contraceptives, pregnancy, head injury, infection, and prothrombotic states such as heparin-induced thrombocytopenia (HIT). HIT is a prothrombotic state leading to thrombosis in several distinct locations including CVST requiring prompt discontinuation of heparin and initiation of nonheparin anticoagulation to prevent catastrophic consequences. Very rarely, HIT can complicate the ongoing CVST leading to worsening thrombosis and clinical deterioration. We here report an exceedingly rare case of CVST complicated by HIT in a 22-year-old female patient who showed remarkable clinical improvement after discontinuation of heparin and initiation of argatroban.

First Case of Nodular Localized Primary Cutaneous Amyloidosis Treated With Bortezomib and Dexamethasone.

Nodular localized cutaneous amyloidosis is a rare form of cutaneous amyloidosis and is characterized by an extracellular deposition of insoluble amyloid fibrils which are either primarily cutaneous or a manifestation of an underlying systemic amyloidosis. Biopsy of the lesion is mandatory for the diagnosis, and histopathology shows diffuse amyloid deposits with plasmacytic infiltration. Apple-green birefringence characteristic of amyloidosis is observed when stained with Congo red and viewed under polarized light. Amyloid subtyping is done with laser microdissection followed by mass spectrometry. Majority of these lesions do not require any treatment but surgical excision, shave excision, laser therapy, and radiotherapy can be considered for symptomatic nodular localized primary cutaneous amyloidosis (NLPCA). We present a case of recurrent NLPCA in a 64-year-old woman who was treated with bortezomib and dexamethasone after failing several local therapies with excellent response.

Hormone Receptor Positive/HER2 Negative Breast Cancer With Isolated Bladder Metastasis: A Rare Case.

Breast cancer is the most common cancer diagnosed in women in North America. Hormone receptor positive (HR+) and HER2 negative (HER2-) breast cancers account for at least 60% to 70% of all breast cancer cases. They usually metastasize to lymph nodes, bones, liver, lungs, and brain. Urinary bladder is a very unusual site for metastatic HR+/HER2- breast cancer and occurs in only 2% of all metastatic disease. In this article, we present a case of a 63-year-old female with locally advanced breast cancer who underwent mastectomy, adjuvant chemotherapy, radiation, and hormonal therapy. She was in remission for almost 17 years and subsequently presented with hematuria and lower abdominal pain. Cystoscopy was performed, which showed evidence of bladder wall thickening. Histopathology showed metastatic HR+/HER2- breast cancer consistent with her history of breast primary. Imaging studies did not show any other evidence of metastatic disease. She was started on cyclin D kinase 4/6 inhibitor, palbociclib, in combination with an aromatase inhibitor, letrozole. This is an exceedingly rare case of HR+ and HER2- breast cancer with metastasis to the urinary bladder. The late onset of recurrence with bladder metastasis makes this case very unique and to our knowledge only few similar cases have been reported in the literature.

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib.

Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma associated with a monoclonal immunoglobulin M protein. Extranodal involvement in WM is not very common. In this article, we present a rare case of WM with kidney and central nervous system involvement. Bing-Neel syndrome is a distinct complication of WM where lymphoplasmacytic cells involve the central nervous system (CNS). Our patient was initially treated with dialysis and steroids with improvement in his kidney function. He was then started on systemic treatment with rituximab, cyclophosphamide, and dexamethasone with stable kidney function but persistent CNS symptoms. Due to rarity of cases, there is no standard treatment for Bing-Neel syndrome. His treatment was switched to ibrutinib with dramatic improvement in his CNS symptoms as well as radiological findings on magnetic resonance imaging.

Testicular Plasmablastic Lymphoma in an HIV-Negative Patient: A Rare Case Presentation.

Plasmablastic lymphoma (PBL) is a very rare disease and it is usually considered a human immunodeficiency virus (HIV)-related B-cell lymphoma that carries a poor prognosis. It mostly involves the oral cavity, lungs, nasal cavity, gastrointestinal tract, lymph node, and skin. Therapeutic regimens like dose-adjusted etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone (DA-EPOCH) have shown better results in these aggressive lymphomas. We report a rare case of PBL in an HIV-negative patient who presented to the clinic with a complaint of left testicular swelling for 3 months. Ultrasound showed an enlarged left testicle. He underwent a left orchiectomy and the pathology showed PBL with involvement of the spermatic cord margin. Positron emission tomography scan showed hypermetabolic mediastinal and hilar lymph nodes. He was started on DA-EPOCH but showed no response. Accordingly, salvage therapy with bortezomib in addition to ifosfamide carboplatin and etoposide (B-ICE) chemotherapy was initiated with remarkable response. Several other regimens can be used in the refractory setting; however, the evidence is mostly based on retrospective analysis.

Immunotherapy-Induced Sarcoid-Like Reaction: A Shrewd Imitator.

A 40-year-old male with a right-sided neck mass was diagnosed with metastatic melanoma. A repeat positron-emission tomography after treatment with combination immunotherapy demonstrated increased hypermetabolic activity in the right supraclavicular, hilar, and mediastinal regions. Immunotherapy was discontinued and a BRAF/MEK inhibitor combination was started. Repeat imaging showed a decrease in size of the neck mass; however, hilar and mediastinal lymph nodes increased in size. A fine needle aspiration of mediastinal lymph nodes was consistent with a granulomatous process. A diagnosis of a sarcoid-like reaction (SLR) was made, and he was started on steroids. A follow-up positron emission tomography showed decreased hilar and mediastinal lymph node hypermetabolic activity. We, therefore, report this rare case of immunotherapy-induced SLR to the expanding literature on immunotherapy-related adverse effects and would like to highlight that SLR can occur in conjunction with disease progression making it challenging to distinguish between the two.

Clinical, epidemiological, and diagnostic characteristics of esophageal carcinoma in a Pakistani population.

BACKGROUND: Esophageal cancer ranks eighth among the most prevalent cancers globally and is the sixth leading cause of mortality from malignancy worldwide; it is the 7th most prevalent malignancy in males and the 6th most prevalent malignancy in females. In Pakistan, the incidence is 4.1 per 100 000 with the province of Baluchistan having the greatest incidence. OBJECTIVE: Report trends and characteristics of esophageal cancer in Pakistan over the past 10 years. DESIGN: Cross-sectional, retrospective review of medical records. SETTING: Tertiary care hospital. PATIENTS AND METHODS: The study included all patients admitted with a diagnosis of esophageal carcinoma with a mass lesion or luminal narrowing. The records were for the period from January 2011 to September 2020. MAIN OUTCOME MEASURES: Gender, histopathological types/differentiation along with clinical/laboratory findings. SAMPLE SIZE: 1009 with a mean (standard deviation) age of 49.3 (14.2) and a median (interquartile range of 50 (22) years (443 males and 566 females with age of 51.0 [20] years and 47.9 [23.8] years, respectively). The male-to-female ratio was 1:1.2. RESULTS: Most patients (82.7%) had squamous cell carcinomas with a male-to-female ratio of 1:2; the remainder had adenocarcinomas with a male-to-female ratio of 4:1 (P<.001). Dysphagia, weight loss, and vomiting were the most prevalent symptoms. More adenocarcinoma masses were located distally compared with squamous cell carcinomas (P=.030), lesions were most likely to be ulcerated (P=.910). Luminal narrowing was slightly more frequent in squamous cell carcinoma (P=.215), thickening was more prominently circumferential in the adenocarcinomas. In squamous cell carcinoma, the most common variant was moderately differentiated while moderate to poorly differentiated variants were more common in adenocarcinoma. In the survival analysis, squamous cell carcinoma (P=.014 vs adenocarcinoma), particularly the well-differentiated type (P=.018 vs other variants), projected a better prognosis. CONCLUSION: Our study reports the most recent trends of esophageal carcinoma in this region. LIMITATIONS: Lack of metastatic workup, TNM staging, and mode of treatment, along with the overlapping pattern of histological variants. CONFLICT OF INTEREST: None.

An updated review of epidemiology, risk factors, and management of male breast cancer.

Unlike female breast cancer, male breast cancer (MBC) is rare and not very well understood. Prospective data in the management of MBC are lacking and majority of treatment strategies are adopted from the established guidelines for breast cancer in women. The understanding of biology, clinical presentation, genetics, and management of MBC is evolving but there still remains a large knowledge gap due to the rarity of this disease. Older age, high estradiol levels, klinefelter syndrome, radiation exposure, gynecomastia, family history of breast cancer, BRCA2 and BRCA1 mutation are some of the known risk factors for MBC. Routine screening mammography is not recommended for asymptomatic men. Diagnostic mammogram with or without ultrasound should be considered if there is a suspicion for breast mass. Majority of men with early-stage breast cancer undergo mastectomy whereas breast conserving surgery (BCS) with sentinel lymph node biopsy (SLNB) remains an alternative option in selected cases. Since the majority of MBC are hormone receptor positive (HR+), adjuvant hormonal therapy is required. Tamoxifen for a total of 5 to 10 years is the mainstay adjuvant hormonal therapy. The role of neoadjuvant and adjuvant chemotherapy for early-stage breast cancer is uncertain and not commonly used. The role of gene recurrence scores like oncotype Dx and mammaprint is evolving and can be used as an aid for adjuvant chemotherapy. Majority of metastatic MBC are treated with hormonal therapy with either tamoxifen, gonadotropin-releasing hormone agonist (GnRH) with aromatase inhibitors (AI), or fulvestrant. Chemotherapy is reserved for patients with visceral crisis or rapidly growing tumors.

Cutaneous Disease as a Rare Presentation of Follicular Lymphoma Progression.

Follicular lymphoma, the third most common lymphoid malignancy, is considered indolent but incurable non-Hodgkin lymphoma. Isolated cutaneous relapse from follicular lymphoma is very uncommon, and very few cases have been reported in the literature. In this article, we present a case of an adult patient with a history of treated follicular lymphoma who presented with a skin lesion on his face and scalp. Further workup, including biopsy, led to the diagnosis of relapsed follicular lymphoma with no progression of disease elsewhere. We reviewed cases of follicular lymphoma, which relapsed with isolated cutaneous involvement. Treatment options for relapsed follicular lymphoma include observation, anti-CD 20 antibody alone, or in combination with chemotherapy, radio-immunotherapy, and stem cell transplantation in selected patients. Increased awareness of disease evolution and prompt diagnosis of this form of relapse from follicular lymphoma will improve the effectiveness and outcome of its management.

Hematological parameters predicting severity and mortality in COVID-19 patients of Pakistan: a retrospective comparative analysis.

BACKGROUND AND OBJECTIVES: COVID-19 is a global pandemic. In our study, we aimed to utilize the hematological parameters in predicting the prognosis and mortality in COVID-19 patients. MATERIALS AND METHODS: A retrospective, observational study was conducted to include all the admitted patients (n = 191) having COVID-19 Polymerase chain reaction (PCR) positive, and evaluated those for prognosis and disease outcome by utilizing several biochemical and hematological markers. RESULTS: Amongst the patients admitted in the ward versus in the intensive care unit (ICU), there were significant differences in mean hemoglobin (P = 0.003), total leukocyte count (P = 0.001), absolute neutrophil and lymphocyte counts (P < 0.001), absolute monocyte count (P = 0.019), Neutrophil-to-Lymphocyte ratio (NLR) and Lymphocyte-to-Monocyte ratio (LMR) (P < 0.001), Platelet-to-Lymphocyte ratio (PLR) and Lymphocyte-to C-reactive protein ratio (LCR) (P = 0.002), and C-reactive protein (CRP) levels (P < 0.001). Amongst the deceased patients, there was significant leukocytosis (P = 0.008), neutrophilia and lymphopenia (P < 0.001), increased NLR (P = 0.001), decreased LMR (P < 0.001), increased PLR (p = 0.017), decreased LCR (p = 0.003), and elevated CRP level (P < 0.001). A receiver operating characteristic curve obtained for the above parameters showed NLR (AUC: 0.841, PPV: 83.6%) and PLR (AUC: 0.703, PPV: 81.8%) for ICU patients, while NLR (AUC: 0.860, PPV: 91.1%) and PLR (AUC: 0.677, PPV: 87.5%) for the deceased patients had significant accuracy for predicting the disease severity of COVID-19 in comparison to survivors. CONCLUSION: The inflammatory markers and hematological indices are a good guide for predicting the severity and disease outcome of coronavirus disease. NLR and PLR are elevated in severe disease while LMR and LCR are inversely correlating with disease severity.

Renal limited ANCA-positive vasculitis: a rare manifestation of a rare disease.

Pauci-immune crescentic glomerulonephritis is the most common variant of rapidly progressive glomerulonephritis, accounting for approximately 80% of total cases. Most of the cases are associated with the presence of anti-neutrophil cytoplasmic antibody (ANCA) and are usually referred to as ANCA-associated vasculitis. A 68-year-old male with no previous renal history presented with complaints of shortness of breath, cough, and bilateral leg swelling for 2 weeks. Initial workup was significant for creatinine elevated at 2.9 mg/dL, blood urea nitrogen at 65 mg/dL, and glomerular filtration rate of 27 mL/min. Further workup was unremarkable for any significant abnormality. Subsequently patient's kidney function worsened, and temporary hemodialysis was started. Kidney biopsy was performed, which later came back significant for necrotizing arteritis, multifocal, with focal necrotizing and crescentic glomerulonephritis, pauci-immune type. High-dose corticosteroids were administered, and good clinical response was noticed. This is a very rare case of renal limited pauci-immune crescentic glomerulonephritis with annual incidence of 7 to 10 cases per million every year in the United States. The absence of involvement of other organs makes our case even rarer. Mortality is as high as 90% in untreated patients and aggressive therapy with glucocorticoids and cyclophosphamide or rituximab are the mainstay of treatment. The presence of significant renal impairment in the absence of other organs involvement in our patient makes it a very unique presentation of ANCA-positive vasculitis.

High-Grade Malignant Spindle Cell Sarcoma of the Pelvis Presenting With Bloody Diarrhea and Urinary Retention: A Rare Case With a Rare Presentation.

Soft tissue sarcomas (STSs) are rare malignant tumors originating from mesenchymal cells. Extremities are the most commonly affected anatomical sites, and majority of them present as a painless mass. We present a very interesting case of high-grade spindle cell sarcoma of the pelvis manifested as urinary retention and bloody diarrhea. A 68-year-old male presented to the emergency department with abdominal pain, inability to void urine, and bloody diarrhea. Straight urinary catheterization retrieved 900 mL of urine, and a Foley catheter was placed. All laboratory workup including complete blood count, complete metabolic panel, and urinalysis were within normal limits, but computed tomography (CT) of the abdomen and pelvis with contrast was remarkable for bilateral moderate hydronephrosis and a large 14 x 9.1 cm pelvic mass fistulizing into the rectum. To better identify the extent of disease, magnetic resonance imaging (MRI) with contrast was performed, which also revealed a similar large pelvic mass fistulizing into the rectum. Core needle biopsy of the mass was performed, which showed malignant spindle and epithelioid neoplasm with necrosis consistent with high-grade sarcoma. This is a very rare presentation of STS, and, to the best of our knowledge, only few similar cases have been reported thus far.

Bullous Skin Rash: A Rare Case of Palbociclib-Induced Dermatological Toxicity.

Palbociclib is an FDA-approved cyclin-dependent kinase inhibitor to treat hormone-positive, HER2-negative metastatic breast cancer. Severe skin toxicities are rare but important adverse events associated with these agents. Early detection of severe forms of skin lesions is crucial to permit the immediate discontinuation of palbociclib in order to avoid unacceptable risk level in the form of severe cutaneous toxicities like Steven-Johnson Syndrome. In such cases, palbociclib should be abruptly discontinued and an early aggressive support should be initiated. We here present a case of 50-year-old Caucasian female, who developed acute onset blistering skin lesions one to two weeks after she was started on palbociclib. We sought to report this case given the unusual toxicity and to emphasize the importance of identifying the acute onset of blistering skin lesions, regardless of their extension, should prompt awareness of their potential severity.

Atypical Takotsubo Cardiomyopathy Secondary to Combination of Chemo-Immunotherapy in a Patient With Non-Small Cell Lung Cancer.

Takotsubo cardiomyopathy (TC) also known as broken heart syndrome or stress-induced cardiomyopathy is a relatively rare and transient form of cardiomyopathy. It usually mimics myocardial infarction in terms of clinical and electrocardiographic presentation, but coronary angiography usually does not reveal any evidence of coronary artery occlusion. Even though many underlying causes including emotional, physical or physiological stress have been identified, the exact pathogenesis remains uncertain. Few of anticancer therapies have been reported as an emerging cause of TC; however, no strong evidence of immunotherapy causing cardiomyopathy. We here present a very rare case of atypical TC in a 57-year-old female with advanced stage non-small cell lung cancer who underwent combined cytotoxic chemotherapy and immunotherapy with carboplatin, pemetrexed and pembrolizumab.

Sinking Skin Flap Syndrome: Phenomenon of Neurological Deterioration after Decompressive Craniectomy.

Sinking skin flap syndrome is rare phenomenon that occurs in patients with large craniectomies. Alteration in normal anatomy and pathophysiology can result in wide variety of symptoms including altered mental status, hemodynamic instability, and dysautonomias. Management is largely conservative. We here present a case of a patient with large craniectomy who was admitted to our hospital with pneumonia. Later on, he developed worsening mental status and CT head revealed sinking skin flap with significant midline shift. This is a very rare case of neurological deterioration after craniectomies, commonly known as sinking skin flap syndrome. To our knowledge, only few cases have been reported so far.

Slipping Rib Syndrome in a Female Adult with Longstanding Intractable Upper Abdominal Pain.

Slipping rib syndrome is a rare cause of abdominal or lower chest pain that can remain undiagnosed for many years. Awareness among health care personnel of this rare but significant disorder is necessary for early recognition. Prompt treatment can avoid unnecessary testing, radiographic exposure, and years of debilitating pain. A 52-year-old female was evaluated for a 3-year history of recurrent abdominal and lower chest pain. Pain was sharp, primarily located in the lower chest and subcostal region left more than right, waxing and waning, nonradiating, and aggravates with specific movements. She underwent frequent physical therapies, treated with multiple muscle relaxants and analgesics with minimal improvement. Imaging modalities including CT scan, MRI, and X-rays performed on multiple occasions failed to signify any underlying abnormality. Complete physical examination was unremarkable except for positive hooking maneuver. Dynamic flow ultrasound of lower chest was performed which showed slipping of the lowest rib over the next lowest rib bilaterally left worse than right, findings consistent with slipping rib syndrome. Slipping rib syndrome is caused by hypermobility of the floating ribs (8 to 12) which are not connected to the sternum but attached to each other with ligaments. Diagnosis is mostly clinical, and radiographic tests are rarely necessary. Hooking maneuver is a simple clinical test to reproduce pain and can aid in the diagnosis. Reassurance and avoiding postures that worsen pain are usually helpful. In refractory cases, nerve block and surgical intervention may be required.

Non-Atherosclerotic Obliteration Of Bilateral Supraclinoid Internal Carotid Arteries; A Classic Case Of Moyamoya Disease.

Moyamoya disease is an idiopathic progressive vasculopathy of distal internal carotid artery and circle of Willis which leads to the development of characteristic smoky appearance of the vascular collateral network on angiography. With the highest reported incidence among Japanese population, it has been under recognized as a cause of cerebrovascular accidents in Western countries. Here we report a case of a young 20-year-old Caucasian woman who presented to the emergency department with expressive aphasia, right arm weakness and numbness for three days. Imaging modalities confirmed Moyamoya disease.