Headache as a presenting symptom is commonly encountered by the emergency department (ED) physician. The differential diagnosis of headaches is extensive and the etiologies can range from benign to life-threatening. These patients can pose a diagnostic and therapeutic challenge to the treating clinician. This chapter encapsulates the clinical approach, appropriate evaluation, and treatment options in patients presenting with the complaint of headache.

Syphilis is one of the infectious common cause of stroke. It accounted for about 2-5% of all cause of ischaemic stroke especially in the young people lived in human immunodeficiency virus pandemic era. The pathologic pattern is meningo-vasculitis and endarteritis. The lesions relate to the cortical and leptomeningeal superficial vessels and can extend to the arteries of medium and large caliber, but rarely in the small arteries Typically the common clinical presentation is the subacute vascular syndrome with the past history of chancre. The diagnosis is based on the combination of past history of chancre in a young people (particularly in HIV infected patient), systemic inflammatory signs, infarction (rarely hemorrhage) in brain imaging and cerebro-spinal fluid positivity of Venereal Disease Research Laboratory and lymphocytic pleiocytosis. The CSF Fluorescent Treponemal Antibody absorption (FTA-ABS or Nelson test) has a high sensitivity. The treatment includes the use of antibiotics (benzyl-penicillin or ceftriaxon for 10-14 days), the management of vascular risk factors and strategy to avoid other behavior toward sexual transmitted disease

Stroke is a devastating complication of tuberculous meningitis (TBM) and it is associated with higher morbidity and mortality. The common pathologies of stroke in TBM include basal exudate induced inflammatory vasospasm, vasculitis, thrombosis and occlusion of large intracerebral arteries and their penetrating branches. Since TBM associated stroke occurs in a chronic and advanced stage patient, its diagnosis can be missed clinically. Hence, radiologic investigations advance the diagnostic accuracy. We report a case of a 28-year-old right-handed black women who presented to the emergency department with a sudden onset dizziness, postural imbalance, falls and right-side visual obscuration of 1-day duration. She had non-specific constitutional symptoms since the past 3 weeks and recently she got an empiric therapy for pyogenic meningitis without improvement. At presentation, she had fever and meningismus. On neurologic exam, she was lethargic with right side visual field defect and left side cerebellar signs. Her brain magnetic resonance imaging showed multiple posterior circulation acute infarcts. Cerebrospinal fluid analysis showed lymphocytic pleocytosis with increased protein and low glucose, A Gene Xpert test from her cerebrospinal fluid was positive for Mycobacterium tuberculosis. Aspirin and antituberculosis medication with steroid was started and after 4 weeks, she was discharged improved. Stroke is a rare but a fatal complication of TBM. Therefore, early recognition and timely initiation of empiric treatment is vital to avoid disease progression to an advanced stage and prevent such grave complication of TBM

A 17-year-old female was referred with a history of several neurological episodes with seizures, sudden vision loss and transient right- and left-sided hemiparesis over 3 years. Increased acute phase reactants have been associated with these episodes. A presumptive diagnosis of vasculitis was established after detection of fever, myalgia, abdominal pain, livedo reticularis and severe hypertension at last episode. On admission, she was febrile (38.3 °C) and her blood pressure was 170/110 mmHg. Physical examination revealed livedo reticularis on both lower and upper limbs with a normal neurologic and fundoscopic examination. Diffuse muscle tenderness and colicky abdominal pain without rebound tenderness were also observed. Cranial angiography was normal, however higher signal intensity in bilateral parietal regions accompanied by parenchymal volume loss was observed in brain magnetic resonance imaging (MRI). Further investigation with conventional visceral angiography elicited multiple microaneurysms and segmental narrowings in branches of renal, hepatic and mesenteric arteries suggestive of a vasculitis affecting primarily medium-sized arteries. The patient diagnosed with polyarteritis nodosa (PAN) and all of the clinical manifestations including fever, myalgia, abdominal pain and hypertension have resolved by three types of antihypertensive medications and 5 days of pulse methylprednisolone (1 gr/m2/day). At discharge, oral methylprednisolone at a dose of 1 mg/kg/day and monthly intravenous cyclophosphamide infusions (1 gr/m2/month) for 6 months were planned for induction treatment. The patient no longer required antihypertensive medication by the second month of discharge and never had a recurrence of disease

A 16 years old female patient admitted to hospital due to fatigue, myalgia, weight loss and night sweats lasting for 10 days. On examination, patient appeared pale with livedo reticularis on lower extremities. On the 2nd day of hospitalization, she developed acute right hemiparesis, dysarthria and right facial droop. Cranial computerized tomography was performed, and it revealed multiple bilateral lesions consistent with acute intracranial hemorrhage. The cranial MRI showed restricted diffusion in the left internal capsule which was in concordance with clinical signs of acute stroke. Past medical history of the patient revealed recurrent attacks of bronchitis. In the laboratory examination, patient had elevated acute phase markers, leukocytosis, mild anemia, thrombocytosis. Further investigations revealed significantly high c-ANCA and proteinase 3 antibody titers, whereas myeloperoxidase antibody was negative. High-resolution computerized tomography revealed lesions in both lungs. A diagnosis of Granulomatosis with Polyangiitis (GPA) was confirmed. high doses of glucocorticoids (30 mg/kg/day for the consecutive 5 days) was given as an induction part of treatment together with pulse dose of cyclophosphamide (1 gr/m2/month for the consecutive 6 months). The rituximab was added to treatment (375 mg/m2/week for the consecutive 4 weeks) since the patient was considered to have life-threating complication of underlying disease. The patient responded promptly with regression of neurological findings, decline in acute phase markers and significantly improvement in patient’s general condition

Among primary form of eye vasculitis, Eales’ disease – also known as retinal periphlebitis or retinal perivasculitis is a rare inflammatory venous occlusive disease. If first cases were reported in Europe or north America, Eales’ disease is now more common in developing countries, probably because of public health issues and an association with Mycobacterium tubercolosis infection has been suggested. The pathological hallmark of the disease is the involvement of peripheral retinal vessels with a first stage of inflammation (i.e. acute phlebitis) that eventually leads to ischaemia, haemorrhages into the vitreous body and then neovascular proliferation due to circulatory deficiency. Patients may complain of sudden blurring of vision in one or both eyes, decreased vision or floaters, while focal neurological deficits can rarely occur, probably because of vasculitic involvement of cerebral arteries leading to clinical and subclinical strokes. Eales’ disease is considered as a diagnosis of exclusion and confirmed only after ruling out all possible infections and systemic aetiologies. Brain MRI or CT angiography is crucial because it may show multiple and irregular stenosis involving both anterior and vertebrobasilar circulation. Fundus oculi examination and fluorescein angiogram may reveal retinal vascular leakage. Finally, the treatment of Eales’ disease depends on the stage of the disease, so in the acute inflammatory stage oral corticosteroids are the mainstay of therapy. As other autoimmune disorders, immunosuppressive therapy, as Cyclosporine, Methotrexate and Azathioprine, may be employed with the aim of preventing relapses

A 49-year-old female patient was admitted to the emergency room with the left-sided frust hemiparesis and hemihypoesthesia. She has been experiencing intermittent cough, general weakness, and body pain for the past year. Cranial diffusion magnetic resonance imaging (MRI) revealed an acute infarct in the right occipital lobe. Chest X-ray showed bilateral hilar fullness. Mediastinal lymphadenopathy, bilateral micronodular parenchymal infiltrations, thickening of the major fissure and multiple linear reticulonodular patterns were observed on high-resolution computerized tomography (HRCT). Laboratory tests showed a sedimentation rate of 44 mm/1 h; positivity for SS-A, SS-B, Ro-52 recombinant AMA-M2 (+++) direct Coombs IgG, anti-beta-2 glycoprotein 1 IgG and high serum ACE (angiotensin converting enzyme) level. Fluorodeoxyglucose (FDG)-positron emission tomography (PET)/CT showed pathological uptake in the mediastinal and abdominal lymph nodes, ground-glass opacity infiltration located in the bilateral lungs and intramedullary bone uptake. Bone marrow biopsy revealed noncaseating granulomas. With the diagnosis of neurosarcoidosis (NS), oral corticosteroid and azathioprine were administered. Sarcoidosis is a chronic, idiopathic, inflammatory and granulomatous disease with multisystem involvement. Noncaseating epithelioid granulomas are the most characteristic findings. NS frequently occurs in patients with active disease and systemic involvement. A sarcoidosis patient may develop neurological symptoms, or the initial symptom of a patient may be typical for NS. Very rare presentations of NS are ischaemic stroke, transient ischaemic attacks (TIAs), intracranial haemorrhage and venous thrombosis. NS should be considered especially in young patients, experiencing recurrent TIAs, ischaemic or haemorrhagic strokes, and having MRI findings suggestive of NS

Vascular disorders of the gastrointestinal (GI) tract range from non-symptomatic lesions to acute life-threatening disorders. Generally, they can be classified as ischemic or bleeding, although this is often a superficial distinction with much overlap. Primary vascular disorders of the GI tract are assessed by pathologists at the time of biopsy or surgical resection, as well as at autopsy. Diagnoses requires a multidisciplinary approach, but pathology often plays a particularly important role in suggesting or confirming a diagnosis. Careful attention to pathologic specimens is important, as the distinguishing features of each entity may be subtle and difficult to appreciate. Likewise, patient management may differ significantly among conditions with overlapping diagnostic features. This chapter will provide a concise review of the diagnostic elements to consider in vascular disorders of the GI tract, and will discuss practical aspects that should help the pathologist to arrive at the best diagnosis for each case.

A brief reprise of normal coronary artery structure is followed by a discussion of normal anatomical variants of the coronary arteries. The commoner abnormal variants, including origin of the left coronary artery from the pulmonary artery and intramural course of a coronary artery, are described and illustrated, followed by a discussion of coronary fistula and atresia. A section is devoted to the variations in coronary anatomy associated with the commoner forms of congenital heart disease. Coronary arteritis is discussed, chiefly in the context of Kawasaki disease, but polyarteritis and eosinophilic arteritis are also described. Fibromuscular dysplasia is treated in some detail and idiopathic arterial calcification rounds off the chapter.