Although the diagnosis of peripheral arteriopathy is fundamentally clinical (in this patient the picture is very typical), we can objectively assess the severity of the disease with the use of bloodless techniques. The most important is the ankle-brachial index. Therefore, answer 2 is correct. Imaging techniques (arteriography, angioCT or angioMRI) are used when surgical repair of arterial occlusion is considered.

Although the diagnosis of peripheral arteriopathy is fundamentally clinical (in this patient the picture is very typical), we can objectively assess the severity of the disease with the use of bloodless techniques. The most important is the ankle-brachial index. Therefore, [HIDDEN]. Imaging techniques (arteriography, angioCT or angioMRI) are used when surgical repair of arterial occlusion is considered.

A 64-year-old man consults for right calf pain when climbing hills and stairs and when walking on level ground for a prolonged period of time. He reports that the pain becomes so intense with exertion that he is forced to stand up, which causes the pain to improve in a matter of minutes. As personal history, the patient is a smoker of 20 cigarettes a day, diabetic on metformin and hypertensive. On examination, there is good peripheral perfusion of the right foot, but the patient has a decreased pedal pulse. Which of the following complementary tests should be ordered initially for the diagnosis and assessment of the severity of the patient's disease?

Computerized axial tomography with vascular reconstruction of lower extremities.

Peripheral vascular examination

Special maneuvers Ankle-brachial pressure index (ABPI) assesses peripheral vascular disease. It may however be unreliable in patients with calcified arteries in the calf (often diabetic patients) or those with extensive oedema, in which case toe pressure or Toe-brachial pressure index (TBPI) should be measured to aid in the diagnosis. Venous refill with dependency (should be less than 30 seconds) – the vein should bulge outward within 30 seconds of elevation for one minute. Buerger's test (assessment of arterial sufficiency):With the patient supine, note the colour of the feet soles. They should be pink. Then elevate both legs to 45 degrees for more than 1 minute. Observe the soles. If there is marked pallor (whiteness), ischemia should be suspected. Next check for rubor of dependency. Sit the patient upright and observe the feet. In normal patients, the feet quickly turn pink. If, more slowly, they turn red like a cooked lobster, suspect ischemia.

Peripheral vascular disease: diagnosis and treatment.

Peripheral vascular disease is a manifestation of systemic atherosclerosis that leads to significant narrowing of arteries distal to the arch of the aorta. The most common symptom of peripheral vascular disease is intermittent claudication. At other times, peripheral vascular disease leads to acute or critical limb ischemia. Intermittent claudication manifests as pain in the muscles of the legs with exercise; it is experienced by 2 percent of persons older than 65 years. Physical findings include abnormal pedal pulses, femoral artery bruit, delayed venous filling time, cool skin, and abnormal skin color. Most patients present with subtle findings and lack classic symptoms, which makes the diagnosis difficult. The standard office-based test to determine the presence of peripheral vascular disease is calculation of the ankle-brachial index. Magnetic resonance arteriography, duplex scanning, and hemodynamic localization are noninvasive methods for lesion localization and may be helpful when symptoms or findings do not correlate with the ankle-brachial index. Contrast arteriography is used for definitive localization before intervention. Treatment is divided into lifestyle, medical, and surgical therapies. Lifestyle therapies focus on exercise, smoking cessation, and dietary modification. Medical therapy is directed at reducing platelet aggregation. In addition, patients with contributing disorders such as hypertension, diabetes, and hyperlipidemia need to have these conditions managed as aggressively as possible. Surgical therapies include stents, arterectomies, angioplasty, and bypass surgery.

Noninvasive Diagnostic Methods for Better Screening of Peripheral Arterial Disease.

Peripheral arterial disease (PAD) is a common circulatory problem in which narrowed arteries reduce blood flow to extremities, usually legs. It does not receive enough blood flow to keep up with demand. This causes symptoms, most notably leg pain while walking which is known as claudication. It is a common manifestation of type-2 Diabetes, but the relationship between other vascular diseases and lower limb peripheral arterial disease (LLPAD) has been poorly understood and investigated. When assessing a patient with clinical LLPAD, 2 methods are used to establish a diagnosis: one is noninvasive testing and other is invasive. Invasive methods are painful and get so bad that some people need to have a leg surgery. People with diabetes are at increased risk for amputation, and it is used only when the damage is very severe. Diagnosis of LLPAD begins with a physical examination, patient history, and certain questionnaire, and noninvasive mode of diagnosis is started for the screening of patients. Clinicians check for weak pulses in the legs and then decide for further diagnosis. This article discusses the prevalence of LLPAD worldwide and in India, along with the clinical effectiveness and limitations of these methods in case of diabetes. The focus of this review is to discuss only those noninvasive methods that are widely used for screening of LLPAD, such as ankle-brachial index (ABI), toe-brachial index (TBI), and use of photoplethysmogram (PPG) especially in case of diabetic patients. Also, this article gives an overview of the work carried out using ABI, TBI, and PPG for detection of LLPAD. These tests are not painful and could be performed in a cost-effective manner to avoid delays in screening/diagnosis and also reduce costs.

Intermittent claudication

Intermittent claudication, also known as vascular claudication, is a symptom that describes muscle pain on mild exertion (ache, cramp, numbness or sense of fatigue), classically in the calf muscle, which occurs during exercise, such as walking, and is relieved by a short period of rest. It is classically associated with early-stage peripheral artery disease, and can progress to critical limb ischemia unless treated or risk factors are modified. Claudication derives from the Latin verb claudicare, "to limp". Signs and symptoms One of the hallmarks of arterial claudication is that it occurs intermittently. It disappears after a very brief rest and the patient can start walking again until the pain recurs. The following signs are general signs of atherosclerosis of the lower extremity arteries: cyanosis atrophic changes like loss of hair, shiny skin decreased temperature decreased pulse redness when limb is returned to a "dependent" position (part of Buerger's test)

Intermittent claudication

The six "P"s of ischemia Pain Pallor (increased) Pulse (decreased) Perishing cold Paraesthesia Paralysis Causes Most commonly, intermittent (or vascular or arterial) claudication is due to peripheral arterial disease which implies significant atherosclerotic blockages resulting in arterial insufficiency. Other uncommon causes are coarctation of the aorta, Trousseau disease and Beurger's disease (Thromboangiitis obliterans), in which vasculitis occurs. Raynaud's phenomenon functional vasospasm. It is distinct from neurogenic claudication, which is associated with lumbar spinal stenosis. It is strongly associated with smoking, hypertension, and diabetes. Diagnosis Intermittent claudication is a symptom and is by definition diagnosed by a patient reporting a history of leg pain with walking relieved by rest. However, as other conditions such as sciatica can mimic intermittent claudication, testing is often performed to confirm the diagnosis of peripheral artery disease.

Leg symptoms, the ankle-brachial index, and walking ability in patients with peripheral arterial disease.

To determine how functional status and walking ability are related to both severity of lower extremity peripheral arterial disease (PAD) and PAD-related leg symptoms. Cross-sectional study. Academic medical center. Patients aged 55 years and older diagnosed with PAD in a blood flow laboratory or general medicine practice (n = 147). Randomly selected control patients without PAD were identified in a general medicine practice (n = 67). Severity of PAD was measured with the ankle-brachial index (ABI). All patients were categorized according to whether they had (1) no exertional leg symptoms; (2) classic intermittent claudication; (3) exertional leg symptoms that also begin at rest (pain at rest), or (4) exertional leg symptoms other than intermittent claudication or pain at rest (atypical exertional leg symptoms). Participants completed the 36-Item Short-Form Health Survey (SF-36) and the Walking Impairment Questionnaire (WIQ). The WIQ quantifies patient-reported walking speed, walking distance, and stair-climbing ability, respectively, on a scale of 0 to 100 (100 = best). In multivariate analyses patients with atypical exertional leg symptoms, intermittent claudication, and pain at rest, respectively, had progressively poorer scores for walking distance, walking speed, and stair climbing. The ABI was measurably and independently associated with walking distance (regression coefficient = 2.87/0.1 ABI unit, p =.002) and walking speed (regression coefficient = 2.09/0.1 ABI unit, p =.015) scores. Among PAD patients only, pain at rest was associated independently with all WIQ scores and six SF-36 domains, while ABI was an independent predictor of WIQ distance score. Both PAD-related leg symptoms and ABI predict patient-perceived walking ability in PAD.

Early diagnosis of peripheral arterial disease can save limbs.

Prompt identification and management of patients with peripheral arterial disease can improve quality of life, save limbs and reduce cardiovascular events. The most common initial symptom is leg pain on exertion or intermittent claudication. More severe or critical limb ischaemia can present with pain at rest, ulceration, tissue loss and/or gangrene, In most patients the symptoms remain stable, but approximately 20% will develop limb threatening critical ischaemia. The incidence of peripheral arterial disease increases with age and up to 20% of people aged over 60 are affected to some degree. The incidence is also high in smokers, diabetes patients, and those with coronary disease. A focused history should identify the presence and severity of intermittent claudication and any critical limb ischaemia. Examination should concentrate on the palpation of lower limb arterial pulses and look for signs of critical ischaemia such as ulceration. The key primary care investigation in suspected peripheral arterial disease is measurement of the ankle brachial pressure index. Lifestyle interventions are a key component of management. NICE recommends that a supervised exercise programme is offered to all patients with intermittent claudication. Pharmacological therapy should always include an antiplatelet agent and statin. Vasoactive drugs such as naftidrofuryl oxalate should be considered for symptom control in intermittent claudication when exercise has not led to a satisfactory improvement and the patient prefers not to be referred for revascularisation. Patients with severe and inadequately controlled symptoms should be referred to secondary care with a view to further imaging to assess the appropriateness of revascularisation.

Chronic limb threatening ischemia

Signs and symptoms Critical limb ischemia includes rest pain and tissue loss. Rest pain Rest pain is a continuous burning pain of the lower leg or feet. It begins, or is aggravated, after reclining or elevating the limb and is relieved by sitting or standing. It is more severe than intermittent claudication, which is also a pain in the legs from arterial insufficiency. Tissue loss Tissue loss is the development of arterial insufficiency ulcers or gangrene due to peripheral artery disease. Diagnosis Critical limb ischemia is diagnosed by the presence of ischemic rest pain, and an ulcers that will not heal or gangrene due to insufficient blood flow. Insufficient blood flow may be confirmed by ankle-brachial index (ABI), ankle pressure, toe-brachial index (TBI), toe systolic pressure, transcutaneous oxygen measurement (TcPo2 ), or skin perfusion pressure (SPP).

Ankle brachial index for the diagnosis of lower limb peripheral arterial disease.

Peripheral arterial disease (PAD) of the lower limb is common, with prevalence of both symptomatic and asymptomatic disease estimated at 13% in the over 50 age group. Symptomatic PAD affects about 5% of individuals in Western populations between the ages of 55 and 74 years. The most common initial symptom of PAD is muscle pain on exercise that is relieved by rest and is attributed to reduced lower limb blood flow due to atherosclerotic disease (intermittent claudication). The ankle brachial index (ABI) is widely used by a variety of healthcare professionals, including specialist nurses, physicians, surgeons and podiatrists working in primary and secondary care settings, to assess signs and symptoms of PAD. As the ABI test is non-invasive and inexpensive and is in widespread clinical use, a systematic review of its diagnostic accuracy in people presenting with leg pain suggestive of PAD is highly relevant to routine clinical practice. To estimate the diagnostic accuracy of the ankle brachial index (ABI) - also known as the ankle brachial pressure index (ABPI) - for the diagnosis of peripheral arterial disease in people who experience leg pain on walking that is alleviated by rest. We carried out searches of the following databases in August 2013: MEDLINE (Ovid SP),Embase (Ovid SP), the Cumulative Index to Nursing and Allied Health Literature (CINAHL) (EBSCO), Latin American and Caribbean Health Sciences (LILACS) (Bireme), Database of Abstracts of Reviews of Effects and the Health Technology Assessment Database in The Cochrane Library, the Institute for Scientific Information (ISI) Conference Proceedings Citation Index - Science, the British Library Zetoc Conference search and Medion. We included cross-sectional studies of ABI in which duplex ultrasonography or angiography was used as the reference standard. We also included cross-sectional or diagnostic test accuracy (DTA) cohort studies consisting of both prospective and retrospective studies.Participants were adults presenting with leg pain on walking that was relieved by rest, who were tested in primary care settings or secondary care settings (hospital outpatients only) and who did not have signs or symptoms of critical limb ischaemia (rest pain, ischaemic ulcers or gangrene).The index test was ABI, also called the ankle brachial pressure index (ABPI) or the Ankle Arm Index (AAI), which was performed with a hand-held doppler or oscillometry device to detect ankle vessels. We included data collected via sphygmomanometers (both manual and aneroid) and digital equipment. Two review authors independently replicated data extraction by using a standard form, which included an assessment of study quality, and resolved disagreements by discussion. Two review authors extracted participant-level data when available to populate 2×2 contingency tables (true positives, true negatives, false positives and false negatives).After a pilot phase involving two review authors working independently, we used the methodological quality assessment tool the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2), which incorporated our review question - along with a flow diagram to aid reviewers' understanding of the conduct of the study when necessary and an assessment of risk of bias and applicability judgements. We screened 17,055 records identified through searches of databases. We obtained 746 full-text articles and assessed them for relevance. We scrutinised 49 studies to establish their eligibility for inclusion in the review and excluded 48, primarily because participants were not patients presenting solely with exertional leg pain, investigators used no reference standard or investigators used neither angiography nor duplex ultrasonography as the reference standard. We excluded most studies for more than one reason.Only one study met the eligibility criteria and provided limb-level accuracy data from just 85 participants (158 legs). This prospective study compared the manual doppler method of obtaining an ABI (performed by untrained personnel) with the automated oscillometric method. Limb-level data, as reported by the study, indicated that the accuracy of the ABI in detecting significant arterial disease on angiography is superior when stenosis is present in the femoropopliteal vessels, with sensitivity of 97% (95% confidence interval (CI) 93% to 99%) and specificity of 89% (95% CI 67% to 95%) for oscillometric ABI, and sensitivity of 95% (95% CI 89% to 97%) and specificity of 56% (95% CI 33% to 70%) for doppler ABI. The ABI threshold was not reported. Investigators attributed the lower specificity for doppler to the fact that a tibial or dorsalis pedis pulse could not be detected by doppler in 12 of 27 legs with normal vessels or non-significant lesions. The superiority of the oscillometric (automated) method for obtaining an ABI reading over the manual method with a doppler probe used by inexperienced operators may be a clinically important finding. Evidence about the accuracy of the ankle brachial index for the diagnosis of PAD in people with leg pain on exercise that is alleviated by rest is sparse. The single study included in our review provided only limb-level data from a few participants. Well-designed cross-sectional studies are required to evaluate the accuracy of ABI in patients presenting with early symptoms of peripheral arterial disease in all healthcare settings. Another systematic review of existing studies assessing the use of ABI in alternative patient groups, including asymptomatic, high-risk patients, is required.

Seropositive Neuromyelitis Optica in a Case of Undiagnosed Ankylosing Spondylitis: A Neuro-Rheumatological Conundrum.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels. Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases. However, AQP-4-antibody-positive NMOSD coexisting with ankylosing spondylitis (AS) is rare. AS is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27. In this study, a 35-year-old Indian man with an undiagnosed progressive axial spondyloarthropathy (i.e., AS) is reported presenting with acute-onset longitudinally extensive transverse myelitis, a clinical subset of NMOSD. Neuromyelitis optica spectrum disorder (NMOSD), a primary demyelinating disorder of the central nervous system (CNS), is an autoimmune astrocytopathy against foot processes of aquaporin-4 (AQP4) water channels, which manifests with optic neuritis, longitudinally extensive transverse myelitis (LETM), area-postrema syndrome, brainstem syndrome diencephalic syndrome, and cerebral syndrome.<sup1-4</sup Ankylosing spondylitis (AS) is an immune-mediated disorder, a subset of axial spondyloarthropathies, which commonly manifests as chronic inflammatory back pain in young people, and it has a strong association with HLA-B27.<sup5,6</sup AS characteristically targets the axial skeleton, peripheral joints, entheses (connective tissues between tendons/ligaments and bones), and gut.<sup5,6</sup Patients with NMOSD tend to have other coexisting autoimmune/connective tissue diseases.<sup7</sup For example, cases with NMOSD and multiple sclerosis, which are other autoimmune primary demyelinating disorders of the CNS, have been reported.<sup8,9</sup However, concurrent existence of AS and NMOSD in the same patient even over years of disease course is rare.<sup10,11</sup In addition, studies describing neurological manifestations of AS are limited,<sup12</sup and they focus on joint inflammation and long-standing bony pathology (ankylosis) related to compressive myelopathy, myelo-radiculopathy, and cauda equina syndromes.<sup12,13</sup The authors present a case of a young Indian man with an undiagnosed progressive AS (misdiagnosed and mismanaged by an indigenous medical practitioner) presenting with acute-onset LETM variant of AQP4-positive NMOSD. A 35-year-old healthy, non-comorbid man from rural India came to the outpatient department with complaints of persistent tingling, numbness, and weakness of both lower limbs (right more than left) for 10 days. The clinical picture showed acute-onset urinary retention, which was relieved by urinary catheterization. An indigenous medical practitioner had prescribed drugs to treat a urinary tract infection. His weakness gradually progressed over the following week, causing him to become bedridden. During the removal of the catheter, he felt urgency, increased frequency of micturition, and overt urinary incontinence. He gave no history suggestive of any girdle-like sensations, root/radicular/tract pain, vertebral pain, trauma, recent vaccination, and diarrheal or febrile illness. For the last 8 months, he had a complaint of an insidious-onset, persistent, bilateral, dull aching pain in the gluteal region accompanied by low-back pain and morning stiffness up to 1 h, which markedly improved with activity and reoccurred following long periods of inactivity. He sometimes had to rise in the middle of the night because of excruciating pain, which could be relieved after moving around the room and corridors for half an hour. He was taking over-the-counter diclofenac tablets for pain relief prescribed by some indigenous medical practitioners who told him that it was due to overwork in agricultural fields, that is, mechanical back pain. He also had a normal X-ray of the lumbosacral spine. He had no addiction liabilities, and none of the family members had ever suffered from a similar kind of illness. He had never consulted any trained medical practitioner, as his previous back-pain-related symptoms responded well to the tablets prescribed by the indigenous medical practitioner(s). During examination, he was found to have recent-onset, asymmetric spastic paraparesis (right more than left) with upper motor neuron-type urinary bladder symptoms. Cognitive assessment (assessed by the Montreal cognitive assessment test) was normal, and posterior column sensations were preserved. Sensory system examination revealed no definite sensory level. Except for the paretic lower limbs, cerebellar functions were normal in other regions. Neuro-ophthalmological examinations were also normal, and no signs of meningeal irritation were observed. The history and course of the disease and clinical examinations were analyzed. Selective tractopathy (early and predominant motor and autonomic tract affection) was suggested for an intramedullary demyelinating pathology affecting the anterior central cord. This case was initially classified as acute-onset non-compressive myelopathy at the lower cervical/upper dorsal region level in a patient with a pre-existing axial spondyloarthropathy. Complete blood cell count; liver, kidney, and thyroid function tests; and plasma glucose and electrolytes were normal, except for an increased erythrocyte sedimentation rate (66 mm in the first hour). Magnetic resonance imaging (MRI) of the spinal cord revealed a demyelinating LETM from C5 to D4 level (Figure 1). Meanwhile, an MRI of the sacroiliac joints revealed bilateral sacroiliitis. Brain and orbital MRIs were devoid of any lesions. Anti-aquaporin 4 (AQP-4) antibodies were tested by cell-based assay in serum and cerebrospinal fluid (CSF), and both were positive. CSF further revealed lymphocytic pleocytosis and increased intrathecal protein production. Visually evoked potential recordings were also normal. In addition, anti-myelin oligodendrocyte glycoprotein antibodies were negative. Anti-nuclear antibody (ANA), ANA-profile, autoimmune vasculitis profile (c-ANCA, p-ANCA), neurovirus panel (i.e., polymerase chain reaction for adenovirus, Epstein-Barr virus, herpes simplex viruses 1 and 2, human herpesviruses 6 and 7, cytomegalovirus, enteroviruses, varicella-zoster virus, Japanese encephalitis, and dengue virus), CSF-polymerase chain reaction for <iMycobacterium tuberculosis</i, angiotensin-converting enzyme, anti-phospholipid, and anti-thyroid antibodies were negative. Anti-CCP-antibody and rheumatoid factor were also negative, including creatine phosphokinase level and serum vitamin B12. Moreover, serologies for hepatitis B, C, human immunodeficiency virus, and scrub typhus were negative. However, HLA-B27 assay was positive. The final diagnosis was AQP4-positive NMOSD associated with AS. He was placed on pulse intravenous methylprednisolone (1 g/day for 5 days). Consequently, his lower limb power improved remarkably. Cyclical rituximab therapy was initiated to prevent relapses. At 3-month follow-up, he had no residual neurological deficit except for persistence of paresthesias. Neuroimaging and visually evoked potential studies revealed no active or new lesions. After 6 months of therapy, a subjective and objective improvement was observed in disease severity based on the Ankylosing Spondylitis Disease Activity Score. Our patient satisfied the new Assessment of SpondyloArthritis International Society diagnostic/classification criteria for AS and the Wingerchuk criteria for NMOSD,<sup4,14</sup an association that has been rarely reported.<sup10,11</sup Amid the extra-articular complications of long-standing AS, neurological manifestations are considered infrequent.<sup15</sup However, subclinical neurological complications may be frequent in AS.<sup12</sup Common neurological manifestations result from bony (vertebral) ankylosis, subluxation of joints, ossification of anterior and posterior longitudinal ligaments, secondary spinal canal stenosis, bony (vertebral) fractures, and subsequent compressions over nerve radicles/roots/cauda equina, and inflammation-related (entrapment) peripheral neuropathies.<sup12,16,17</sup Acute transverse myelitis can occur as a subset of several primary demyelinating disorders of the CNS (i.e., multiple sclerosis, NMOSD, myelin oligodendrocyte glycoprotein antibody disease, and acute disseminated encephalomyelitis) and various systemic autoimmune connective tissue disorders (i.e., systemic lupus erythematosus, mixed connective tissue disease, Sjögren syndrome, inflammatory bowel disease, and neurosarcoidosis).<sup18</sup Acute transverse myelitis (short or long segment) is an infrequent extra-articular complication of AS.<sup18</sup It has been reported to evolve either as a distinct neurological complication of AS, or it may develop secondary to TNF-alpha-inhibitor therapy for the treatment of AS.<sup18,19</sup AS is a heritable inflammatory spondyloarthropathy that primarily affects the axial skeleton, which is mediated by T-cells; B-cells only play a minor role.<sup5</sup On the contrary, the key for the pathogenesis of NMOSD is the production of autoantibodies against AQP-4 channels expressed on astrocytes, leading to complement-mediated damage, with ensuing demyelination. Myelitis usually shows high signal intensity on the tbl2-weighted image and contrast enhancement in the spinal cord.<sup1-4</sup Despite the difference in molecular mechanisms, the diagnosis of these diseases in the same individual may not be coincidental. Recent evidence has shown T-cell-mediated inflammatory responses in cases of NMOSD.<sup20</sup In particular, Th17 and Th2-related cytokines are elevated in the CSF of NMO patients.<sup20</sup Environmental factors such as <iEscherichia coli</i have also been proven to aggravate autoimmunity in AS and NMOSD (however, body fluid cultures for <iEscherichia coli</i, performed in our patient, showed similar association, and they were found negative two times).<sup21,22</sup Although large-scale epidemiological studies investigating the underlying pathogenesis related to these diseases are lacking, studies have demonstrated an increased incidence of optic neuritis among patients with AS.<sup23</sup Systemic sclerosis and mixed and undifferentiated connective tissue diseases were excluded after expert opinions (from two board-certified rheumatologists and two dermatologists) because of the lack of suggestive clinical findings (e.g., absence of skin thickening, salt-and-pepper appearance, nail changes, Mauskopf facies, sclerodactyly, calcinosis cutis, Raynaud's phenomenon, other cutaneous manifestations, pulmonary arterial hypertension/interstitial lung disease, dysphagia, muscular pain/weakness renal impairments, absence of ANA, anti-centromere antibodies, anti-Scl-70, PM-Scl antibodies, anti-ds DNA, PCNA, CENP-B, anti-nucleosomes, anti-Smith, anti-U1-RNP, anti-Jo1, anti-Mi2, anti-Ro52, anti-La antibodies, and normal C3 and C4 complement levels) (The European League Against Rheumatism and the American College of Rheumatology classification criteria 2019).<sup24</sup Finally, our patient was treated with intravenous steroids followed by rituximab infusions, a monoclonal anti-CD20 antibody directed against B-cells. In particular, this patient clinically and radiologically responded to immunomodulatory drugs, which might support a possible common pathogenic basis of the two processes. TNF-alpha inhibitors are commonly used as novel therapeutics in AS; however, they can potentially result in serious complications, that is, secondary demyelinating disorders.<sup25</sup However, such inhibitors in this patient were not used. When used in cases of AS, they show satisfactory results.<sup25,26</sup Therefore, it was decided to treat him with rituximab only without adding any second immunomodulatory. Other possible therapeutic options include cyclophosphamide and mycophenolate mofetil, but they were not used because of their low efficacy-safety balance. Moreover, plasmapheresis was not available in our specific setting, despite solid evidence that early treatment with therapeutic strategy (5-7 courses) provides good long-term outcomes in patients with NMOSD.<sup27</sup Therefore, when dealing with a case of acute non-compressive myelopathy, history and clinical examination are important to determine the potential underlying etiology and identify an undermined systemic disorder with apparently unrelated non-specific features. Connective tissue disorders should always be considered as a differential diagnosis and be ruled out in all cases of either seropositive or seronegative NMOSD. A diagnosis of AS should be considered in relevant circumstances when dealing with a case of isolated seronegative LETM. Moreover, early diagnosis and treatment of AS are quintessential to prevent lifelong distressing disabilities. However, whether patients with AS have any extra predilection to develop NMOSD throughout their life requires further studies.

[Contribution of the maximal exercise test to diagnosis the vascular origin of leg pain in athletes].

Leg pain is a common debilitating symptom in athletes. Vascular disease is not often proposed as a possible cause. Maximal exercise with measure of the ankle-brachial index after exercise can be an interesting diagnostic test. We report an illustrative case where an athlete presented leg pain revealing arterial disease disclosed by exercise. Interestingly, sub-maximal exercise did not cause pain, causing a delay in diagnosis. The vascular origin of leg pain can be detected with a maximal exercise test that induces the symptomatic pain or at least clinical discomfort.

The correlation between symptoms and non-invasive test results in patients referred for peripheral arterial disease testing.

The WHO/Rose questionnaire has served as the epidemiologic and clinical standard in the assessment of leg pain in patients with peripheral arterial disease (PAD) for over three decades. However, the structure of this questionnaire does not allow assessment of leg-specific (i.e. right versus left) symptoms. We studied 508 patients aged 39-95 years (mean 68 years), initially referred for PAD non-invasive testing. A revised questionnaire, the San Diego Claudication Questionnaire, was administered which allowed determination of leg-specific symptoms and evaluated thigh and buttock as well as calf pain. Leg-specific symptoms were categorized into no pain, pain at rest, non-calf claudication, non-Rose calf claudication, and Rose claudication. At the same visit, the ankle brachial index, the toe brachial index, and peak posterior tibial flow velocity were measured by Doppler ultrasound and five categories of non-invasive results by type and severity of PAD were defined. Legs with previous intervention (Rx), surgery or angioplasty, were evaluated separately. Claudication was reported in 42% of no Rx legs and 50% of Rx legs; 40% of claudication was atypical (not Rose); 64% of no Rx and 81% of Rx legs had PAD by non-invasive testing, and 27% of affected legs had severe PAD. The correlation between the severity of symptoms and the severity of ipsilateral PAD in no Rx legs was r = -0.40, p < 0.001. In Rx legs, this correlation was somewhat less (r = 0.27, p < 0.001) due to more symptomatology at lesser degrees of PAD, suggesting reporting bias and/or more residual disease than evident from non-invasive testing. To our knowledge, these results provide the first comparison between a standardized assessment of leg pain and the severity of ipsilateral PAD by non-invasive testing.

Does the clinical examination predict lower extremity peripheral arterial disease?

Lower extremity peripheral arterial disease (PAD) is common and associated with significant increases in morbidity and mortality. Physicians typically depend on the clinical examination to identify patients who need further diagnostic testing. To systematically review the accuracy and precision of the clinical examination for PAD. MEDLINE (January 1966 to March 2005) and Cochrane databases were searched for articles on the diagnosis of PAD based on physical examination published in the English language. Included studies compared an element of the history or physical examination with a reference standard of ankle-brachial index, duplex sonography, or angiogram. Seventeen of the 51 potential articles identified met inclusion criteria. Two of the authors independently extracted data, performed quality review, and used consensus to resolve any discrepancies. For asymptomatic patients, the most useful clinical findings to diagnose PAD are the presence of claudication (likelihood ratio [LR], 3.30; 95% confidence interval [CI], 2.30-4.80), femoral bruit (LR, 4.80; 95% CI, 2.40-9.50), or any pulse abnormality (LR, 3.10; 95% CI, 1.40-6.60). While none of the clinical examination features help to lower the likelihood of any degree of PAD, the absence of claudication or the presence of normal pulses decreases the likelihood of moderate to severe disease. When considering patients who are symptomatic with leg complaints, the most useful clinical findings are the presence of cool skin (LR, 5.90; 95% CI, 4.10-8.60), the presence of at least 1 bruit (LR, 5.60; 95% CI, 4.70-6.70), or any palpable pulse abnormality (LR, 4.70; 95% CI, 2.20-9.90). The absence of any bruits (iliac, femoral, or popliteal) (LR, 0.39; 95% CI, 0.34-0.45) or pulse abnormality (LR, 0.38; 95% CI, 0.23-0.64) reduces the likelihood of PAD. Combinations of physical examination findings do not increase the likelihood of PAD beyond that of individual clinical findings. However, when combinations of clinical findings are all normal, the likelihood of disease is lower than when individual symptoms or signs are normal. A PAD scoring system, which includes auscultation of arterial components by handheld Doppler, provides greater diagnostic accuracy. Clinical examination findings must be used in the context of the pretest probability because they are not independently sufficient to include or exclude a diagnosis of PAD with certainty. The PAD screening score using the hand-held Doppler has the greatest diagnostic accuracy.

Successful management of critical limb ischemia with intravenous sodium thiosulfate in a chronic hemodialysis patient.

Vascular calcification is common among hemodialysis (HD) patients and contributes to the development of peripheral arterial disease. A 57-year-old Japanese man who had been on HD for 30 years was referred to us for severe pain with multiple ulcers on his toes and fingers. He was an ex-smoker and had no diabetes mellitus. On admission, he had ulcers on his big toes bilaterally and right 2nd - 4th fingers. Peripheral pulses were strong and his ankle-brachial pressure index was above 1.3. Laboratory data were as follows: calcium 9.9 mg/dl, albumin 3.3 g/dl, phosphate 3.0 mg/dl, Ca x P product 30, and parathyroid hormone 98 pg/ml. He had a parathyroidectomy in 1998 and 1999. X-rays of his hands and legs showed diffuse subcutaneous arteriolar calcification. Angiography revealed no local stenotic lesions. Despite intensive therapies including hyperbaric oxygen therapy, painful gangrene developed on his right big toe and the pain was so intense that he could not go to sleep in a supine position. We infused intravenous sodium thiosulfate (20 g) 3 times weekly, based on previous reports. Within 4 - 5 days, he experienced rapid and dramatic symptom relief. The score of the visual analogue pain scale improved from 10/10 - 2/10. The signs of ischemia, measured by transcutaneous partial oxygen pressure and thermography, improved significantly. During the infusion of sodium thiosulfate, the patient complained of nausea, vomiting and hyperosmia. These adverse symptoms were resolved after discontinuation of the infusion. Pain relief was sustained and he could walk after 2 weeks of infusion. Our case supports the use of sodium thiosulfate as a novel therapeutic choice for critical limb ischemia with severe vascular calcification in chronic HD patients.

Exercise testing criteria to diagnose lower extremity peripheral artery disease assessed by computed-tomography angiography.

The sensitivity and specificity of exercise testing have never been studied simultaneously against an objective quantification of arterial stenosis. Aims were to define the sensitivity and specificity of several exercise tests to detect peripheral artery disease (PAD), and to assess whether or not defined criteria defined in patients suspected of having a PAD show a difference dependent on the resting ABI. In this prospective study, consecutive patients with exertional limb pain referred to our vascular center were included. All patients had an ABI, a treadmill exercise-oximetry test, a second treadmill test (both 10% slope; 3.2km/h speed) with post-exercise pressures, and a computed-tomography-angiography (CTA). The receiver-operating-characteristic curve was used to define a cut-off point corresponding to the best area under the curve (AUC; [CI95%]) to detect arterial stenosis ≥50% as determined by the CTA. Sixty-three patients (61+/-11 years-old) were included. Similar AUCs from 0.72[0.63-0.79] to 0.83[0.75-0.89] were found for the different tests in the overall population. To detect arterial stenosis ≥50%, cut-off values of ABI, post-exercise ABI, post-exercise ABI decrease, post-exercise ankle pressure decrease, and distal delta from rest oxygen pressure (DROP) index were ≤0.91, ≤0.52, ≥43%, ≥20mmHg and ≤-15mmHg, respectively (p<0.01). In the subset of patients with an ABI >0.91, cut-off values of post-exercise ABI decrease (AUC = 0.67[0.53-0.78]), and DROP (AUC = 0.67[0.53-0.78]) were ≥18.5%, and ≤-15mmHg respectively (p<0.05). Resting ABI is as accurate as exercise testing in patients with exertional limb pain. Specific exercise testing cut-off values should be used in patients with normal ABI to diagnose PAD.

Complex Regional Pain Syndrome Caused by Lumbar Herniated Intervertebral Disc Disease.

Most cases of complex regional pain syndrome (CRPS) occur after some inciting injury. There are a few cases of CRPS after an operation for disc disease. CRPS from a mild herniated intervertebral disc (HIVD) without surgical intervention is even rarer than CRPS after an operation for disc disease.A 22-year-old man was transferred to a pain clinic. He had continuously complained about back and right leg pain. He presented with a skin color change in the right lower leg, intermittent resting tremor, stiffness, and swelling in the right leg. He complained of a pulling sensation and numbness in his right buttock, posterior thigh, lateral calf, and ankle. This symptom was in accordance with L4/5 radiculopathy. Magnetic resonance imaging (MRI) also showed L4/5 HIVD that was central to the bilateral subarticular protrusion.He was diagnosed as having CRPS, which fits the revised International Association for the Study of Pain (IASP) criteria. He fulfilled 4 symptom categories (allodynia, temperature asymmetry and skin color change, sweating changes, decreased range of motion and motor dysfunction) and 3 of 4 sign categories (allodynia, temperature asymmetry and skin color changes, decreased range of motion and motor dysfunction). The bone scan and thermography also revealed CRPS.For the past 2 months, we have performed intensive treatments. But, he never became pain-free and walking for 5 minutes led to persistent leg pain. We decided to perform percutaneous nucleoplasty, which can directly decompress a HIVD. On the next day, he achieved dramatic symptom relief. The visual analog scale (VAS) score improved to 3, compared to the VAS score of 9 at the first visit. The skin color change, allodynia, and tremor in the right leg disappeared, and the temperature asymmetry normalized. Motor weakness of the right leg also recovered.We report an unusual case of CRPS that was caused by L4/5 HIVD without a history of trauma or surgery. It has a clear causal relationship between HIVD and CRPS and definitively fits in the newly revised IASP criteria. In conclusion, mild HIVD can cause CRPS without any trauma. And percutaneous nucleoplasty can be considered as a treatment option.

[Early diagnosis of arteriopathy of the legs using measures adapted to general practitioners: the systolic index and pulse perception].

Atherosclerotic lower-limb arteriopathy is a serious disease. Its prevalence often underestimated when relying only on questioning the patient. A simple early detection method is the systolic index, i.e. the ratio of systolic ankle over brachial pressure. This ratio should normally be greater than 0.9. Lower values indicate detectable arterial obstruction as revealed by reduced peripheral perfusion. Under 0.75 patients are considered as suffering from peripheral vascular disease and require further investigations and specialist opinion. With an index between 0.75 and 0.90 patients are classified as stage I or II of the disease. In a survey by 180 General Practitioners, which were trained by angiologists to measure ankle pressure, more than 1,400 patients, between 40 and 75-years old, with at least one vascular risk factor (hypertension, diabetes, lipids, tobacco) were selected. Tobacco was the most prevalent vascular risk factor, then diabetes (particularly in men). 23.8% of patients recorded values between 0.90 and 0.75 and thus suffering from peripheral vascular disease (certainly age dependent). Clinically absent pulses at the posterior tibial and the dorsalis pedis artery were found is 19 and 27% of patients respectively. Most of the patients received a pharmacological substance allowing to check this index sensitivity. Thus systolic index is a simple low cost method for early detection and care and more general utilisation is proposed.

Diabetic foot infection

History and physical History should be taken for known recent foot trauma, and the lower extremities should be inspected for signs of recent trauma, including redness, induration, edema, visible ulceration with exudate or pus, or bony deformity. Ulcers do occur in the absence of pathological infection. Diagnosis of an infected wound is classically made with ≥2 signs of inflammation or purulence. Peripheral pulses should also be evaluated (posterior tibial and dorsal pedis), and if not palpable, should be further evaluated using ultrasound. In patients with non-palpable pulses, evaluation of PAD with an ankle-brachial index should also be performed. Ulceration or deeper wounds should be probed to identify the depth of penetration and determine involvement of bone, which would indicate osteomyelitis. Neurologic testing includes testing peripheral sensation to vibratory stimuli, temperature, pain, along with deep tendon reflexes.

Exercise hyperemia for the study of peripheral circulation.

Blood flow in the calf was measured during postexercise hyperemia in normal subjects and in PAD patients by means of a foot ergometer that gives direct reading of the work performed. In normals, first and peak flow increased with the rise of work load up to 100 KGM. The duration of hyperemia increased with a work load of 30 to 200 KGM. In PAD patients, first flow did not coincide with peak flow. Peak flow was lower and delayed, and the duration of hyperemia was more prolonged than in normal subjects. In patients with intermittent claudication, first flow, peak flow, and work load were higher than in patients with rest pain or impending gangrene. Exercise hyperemia appears as a useful test for screening normal limbs from those with arterial obstruction. Since in PAD patients exercise is interrupted when muscular pain appears, it is evident that the earlier the arrest of work and the appearance of pain, the greater is the involvement in the arterial tree of the leg. Therefore the exercise hyperemia test can be used also as a means of evaluating the different stages of PAD.

Imaging features of periostitis as a manifestation of IgA vasculitis: A case report.

Periostitis in systemic vasculitis is very rare with only a few previously reported cases. The reported cases were seen in polyarteritis nodosa or Takayasu arteritis. We report the first case of periostitis associated with IgA vasculitis with demonstration of computed tomography (CT), magnetic resonance imaging (MRI) features, and serial changes of radiographs. A 74-year-old man visited an orthopedic outpatient clinic for pain in both lower legs and left ankle pain. He underwent a total ankle arthroplasty of the left ankle 3 years ago. His medical history disclosed IgA vasculitis/nephropathy caused by cephalosporin antibiotic class 5 months earlier. Plain radiography, MRI of the right lower leg, CT scan of the left ankle showed single lamellar to spiculated periosteal reactions at both tibia, fibula and left calcaneus. There was no evidence of bone or soft tissue mass lesions or cortical destruction. We concluded that this was a case of periosteal reactions associated with IgA vasculitis for the following reasons: (1) periosteal biopsy was negative for tumor. (2) there was no pulmonary lesion on chest radiography and CT, no history of trauma, inflammatory arthritis, metabolic disease, or genetic disease that could cause periostitis at multiple bones, (3) the initial MRI showed predominant signal changes around the tibial and fibular shafts, possibly explaining subsequent periosteal reactions, and (4) symptoms subsided with conservative treatment and follow-up radiographs showed remodeling of periosteal reactions. The patient took conservative management. His calf pain improved, and a radiograph 7 months later showed remodeling to the solid or single lamellar periosteal reaction along the both tibia and left fibula. Awareness of this uncommon manifestation would help differential diagnosis of periostitis and could help decrease the patient's anxiety. It should also be noted that periosteal reactions by benign entities could cause aggressive-looking periosteal reactions in post-operative regions.

Usefulness of stress-loading test for ankle brachial index using an originally developed exercise device to detect peripheral arterial disease.

In the detection of peripheral arterial disease (PAD), the post-exercise ankle brachial index (ABI) using a treadmill is useful although time consuming. We developed a stress-loading device that induces fatigue in the calf muscles while remaining in the supine position, which is designed to reduce delay between completion of the exercise and measurement and to reduce the potential for cardiac and cerebrovascular accident in at-risk persons. Using our original device, we measured post-stress-loading ABI in 536 patients (1,072 legs) with lower extremity symptoms such as intermittent claudication. ABI was measured at rest and after stress loading by VaSera. The sensitivity and specificity of resting ABI using a cutoff of 0.9 was 74.8% and 99.4%, respectively, and of post-stress-loading ABI was 85.0% and 99.6%, respectively. The absolute and percent decrease of ABI after stress loading was 0.09+/-0.10 and 13.6+/-13.9%, respectively, in the PAD group and 0.04+/-0.05 and 3.2+/-4.0%, respectively, in the non-PAD group, a statistically significant difference (P<0.01). Our originally developed stress-loading device for measurement of ABI under load simplifies the test procedures and increases the testing precision to a level higher than that of resting ABI and to that comparable to the conventional treadmill method.

[Pulmonary thromboembolism. A clinical case with unusual presentation].

The authors describe a rare case of pulmonary thromboembolism with unusual clinical findings and emphasized the large difficulty encountered in formuling a correct diagnosis in a reasonable time. A man, 60 years old, was admitted to a Medical Division of our hospital for the appearance of chest pain and epigastric pain during effort in the last year. He smoked 20 cigarettes a day and drank wine (1 or 2 litres a day). He was affected by hypercholesterolemia and in the past reported relapsed thrombophlebitis in the left leg. Four years before admission to our hospital he underwent large and small left saphenectomy. He had no cardiac events in the past. After a non significant exercise stress test the patient was treated with nitrates and asa and was discharged from the hospital. At home the symptoms increased and after 8 months the patient was admitted again to the Cardiologic Division of the hospital. At admission he reported dyspnea and chest pain at rest, not only during effort and the ECG showed negative T waves in anterior and inferior leads. Intravenous heparine, nitrates and calcium antagonists stabilized the clinical picture. The following examinations revealed: reduction of the T wave negativity at the ECG registered during chest pain; mild enlargement of the heart at the chest roentgenogram; normal value of the left ventricle and apical and midseptal by ipokinesia at the transthoracic echocardiogram; normal coronary artery at the coronary arteriography. "Vasospastic angina" was diagnosed and the patient was discharged after 20 days, asymptomatic. After 15 days he returned to the hospital again for chest pain, dyspnea, hypotension and syncope despite therapy. At physical examination he showed a painful left tibio-tarsal tumefaction, an increased and splitting second heart sound in the pulmonary area and a systolic murmur in the third and fourth left interspace. The ECG showed a severe anterior ischemia, while a new transthoracic echocardiogram revealed a considerable dilatation of the right atrium, right ventricle and the main pulmonary artery with severe tricuspid regurgitation and pulmonary hypertension (mean PAP about 50 mmHg). The following pulmonary perfusion scintigraphy confirmed the diagnosis of pulmonary embolism and the selective right and left pulmonary arteriography exhibited multiple thrombi and large intravascular filling defects. The right heart catheterization confirmed a chronic precapillary pulmonary hypertension (mean PAP = 55 mmHg). About 24 hours after these examinations the patient died because of a cardiac arrest with electromechanical dissociation. Pulmonary thromboembolism is a potentially fatal disease characterized by a largely variable clinical presentation. Frequently pulmonary embolism diagnosis is difficult especially when clinical findings are unusual. In the case observed the "typical" chest and epigastric pains associated with the electrocardiographic findings directed diagnosis towards myocardial ischemia. Also after the coronary arteriography that showed normal coronary artery, the erroneous diagnosis persisted. Pulmonary embolism was correctly diagnosed too late to begin an effective therapy. These unusual clinical findings and diagnostic mistakes are stressed and critically reviewed in the article.

Assessment of noninvasive lower extremity arterial testing versus pulse exam.

Palpation of pedal pulses was compared to noninvasive testing in 100 patients referred to a vascular laboratory. Subjects were 65 +/- 13 (mean +/- s.d.) years old. The right dorsalis pedis (DP) artery served as the reference artery for comparison of Doppler studies with physical examination of the pulses. Absolute Doppler pressures in the right DP were 129 +/- 50 mm Hg. The right ankle:brachial index (ABI) was 0.86 +/- 0.32. There were significant differences in ABI in those with (0.68 +/- 0.28) vs without (0.95 +/- 0.31) claudication in either extremity (p < 0.001). Rest pain was also associated with lower ABI (P < 0.04). Diabetics, hypertensives, claudicants and those with ischemic rest pain were less likely to have palpable pulses (P < 0.035). With right DP pressure >/= 118 mm Hg, 63 per cent of subjects had a palpable DP pulse, whereas 68 per cent with ABI > 0.82 had a palpable right DP. Of those (n = 35) with a right DP pressure < 118 mm Hg, only 6 per cent (n = 2) had a palpable pulse, whereas 5 per cent (2/40) with ABI </= 0.82 had palpable DP pulses. Based on these findings, it was predicted that a pulse would likely not be palpable in the left DP with a pressure < 118 mm Hg or with AB </= 0.82. The prediction based on systolic ankle pressures was correct, with 66 per cent sensitivity and 91 per cent specificity. The predication that a left DP pulse would not be palpable with AB </= 0.82 was more accurate (78% sensitive, 97% specific). Presence of a palpable DP pulse suggests the presence of a Doppler pressure >/= 188 mm Hg and ABI > 0.82. The range of ankle pressures with palpable right DP pulses was 64-220 mm Hg, whereas the range with nonpalpable DP was 42-300 mm Hg. Given the frequent disparity of pulse exam and ankle pressures, noninvasive Doppler testing may be necessary for many patients to accurately assess the vascular status of the leg.

Peripheral artery disease

In individuals with suspected PAD with normal ABIs can undergo exercise testing of ABI. A baseline ABI is obtained prior to exercise. The patient is then asked to exercise (usually patients are made to walk on a treadmill at a constant speed) until claudication pain occurs (for a maximum of 5 minutes), after which the ankle pressure is again measured. A decrease in ABI of 15%–20% would be diagnostic of PAD.

Exercise transcutaneous oximetry significantly modifies the diagnostic hypotheses and impacts scheduled investigations or treatments of patients with exertional limb pain.

In lower extremity peripheral artery disease (PAD), transcutaneous oximetry at exercise (Ex-TcpO2) has been largely validated in research practice, but evidence of routine practice in various vascular laboratories is missing. We hypothesized that Ex-TcPO2 would change the diagnosis hypotheses, investigations and treatments for patients referred for exertional limb pain. A multicenter prospective trial was conducted in nine different referral centers. Investigators performed Ex-TcpO2 and recorded investigations and treatments already scheduled for the patient. We encoded referral physician's diagnostic hypothesis. Before Ex-TcpO2, vascular physicians were asked to give their diagnosis hypotheses. A minimal decrease from rest of oxygen pressure (DROP)<minus 15mmHg defined the presence of exercise-induced ischemia on the area of interest. After Ex-TcPO2, we recorded post-test diagnostic hypothesis and investigations and treatments to be cancelled or performed. We compared the diagnosis hypotheses, scheduled investigations and treatments, before and after the Ex-TcpO2. We included 603 patients (485 males: 80.4%), aged 64.7±9.8years. The post-test diagnosis hypothesis differed in 266 patients (44.1%; p<0.0001) and in 96 patients (15.9%) from the pre-test hypothesis of referring and vascular physician, respectively. This led to the recommendation to cancel 27 scheduled investigations or treatments of a total cost of ~130,000 euros. Ex-TcPO2 in patients with exertional limb pain is applicable in various vascular institutions, and significantly modifies the diagnostic hypotheses and impacts scheduled investigations or treatments of patients with exertional limb pain.

Surgery_Schwartz

arteries (Fig. 23-1). Normal is more than 1. Patients with clau-dication typically have an ABI in the 0.5 to 0.7 range, and those with rest pain are in the 0.3 to 0.5 range. Those with gangrene have an ABI of less than 0.3. These ranges can vary depending on the degree of compressibility of the vessel. The test is less reliable in patients with heavily calcified vessels. Due to non-compressibility, some patients, such as diabetics and those with end-stage renal disease, may have ABI ≥1.40 and require addi-tional noninvasive diagnostic testing to evaluate for peripheral artery disease. Alternative tests include toe-brachial pressures, pulse volume recordings, transcutaneous oxygen measurements, or vascular imaging (duplex ultrasound).Segmental Limb Pressures. By placing serial blood pressure cuffs down the lower extremity and then measuring the pressure with a Doppler probe as flow returns to the artery below the cuff, it is possible to determine segmental pressures down the leg. This

Pulmonary artery ablation to treat pulmonary arterial hypertension: a case report.

Idiopathic pulmonary arterial hypertension is defined as a group of diseases characterized by a progressive increase in pulmonary vascular resistance that results in right heart failure and premature death. Although therapies exist to improve hemodynamic instability and symptoms, there is no cure for pulmonary arterial hypertension and it remains a life-threatening condition. A recent study performed in China reported, for the first time, the effect of pulmonary arterial denervation on functional capacity and hemodynamics in patients with refractory idiopathic pulmonary arterial hypertension. We report a case of a 60-year-old white Brazilian man, with controlled hypertension and stage 2 obesity who complained of progressive fatigue with moderate to light exertion of approximately 1 year's duration. During this period, he underwent myocardial perfusion scintigraphy without evidence of obstructive ischemic disease. He had no clinical evidence of systolic heart failure. He had undergone biological mitral valve replacement 3 years previously for mitral valve stenosis and ablation of atrioventricular nodal reentry tachycardia 18 months previously. At the time of valve replacement, he had no reported evidence of pulmonary arterial hypertension. His echocardiogram showed normal function of a mitral prosthesis, normal global left ventricular systolic function (left ventricular ejection fraction 62 % measured using the Teichholz method), stage I diastolic dysfunction, and a mean systolic pulmonary arterial blood pressure of 50 mmHg. In the 6-minute walk test, the patient walked 104 meters. Catheterization of his right heart chambers and pulmonary arteries confirmed the diagnosis of pulmonary hypertension. Electroanatomic reconstruction of the right ventricular outflow tract and pulmonary artery was performed under direct fluoroscopic visualization, and a merger was made with a formatted image of cardiac computed tomography angiography. Then we performed irrigated cardiac catheter ablation of the pulmonary trunk. At the patient's 3-month follow-up, he showed improvement in functional class for fatigue on major exertion, increased distance walked in the 6-minute walk test, and reductions in pressure of both the right cavities and the pulmonary artery. Currently, with 6 months of clinical follow-up, the patient has maintained his functional classification and is pedaling his bicycle.

[Intermittent arterial claudication. Clinical aspects--hemodynamic correlation].

Intermittent arterial claudication is the master symptom in lower limbs arteriopathy. It is used as a reference in invasive or non-invasive arterial explorations. It allows to assess the evolutive degree of the disease and above all helps the therapeutic decision. The seat of claudication is traditionally acknowledged as being located immediately downstream of the significant arterial lesion. The purpose of our work was to verify such a notion and ascertain the relation between spontaneous and tread-mill induced claudication. 60 stage-2 arteritic patients were included in a prospective mode study. They were all taken in hospital for arteriographic check-up and vascular functional explorations: Doppler velocimetry, segment pressures, treadmill tests and pressure measurements after stress. The patients were classified according to age (table I), time of claudication record (table II) and lesions location (table III). All clinical and paraclinical data were recorded with a view to correlations interpretation. The relation between spontaneous claudication and tread-mill induced claudication was studied with respect to lesion sites (table IV). Among 37 patients 46% of the claudications observed appeared at iliac stage in both cases (fig. 1). In 35 cases, femoral claudication was observed in 48% (fig. 2). As for distal claudication among 6 patients, 3 showed calf claudication in both cases, i had foot claudication and 2 showed discrepant claudication types. The relation between claudication and lesion site was not confirmed in all cases. Buttock claudication always appeared in relation with a lesion upstream or an internal iliac lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Peripheral Arterial Disease -- Evaluation

Making the diagnosis of PAD should factor in the patient’s history, physical exam, and objective test results. Key points in the history include an accurate assessment of the patient’s walking ability. Many patients do not explicitly report the classic claudication symptoms of cramping calf pain after walking 2 blocks that resolves with rest. Some patients may walk very little because of other physical ailments or limitations. Other patients may not volunteer any information about walking unless specifically asked. Important aspects of the history also include cardiovascular risk factors such as smoking, hypertension, high cholesterol, and diabetes. [7] [8] [9]

[A 22-year-old man with long-standing weakness and atrophy predominantly in the lower extremities].

We report a right-handed 22-year-old man with muscle atrophy. His prenatal course and the delivery were uneventful, but he walked unsupported at 15 months of the age for the first time. He was apparently well but he was in the slowest group in running in schools. He noted a difficulty in climbing up stairs at 19 years of the age, and he was admitted to our hospital for the work up when he was 22-year-old. His family history and past medical history were unremarkable. On admission, he was a slender and tall guy in no acute distress. General physical examination was unremarkable, but he had high-arched palate and high-arched feet. On neurologic examination, mental status and higher cerebral functions were normal. Cranial nerves appeared intact, however, he had a thin and long face without weakness. The sternocleidomastoid muscles appeared somewhat atrophic and were moderately weak. He was able to walk normally, however, he needed a handrail when he went up stairs. Thigh muscles and triceps surae muscles were atrophic and slightly weak (4/ 5). Muscle tone was hypotonic and no deep tendon reflexes were elicited except for jaw jerk. No ataxia or involuntary movements were seen; sensation was intact. Laboratory examination was unremarkable except for slight increase in serum CK to 145 IU/L. An ischemic forearm exercise test revealed slight elevation of lactate and pyruvate in that base line levels were 5.4 mg/dl and 0.52 mg/dl, respectively, which rose to 11.4 mg/dl and 0.85 mg/dl, respectively, 20 minutes after the initiation of the ischemic exercise. The base line serum ammonia was 102.5 micrograms/dl which decreased to 64.8 micrograms/dl at 20 minutes. A diagnostic biopsy was performed from the left quadriceps femoris muscle. The patient was discussed in a neurologic CPC, and the chief discussant arrived at the conclusion that the patient had nemaline myopathy. Opinions were divided between nemaline myopathy and debranching enzyme deficiency. The results of the ischemic exercise was not typical of glycogen storage disease, but elevations of lactate and pyruvate did not appear to be sufficient to be interpreted as normal. Histologic examination of the biopsied specimen revealed marked type I fiber predominance and abundant nemaline rods. Cytoplasmic bodies were also seen. Histologic characteristics were consistent with the diagnosis of nemaline myopathy. The possibility of concomitant presence of AMP deaminase deficiency was discussed, because serum ammonia did not elevate in the ischemic exercise test.

[Practical diagnosis of arterial occlusive diseases of the lower extremities (author's transl)].

A peripheral arterial occlusive disease can be diagnosed by a clinical examination. Technical methods serve for documentation, definition of localization and differentiation of doubtful findings and special therapeutic measures. A definite sequence of diagnostic procedures simplifies diagnosis. History (risk factors), individual clinical or biochemical findings, intermittent claudication, pain at rest are important for the assessment of peripheral arterial circulation when considered with the appropriate differential diagnosis of various internal disease conditions, inspection, palpation and especially auscultation at rest and on effort. Ratschow's circulation test provides information on the degree of compensation. Mechanical or electronic oscillography and Doppler echography have proved valuable as technical methods for measuring post-stenotic pressure.

Medial malleolar stress fracture resulting from repetitive stress caused by lateral ankle instability: A case report.

Stress fractures are most commonly sustained in the lower extremities owing to the repetitive weight-bearing forces. They are overuse injuries that are seen often in athletes, but rare in the general population, so early diagnosis and proper treatment are important to enable athletes to return to activity. This is a rare example of successful treatment of a medial malleolar stress fracture with lateral ankle instability. A 16-year-old athlete presented with acute-onset left ankle pain. He was a baseball pitcher who had previously sprained left ankle while training. Subsequently, the ankle pain worsened, and he had tenderness on the medial aspect of his left ankle. The symptoms were mild at rest, but increased upon walking and training. Three years previously, he had sprained his ankle several times during baseball training. Plain standing radiographs of the left ankle showed a vertical fracture line in the medial malleolus. Computed tomography also showed the vertical fracture in the medial malleolus. Magnetic resonance imaging revealed mild bone marrow edema in the medial malleolar area and total rupture of the anterior talofibular ligament. Surgery was performed under general anesthesia, and we checked the instability of his ankle using a C-arm image intensifier, and the varus talar tilt angle was increased (10.3°). The medial malleolus stress fracture was fixed using tension bend wiring, and an arthroscopic modified Broström procedure was done. Two months postoperatively, the patient started walking, and raised-heel squatting. The medial malleolar fracture was completely united at the 3 months postoperatively on plain radiography, and return to full activity was achieved by 3 months postoperatively. Then the hardware was removed 1-year after operation and both the anterior drawer and external rotation stress tests were negative. Medial malleolar stress fractures are rare, so they can be overlooked. With these fractures, plain radiographs are frequently normal initially because the medial malleolus consists mainly of cancellous bone. Physicians require a high level of suspicion when taking the patient's history and doing the physical examination. Because most occur in athletes, early diagnosis and proper treatment are important for patients' subsequent athletic performance.