Neoplasms and related disorders.

Spinal neoplasms may be primary or metastatic, benign or malignant. In adults, metastatic involvement of the spine will represent the most important neoplastic disease of this region. However, hemangiomas are the most common spinal neoplasms. The vast majority of intradural, extramedullary neoplasms that will be identified in the spine are meningiomas and neurofibromas. Both lesions may be sporadic or associated with phakomatoses. Intramedullary spinal cord and filum terminale tumors are relatively rare and are far less common than intramedullary brain tumors. As is the case in the brain, these are overwhelmingly glial neoplasms, with ependymomas and low-grade astrocytomas representing the majority of the lesions. Hemangioblastoma deserves mention because of its often characteristic imaging findings and its association with von Hippel-Lindau disease.     

Cocaine and opiate related disorders

Cocaine and the opiates are--together with alcohol--involved in the genesis of the "three great addictions". The medical conceptualisation of the dependence phenomenon has evolved on the grounds of a critical observation of different ways of use and treatment endeavours, mainly during the last three centuries. Whereas the prohibitionists favoured (and still favour) total abstinence from all psychotropic drugs, defect theoreticians emphasise analytically-oriented psychotherapeutic procedures aiming at healing a supposed underlying self-defect. Contrarily, proponents of the "Metabolic Theory" of the addictions suggest a mainly medication-based approach in order to normalise a--in their eyes causal--still in some aspects hypothetical metabolic defect. Based on a short survey of recently emerging trends of consumption patterns (speed-balling, chasing, multi-substance-use) in Europe, the most important newer treatment types and forms are presented. Finally, a multimodal, integrated, and individualized approach is recommended.     

Pyoderma gangrenosum and related disorders

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition with distinctive clinical characteristics and a frequent association with systemic diseases. Most frequently among the associated disease list are: inflammatory bowel disease, arthritis, and a variety of hematologic disorders. There is no specific treatment of PG; however, the effective treatment of an accompanying systemic disease, local therapy, and/or the use of topical or systemic agents known to be useful in neutrophil disorders, have been beneficial in most patients.     

Alzheimer's disease and related disorders

The cholinesterase inhibitors provide the first clearly effective treatments for the cognitive deficits of AD and appear to have a beneficial effect on activities of daily living function and noncognitive behavior. There is increasing support for starting donepezil, rivastigmine, or galantamine early in the disease course and maintaining treatment at least during the early and middle stages of AD. Depressive signs and symptoms complicating AD are treated best with SSRIs. Placebo-controlled trials support the use of citalopram and sertraline in AD complicated by depression. The atypical antipsychotics are the first choice for managing psychosis and disruptive agitation in AD and particularly in the Lewy body variant of AD. Studies suggest that low-dose treatment with risperidone, 1 mg/d, or olanzapine, 5 mg/d, offers the optimal ratio of therapeutic to adverse effects.     

Sleep apnea and related disorders

This article has ranged far and wide, from the pickwickian patient weighing 500 pounds to the tiny 4-lb premature baby, but the common thread is cessation or diminution of breathing during sleep and consequent oxygen desaturation. Associations have been made between abnormalities of sleep and breathing and multiple diseases--some causative and some fortuitous. Despite relating this information, we are only beginning to understand sleep and breathing. Although we spend a third of our lives asleep, very little attention was paid to the study of sleep until the mid 1970s. Even to this day, there is little interest in snoring and its adverse effects. The situation may be a sexist one in which men have done the research and the snoring, but the snoring has bothered only women. I predict that the next 15 years will see a vast increase both in studies of sleep and in our understanding of the importance of breathing during sleep--to the well-being of all of us.     

Heat stroke and related heat stress disorders

Medical disorders related to environmental heat exposure are exceptionally common in persons who perform hard work in hot climates. They are also common in competitive athletes as well as in persons who participate in casual exercise to maintain health. The important issue of salt and water disturbances consequent to heavy sweating in hot climates is discussed in detail as are mechanisms of potassium deficiency and its implications. The major forms of environmental heat illness including heat syncope, heat cramp, heat exhaustion, and heat stroke are presented in detail with relevant clinical examples. A discussion of the differential diagnosis of hyperthermia and rhabdomyolysis follows. Because of the difference in treatment and complications, heat stroke is subdivided into the classic variety that affects the elderly and very young and that form that follows heavy physical work and is always associated with rhabdomyolysis. Because severe heat exhaustion and heat stroke are life-threatening disorders, the chapter includes a detailed discussion of complications and plans for treatment.     

Androgen resistance related to receptor disorders]

It is the review of literature data on various phenotypic anomalies consequent to the absent or reduced ability of the target tissues to response to androgens (A) due to defects in receptor A structure and function. Clinical forms of resistance to A, quantitative and qualitative abnormalities in hormone-receptor interaction detectable in relevant patients are described as well as markers of target tissue sensitivity to A action suggested for use in prognosticating the response to A treatment.     

Environmentally related disorders of the cardiovascular system

The patient's level of activity is typically the only work-related concern considered in clinical medicine. This article discusses why it is important for clinicians to expand their concern to include the possibility of exposure to various gases, metals, and chemicals when evaluating a patient with cardiovascular disease. Both at the time of diagnosis and when deciding whether a patient can return to work, the workplace exposures need to be reviewed for their potential to exacerbate or cause cardiac symptoms or even death. An appropriate exposure history with confirmatory laboratory tests will allow the clinician to diagnose and manage environmentally related cardiovascular disease.     

Environmentally related disorders of the nervous system

Specific physical and chemical agents found in the workplace and in the general environment are responsible for characteristic pathologic processes within the nervous system. It has been shown that many neurotoxic agents produce a dose-related spectrum of impairment ranging from mild slowing of nerve conducting velocity or prolongation in reaction time to neuropathy and frank encephalopathy. Clinical manifestations are determined by the agent involved, by the dose of exposure, the vulnerability of the cellular target, the ability of the organism to metabolize and excrete the agent, and the ability to repair damage. An occupational history, including evaluation of evidence of specific agents and job history, is a critical component in the clinical management of individuals with suspect neurotoxic disease. Environmentally-induced disorders can be prevented by appropriate environmental controls. Prevention of neurotoxic disease is a complex process requiring continuous involvement of public health agencies and strong scientific research.     

Paraproteins in rheumatoid arthritis and related disorders

Summary There is evidence that the prevalence of monoclonal parparoteinaemia is slightly increased in patients with rheumatoid arthritis. The possibility that this may be a marker of the development of later malignancy in such patients is explored. Mortality rates in rheumatoid arthritis are increased although the development of lymphoreticular malignancy contributes only a small percentage of this increase. However, it does seem likely that patients with longstanding severe rheumatoid arthritis are more at risk of developing myeloma or lymphoma if they have a monoclonal paraprotein band in their serum. IgA paraprotein seems to carry a higher risk than IgG whilst other factors such as urinary free light chains and the presence of secondary Sj?gren's syndrome are of less prognostic significance. Similarly a monoclonal para-protein may identify patients with primary Sj?gren's syndrome who have a particular risk of later lymphoma whilst this risk does not appear to extend to patients with systemic lupus erythematosus. Patients with an overlap syndrome do not appear to be at greater risk than those with “pure disease”. The association of other rheumatological disease and paraproteinaemia is briefly discussed.     

Paraproteins in rheumatoid arthritis and related disorders.

There is evidence that the prevalence of monoclonal parparoteinaemia is slightly increased in patients with rheumatoid arthritis. The possibility that this may be a marker of the development of later malignancy in such patients is explored. Mortality rates in rheumatoid arthritis are increased although the development of lymphoreticular malignancy contributes only a small percentage of this increase. However, it does seem likely that patients with longstanding severe rheumatoid arthritis are more at risk of developing myeloma or lymphoma if they have a monoclonal paraprotein band in their serum. IgA paraprotein seems to carry a higher risk than IgG whilst other factors such as urinary free light chains and the presence of secondary Sj?gren's syndrome are of less prognostic significance. Similarly a monoclonal paraprotein may identify patients with primary Sj?gren's syndrome who have a particular risk of later lymphoma whilst this risk does not appear to extend to patients with systemic lupus erythematosus. Patients with an overlap syndrome do not appear to be at greater risk than those with "pure disease". The association of other rheumatological disease and paraproteinaemia is briefly discussed.     

Clinical and diagnostic aspects of gluten related disorders

Gluten is one of the most abundant and widely distributed components of food in many areas. It can be included in wheat, barley, rye, and grains such as oats, barley, spelt, kamut, and triticale. Gluten-containinggrains are widely consumed; in particular, wheat is one of the world's primary sources of food, providing up to 50% of the caloric intake in both industrialized and developing countries. Until two decades ago, celiac disease(CD) and other gluten-related disorders were believed to be exceedingly rare outside of Europe and were relatively ignored by health professionals and the global media. In recent years, however, the discovery of important diagnostic and pathogenic milestones led CD from obscurity to global prominence. In addition, interestingly, people feeding themselves with glutenfree products greatly outnumber patients affected by CD, fuelling a global consumption of gluten-free foods with approximately $2.5 billion in United States sales each year. The acknowledgment of other medical conditions related to gluten that has arisen as health problems, providing a wide spectrum of gluten-related disorders. In February 2011, a new nomenclature for gluten-related disorders was created at a consensus conference in London. In this review, we analyse innovations in the field of research that emerged after the creation of the new classification, with particular attention to the new European Society for Paediatric Gastroenterology, Hepatology and Nutrition guidelines for CD and the most recent research about non-celiac gluten sensitivity.     

Influenza encephalopathy, related disorders and differential diagnosis

Influenza encephalopathy and related disorders such as Reye's syndrome, acute necrotizing encephalopathy and acute hemorrhagic encephalopathy are still undefined clinical entities because the pathophysiology is not clearly established. Even Reye's syndrome which has been extensively studied during the last two decades, its real picture has not been well established. Moreover, Reye's syndrome is going to disappear just like Ekiri syndrome. Acute necrotizing encephalopathy and acute hemorrhagic encephalopathy need further investigation to be recognized as single clinico-pathological entity because decreased cerebral blood flow is closely linked with brain edema including basal ganglia lesion and hepato-renal dysfunction.     

Future direction of therapies for sleep related breathing disorders

There are several mechanisms by which pharmacologic agents might improve sleep related breathing disorders. Upper airway muscle atony during sleep and fat deposition on the upper airway walls are critical in the pathogenesis of upper airway obstruction. As central neuromediators that regulate upper airway muscle activity, serotonin, gamma amino-butyric acid and thyrotropin releasing hormone are reviewed. Although agonists or antagonists of these mediators changed the upper airway muscle activity, no agents have successfully improved sleep related breathing disorders. Leptin is a protein produced by adipose tissue that interacts with receptors in the hypothalamus to inhibit eating. Leptin might have therapeutic potential for obesity-related breathing disorders related to a relative deficiency in leptin, or a leptin resistance.