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How people construct families varies greatly from one society to arteria lacrimalis purchase zestoretic 17.5mg amex another blood pressure medication that doesn't cause cough purchase zestoretic 17.5 mg overnight delivery, but there are patterns across cultures that are linked to blood pressure chart generator buy zestoretic 17.5 mg on line economics, religion, and other cultural and environmental factors. The study of families and marriage is an important part of anthropology because family and household groups play a central role in defining relationships between people and making society function. While there is nothing in biology that dictates that a family group be organized in a particular way, our cultural expectations leads to ideas about families that seem "natural" to us. Why is it important for anthropologists to understand the kinship, descent, and family relationships that exist in the cultures they study? Status and role define the position of people within the family as well as the behaviors they are expected to perform. In this chapter, Gilliland describes several different patterns of family organization including nuclear families, extended families, and joint families. While small nuclear families are common in the United States, larger families are common in many other societies. What do you think are some of the practical effects of both small and large families on everyday life? Clan: a group of people who have a general notion of common descent that is not attached to a specific biological ancestor. Descent groups: relationships that provide members with a sense of identity and social support based on ties of shared ancestry. Domestic group: a term that can be used to describe a group of people who live together even if members do not consider themselves to be family. Endogamy: a term describing expectations that individuals must marry within a particular group. Exogamy: a term describing expectations that individuals must marry outside a particular group. Family: the smallest group of individuals who see themselves as connected to one another. Family of procreation: a new household formed for the purpose of conceiving and raising children. Kinship: term used to describe culturally recognized ties between members of a family, the social statuses used to define family members, and the expected behaviors associated with these statuses. Kinship diagrams: charts used by anthropologists to visually represent relationships between members of a kinship group. Kinship system: the pattern of culturally recognized relationships between family members. Matrilineal descent: a kinship group created through the maternal line (mothers and their children). Neolocal residence: newly married individuals establish a household separate from other family members. Nuclear family: a parent or parents who are in a culturally-recognized relationship, such as marriage, along with minor or dependent children. Patrilineal descent: a kinship group created through the paternal line (fathers and their children). Polygamous: families based on plural marriages in which there are multiple wives or, in rarer cases, multiple husbands. Role: the set of behaviors expected of an individual who occupies a particular status. Sororate marriage: the practice of a man marrying the sister of his deceased wife. Status: any culturally-designated position a person occupies in a particular setting. Stem family: a version of an extended family that includes an older couple and one of their adult children with a spouse (or spouses) and children. Her primary research took place in the former Yugoslavia (1982-4, 1990-1), Croatia (1993, 1995, 1996-7) and with displaced Bosnians, Croats and Serbs in the United States (2001-3). In Croatia, Mary Kay was affiliated with the Filozofski Fakultet in Zagreb, the Ethnographic Museum in Slavonski Brod (Croatia/Yugoslavia), and with the Institute for Anthropological Research (Zagreb, Croatia both pre- and post-independence). Continuing affiliation as member of Editorial Board for the Collegium Antropologicum: the Journal of the Institute for Anthropological Research, and named a Lifetime Member of the Croatian Anthropological Society. Her areas of research interest and publication include culture and social change, gender and ethnic identity, family, marriage and intergenerational relationships.
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The differential here rests on the overall course: in the personality disorder there is no deterioration heart attack yahoo answers generic 17.5mg zestoretic otc, whereas in simple schizophrenia one sees a very slow progression heart attack yawning purchase zestoretic 17.5 mg free shipping. Schizophreniform disorder and brief psychotic disorder (also known as brief reactive psychosis) are both characterized by symptoms that are similar to blood pressure medication images purchase zestoretic 17.5mg on line those seen in schizophrenia; however, where they differ is in their supposed course. Patients who experience a full, complete, and spontaneous remission within 1 month are said to have brief psychotic disorder, whereas those whose illness lasts longer than 1 month but less than 6 months are said to have schizophreniform disorder. Certainly, there are patients with schizophrenia who are treated with antipsychotics early in the course of the illness and who experience a complete, antipsychotic-induced remission of symptoms; however, in these cases, if treatment is discontinued, symptoms gradually recur. What is at issue here is whether there are, in fact, cases in which symptoms spontaneously and completely undergo a lasting remission without treatment. I have never seen such a case, nor am I aware of any such well-documented cases in the literature. Treatment In almost all cases, treatment involves the use of an antipsychotic drug. These agents may be broadly divided into two different categories, namely first-generation and second-generation or, as they are often also termed, typical and atypical agents. High-potency drugs require lower milligram doses and are more likely to cause extrapyramidal side-effects. Low-potency drugs require higher doses and are less likely to cause extrapyramidal sideeffects, but are prone to cause sedation, hypotension, and anticholinergic effects. Medium-potency drugs, as might be expected, fall in-between regarding both milligram dosage and side-effects. All other things being equal, of the first-generation agents, haloperidol is probably a reasonable first choice. Second-generation agents include clozapine, olanzapine, risperidone, quetiapine, aripiprazole, and ziprasidone. With regard to effectiveness, clozapine is clearly superior; however, its side-effect profile, especially the risk of agranulocytosis, limits its use to treatment-resistant cases. Of the other agents, although there is controversy here, it appears that olanzapine may have an edge in terms of overall effectiveness (Lieberman et al. This advantage, however, is severely tempered by the tendency of olanzapine to cause metabolic derangements, including weight gain, hyperlipidemia, and diabetes. Aripiprazole and ziprasidone stand out in that they are not associated with metabolic changes. Overall, and all other things being equal, if a secondgeneration agent is used, it may be reasonable to start with risperidone; however, again, it must be acknowledged that this is an area of great controversy. In deciding which antipsychotic, whether first or second generation, to prescribe, the first step is to obtain an accurate treatment history, and this may require not only questioning the patient but also reviewing records and interviewing family members. If there is a history of a good response combined with good tolerability, then it makes sense to use the same drug again. In treatment-naive patients, or in cases in which prior treatments were unsatisfactory, other considerations come into play. Although not without controversy, it appears that, overall, second-generation agents are more effective and better tolerated than first-generation ones, and, consequently, it may be reasonable to select a second-generation agent. It must be emphasized, however, that the choice of an agent is not simple or straightforward, and often multiple trials of different agents must be performed before a regimen is found that is reasonably effective and well-tolerated. In general, assuming an adequate dose is used, one should observe the patient for at least 2 weeks to get a reasonable idea as to response. In cases characterized by significant agitation, one may, as described in Section 6. If the response to the first agent is poor, or the side-effects are unacceptable, then a trial with a different agent should be considered. Should patients fail to get a good response to adequate trials of two agents, one may be dealing with a treatment-resistant case. As regards effectiveness, clozapine is clearly head and shoulders above all other antipsychotics in treatment-resistant cases, and indeed may yield some of the most gratifying treatment responses in all of medical practice; however, its side-effect profile gives pause to many patients and physicians. In some cases, however, patients will opt to stay with a regimen that, although perhaps providing less relief than is hoped for, is at least tolerable. Initially, patients should be maintained on a dose that is similar to, if not identical to, that utilized during initial treatment.
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They have also attempted technical debiasing hypertension 6 months pregnant order 17.5 mg zestoretic amex, working to blood pressure chart normal blood pressure range zestoretic 17.5 mg line "fix" algorithms and diversify data sets hypertension over 60 discount zestoretic 17.5 mg, even though these approaches have proven insufficient and raise serious privacy and consent concerns. Affect recognition, which claims to "read" our inner emotions by interpreting physiological data such as the micro-expressions on our face, tone of voice, or gait, has been a particular focus of growing concern in 2019-not only because it can encode biases, but because we lack a scientific consensus as to whether it can ensure accurate or even valid results. Key concerns have also emerged regarding how algorithmic health-management tools are impacting patient data and well-being, and the lives of those who care for them. Most algorithmic management tools, like most algorithmic decision systems, lack meaningful opportunities for workers to understand how the systems work or to contest or change determinations about their livelihood. In many warehouses, termination is an automated process (not unlike being "kicked off" a gig-economy platform). Many workers in the Amazon warehouse where he organizes are Somali immigrants, whose work at Amazon helps send money home. The typical worker had total annual earnings in 2017 of $20, 585, which is slightly over half of the living wage. Chris Ramsaroop, a founding member of the organization Justicia for Migrant Workers, documents the integration of tracking and productivity technologies in the agriculture sector in Canada, finding that "surveillance technologies are utilized to regiment workers to determine their pace at work and their production levels, much like what we see in warehouses. This mandates a rate of productivity as part of the contractual agreement and enforces that rate through an algorithm, instead of through on-site supervisors. Such rate-setting systems rely on pervasive worker surveillance to measure how much they are doing. Systems to enable such invasive worker monitoring are becoming more common, including in traditionally "white-collar" working environments. For example, the start-up Humanyze incorporates sensors into employee badges to monitor employee activities, telling employers where workers go, whom they interact with, and how long they stay in a given place. Another company called Workplace Advisor uses heat sensors to achieve a similar aim. And though the usefulness of these products is disputed, 15 they reflect an increasing willingness to engage in invasive surveillance of workers in the name of workplace control and eking out incremental gains in productivity. Algorithmic Wage Control Algorithmic worker management and control systems have also had a severe negative impact on wages across the so-called "gig economy. Similar to other algorithmic management systems, these function by pooling information and power together for the benefit of owners, managers, and a handful of developers, allowing companies to optimize such systems in ways that maximize revenue without regard to the need for stable and livable wages or predictable incomes, schedules, and availability of work. Indeed, many workers have reported being abruptly "kicked off" a gig work platform, and finding themselves unable to work without warning. Abrupt changes intended to increase revenue for the company can result in significant losses for workers. In one example, Instacart made changes to its interface that misled customers into thinking they were leaving a tip for workers, when in fact they were paying a service fee to the company. The ability of automated management platforms to manipulate (and arbitrarily cut) wages has been at the heart of worker grievances. Instacart workers report that their earnings decreased precipitously over the last year. Yet at this point, such claims amount to marketing statements and are unsupported by peer-reviewed research. What is missing from both conflicting narratives is the more nuanced prediction of who will be harmed and who will benefit from labor automation in the years to come. One such study from the Brookings Institute predicts that certain demographic groups will likely bear more of the burden of adjusting to labor automation than others, implying that the benefits of automation-increased efficiency and profit-are not shared with all workers, but accrue to those at the top. This has serious implications in terms of the risk exposure faced by certain communities. Black, Native American, and Latinx workers who make up a larger proportion of the workforce in occupations like construction, agriculture, and transportation50 face average task-automation potentials of 44 to 47 percent. We may "have no idea how many jobs will actually be lost to the march of technological progress, "57 but we can begin to answer who will lose their jobs based on the power dynamics and economic disparities that already exist today. The vast majority of these documents were generated from countries and organizations in the global North. Scholars and advocates have increasingly called attention to the gap between high-level statements and meaningful accountability. This lack of professional and legal accountability undermines corporate ethics approaches. Advocates for this approach describe human rights as ethics "with teeth, " or an alternative to the challenge of operationalizing ethics.
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The task force noted that proposed global solutions focused on top-down management strategies that did not take existing social issues of "poverty blood pressure medication safe for pregnancy cheap 17.5mg zestoretic with visa, marginalization blood pressure medication that starts with an l buy zestoretic 17.5 mg without prescription, lack of education and information prehypertension with low heart rate buy 17.5 mg zestoretic visa, and loss of control over resources" that structure vulnerability of different populations to the impacts of a warming planet into account. At the end of the report, the task force recommended actions anthropologists could take to contribute to efforts to address global climate change, including reducing the carbon footprint of anthropological meetings, working with interdisciplinary research teams to continue research, and maintaining a research agenda that stresses the importance of anthropological contributions to discussions of climate change. The anthropological 377 focus on local communities is a welcome change of perspective when, by definition, the scale of global climate change seems to preclude local involvement and solutions. They also work for smaller conservation organizations, urban planning initiatives, environmental education groups, environmental activist networks, and other initiatives aimed at reducing our negative impact on the planet. Cultural Resources Management Management of cultural resources is a growing field of anthropology that catalogs and preserves archaeological sites and historic places threatened by development, bringing together various principles developed in anthropology over the years. First, it recognizes the need to preserve both "natural" ecosystems and ecosystems shaped by past human activities. Second, cultural resource managers recognize the need for continuing involvement of indigenous communities with archaeological sites and seek their input to inform management plans and practices. As cultural resource management has become standard operating procedure, archaeologists have begun to meet with members of the local community and others who have a stake in their research. These interactions improve archaeological research and create the kind of cross-cultural bridges that strengthen the discipline. Finally, destruction of historical places and archaeological sites is a form of environmental destruction that, like climate change and species extinctions, requires us to critically examine the cultural values underlying that destruction. Anthropologists are hard at work with governments, conservation organizations, and community groups to understand and solve complex environmental problems. I hope this discussion has challenged you to think about the environment and conservation in a new way, allowing you to help reframe these debates and develop innovative solutions to the complex problems that confront us. In what ways have anthropologists examined human interactions with the environment over time? How has research in political ecology challenged traditional conservation efforts? What are some of the problems with promoting parks or ecological reserves as solutions to environmental problems? How has research in anthropology contributed to an improved understanding of how humans interact with the "natural" world? What insights from anthropology do you think would be most useful to the public, environmental activists, and government officials when considering policies related to current environmental challenges? There is some disagreement about when the Anthropocene period began-most likely, it began with industrialization. Cultural ecology: a subfield of cultural anthropology that explores the relationship between human cultural beliefs and practice and the ecosystems in which those beliefs and practices occur. Cultural evolutionism: a theory popular in nineteenth and early twentieth century anthropology suggesting that societies evolved through stages from simple to advanced. Ecocide: destruction of an environment, especially when done intentionally by humans. Eco-justice: a movement to recognize and remedy the adverse relationship between social inequality and the harms and risks that come from environmental destruction and pollutants. Ethnocide: destruction of a culture, often intentionally, through destruction of or removal from their territory, forced assimilation, or acculturation. Ethnoecology: the relationships between cultural beliefs and practices and the local environment. Extractive reserves: community-managed protected areas designed to allow for sustainable extraction of certain natural resources (such as fish, rubber, Brazil nuts, and rattan) while maintaining key ecosystems in place. Exurban: migration of generally affluent people from urban areas to rural areas for the amenities of nature, recreation, and scenic beauty associated with rural areas. Historical particularism: the theory that every culture develops in a unique way due to its history, including the interaction of people with the natural environment. Homeostasis: the movement of a particular system (a human body, an ecosystem) towards equilibrium. In ecology this is associated with the idea that ecosystems should remain at a climax ecosystem associated with an area.
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The document indicates that the notice of termination was served less than two or more than ten years before the effective date of termination heart attack 34 years old proven 17.5mg zestoretic. The document was received by the Office on or after the effective date of termination prehypertension follow up buy zestoretic 17.5 mg without prescription. The notice of termination was issued under Section 203 arteria buccinatoria generic zestoretic 17.5 mg without a prescription, but the document indicates that the date of execution for the grant falls before January 1, 1978. The date of recordation for a notice of termination is the date when the Office receives the proper filing fee and a notice that satisfies the relevant requirements set forth in Section 201. Likewise, if the notice is returned to the remitter for correction, the date of recordation is based on the date that the corrected notice is received by the Office. Copyright Office is a mandatory requirement for terminating a grant under Sections 203, 304(c), or 304(d). However, the fact that the Office recorded a notice of termination does not create a legal presumption that the termination has been properly effected or that the notice is valid. As a general rule, an error may be considered harmless if it "do[es] not materially affect the adequacy of the information required" under sections 203, 304(c), or 304(d) of the statute. The following are representative examples of harmless errors that will not affect the validity of a notice, provided that the errors were made in good faith and without any intention to deceive, mislead, or conceal relevant information: Errors in identifying the date of registration or registration number. Errors in describing the precise relationships between the author and his or her heirs. If it turns out that the date of execution specified in the notice of termination is not the actual date of execution of the grant, the error may be considered harmless if it is as accurate as the terminating party is able to ascertain, and if the date is provided in good faith and without any intention to deceive, mislead, or conceal relevant information. Providing an erroneous date of execution, however, may not be considered harmless if the grant would have properly been subject to termination under section 203, rather than section 304 (or vice versa). Copyright Office-must determine whether a grant is eligible for termination under Sections 203, 304(c), or 304(d). The Office cannot provide specific legal advice on the rights of persons, issues involving a particular use of a copyrighted work, or other matters of a similar nature. If the answer is "yes, " the grant cannot be terminated under Sections 304(c) or 304(d). Copyright Office has created a separate "work made for hire questionnaire, " which may be useful in determining whether a particular work may be considered a work made for hire. If the answer is "yes, the work is a work made for hire, " the grant cannot be terminated under Sections 203, 304(c), or 304(d). To determine if the grant may be eligible for termination under Sections 304(c) or 304(d), proceed to Question 6. If the answer is "no, the grant was made on or before December 31, 1977, " proceed to Question 5. If the answer is "no, the work was created on or before December 31, 1977, " the grant cannot be terminated under Section 203. If the answer is "no, the work was first published on or after January 1, 1978" proceed to Question 10. If the answer is "no, the work was first published on or before December 31, 1951, " the grant cannot be terminated under Sections 203 or 304(c). To determine if the grant may be eligible for termination under Section 304(d), proceed to Question 13. Question 11: Was the work first registered with the Copyright Office on or before December 31, 1977? If the answer is "no, the work was first registered on or after January 1, 1978, " the grant cannot be terminated under Sections 203, 304(c), or 304(d). Question 12: Was the work first registered with the Copyright Office on or after January 1, 1952? If the answer is "no, the work was first registered on or before December 31, 1951, " the grant cannot be terminated under Sections 203 or 304(c). To determine if the grant may be terminated under Section 304(d), proceed to Question 13.
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Efficacy of modafinil compared to pulse pressure is trusted 17.5 mg zestoretic dextroamphetamine for the treatment of attention deficit hyperactivity disorder in adults blood pressure medication and zinc buy cheap zestoretic 17.5 mg on-line. Comparing guanfacine and destroamphetamine for the treatment of adult attention-deficit/hyperactivity disorder diastolic blood pressure 0 buy cheap zestoretic 17.5mg. A note on the association of extensive haemangiomatous naevus of the skin with cerebral (meningeal) haemangioma especially cases of facial vascular naevus with contralateral hemiplegia. Carbamazepine-induced heart block in a child with tuberous sclerosis and cardiac rhabdomyoma: implications for evaluation and follow-up. A positron emission tomography (18F) deoxyglucose study of developmental stuttering. Thus conceived, multi-infarct dementia is, in all likelihood, a common cause of dementia in the elderly. Vascular dementia is an umbrella term that includes not only multi-infarct dementia but also lacunar dementia (Section 10. Although in some cases the dementia may be rather nonspecific, with mere difficulties in memory, calculations, and abstractions, etc. Hallucinations are generally visual, and delusions tend to be either of persecution or misidentification. Focal signs, as noted, are common and may include aphasia, apraxia, neglect, and hemiparesis etc. Course As noted, the classic course of multi-infarct dementia is stepwise, with successive strokes bringing the patient down yet another step into further cognitive deterioration. Importantly, many of these steps are characterized by a delirium, which gradually resolves concurrent with the resolution of peri-lesional edema. In some cases, the course may be marked by one giant step down, as in cases of multiple simultaneous infarcts or with one infarct occurring in an exquisitely strategic location. Clinical features the onset of multi-infarct dementia corresponds to the age of greatest risk for stroke, and hence most patients are in their sixties or older. The multiple causes of these infarctions (and hemorrhages) are discussed in Section 7. There is also evidence that both the cholinesterase inhibitors donepezil (Black et al. Concurrent with symptomatic treatment, steps should be taken to prevent future strokes if possible, as discussed in Section 7. Although the prevalence of lacunar dementia is not known with any precision, the clinical impression is that it is not uncommon. Differential diagnosis the diagnosis of multi-infarct dementia should be considered in any patient with dementia and a history of stroke. In weighing this history, however, one must take into account the location of the lesion: whereas infarcts in such cognitively strategic locations as the frontal, parietal, or temporal cortices might be expected to cause dementia, one would be hard-pressed to attribute a dementia to infarctions occurring in the occipital lobes. Lacunar dementia may also present with a history of stroke; however, here the strokes tend to be of the lacunar variety, such as pure motor stroke. Furthermore, and in contrast to multi-infarct dementia, lacunar dementia tends to be characterized by a frontal lobe syndrome. In some instances, it may not be possible to disentangle the effects of each of these separate processes, and in such cases, one may have to be content with merely making a diagnosis of vascular dementia. Such a diagnosis should be considered in cases in which the course is mixed, being composed of sequential downward steps occurring on a background of a steady, gradual decline. Clinical features In addition to cognitive deficits such as decreased shortterm memory, slowed thinking, and disorientation (Mok et al. As might be expected, there is typically also a history of lacunar syndromes, such as pure motor stroke, ataxic hemiparesis, dysarthriaclumsy hand or pure sensory stroke. Furthermore, and in advanced cases of the lacunar state, it is common to see a psuedobulbar palsy with, as described in Section 4. Clinical features Etiology In all likelihood, lacunes cause cognitive deficits by interrupting the circuit that runs from the frontal cortex to the basal ganglia, then to the thalamus and finally back to the frontal lobe, and this may account for the frequency with which the frontal lobe syndrome accompanies this dementia. A discussion of the mechanisms underlying the infarctions that create these lacunes is provided in Section 7. Notably, both tremor and cogwheeling are generally absent, and, as discussed below, the response to levodopa is generally poor. Magnetic resonance scanning reveals multiple lacunes, with at least some of them involving either the basal ganglia or the mesencephalon (Zijlmans et al. As noted earlier, it is not uncommon to find evidence of other vascular pathology and, in cases in which there are also large, territorial cortical infarcts or extensive white matter disease in addition to lacunes, clinical judgment must come into play in deciding how important the lacunes are in the development of the dementia.
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Finally prehypertension heart attack buy zestoretic 17.5mg cheap, in severe and treatment-resistant cases blood pressure chart for age 50+ cheap 17.5mg zestoretic otc, neurosurgical intervention arteria zigomatica buy discount zestoretic 17.5 mg on line, for example deep brain stimulation of the globus pallidus, may be considered (Shahed et al. The original name, bestowed by Batten and Gibb (1909), was myotonia atrophica; a similar one is myotonia dystrophica. Subsequently, this disease was referred to as myotonic muscular dystrophy or, simply, myotonic dystrophy. Clinical features the onset is gradual and insidious and, although most patients fall ill in their late teens or early twenties, the range of age of onset is wide, from childhood up to the sixth decade. Myotonia may go unnoticed by the patient, or may manifest in difficulty in letting go of, for example, a doorknob, or in disengaging from a handshake. On physical examination, myotonia may be elicited by tapping the thenar eminence with a percussion hammer and observing for the characteristic muscle dimpling. Weakness is eventually accompanied by atrophy and, importantly, this is more prominent distally, being seen first in the upper extremities. Cataracts are seen in over 90 percent of patients, and there may be deafness (Wright et al. Cardiac conduction abnormalities and arrhythmias may occur, including atrioventricular block, fascicular block, intraventricular conduction block, atrial fibrillation, and ventricular premature contractions; a cardiomyopathy with congestive heart failure may also occur, but is less common. Personality change may also occur and, although it tends to be mixed in type, avoidant traits may be predominant (Delaporte et al. Apathy appears to be more common than among comparable controls (Rubinsztein et al. Although some of these hypersomnic patients demonstrate sleep apnea (of either the central or obstructive type), the number of apneic episodes is not high enough to account for the degree of hypersomnolence (van der Meche et al. Course the disease is gradually progressive and, although those with later onsets and milder symptoms may experience a normal lifespan, cases of early onset and severe symptoms are often associated with premature death in early or middleadult years, often from cardiac or respiratory causes. Etiology Myotonic muscular dystrophy is inherited in an autosomal dominant fashion with almost 100 percent penetrance but a quite variable expressivity, even within the same family (Pryse-Phillips et al. Although anticipation is more likely with maternal transmission, it has been noted in paternal cases (Nakagawa et al. Neuronal heterotopias have been noted in the cerebral cortex (Rosman and Kakulas 1966) and neurofibrillary tangles in the hippocampus (Maurage et al. In cases characterized by hypersomnolence, cell loss was noted in the superior central nucleus of the midbrain (Ono et al. Because the phenomenon of myotonia may be the only symptom of myotonic muscular dystrophy for many years, the clinical differential between myotonic muscular dystrophy and myotonia congenita may depend on long-term follow-up, watching closely for other symptoms to appear (Maas and Paterson 1950). Hypersomnolence, which, in some cases, may be the most distressing feature of the disease, responds to modafinil (MacDonald et al. Myotonia may respond to various medications, including phenytoin, disopyramide, procainamide, and nifedipine, but given that myotonia rarely causes distress or disability, these agents are typically not required. As noted earlier, alveolar hypoventilation may occur; these patients do not appear to tolerate general anesthesia well, hence surgery, if possible, should be avoided. Genetic counseling is appropriate and, given the variable expression of the disease, it may be appropriate to offer testing to apparently unaffected relatives. Although the disease may present with any one of these various features, in most cases the presentation will be with juvenile cataracts or with chronic, intractable diarrhea. Serum cholestanol levels are grossly increased; cholesterol levels are either normal or decreased (Salen 1971). In those with an early age of onset and severe symptoms, death typically occurs within 10 to 20 years, whereas those with later onsets and milder symptoms may experience a normal lifespan. With defective activity of this enzyme, cholestanol accumulation occurs in the cerebral and cerebellar white matter, the cerebellar dentate nuclei, and peripheral nerves, tendons, and corneae (Menkes et al. Within the brain, widespread demyelinization occurs and, in some cases, actual xanthomas may form (Schimschock et al. Differential diagnosis Whenever dementia occurs in the setting of juvenile cataracts, chronic diarrhea, or, classically, Achilles tendon enlargement, the diagnosis of cerebrotendinous xanthomatosis is very likely. When these are not present, or are overlooked, consideration may be given to metachromatic leukodystrophy in children, and to spinocerebellar ataxia in adults. Clinical features the onset is very gradual and, although most patients begin to have symptoms in late childhood or early adolescence, the range in age of onset is wide, from infancy to middle years (Swanson and Cromwell 1986). It is of particular interest in that, when appearing in adolescents or adults, it may present with a psychosis, personality change, or a dementia.
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