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Within this circle catapres 100 mcg otc hypertension bench, of family members and friend associated fellowships order 100 mcg catapres with amex arrhythmia icd 9 codes, is the comfort zone for giving and receiving help and support when needed order catapres 100mcg blood pressure blurry vision. This may lead to purchase catapres 100 mcg otc blood pressure medication benicar lack of social participation, loneliness, weaker economy and negative stress, all factors that affect social, psychological and physiological environmental determinants (Nss, Rognerud, Strand 2007, Sund, Krogstad, 2005). More research is needed concerning the interplay of socio economic conditions and health risk factors in a life span perspective. Still there are indications pointing to social support as a considerable buffer against diseases and psychological stress (Cohen, S. Although results are not clear, there are strong indications that the risk for experiencing unwanted loneliness increases with age (Halvorsen, 2005, Holmen, & Furukawa, 2002, Thorsen, 2005). An interesting gender difference is found, as elderly men tend to live with significantly younger women (Nolen Hoeksema, & Rusting, 2000). Being single, feeling lonely may be a barrier difficult to break, according to our informants. In focus group interviews, most participants more or less “confessed” that they are not good at welcoming or even speaking to newcomers. Some had experienced how difficult it may be to “join in” among strangers by going to a dance activity. According to our informants, men put restrictions on themselves by demanding some basic skills or knowledge prior to participation, and as result, many prefer to stay at home. Other given reasons for not attending cultural and other leisure activities, is a lack of interest in earlier years, and a notion that most activities are run “on women’s terms. Today’s pensioners represent a political epoque where socio democratic egalitarianism and development of a strong national welfare state has had first priority. Norwegian citizens have enjoyed solid predictability and security at the job marked. Access to work, a decent income, material goods, education, individual and colloquial security are also considered important national health factors (Strand, Kunst, 2006). Over the past few decades we have seen a tendency of higher cultural participation among working class groups, especially among women (Loga, 2010, Vaage, 2010). In our studies, the majority of informants are culturally and socially active, with small demands for health care services. Over the past 30 years, social inequality has been on the rise, and with it, a growing tendency towards socio 290 economic exclusion. The most resourceful groups tend to keep themselves busy with friends and family based activities as well as participation in various activities according to individual interests (Wilhelmsen, 2009). Among repercussions of the “globalized” market economy are pressures on democracy and national sovereignty (Smedshaug, 2011, Brox, 1994). A continuous growth in socio economic differences is a future threat to public health standards. In this perspective, common access to stimulating and inclusive cultural arenas may be of crucial importance for future public health strategies. Closing comments Although results from these surveys should be interpreted with caution, they fall in line with results from larger national and international studies, highlighting the importance of enriched environments, participation and association in meaningful cultural and social activities as major life quality and health promotional factors. The cultural programs offered by Trondheim Municipality seem to be of great significance for its participants. The vast majority of informants live an active life with little hindrance from practical, economical as well as physical and psychological health related matters. The opportunity to pick up on old interests after retirement seems to have contributed to more joy, growth, social participation, meaning and purpose in life. During the survey period for Study 1, several concerts and the like were arranged at nursing homes, health centers, and other geriatric institutions. In accordance with the “Culture Law Regulation” passed by the Parliament in 2007 (Stortingsmelding Nr. More knowledge concerning the use of cultural activities in health promotion may turn out to be of great value for future public health, environmental and socio economic development strategies. More research is needed, including longitudinal population studies, focusing on quality of life as well as on biomedical effects. Unequal in death: Attendance at cultural events, reading books or periodicals, and making music or singing in choir as determinants for survival. Cultural participation and health: A randomized controlled trial among medical care staff. The Impact of Professionally Conducted Cultural Programs on the Physical Health, Mental Health, and Social Functioning of Older Adults. Den kulturelle spaserstokken, arena for kulturell deltakelse for seniorer i Trondheim kommune. Et bidrag til kartlegging av kultur – og fritidsinteresser blant hjemmeboende men i alderen 70+ i Trondheim kommune. Childhood socioeconomic status and suicide mortality in early adulthood among Norwegian men and women. A prospective study of Norwegians born between 1955 and 1965 followed for suicide from 1990 to 2001. Resultater fra kultur og mediebruksundersokelsene fra 1991 til 2008 og Levekarsundersokelsen 2007. Dokumentasjonsrapport (2009/40): Levekarsundersokelsen 2008: helse, omsorg og sosial kontakt.
In those destined activities buy catapres 100mcg overnight delivery blood pressure medication joint pain, the bedroom should be darkened and quiet buy 100mcg catapres with amex heart attack 51, and to buy generic catapres 100mcg line blood pressure hypertension develop psychophysiologic insomnia cheap catapres 100mcg otc arrhythmia pac, however, a certain the bed should be reserved for sleep or sexual activity. This sleep does not come, patients should do something else, anxiety then makes sleep less likely to occur and a vicious perhaps reading, until drowsiness occurs. Whether insom cycle may be established in which a failure to sleep engen nia occurs or not, the wake up time should be strictly ders more anticipatory anxiety, which in turn makes the adhered to. Once this pattern is firmly established, the Should insomnia persist despite good sleep hygiene, bed, rather than being seen as a place for relaxation and consideration may be given to cognitive behavioral restoration, becomes an anxiety provoking stimulus in therapy, which is not only effective acutely (Edinger et al. Interestingly, in such cases patients may sleep better 2001) but also confers enduring benefits (Backhaus on the couch or in a hotel. Should cognitive behavioral therapy be inef factors exist in psychophysiologic insomnia is not clear; a fective or impractical, pharmacologic treatment may be recent report, however, did note reduced nocturnal mela considered. Differential diagnosis Melatonin was effective in one double blind study (Zhdanova et al. Ramelteon, a selective melatonin receptor ago less legs syndrome, periodic leg movements of sleep, and nist, also appears to be effective (Erman et al. Trimipramine, another tricyclic antidepressant, disorder, post traumatic stress disorder, and schizophre is also effective in doses of 100–200 mg h. Trazodone is cause insomnia, and insomnia is universal and often very very widely used in doses ranging from 25–100 mg h. Given the excellent tolerability of making this diagnosis, however, it must be borne in mind melatonin, starting with this agent, using a dose of 3–6 mg that some patients with psychophysiologic insomnia sleep in the evening, is a reasonable choice. When this is sus no comparative studies of melatonin and ramelteon; the pected, it may be appropriate to perform polysomno latter agent, however, represents another reasonable first graphy at the patient’s home before making the diagnosis. Should melatoninergic agents fail, consideration ‘Short sleepers’ are individuals who, despite getting little may be given to zolpidem or eszopiclone; doxepin and sleep, awake refreshed and have no complaints. Treatment the foregoing discussion of pharmacologic treatment concerns the psychophysiologic form of primary insom Good sleep hygiene is essential. Naps should not be taken, nia; in cases of idiopathic insomnia in children, melatonin, and patients should get some exercise every day. Narcolepsy is also often Primary or essential hypersomnia is a rare disorder charac considered; however, the naps seen in narcolepsy, unlike terized by chronic persistent hypersomnolence (Bassetti those of primary hypersomnia, come in irresistible attacks and Aldrich 1997, Billiard et al. Clinical features Excessive daytime sleepiness may also be seen in myotonic muscular dystrophy, hypothyroidism, as a sequela the onset is gradual, typically occurring in late adoles to infectious mononucleosis, and with lesions of the hypo cence. Chronic use of sedat are typically groggy and have trouble ‘getting going’; a ing medications, such as benzodiazepines, tricyclic anti minority may also experience sleep drunkenness. Patients depressants, certain anti epileptic drugs, antihistamines, report persistent drowsiness during the day and often take and opioids must also be considered on the differential. Importantly, these naps do not occur as irre who require more than 8 hours of sleep a night. In contrast sistible attacks but rather are preceded by a gradually to patients with primary hypersomnia, however, these increasing drowsiness, which can often be resisted. Treatment Course There are no controlled studies; case series (Bassetti and For most, primary hypersomnia is a chronic condition; Aldrich 1997) suggest that some patients improve with spontaneous improvement is seen in only about 10 percent. Narcolepsy associated with lesions of the also intermittent forms of hypersomnia under the rubric of diencephalon. Augmentation of the restless legs syndrome mary hypersomnia is reserved for chronic cases only. Kleine–Levin syndrome: a taking the history one must be alert to other vegetative systematic review of 186 cases in the literature. Once it is established that the patient indeed has chronic Ayalon L, Borodkin K, Dishon L et al. Neurology disorders characterized by excessive daytime sleepiness 2007; 68:1136–40. Long term legs syndrome, periodic limb movement disorder, painful effectiveness of a short term cognitive behavioral group p18. Pulmonary function in a typical case of response and complications of extended treatment in 49 Pickwickian syndrome. J Clin behavior disorder pathophysiology in brainstem predominant Psychiatry 1987; 48:209–10. Neurology 1988; scheduled dark, sunglasses, and melatonin to facilitate 38:1845–8. Protriptyline in resetting the circadian clocks of patients with delayed sleep obstructive sleep apnea: a double blind trial. Treatment of night terrors and darkness to treat physiologic maladaptation to night with imipramine. Modafinil for excessive associated with alveolar hypoventilation: a Pickwickian daytime sleepiness associated with shift work sleep disorder. An autoimmune hypothesis based on clinical and Kleine–Levin syndrome with localized encephalitis. Disturbed hypothalamic due to prolonged half life in 18 patients with nocturnal pituitary axis in idiopathic recurring hypersomnia syndrome. A psychophysiological study of an acromegalic patient who underwent pituitary irradiation. Obstructive sleep apnea motor vehicle mishaps in persons taking pramipexole and associated with cerebral hypoxemia and death. The Kleine–Levin syndrome: hypersomnia, bulimia, and Neurosurg Psychiatry 2003; 74:127–30. Hypersomnia associated behavioral therapy for treatment of chronic primary insomnia: with a focal pontine lesion.
Clinical Practice Guideline: Insomnia in excess of placebo cheap catapres 100 mcg on-line prehypertension 139, with the possible exception of excessive Bowen100 compared triazolam 0 discount 100 mcg catapres with visa heart attack vomiting. This assessment refects the task received one of three interventions nightly for three weeks in force’s clinical judgment catapres 100mcg pulse pressure 25, based on zolpidem’s effcacy for a parallel group design cheap 100mcg catapres with mastercard blood pressure stress. Outcome variables were patient sleep onset and maintenance, and its relatively benign side ef reported. Hajak104 treated 1,507 subjects with sleep onset or maintenance Triazolam for the Treatment of Chronic Insomnia insomnia with triazolam 0. The triazolam versus placebo comparison groups totaled 605 sub Recommendation 5: We suggest that clinicians use jects, who received drug or placebo for 28 consecutive nights triazolam as a treatment for sleep onset insomnia (versus and reported sleep variables on visual analog scales. The triazolam and placebo Because only one study96 contained data of suffcient quality, groups included 28 subjects who completed daily sleep diaries. The quality of evidence for Rickels107 studied 50 subjects with sleep onset or maintenance this study was high. This study, consisting of patient reported insomnia who received either triazolam 0. Sleep latency: In the only study with adequate data for Number of awakenings was insignifcantly decreased. This reported sleep diary reductions from baseline placebo levels was followed by 14 nights in which subjects chose to self admin of 55 min and 24 min in two separate triazolam 0. Rickels107 reported similar subjective improvement on ratings Thirteen additional studies met general inclusion and exclu of sleep induction for triazolam 0. Many included dosages which fell below the clinical signifcance threshold of 30 min and was are higher than current recommended dosages. Clinical Practice Guideline: Insomnia In additional studies, Hajak104 found no signifcant differ patientS’ valueS and preferenceS: the task force ence between triazolam 0. This was not a clinically signifcant difference from number of aWakeningS: Roehrs96 reported a reduction in placebo and falls below the threshold for clinical signifcance. In summary, meta analysis data are available for temaze Bowen100 observed a statistically signifcant reduction in sub pam 15 mg only. Hajak104 studies (see below), the task force judged that the benefts of te reported that “speech disorder” was the only adverse effect, mazepam 15 mg appear to be greater than the potential harms. Wilson117 conducted an mazepam in the treatment of insomnia was included in the sta actigraphic evaluation of 38 subjects with “complaints of poor tistical analysis. Subjective results from patient jects underwent a crossover study of two weeks of treatment diaries as well as actigraphic results were averaged over the with placebo, temazepam 15 mg, or diphenhydramine 50 mg respective periods. For the purpose of this analysis, on subjective sleep latency from patient diaries. However, it is noteworthy that in this ceive temazepam 15 and 30 mg or placebo for a single night sample of “primary sleep onset insomnia” patients, both te using a within subjects crossover design. Subjects received a single night of placebo and a single night At the 20 mg dosage, three trials92,113,117 reported subjective of temazepam 20 mg with one week intervening washout. Of note, the subjects in this study were de on sleep onset, total sleep time, awakenings, sleep effciency, scribed as exhibiting “sleep onset insomnia. Over, benefts were deemed to outweigh harms for te Quality of Sleep: Meta analysis was conducted for sleep mazepam 15 mg. Two studies found statistically signifcant improvement in Melatonin agonists sleep quality ratings for temazepam 30 mg. There was no evidence of signifcant difference from placebo for any overall Quality of evidence: the overall quality of adverse events, based on available side effect data. Although evidence in the meta analytic data from the two available stud the evidence for effcacy is marginal, the benefts appear to be ies was moderate for temazepam 15 mg due to imprecision. Based on clinical judgment, the task force determined that the majority of well HarmS: Limited data on adverse effects of temazepam 15 informed patients would use ramelteon over no treatment. Heffron115 found eon in the treatment of chronic primary insomnia was included 329 Journal of Clinical Sleep Medicine, Vol. Roth and colleagues120 studied 100 older adults (age > 65 in meta analysis supported these results. Meta analysis of the grouped evidence demonstrated mar Quality of Sleep: Sleep quality ratings showed virtually ginal improvement in this critical outcome. The quality of evidence and therefore the quality of evidence was downgraded for im was downgraded to low due to heterogeneity and the risk of pub precision. It was downgraded further for the high degree of lication bias since all these studies were funded by industry. Ad heterogeneity across studies (I2 = 96%), and due to the risk of ditional studies which assessed subjective sleep quality found no publication bias since all these studies were funded by indus difference between ramelteon and placebo groups. Subjective sleep latency from these investiga falling well below the clinically signifcance threshold for ob tions was comparable to objective latencies with mean differ jective sleep effciency of 5%. This was likewise cally signifcant difference between ramelteon 8 mg and pla the case for sub group analysis of subjects with primary sleep cebo (+0. Substantial het Remarks: this recommendation is based on trials of 3 mg erogeneity across studies was noted for multiple outcomes. The data were also downgraded for imprecision, due to the rel Summary atively large confdence intervals, which cross the clinical sig nifcance thresholds for multiple outcomes. The quality of evi quality of evidence for both dosages was low due to poten dence for individual outcomes ranged from moderate to very tial publication bias and imprecision. The evidence suggests low; therefore, the overall quality of evidence was very low. The overall evidence was HarmS: Meta analytic data on adverse effects showed a rela graded as weakly in favor of doxepin’s effcacy in improving tively low frequency of adverse effects overall and none which sleep maintenance. Roth and colleagues reported on next day re Meta analysis of side effects included headache, diarrhea, sidual pharmacological effects of ramelteon in an older adult somnolence and upper respiratory infection at 3 mg, and head population. Results suggest mild recall, subjective alertness, and concentration showed no sig increase in somnolence at 6 mg.
Only a few case reports recommended because other therapies are more cost effective order 100 mcg catapres with visa heart attack zippo. In milder disease buy cheap catapres 100mcg line high blood pressure medication and lemon juice, treatment includes addressing the un organ specic autoimmune diseases derlying hyperthyroidism discount 100mcg catapres with amex blood pressure medication low blood pressure, and symptomatic care generic 100mcg catapres otc hypertension 8 weeks pregnant. Additionally, B endorsed by the International Consensus Report and the cell depletion with rituximab is emerging as an alternative, American Society of Hematology 2011 evidence based especially in severe disease, because it efciently decreases 158,159 guidelines. Multispecialty management, including endocri and are therefore without randomized studies. Current guidelines recommend a corticosteroid as the discussed, data are limited to open label or retrospective studies rst line treatment, with the addition of an immunosuppressive and case reports. For the most part, the efcacy of immunoglob agent in corticosteroid resistant cases or for corticosteroid ulin therapy in patients with organ specic autoimmune disease sparing effects. Newer biologics are also being considered, 259,260 or various forms of autoimmune vasculitides is limited, and depending on the type of autoimmune uveitis. Importantly, new biologic therapies have emerged autoimmune posterior uveitis that frequently requires immuno recently as better alternatives or even as primary therapies for suppressive therapy. Autoimmune hepatitis is responsiveness, airow limitation, respiratory symptoms, and typically treated with a corticosteroid and azathioprine or another disease chronicity. Atopy is the strongest identiable predisposing immunosuppressive agent, such as rituximab, in refractory dis factor for developing asthma. Patients who showed normalization of liver enzymes, undetectable with these symptoms are occasionally found to have antibody 267 274 circulating immune complexes, and improvement in periportal deciency. Insome patients withimmuneabnormalities and mononuclear cell inltrates after treatment. This, in turn, may decrease 274,275 variety of other available second line immunosuppressive agents the symptoms and morbidity of asthma. Inammatory bowel deciency and not of asthma, although the benet of this comorbid diseases are chronic inammatory disorders involving the tissues diagnosis can be substantive. Multiple open label trials have examined the effects of late mofetil or omalizumab have failed. However, small showed no signicant effect of immunoglobulin therapy numbers of patients have severe resistant disease despite 283,284 in asthma, while the third reported a signicant receiving second line therapies. In addition, these patients can corticosteroid sparing effect in a subgroup that required relatively develop unacceptable adverse events from therapy. A similar trend was seen in the allergic inamma high doses of systemic corticosteroids may be of interest. Urticaria Dosing in each patient varied from 300 mg/kg to 2 g/kg, and Chronic urticaria is a disorder that is often difcult to treat, duration ranged from 6 to 39 months. Time to response seen although advances in the understanding of the underlying was 3 6 months. Signicant decreases in serum IgE and mechanisms have provided new insights and therapeutic ratio eosinophils were seen at the 3 month time point, and the 304 nale. An autoimmune process is implicated in about one third of decrease in serum IgE persisted after discontinuation. Slight improvement in skin disease was observed in 6 trial; one third of the enrolled patients experienced remission, patients; no improvement, in 2 patients; and worsening, in 1 another third experienced some benet, and the rest did not patient. The cause of provided conicting results, and the majority of successful reports illness remains unknown but several clinical, laboratory, and were case series. Long term benets following discontinuation of may be associated with even better outcomes, but these data 314 treatment are conicting, and additional randomized, placebo have been challenged. All patients should be given a single 315 controlled studies with longer follow up are needed. Another meta analysis of data from >3400 patients for which no specic chemotherapy exists. Age, duration of illness, immunoglobulin products with high concentrations of specic neutrophil and platelet counts, elevated aspartate aminotrans antibodies to pathogens such as those causing tetanus, ferase and C reactive protein, and hyponatremia have been pro 322 325 rabies, and diphtheria have been made available in the United posed to predict resistance to treatment. A larger scale study in 3493 infants receiving not responding to initial dosing within 48 72 hours (ie, when antibiotics for the treatment of sepsis did not show differences in neutrophil counts, C reactive protein, and N terminal of the mortality or major disability at 2 years between patients who prohormone brain natriuretic peptide, which are independent 343 received immunoglobulins and those who received placebo. Immunoprophylaxis with 5 332 coccal disease in newborns, streptococcal toxic shock/invasive monthly doses of palivizumab is an effective intervention that 333 337 338 streptococcal syndromes, postoperative sepsis, trauma has been reported to reduce hospitalization by 39 82% among 339 340 351 associated sepsis, and neonatal sepsis. Category Ib evidence exists to support the retrospective study, in 9 of 14 patients with refractory C. Those probably benecial in the treatment of neonatal sepsis (Ia), but not studies were of relatively small sample size and used different in prophylaxis of infection. It is thought to result from 369,370,373,374 371 were common and treatment failures did occur, immunologic destruction of myelin or Schwann cells within the but the latter approach was associated with long term eradication peripheral nervous system. None of the treatments signicantly reduced mor tients with autonomic instability. Nonetheless, a randomized, placebo controlled 414 remain unclear, although genetic factors may play a role. A retrospective chart review of data from 53 patients 416 418 conduction block or Medical Research Council scores. Limited but moderate to high quality ev now the recommended therapy for this neurologic disease. A similar response and lack of serious and other centralnervous system syndromes) that is associated with adverse events have been reported in additional case reports and autoantibodies against the astrocyte water channel called aqua 443 445 461 uncontrolled trials. It may thus be used as an alternative prine, mycophenolate mofetil, or rituximab, based on retrospective treatment in patients who fail to respond or do not tolerate other and prospective open label studies only. When larger doses were tried (1 g/kg/d for Intractable childhood epilepsy 2 days at 4 week intervals), 65% (of 25 patients) had no There is some evidence that an aberrant immune response is 449 exacerbations in 6 months versus 35% of the control group. However, event suggesting demyelinating disease signicantly lowered the there are 2 randomized placebo controlled trials that have been 450 prevalence of a second attack and reduced disease activity.
Increases in repeat Magnetic resonance scanning may reveal both cerebral length are likely during oogenesis and purchase catapres 100mcg without prescription prehypertension journal, consequently order 100mcg catapres blood pressure 9058, a and cerebellar cortical atrophy generic 100mcg catapres with visa heart attack 50 years. The most characteristic daughter may have passed on a ‘full’ mutation to buy catapres 100mcg with mastercard arrhythmia with normal ekg one of her finding, however, is the ‘middle cerebellar peduncle’ sign: children, who, in turn, might express the fragile X syn on T1 weighted scans these peduncles show decreased sig drome with mental retardation. This sign, although severe; this is probably due to the fact that females who do not universally present, becomes more likely later in the have the ‘pre mutation’ also have a normal gene on the course of the disease. Treatment Etiology the general treatment of dementia is described in Section As might be gathered from the name of this syndrome, 5. The mutation in question Senile chorea is a rare disorder characterized by the gradual involves an increase in the number of these repeats beyond onset of chorea late in life, in the absence of a family history. Increases into the range of 55 to 200 repeats have traditionally been termed a ‘premutation’ Clinical features whereas increases above 200 repeats have been considered to constitute a ‘full’ mutation. Individuals with full muta the onset is insidious and generally in the seventh decade. Until recently, it had been chorea, generally involving the upper extremities or the thought that those with a ‘premutation’ were merely carri face (Delwaide and Desseilles 1977; Klawans and Barr ers who remained free of any symptoms. Indeed, among men with the premutation, the penetrance progressively rises from roughly 20 percent Course in those from 50 to 59 years of age up to 75 percent in those aged 80 or above (Jacquemont et al. Intranuclear inclusions are found in both astrocytes Microscopically, neuronal loss is noted in the caudate. The autosomal dominant pattern displayed, disease, the anti phospholipid syndrome, tardive dyskine and the course, however, are quite distinctive. Edentulousness should also be Treatment considered, as it may be accompanied by orofacial move ments that resemble chorea (Koller 1983). There are no established treatments; in case reports, lev odopa was beneficial (Asmus et al. Consideration may also be given to treatment with antipsychotics, as dis Treatment cussed for Huntington’s disease. In those rare cases in which the chorea becomes severe enough to interfere with functioning, consideration 8. The unwieldy name of this disorder is derived from the main structures that show pathological changes, namely 8. There is no dementia (Behan and the disorders are considered separately in this text. Course Clinical features After a period in which the chorea becomes established, the onset is gradual, regardless of whether it is early or late, although in most cases there is no change over time, one and the clinical features are strongly influenced by the age may see in a minority either a mild progression (Kleiner of onset. Magnetic reso 2005) and astrocytosis has been noted in the globus pal nance scanning may reveal atrophy of the superior cerebel lidus, thalamus, hypothalamus, and periaqueductal gray lar peduncle and, on T2 weighted scans, increased signal (Kleiner Fisman et al. Other symptoms include seizures, a Kayser–Fleischer ring, Etiology hepatitis, and anemia. Chorea may involve either the notypic heterogeneity both within and between families upper or the lower extremities. Neuronal loss is found not only in the mic or irregular; at times, one may see the classic ‘wing namesakes of this disorder (the dentate nuclei, red nucleus, beating’ tremor in which a rhythmic elevation and lowering globus pallidus, and subthalamic nucleus) but also in the of the upper extremities, combined with flexion at the striatum, substantia nigra, inferior olives, thalamus, and elbows, gives an overall appearance of a frightened bird cerebral cortex (Becher et al. In surviving neurons, Personality change is generally characterized by lability, intranuclear inclusions, formed of abnormal atrophin 1, disinhibition, and, at times, bizarre behavior (Bridgman may be found. Psychosis is characterized by hallucinations and delu Differential diagnosis sions (Dening and Berrios 1989; Gysin and Cooke 1950) and may be quite bizarre, with Schneiderian first rank In adults, the differential is influenced by the nature of the symptoms. Indeed, one of Wilson’s (1912) patients heard movement disorder: when chorea is prominent, consider ‘God and the devil talking to him simultaneously’ and said ation is given to Huntington’s disease, and when ataxia is that he was ‘influenced, willed or hypnotized to do certain foremost, spinocerebellar ataxia enters the differential. In one case (Davis and Borde 1993), stuporous Genetic testing is often required to make the differential. Seizures may occur but are rare, occurring in about 5 percent of patients (Dening et al. Treatment the Kayser–Fleischer ring is a golden brown discol oration of the corneal limbus, visible either on slit There is no specific treatment. With significant hepatic dysfunction, hepatic encephalopa Wilson’s disease, also known as hepatolenticular degenera thy may occur (Starosta Rubinstein et al. Although relatively rare, occurring in perhaps both low, but the serum free copper is elevated, as is the 1–2/100 000 population, it is an important diagnosis given 24 hour urinary copper; in a small minority, the ceruloplas its eminent treatability. Magnetic resonance scanning may reveal charac Clinical features teristic findings (Sinha et al. On T2 weighted scans, increased signal intensity may be seen in the head of the the clinical features of Wilson’s disease have been caudate and lateral aspect of the putamen, with decreased described in several large series (Dening and Berrios 1989; signal intensity in the globus pallidus; in the midbrain, Machado et al. The onset is typi sign, with increased signal intensity in the tegmentum and cally between late childhood and early adulthood, although decreased signal intensity in the red nuclei. Given the large number of possible mutations, genetic Treatment is aimed at reducing the total copper burden. Several methods are available, including chelation with penicillamine, trientine, or tetrathiomolybdate; treatment with zinc; and limiting copper intake. Course Chelation has traditionally been accomplished with penicillamine; however, this agent has multiple, severe Although there may be partial, temporary remissions, the side effects and also, in a significant minority of patients, overall course is one of progression, with death within will either exacerbate pre existing symptoms (Starosta 5–10 years. Etiology Consequently, attention has shifted to two other chelating agents, namely trientine and tetrathiomolybdate; of these, Wilson’s disease is essentially a disease of copper metabo tetrathiomolybdate is more effective than trientine lism. In Wilson’s disease biliary Zinc induces intestinal metallothionein, which in turn excretion is deficient and copper accumulates, first in the binds copper and prevents its absorption; it is less effective liver, where it causes hepatitis, and then, once it spills over than chelating agents. Zinc is given in a dose of 50 mg three into the systemic circulation, in the brain, where copper times daily, before meals (Hoogenraad et al.
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