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The prognosis is guarded, with about 10% mortality, 30% remaining permanently paraplegic and 60% showing moderate to good recovery. It is estimated that neurological involvement will occur in about 7% of patients 10-25 years after primary infection, if primary syphilis is untreated. However despite the frequency of primary syphilis in Africa, neurosyphilis remains uncommon in many countries there. The reason for this has been ascribed to the continued widespread use of antibiotics inadvertently treating early infection or altering the natural history of clinical disease. However tabes dorsalis remains a distinctly uncommon clinical presentation in Africa. The serological prevalence rates are 3-6% in some of the worst afected communities. However this occurs in only 1-5% of persons infected with the virus with the majority being asymptomatic carriers. It occurs predominantly in females in their late teens or early 20s in Africa but may afect older women in their 30s and 40s in high income countries. The optic neuritis typically precedes the transverse myelitis but can also occur concurrently with it. The relapses are severe, occurring usually within 6 months of the frst and subsequent episodes. Long term prophylaxis with a combination of steroids and azathioprine or other forms of immunosuppression has been tried but the response is variable. Residual disability after an attack is usual with many patients becoming wheelchair or bed bound within 1 to 2 years of onset. The main spinal fndings are those of a myelopathy with brisk knee refexes and up going plantars. Tese typically occur in combination with signs of neuropathy, including absent ankle jerks and loss of peripheral sensation including joint position sense. Malabsorption may be caused either by a lack of intrinsic factor which seems to be quite uncommon in Africa and also by diseases of the terminal ileum. Diagnosis Diagnosis is by fnding the typical neurological fndings in combination with evidence of megaloblastic anaemia i. The dose is 1 mg on alternate days for a total of fve injections followed by 1 mg injections every 3 months for life. If intramuscular injections are not available then B-12 can sometimes be given orally, in a dose of 1 mg po daily. William Howlett Neurology in Africa 245 Chapter 10 paraplegia non traumatiC this may provide adequate replacement particularly in cases secondary to nutritional or dietary defciency. Tese disorders represent a distinct group of community based myeloneuropathies which result in paraplegia.

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Ursodiol for the long-term treatment of progression of liver stiffness as determined by Fibroscan in patients with primary biliary cirrhosis. The Canadian Multicenter Double-blind Randomized Controlled Trial Determination of reliability criteria for liver stiffness evaluation by of ursodeoxycholic acid in primary biliary cirrhosis. A two and histologic features in asymptomatic and symptomatic primary biliary year controlled trial examining the effectiveness of ursodeoxycholic acid in cirrhosis. A randomized, double-blind, placebo-controlled trial of ursodeoxy- primary biliary cirrhosis. A randomized trial in primary biliary cirrhosis mofetil in patients with features of severe primary biliary cirrhosis not comparing ursodeoxycholic acid in daily doses of either 10 mg/kg or 20 Journal of Hepatology 2017 vol. Randomised controlled trials of [143] Hosonuma K, Sato K, Yamazaki Y, Yanagisawa M, Hashizume H, Horiguchi ursodeoxycholic-acid therapy for primary biliary cirrhosis: a meta-analy- N, et al. Ursodeoxycholic acid alkaline phosphatase in primary biliary cirrhosis patients with incomplete for primary biliary cirrhosis. Drug insight: Mechanisms and sites of action of ursodeoxycholic primary biliary cirrhosis. Effect of ursodeoxycholic acid on bile acid profiles and effective adjunctive therapy in the treatment of primary biliary cirrhosis: A intestinal detoxification machinery in primary biliary cirrhosis and health. Efficacy of ursodeoxycholic acid in treating intrahepatic cholestasis of [149] Yano K, Kato H, Morita S, Takahara O, Ishibashi H, Furukawa R. Investiga- analysis of randomized clinical trials using Bayesian approach as sensitivity tion into the efficacy of bezafibrate against primary biliary cirrhosis, with analyses. Combination therapy of Efficacy of obeticholic acid in patients with primary biliary cirrhosis and fenofibrate and ursodeoxycholic acid in patients with primary biliary inadequate response to ursodeoxycholic acid. Good A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. Pharmacokinetics and pharmacodynamic action of budesonide in early- Rifampicin in the treatment of severe intrahepatic cholestasis of preg- and late-stage primary biliary cirrhosis. Overlap syndromes: the International Autoimmune Hepatitis cirrhosis: results of a prospective double-blind trial. Oral [159] Lindgren S, Glaumann H, Almer S, Bergquist A, Bjornsson E, Broome U, et al. Development of regimens for primary biliary cirrhosis: a systematic review and network autoimmune hepatitis in patients with typical primary biliary cirrhosis. Network meta- [161] Efe C, Ozaslan E, Heurgue-Berlot A, Kav T, Masi C, Purnak T, et al.

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This process should include an interdisciplinary tinued evolution of best practices for nutritional as- team approach and organizational standards of care sessment and treatment. Optimizing nutritional sup- with policies and procedures that ensure implementa- port and care of the critically ill and patients with acute tion, continuous assessment, and monitoring of the nu- and chronic respiratory disorders will contribute to im- trition care plan. Adult Nutrition Mid-arm muscle area is a better predictor of mortality than body Support Core Curriculum, 2nd edition. Nutritional strategies to attenuate muscle body mass index explains obesity-related health risk. C-reactive protein: the best laboratory indicator and Enteral Nutrition: characteristics recommended for the identifi- available for monitoring disease activity. Cleve Clin J Med 1989; cation and documentation of adult malnutrition (undernutrition). Expression and secretion before and after the implementation of an evidence-based nutri- of procalcitonin and calcitonin gene-related peptide by adherent tional management protocol. Nutritional status classification tion and disease-related malnutrition: a proposal for etiology-based in the Department of Veterans Affairs. J Am Diet Assoc 2001; diagnosis in the clinical practice setting from the International Con- 101(7):786-792. Obesity hypoventilation syndrome: a state-of-the-art Care Med 2002; 30(3):586-590. A comparison of early gastric and Association/National Heart, Lung, and Blood Institute Scientific post-pyloric feeding in critically ill patients: a meta-analysis. Comparison of postpyloric tube feed- sleep apnea: a cardiometabolic risk in obesity and the metabolic ing and gastric tube feeding in intensive care unit patients: a meta- syndrome. Checking gastric residual volumes: a associated with obesity and metabolic syndrome in adults. Diagnosis and manage- volumes as a marker for risk of aspiration in critically ill patients. Obstructive sleep ap- volume and aspiration in critically ill patients receiving gastric feed- noea is independently associated with an increased prevalence of ings. Obesity, obstructive sleep apnoea and bility of early enteral nutrition with immediate or gradual introduc- metabolic syndrome.

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Pearls & Oy-sters: selective postictal aphasia: cerebral language organization in bilingual patients. Maurice Ravel and right-hemisphere musical creativity: influence of disease on his last musical worksfi Global aphasia without hemiparesis: lesion analysis and its mechanism in 11 Korean patients. Comparison of aphasia profiles and language recovery in nonright-handed and matched right-handed patients. Anatomoclinical correlations of the aphasias as defined through computerized tomography: exceptions. Primary lateral sclerosis presenting with isolated progressive pseudobulbar syndrome. Dissociations of language functions in aphasics with speech automatisms (recurring utterances). The new classification of primary progressive aphasia into semantic, logopenic, or nonfluent/agrammatic variants. And all the daughters of Musick shall be brought low: language function in the elderly. Aphasia in border-zone infarcts has a specific initial pattern and good long-term prognosis. Anomalous anatomy of speech-language areas in adults with persistent developmental stuttering. The aprosodias: further functional-anatomical evidence for the organisation of affective language in the right hemisphere. Variability in subcortical aphasia is due to variable sites of cortical hypoperfusion. Action naming in anomic aphasic speakers: effects of instrumentality and name relation. Transcortical sensory aphasia following a left frontal lobe infarction probably due to anomalously represented language areas. The existence of phonatory instability in multiple sclerosis: an acoustic and electroglottographic study. Strokes in the subinsular territory: clinical, topographical, and etiological patterns.

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