A. Cronos. University of Georgia.
Eg Oral or manual automatisms Seizure location: Frontal: focal tonic or clonic motor activity discount avana online amex erectile dysfunction pump how to use, posturing purchase avana 200mg visa impotence questions, prominent motor automatisms but no orofacial or experiential automatisms Central: focal clonic seizures with preservation of awareness Temporal: experiential buy avana with visa erectile dysfunction in young, gustatory or olfactory hallucination. Takes about 5 days to stabilise a change in dose Monitor drug level: for other than phenytoin, this is to check compliance. Especially Valproate Rarely bone marrow suppression Pregnancy: Epilepsy often worsens during pregnancy Plasma concentration of drugs falls due to pharmokinetic changes and compliance Teratogenic: 3% risk of malformation on 1 drug (also, epilepsy itself can be teratogenic -? If no response give clonazepam 1 4 mg iv Phenytoin 50 mg/min iv (25 mg/min in cardiovascular disease), usual adult dose 1250 mg in 100 mls saline over no more than 20 minutes. Low grade have a tendency to become high grade and are also hard to cure due to their infiltrative nature Grossly: Infiltrative. Include: Gangliocytomas Gangligliomas: better prognosis Cerebral neuroblastoma: Rare, in children. Resemble peripheral neuroblastomas small round blue-cell tumours Oligodendroglioma: 5 15% of gliomas Radiographically, well demarcated and often show calcification (key differential) Grossly: gelatinous masses +/- cysts and/or haemorrhage Ependymoma: usually in fourth ventricle outflow obstruction. Significant histological features: true rosette and perivascular pseudo-rosette. Cauliflower type projections into ventricular lumen Neuro-sensory 137 Pineal Neoplasma: Intrinsic tumours are Pineocytoma and Pineoblastoma. Differential: lymphoma or metastatic cancer Hemangioblastoma: Highly vascularised, cystic tumours, mainly in the cerebellar hemispheres Craniopharyngioma: Arise from the epithelium of Rathkes pouch part of the embryonic nasopharynx the forms the anterior lobe of the pituitary. Occurs exclusively in the cerebellum, mainly in children, mainly as a midline mass. Usually B-cell lymphomas Pituitary Pituitary Adenoma: benign neoplasm in anterior lobe of the pituitary Present with either mass effects (including on the rest of the pituitary) and excess hormone secretion At any age or sex, but most common in men aged 20 50 Classified on the basis of hormones they secrete by immunocytochemistry. Poor correlation between acidophils, basophils and chromophobes and the hormones secreted Carcinomas are rare. Diagnosis requires gross brain invasion or discontinuous spread Metastatic Brain Tumours 20% have intracranial mets at autopsy In 15% primary organ not found Surgery for solitary met if primary site controlled or for symptomatic control or for diagnosis Most mets are carcinomas. Also haemorrhage (epidural haematoma) or epidural abscess Key questions: where is the lesion, what is the lesion (eg weight loss, past cancer history cancer) th th 138 4 and 5 Year Notes If there is a clear level below which these is sensory abnormality spinal chord If both legs then spinal chord (usually). Types: post-coital, ergotamine misuse Cluster headache: clusters of extreme, recurrent non-throbbing deep pain in and around an eye, spreading onto the face. Acyclovir 10 mg/kg 8 hourly (> 20% caused by Herpes Simplex) Temporal arteritis/Giant Cell Arteritis: See Giant Cell arteritis/Temporal Arteritis, page 282 Other th Trigeminal neuralgia: momentary severe shooting pain in one division of 5 nerve due to touching, chewing or speaking. Responds to Tegretol Locked-in syndrome: pontine infarction quadriplegia and variable loss of all reflex and horizontal eye movements. Vertical eye movement or eyelid movement may be the only means of communication Cerebellar infarction/haemorrhage: vertigo, headache, and abnormalities of eye movements (eg saccadic deficits). Also smoking incidence of cataract Physical Examination Trying to work out where is the problem: refractive, obstruction of light through the transparent tissues of the eye, or neural problem Measure Visual Acuity: One at a time. Wear glasses Snellens Chart: Distance of chart (normally 6 metres)/Distance they could read. Swinging light test: Alternate light from one eye to the other, swapping it quickly. If this shows a problem, test for an efferent pupillary defect with the near reflex test Internal inspection with ophthalmoscope: Get patient to look at target a long way away: relaxes accommodation. Dim the light dilation Dilate pupil with Madriasil (not atropine, T too long) Check for: Red reflex defects: eg cataract, intra-ocular blood Reduced transparency (compare two eyes) Cup and disk: Disk is 15 degrees nasal to fixation. To examine macula, get patient to look directly at the light Check disk for distinct margins and symmetry Physiology cup is blood vessels in the centre of the nerve not nerves Normal cup to disc ratio < 1/3 (but lots of variation). Check its the same in both eyes Large and/or deep cup sign of glaucoma (vessels diving into the cup) especially if eyes different. Veins: thicker, straight Amount of melanin in choroid layer variation in pigmentation of retina. Deep green patch = coronial nevus (benign) Clinical usefulness depends on good instrument, good technique, knowledge of normal anatomy and normal variations Anatomy Fovea: dip in middle of retina. Only photoreceptors, neural connections heaped up around it Macula: ill defined area around fovea Uvea = iris, ciliary body and choroid. Iritis = anterior uveitis Reflex pathway: retina optic nerve optic chiasm optic tract lateral geniculate nuclei rd optic radiation and brain stem Edinger Westphal nucleus 3 nerve Loss of Vision Is it bilateral or unilateral? Atrophy of photoreceptors, loss of outer nuclear layer Wet (exudative) macular degeneration: May be due to Choroidal Neovascular Membranes, unilateral with onset over several weeks. Straight lines appear wavy refer as laser treatment slows progression Symptoms: blurred central vision, distortion of straight lines Eye Trauma Mechanical: blunt/sharp, superficial/penetrating Chemical: alkali the worst. Local anaesthetic then irrigate for 30 minutes (less time already irrigated before presentation). Always evert upper key lid to look for further foreign bodies Corneal abrasions: Very painful and photophobic. Pain is due to trigeminal neuralgia Iritis: Frontal headache, photophobia, not watering. May be white cells at bottom of cornea Usually autoimmune: Ankylosing Spondylitis, Crohns. Treat with steroids, and dilating drops to keep iris mobile Rarely infective (eg Tb) Episcleritis: = Localised inflammation of sclera. Eg Haemolytic glaucoma, lens protein glaucoma Secondary closed angle glaucoma: Can be due to neovascularisation zipping up the angle, secondary to ischaemic eyes (eg diabetes, central retinal vein occlusion) Eye Infections Viral Infections: Adenovirus types 8 (epidemic) and 3 and 7 (sporadic). Never give steroids: worse infection permanent damage Bacterial: Usually puss. Always bilateral: Standard bacterial conjunctivitis: treatment chloremphenicol eye drops Trachoma: Due to Chlamydia. Initially the conjunctival epithelium is infected scarring of the eye lid abrasion of cornea over years get panus (fibrovascular layer) over the cornea Gonorrhoea: pre-auricular nodes th th 144 4 and 5 Year Notes Optic Nerve Lesions Optic atrophy: pallor of the optic disk, and damage to the retinal nerve layer, optic nerve or tract leading to visual acuity or field loss. Worsens over hours to days, may lose sight completely, gradually improves over 4 6 weeks. Due to occlusion of the arterioles to the optic nerve head Compressive Optic neuropathy: insidious loss of central vision in one eye, especially colour. Many variants nd 2 most common site of melanoma after the skin Prognosis depends on cell type (Spindle A, Spindle B, Epithelioid or Mixed) and Stage. Hereditary neuroblastoma = inherit one defective gene from parent, with other allele in one cell undergoing spontaneous mutation. From occurrence in eye to spreading down the optic track is ~ 6 months Survivors have a 20% chance of developing malignant tumours at 10 years: osteosarcoma or rhabdomyosarcoma Prognosis: 90% 5-year survival (less if optic nerve invasion). Cover good eye and see if corneal reflection shifts over the pupil of the bad eye Check for equal schlera on either side of iris. Wide bridge of nose may give pseudo squint Can have squint without amblyopia as long as brain alternates which eye it looks through. If preference for one eye, then amblyopia Amblyopia = Lazy eye Affects 2 to 3 per 100 children. Can only occur in childhood while visual pathway still developing Usually unilateral: maybe bilateral if bad astigmatism or hypermetropia. The crossed eye is turned off to avoid double vision Unequal focus (refractive error). One eye is more near/far sighted or astigmatic Visual obstruction: eg Cataract Also caused by ocular motor defects Treatment: force the use of the weak eye by covering the good one (for weeks or months), plus correcting refractive errors with glasses Refractive errors Myopia Neuro-sensory 147 Hypermetropia: if equal and severe then squint due to accommodation Stigmatism Anisometropia: difference between two eyes (especially if one normal and other long sighted) accommodation just makes normal eye go out of focus Other Congenital cataract Can be autosomal dominant Check for red reflex within 6 weeks May be uni or bilateral, part of a syndrome or isolated Congenital epiphora Watery eye.
We set out to determine whether significant differences in type 1 diabetes management outcomes exist between low-carbohydrate diets and higher-carbohydrate com- parators buy discount avana online shakeology erectile dysfunction. We also investigated whether primary nutrition studies of low-carbohydrate diets have different levels of effect depending on the degree of carbohydrate restriction purchase discount avana on line erectile dysfunction 21 years old. Citations and abstracts of all papers retrieved from these searches were downloaded into Endnote reference management software (Endnote X7 purchase avana 100 mg without prescription erectile dysfunction treatment electrical. Dis- agreements were resolved by consensus through adjudication with a third independent researcher. Studies included in the review had to be primary research studies of interventions or exposures including controlled trials, cohort-type studies and case-control trials. In the case of multiple reports from the same study, we used the most complete or recently reported data. For studies investigating different levels of car- bohydrate restriction, the lowest reported or prescribed level of dietary carbohydrate intake was considered the intervention and the highest level was considered the comparator. Risk of bias assessments were conducted for methodological quality of each included study using the critical appraisal tool most appropriate for its design. For randomised controlled tri- als, the Cochrane Collaborations Risk of Bias tool for randomised studies was used . This assesses bias as low risk, high risk or unclear risk across seven domains. For specificity, we separated blinding of participants and blinding of personnel into two separate domains. For pre-post intervention studies, the National Institute of Healths quality assessment tool for before-after studies with no control group was used . This tool evaluates potential flaws in study methods or implementation using twelve closed questions. The ratings (yes/no/other) on the different items are then used by reviewers to assess overall risk of bias as good (low risk of bias), fair (susceptible to some bias) or poor (significant risk of bias). For case-series and case-reports, we used the critical appraisal checklists from the Joanna Briggs Institute . These checklists are a series of 8 to 10 closed questions (yes/ no/unclear/not applicable) which help form an overall appraisal for each study assessed. For standardisation, we used this assess- ment to classify studies as low risk, high risk or unclear risk of bias. If a decision could not be reached on bias assessments, an additional inves- tigator made a decision. This approach specifies four levels of quality; high, moderate, low and very low. Data synthesis and analysis To summarise the effects of low-carbohydrate diets on type 1 diabetes outcomes in controlled trials, we extracted mean outcome values for the intervention and control groups at baseline and follow-up. For other studies with only an intervention group or for trials where only one participant group was relevant to our study, we extracted mean outcome values for the inter- vention or observed group at baseline and follow-up. Standard deviations and/or standard errors, sample sizes, follow-up time and published levels of significance (i. If no P-value was published and raw outcome data were available, the P-value was calculated using the R Statistical Language (R version 3. A meta-analysis was not able to be conducted due to obvious heterogeneity and we used text and tabular format to summarise the outcome data of all low-carbohydrate diets. Where no specific pre- scription was available and compliance to the intervention was reported, studies were classified according to the reported dietary intake data of participants. Results Literature search results The database search identified 2724 possibly relevant studies that were screened by titles and abstracts (Fig 1). A further 2645 records were excluded with 79 full-text articles subsequently assessed for eligibility. Eleven additional records were identified through searching the reference lists of included studies. The duration of exposure to a low-carbohydrate diet ranged from two weeks to five years. The two controlled trials com- pared a low-carbohydrate diet (intervention) to a higher-carbohydrate diet (comparator) using either a crossover  or parallel  design. All other studies compared a low-carbohy- drate diet (intervention) to baseline or usual diet (comparator). Study Details Populationa Interventionb Comparatorb Insulin Protocolc Outcomed Anderson 1991 . Information was provided on the amount of insulin likely needed to match 5075 g of carbohydrate per day. Group education course (whole day pen device that enables delivery of followed by 4 x 3 h sessions over 4 wk). Phone calls and injections, and no more than 9 h office visits used to tailor individual between evening basal dosages and regimen of each patient. Diabetes duration (xD) is given as the mean (to nearest whole year) and range of n participants. Not controlledResearchers did not make an acceptable attempt to control for the effect of insulin and/or only observed insulin protocols (i. All outcomes Results for all primary and secondary outcomes are presented in Table 2. Effect sizes were not calculated because raw outcome data were not available for all studies and most outcomes were inconsistently reported. Results for our primary out- come (HbA1c) were available from eight of nine studies reviewed. HbA1c Eight studies investigated the effect of a low-carbohydrate diet on glycaemic control using HbA1c [8, 10, 2024, 26] (Table 2). One study  reported a follow-up value for HbA1c but did not provide baseline data so was not included for this outcome. Of the two studies that com- pared a low-carbohydrate diet to a higher-carbohydrate diet [10, 20], neither showed a signifi- cant difference between groups at follow-up. Effect of intervention and comparator diets on type 1 diabetes management outcomes (primary and secondary). This was converted to 730 via simple calculation (2 x 365) and may not be an accurate representation of a full year. Levels of significance could not be calculated or obtained in three studies due to inadequate sample size  and lack of raw participant data [8, 24]. Risk of bias in one  was rated low in four domains and unclear in four domains. The other controlled trial  was rated low in seven domains and unclear in one domain. Of the pre-post intervention studies (S9S13 Tables), two were rated as fair quality [8, 21] and two were rated as poor.
Vitamin B12 deciency It is also used in maintaining skin order avana 100mg on-line erectile dysfunction doctor in mumbai, especially in sun- exposed areas discount 50 mg avana fast delivery erectile dysfunction doctors tucson az. Vitamin C deciency Clinical features Pellagra is due to lack of nicotinic acid cheap 200 mg avana mastercard erectile dysfunction doctors in sri lanka, it often occurs Denition as part of a more general nutritional deciency. Pellagra Vitamin C deciency causes scurvy, which was rst de- presents with dermatitis, diarrhoea and dementia. Genetic factors Occurs in the poor, pregnant or those on a peculiar may be involved in predisposing to the development of diet. Vitamin C brillogenesis and amyloidosis: (ascorbic acid) is found in citrus fruits, potatoes, green r Geneticmutationsresultinginproteinswithincreased vegetables and fortied fruit drinks. The precursor protein, the tissue distribution and the amount of amyloid deposited affect the clinical presen- Management tation. Deposition in the coronary arteries can lead Vitamin K deciency to ischaemic heart disease. This form of deposition is particularly seen many of which circulate as constituents of plasma. At least 21 different protein precursors of amyloid brils are now known (see Table 13. Investigations Besides systemic amyloid deposition, organ specic Where possible biopsy and histology is used to con- amyloid may occur in the skin or heart and most no- rm clinical suspicion. Familial amyloidosis Various Autosomal dominant inherited, including familial transthyretin-associated amyloidosis. Denition Management Theporphyriasaregeneticoracquireddecienciesinthe Therapy is aimed at the underlying cause where possi- activity of enzymes in the heme biosynthetic pathway. Differing manifestations such as renal failure require Aetiology/pathophysiology support. Inthehereditaryamyloidoseswheretheprecur- Heme is synthesised from succinyl Co A and glycine (see sor protein is produced by the liver, liver transplantation Fig 13. Enzyme deciencies result in increases in metabolic ing folic acid as a co-factor. It presents in adult life with muscle cell proliferation, activation of clotting factors abdominal pain, vomiting and constipation, polyneu- and a pro-aggregatory effect on platelets. Acuteepisodes also be a pro-inammatory effect by upregulating neu- areprecipitatedbyalcoholanddrugs. Urinary levels of Clinical features uroporphyrinogen (the substrate for the decient en- 1 Homocystinuria presents in childhood with devel- zyme)areraised. Other features include a Marfans r Congenital erythropoietic porphyria is inherited in likesyndrome,ocularabnormalities,thromboembolic an autosomal recessive fashion. The photosensitivity that ischaemicheartdiseaseandstroke,althoughtheeffects results can be controlled with -carotene by an un- are less strong than those of, e. It is more strongly asso- ciated with an increased risk of pulmonary embolism and deep vein thrombosis. Hyperhomocysteinaemia Investigations Denition Homocysteine levels can be measured (normal be- Raised levels of homocysteine (an amino acid formed ing 515 mol/L, moderate 1530 mol/L and severe by the conversion of methionine to cysteine) have been >100 mol/L). A methionine challenge can be given to associated with premature atherosclerotic disease. Increased folic acid intake reduces homocysteine lev- 2 Moderate homocysteinaemia occurs in approximately els. Causes include the following: B6 and vitamin B12 is advocated by some for those with r Genetic defects in enzymes involved in homocys- premature cardiovascular disease and recurrent venous teine metabolism. There is as yet no clear evidence that monly (approximately 10% of the population) is supplements should be given to all those with ischaemic avariant of methylene tetrahydrofolate reductase, heart disease, although several trials are in progress. G enetic syndrom es 1 Patterns of inheritance, 516 Incidence Patterns of inheritance Rises with increasing maternal age (1 in 3000 when mother is less than 30 years to 1 in 300 when mother Autosomal dominant:Mendelian pattern of inheritance is 3540 years and 1 in 30 in women above 45 years). Be- where the presence of a single abnormal allele is able cause of the high birth rate in mothers below 35, half to produce the disease. There may be reduced expres- of all Down syndrome children are born to mothers sion of the condition if the condition does not have full below 35. Autosomal recessive: Mendelian pattern in which Age both genes must be defective to produce the clinical phe- Congenital. M = F There is no male-to-male transmission, daughters of an affected male will be obligate carriers. In X linked domi- Geography nant conditions, females may also demonstrate the clin- All ethnic communities. The additional chromosome 21 is usually follow normal Mendelian patterns of inheritance. In this (94% of cases) the result of non-disjunction of chromo- set of conditions males and females may be affected, but some 21 during the formation of the maternal ovum. In about 3% of cases there is mosaicism with some cells demonstrating a normal Down syndrome karyotype. Denition Pathophysiology Down syndrome is the clinical condition usually result- The Alzheimers disease seen with Down syndrome is ing from a trisomy of chromosome 21 rst described by thought to be due to the presence of three copies of the Langdon Down in 1865. Chronic granulomatous Recessive Cytochrome Neutrophils can phagocytose material, but are unable disease b245 to generate respiratory burst and hence kill bacteria. Duchenne muscular dystrophy Recessive Dystrophin Progressive proximal muscle weakness with calf psuedohypertrophy. Causes progressive intellectual deterioration, loss of purposeful use of hands and jerky truncal ataxia. Leber optic atrophy Multiple loci Sudden onset adult blindness, cardiomyopathy, cardiac conduction defects. Short into adult life, but by 40 almost all have Alzheimers middle phalynx of little nger, single horizontal pal- disease. Klinefelter syndrome r Congenitalheartdiseasein30%,mostcommonlyatri- Denition oventricular septal defects. Tracheo-oesophageal stula, duodenal atresia, annu- lar pancreas, Hirschsprungs disease. Denitive diagnosis is made by chori- more X chromosomes the more severe the phenotype. All appear normal until puberty when hypogo- tions for testing include maternal age and a Down nadism becomes prominent. As there is nor- mally only one copy of the X chromosome, females Incidence suffer from X linked recessive conditions such as 1in5000 live births. O verdose, poisoning 1 and addiction Alcohol and drugs of abuse, 521 Overdose and poisoning, 526 r Alcohol dependence is dened as a maladaptive pat- Alcohol and drugs of abuse tern of use associated with tolerance and withdrawal syndrome despite signicant physical and psycholog- Alcohol abuse and dependence ical problems. Patients often exhibit a stereotyped drinking pattern with alcohol consumption taking Denition preference over other activities. Regular or binge consumption of alcohol sufcient to A history of alcohol consumption should be taken from cause physical, neuropsychiatric or social damage.