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Background: Paroxysmal gluten‐sensitive dyskinesia (PGSD) in border terriers (BTs) results from an immunologic response directed against transglutaminase (TG)2 and gliadin. Recent evidence suggests that PGSD is only one aspect of a range of possible manifestations of gluten sensitivity in the breed. Hypothesis/Objectives: Gluten sensitivity in BTs is a heterogeneous disease process with a diverse clinical spectrum; to characterize the phenotype of PGSD using TG2 and gliadin autoantibodies as diagnostic markers. Animals: One hundred twenty‐eight client‐owned BTs with various disorders. Methods: Prospective study. BTs with paroxysmal episodes and a normal interictal examination were phenotyped using footage of a representative episode and assigned to 3 groups: idiopathic epilepsy (IE), paroxysmal dyskinesia (PD), or other. Owners of each dog completed a questionnaire to obtain information regarding clinical signs. Healthy BTs formed a control group. Serum antibodies against TG2 and AGA were measured in all dogs. Results: One hundred twenty‐eight BTs were enrolled; 45 with PD, 28 with IE, 35 with other conditions, and 20 controls. Three overlapping phenotypes were identified; PD, signs suggestive of gastrointestinal disease, and dermatopathy. AGA‐IgG concentrations were increased in PD, compared with IE (P = 0.012), controls (P < 0.0001) and other (P = 0.018) conditions. Anti‐canine TG2‐IgA concentrations were increased in PD, compared with IE (P < 0.0001), controls (P < 0.0001) and other (P = 0.012) conditions. Serological markers are highly specific for PGSD but lack sensitivity. Conclusions: PGSD appears part of a syndrome of gluten intolerance consisting of episodes of transient dyskinesia, signs suggestive of gastrointestinal disease, and dermatological hypersensitivity. [ABSTRACT FROM AUTHOR]

Background Canine epileptoid cramping syndrome ( CECS) is a paroxysmal movement disorder of Border Terriers ( BTs). These dogs might respond to a gluten-free diet. Objectives The objective of this study was to examine the clinical and serological effect of a gluten-free diet in BTs with CECS. Animals Six client-owned BTs with clinically confirmed CECS. Methods Dogs were prospectively recruited that had at least a 6-month history of CECS based on the observed phenomenology (using video) and had exhibited at least 2 separate episodes on different days. Dogs were tested for anti-transglutaminase 2 ( TG2 IgA) and anti-gliadin ( AGA IgG) antibodies in the serum at presentation, and 3, 6, and 9 months after the introduction of a gluten-free diet. Duodenal biopsies were performed in 1 dog. Results Serum TG2 IgA titers were increased in 6/6 BTs ( P = .006) and AGA IgG titers were increased in 5/6 BTs at presentation compared to those of controls ( P = .018). After 9 months, there was clinical and serological improvement in all BTs with CECS strictly adhering to a gluten-free diet (5/5). One dog had persistently increased antibody titers. This dog scavenged horse manure. On the strict introduction of a gluten-free diet this dog also had an improved clinical and serological response. The diet-associated improvement was reversible in 2 dogs on completion of the study, both of which suffered a relapse of CECS on the re-introduction of gluten. Conclusions Canine epileptoid cramping syndrome in BTs is a gluten-sensitive movement disorder triggered and perpetuated by gluten and thus responsive to a gluten-free diet. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Randomised clinical trial: Gluten may cause depression in subjects with non-coeliac gluten sensitivity: An exploratory randomised clinical study" by S. L. Peters and colleagues in a previous issue.

Aims Immunoglobulin A (IgA) measurement is advocated when case finding for coeliac disease in patients with Type 1 diabetes mellitus. Currently, there is a paucity of contemporary studies assessing IgA deficiency in Type 1 diabetes. This study evaluates the prevalence of IgA deficiency in individuals with Type 1 diabetes, compared with patients with coeliac disease and control subjects. In addition, we evaluate whether routine IgA measurement is justifiable when case finding for coeliac disease in patients with Type 1 diabetes. Methods All patients were assessed using IgA endomysial antibodies, IgA anti-tissue transglutaminase antibodies and total IgA levels. Altogether, 2434 individuals were tested: 1000 patients with Type 1 diabetes, 234 patients with coeliac disease and 1200 population control subjects. Definitive IgA deficiency was defined as total IgA levels < 0.07 g/l. Results The prevalence of IgA deficiency was significantly more common in patients with Type 1 diabetes (0.9%, n = 9/1000; P = 0.036) and coeliac disease (1.29%, n = 3/234; P = 0.041) when compared with population control subjects (prevalence of 0.17%, 2/1200). No statistical difference between Type 1 diabetes and coeliac disease for IgA deficiency was identified ( P = 0.87). Of patients in the group with Type 1 diabetes, 3.3% (33/1000) had coeliac disease, and of those only one patient had IgA deficiency leading to an antibody-negative presentation. Both IgA-deficient individuals within the population control subjects had normal duodenal biopsies and no relevant symptoms. Conclusions IgA deficiency is more common in Type 1 diabetes compared with population control subjects. Despite this, very few individuals with Type 1 diabetes and IgA deficiency appear to have villous atrophy on biopsy. These outcomes question the practice of routine IgA measurement when case finding for coeliac disease in patients with Type 1 diabetes. [ABSTRACT FROM AUTHOR]

Hadjivassiliou M, Wallis LI, Hoggard N, Grünewald RA, Griffiths PD, Wilkinson ID. MR spectroscopy and atrophy in Gluten, Friedreich's and SCA6 ataxias. Acta Neurol Scand: 2012: 126: 138-143. © 2011 John Wiley & Sons A/S. Background - Previous work using proton MR spectroscopy (1H-MRS) of the cerebellum in the ataxias suggested that 1H-MRS abnormalities and atrophy do not necessarily occur concurrently. Aims - To investigate the spectroscopic features of different types of ataxias. Methods - Using a clinical MR system operating at 1.5T, we performed 1H-MRS with a single voxel placed over the right dentate nucleus in 22 patients with gluten ataxia (GA), six patients with Friedreich's ataxia (FA), six patients with spinocerebellar ataxia type 6 (SCA6) and 21 healthy volunteers. Atrophy of the vermis and hemispheres on standard MRI was rated by a neuroradiologist. Any interaction between atrophy and 1H-MRS was analysed for the three groups of patients and controls. Results - Patients with GA had significant atrophy of the vermis and hemispheres as well as abnormal 1H-MRS. Patients with SCA6 had more severe overall atrophy of the vermis and hemispheres, but relatively preserved N-acetyl-aspartate/creatine (NAA/Cr). The FA group showed significant atrophy of only the superior vermis with normal 1H-MRS. Conclusions - This study suggests that 1H-MRS of the cerebellum in patients with ataxia provides information in addition to the presence of atrophy. There are significant 1H-MRS differences amongst different types of ataxia with interesting correlations between atrophy and NAA/Cr. [ABSTRACT FROM AUTHOR]

Hadjivassiliou M, Aeschlimann D, Grünewald RA, Sanders DS, Sharrack B, Woodroofe N. GAD antibody associated neurological illness and its relationship to gluten sensitivity. Acta Neurol Scand: 2011: 123: 175-180. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. The high prevalence of gluten sensitivity in patients with stiff-person syndrome (SPS) lead us to investigate the relationship between gluten sensitivity and GAD-antibody-associated diseases. We used ELISA assays for anti-GAD and for serological markers of gluten sensitivity. Patients were recruited from clinics based at the Royal Hallamshire hospital, Sheffield, UK. Patients with gluten sensitivity were followed up after the introduction of a gluten-free diet and serological testing was repeated. Six of seven (86%) patients with SPS were positive for anti-GAD, mean titre 109 U/ml; This compared with 9/90 (11%) patients with idiopathic sporadic ataxia, mean titre 32 U/ml, 16/40 (40%) patients with gluten ataxia, mean titre 25 U/ml, and 6/10 patients with type 1 diabetes only, mean titre 8 U/ml. None of 32 patients with celiac disease only, and of 40 patients with genetic ataxia were positive for anti-GAD. The titre of anti-GAD reduced following the introduction of a gluten-free diet in patients with SPS who had serological evidence of gluten sensitivity. The same was observed in patients with gluten ataxia and anti-GAD antibodies. This was also associated with clinical improvement. These findings suggest a link between gluten sensitivity and GAD antibody-associated diseases. [ABSTRACT FROM AUTHOR]

Background Factors predicting an unfavourable course of genital warts to treatment have not been determined. Materials and methods Behavioural and baseline disease characteristics were recorded from 246 males with anogenital warts. Urethral swabs were obtained and examined using the Hybrid Capture 2 Microplate assay. Patients were treated for their anogenital warts with cryotherapy, imiquimod cream 5% or podophyllotoxin. They were followed up every 3 months for 1 year. Results Patients with a negative or low-risk initial test tended to respond earlier to treatment than those with a high/intermediate-risk human papillomavirus (HPV) or with a dual infection ( P = 0.028). The response rate was unrelated ( P > 0.05) to the duration, number and anatomical location of the lesions and to the patient's age and sexual orientation, and only marginally to the initial extent of the lesion ( P = 0.046). However, the type of treatment predicted a favourable response (P ≤ 0.001), with patients who received both imiquimod and crotherapy responding worse. Considering all factors simultaneously in logistic regression, only the type of treatment and extent of the disease were found to influence the response rate. Conclusion The type of treatment and extent of the disease were the only factors found critical for patients’ response. [ABSTRACT FROM AUTHOR]

Background – The role of PET in the diagnosis of paraneoplastic neurological syndromes (PNS) has previously been reported in retrospective studies, from specialized neuro-oncology units, often selecting patients with positive paraneoplastic antibodies. Objectives – To prospectively assess the usefulness of PET in detecting malignancy in patients clinically suspected of having PNS. Methods – PET was performed in patients suspected of PNS within 4 weeks of the normal CT body scan. All patients were followed up. Results – Eighty patients suspected of having PNS underwent PET. 18/80 (23%) were abnormal and suspicious of malignancy. The total number of definite and probable PNS with abnormal PET was 11/18 (61%). The total number of definite and probable PNS with a normal PET was 3/62 (5%). Only 50% of patients with biopsy-proven malignancy were positive for paraneoplastic antibodies. The prevalence of abnormal PET in patients presenting with classical PNS was 41% as opposed to 21% in patients with non-classical PNS. The sensitivity and specificity of PET in diagnosing PNS was 75% and 87% respectively. Conclusions – PET is a valuable tool in clinically suspected PNS. Its use should not be restricted to specialized neuro-oncology units or in patients with positive paraneoplastic antibodies. Positive yield is the highest amongst patients with classical PNS. [ABSTRACT FROM AUTHOR]

Objective: Gluten sensitivity typically presents as celiac disease, a chronic, autoimmune-mediated, small-intestinal disorder. Neurological disorders occur with a frequency of up to 10% in these patients. However, neurological dysfunction can also be the sole presenting feature of gluten sensitivity. Development of autoimmunity directed toward different members of the transglutaminase gene family could offer an explanation for the diversity in manifestations of gluten sensitivity. We have identified a novel neuronal transglutaminase isozyme and investigated whether this enzyme is the target of the immune response in patients with neurological dysfunction.Methods: Using recombinant human transglutaminases, we developed enzyme-linked immunosorbent assays and inhibition assays to analyze serum samples of patients with gluten-sensitive gastrointestinal and neurological disorders, and various control groups including unrelated inherited or immune conditions for the presence and specificity of autoantibodies.Results: Whereas the development of anti-transglutaminase 2 IgA is linked with gastrointestinal disease, an anti-transglutaminase 6 IgG and IgA response is prevalent in gluten ataxia, independent of intestinal involvement. Such antibodies are absent in ataxia of defined genetic origin or in healthy individuals. Inhibition studies showed that in those patients with ataxia and enteropathy, separate antibody populations react with the two different transglutaminase isozymes. Furthermore, postmortem analysis of brain tissue showed cerebellar IgA deposits that contained transglutaminase 6.Interpretation: Antibodies against transglutaminase 6 can serve as a marker in addition to human leukocyte antigen type and detection of anti-gliadin and anti-transglutaminase 2 antibodies to identify a subgroup of patients with gluten sensitivity who may be at risk for development of neurological disease. [ABSTRACT FROM AUTHOR]

A great part of the medical wax moulages produced in the beginning of the last century depicts the various clinical signs of syphilis. Based on the study of a large Greek moulage collection, the different stages of syphilis are described. Moulages may still have the original scope as teaching objects, presenting clinical manifestations seldom seen to day and thus more likely of being overseen by the clinicians. [ABSTRACT FROM AUTHOR]

Medical moulages are three-dimensional wax figures made for teaching in the beginning of the last century. A rather unknown hospital museum in Athens, Greece, stores 1660 moulages depicting skin and venereal diseases prevalent at that time such as syphilis, lepra and tuberculosis. The historical background behind the Greek moulages and the art of moulaging are described. [ABSTRACT FROM AUTHOR]

One hundred and sixty HIV-infected Greek patients were prospectively examined and the oral signs and symptoms were recorded. At the time of oral examina tion, 76 patients were asymptomatic seropositive, 47 were in ARC stage and 37 had AIDS. One or more oral findings were recorded in 90.6% of the patients while a total of 33 different lesions were observed. The more common oral lesions (highly suspicious) were candidiasis (61%), hairy leukoplakia (24%), periodontitis (19%), necrotizing gingivitis (11%) and Kaposi's sarcoma (12%). In addition some unclassified lesions or symptoms (xerostomia 26%, burning mouth syndrome 19%, patchy depapillated tongue 16%, hairy tongue 10%, exfoliative cheilitis 4%), were common while submandibular and cervical lymph node enlargement were found in 49% of the patients. Interestingly in 16 patients (10%) the suspicion of HIV infection was exclusively based on oral lesions. Our findings show that oral signs and symptoms are common and some times early manifestations of HIV infection and it is in association to those reported in previous studies. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented about the case of a 32-year-old man with vitiligo in the penis, scrotum and pubic area after the use of imiquimod 5 percent cream.