Cold paresis in multifocal motor neuropathy

Increased weakness during cold (cold paresis) was reported in single cases of multifocal motor neuropathy (MMN). This was unexpected because demyelination is a feature of MMN and symptoms of demyelination improve, rather than worsen, in cold. It was hypothesized that cold paresis in MMN does not reflect demyelination only, but may indicate the existence of inflammatory nerve lesions with permanently depolarized axons that only just conduct at normal temperature, but fail at lower temperatures. We investigated symptoms of cold paresis in 50 MMN patients, 48 chronic inflammatory demyelinating polyneuropathy (CIDP) patients, 35 progressive spinal muscular atrophy (PSMA) patients, and 25 chronic idiopathic axonal polyneuropathy patients. We also investigated symptoms of increased weakness during warmth (heat paresis). Cold paresis was reported more often than heat paresis. Cold paresis was most frequently reported in MMN. Multivariate analysis indicated that MMN patients had a 4- to 6-fold higher risk of reporting cold paresis than CIDP or PSMA patients. Because cold paresis is not consistent with demyelination, the lesions in MMN may involve other mechanisms than demyelination only. In conclusion, symptoms of cold paresis are common in peripheral nervous system disorders, particularly in MMN. This supports the above-described hypothesis.     

Hidradenitis suppurativa is not associated with the metabolic syndrome based on body type: A cross‐sectional study

A body type with a high waist circumference or elevated waist‐to‐hip ratio (WHR), known as the “apple” body type, represents central/visceral obesity and is associated with the metabolic syndrome. The aim of this study was to simultaneously investigate the body mass index (BMI) and WHR in order to classify body types in individuals with hidradenitis suppurativa (HS) compared with a general dermatological population. A hospital‐based cross‐sectional study was performed in the Netherlands. One hundred and six HS patients and 212 controls were included. The BMI was significantly higher in the HS group in comparison with the control group, at 27.8 ± 5.4 and 25.6 ± 4.8, respectively (P < 0.001). The WHR did not significantly differ between HS patients and the control dermatological population (P > 0.05). A more peripheral pattern of bodyweight distribution was seen in 43% of the 37 obese HS individuals, in contrast to 19% of 31 obese patients in the control group (P = 0.036). In conclusion, the body type in obese HS patients, based on the WHR, shows a more peripheral pattern and differs from the WHR in the BMI‐matched general dermatological population.     

Allergen‐specific T cell responses to immunotherapy monitored by CD154 and intracellular cytokine expression

Background A sensitive measurement of low numbers of intracellular cytokine‐expressing antigen‐specific T cells from peripheral blood mononuclear cells (PBMC) is possible using CD154 as a marker of recently activated T cells. This technique may have potential for monitoring peripheral blood T cell responses to immunotherapy. Objective To evaluate the applicability of this method for measuring changes in cytokine production by allergen‐specific T cells in a clinical trial setting. Methods Ex vivo ragweed‐specific CD154 and intracellular cytokine expression were evaluated using a subset of subjects in an environmental chamber study of allergic rhinitis immunotherapy. PBMC were collected and cryopreserved from Amb a 1‐immunostimulatory oligodeoxynucleotide conjugate (AIC)‐treated (n=17) and placebo‐treated (n=15) ragweed‐allergic subjects both after pre‐ and post‐treatment ragweed exposures. In vitro allergen‐stimulated CD3⁺CD4⁺CD154⁺ T cell intracellular IL‐4, IL‐5, IL‐13, and IFN‐γ expression were evaluated by flow cytometry. Results Compared with the T helper type 2 (Th2) cytokine expression measured after pre‐treatment ragweed exposures, placebo‐treated subjects demonstrated a significantly elevated ragweed‐ and Amb a 1‐specific T cell IL‐4 and IL‐13 co‐expression (P=0.005 and P=0.022, respectively) and a significantly elevated ragweed‐specific IL‐5 expression (P<0.001) following post‐treatment ragweed exposures. In contrast, AIC‐treated subjects demonstrated no increases in allergen‐specific Th2 cytokine expression following post‐treatment ragweed exposures. IFN‐γ expression remained low and un‐changed in both groups. Subject reported total nasal symptom scores demonstrated modest but significant correlations with Amb a 1‐ and ragweed‐stimulated intracellular Th2 cytokine responses. Conclusion Combined CD154 and intracellular cytokine staining in PBMC can be used to sensitively monitor changes in antigen‐specific T cell subset frequencies in clinical studies. Antigen‐specific cytokine expression moderately correlated with the reported levels of allergic symptoms. Trial Registration NCT00537355 Cite this as: J. D. Campbell, P. Buchmann, S. Kesting, C. R. Cunningham, R. L. Coffman and E. M. Hessel, Clinical & Experimental Allergy, 2010 (40) 1025–1035.     

Tolerance and immunogenicity of the capsular polyoside vaccine against Haemophilus influenzae type b. A study of 325 children 15 to 71 months of age

The antigenic properties of the capsule polyoside (PRP) from Haemophilus influenzae type b (Hib) are adequate to form the basis for immunization designed to prevent the severe infections caused by this organism. The tolerance and immunogenicity of a vaccine containing either 12.5 or 25 micrograms PRP were studied in 325 healthy children aged 15 to 71 months after informed consent had been obtained from the parents. Each child was given one subcutaneous injection of 0.5 ml vaccine. Antibodies against Hib were assayed before and one month after the injection. Clinical tolerance was outstanding both locally (moderate and transient pain in 13% of cases) and systematically, with only eight (2.6%) febrile reactions in excess of 38.5 degrees C within 24 hours after the injection. A very significant rise in antibody titers was seen in all age groups, but a mean titer of 1 microgram/ml was achieved only in children aged 24 months or more. No significant difference was found between the two dosages. Individual analysis showed that following immunization antibody titers reached 0.15 micrograms/ml or more in 65% of infants aged 15 to 17 months, 71% of infants aged 18 to 23 months, 80% of infants aged 24 to 30 months and 95% of children older than 30 months. Despite the inadequate immune response evidenced in the younger age groups, our results confirm that Hib infections are preventable from the age of 2 years. Our results are consistent with those recorded with a similar vaccine in Finland.     

Hearing loss among construction workers in Edmonton, Alberta, Canada

Hearing acuity among electricians, plumbers and pipefitters, and boilermakers with > or = 20 years of union membership was compared with that of telephone workers. Automated pure tone audiometry was performed and a questionnaire was administered. Most construction workers were exposed to occupational noise and wore hearing protection. Median thresholds for electricians and telephone workers were comparable. Thresholds of plumbers and pipefitters were higher but comparable to expected values. Boilermakers had high levels of hearing loss. Thresholds at 4000 Hz among older workers were similar for all groups and were above expected values, suggesting a cohort effect. Audiometric screening seems to be warranted for some construction workers.     

Interhemispheric reorganization of motor hand function to the primary motor cortex predicted with functional magnetic resonance imaging and transcranial magnetic stimulation

The objective of this study was presurgical assessment of reorganization of motor hand function in an 11-year-old girl with intractable epilepsy and a right-sided hemiplegia resulting from an extensive perinatal left hemispheric stroke. Prior to a left functional hemispherectomy, functional magnetic resonance imaging (MRI) showed that both nonparetic and paretic motor hand function predominantly activated the right primary motor cortex, whereas no activation was found in the left hemisphere. Transcranial magnetic stimulation of the right central area yielded responses in both the nonparetic and the paretic hand, whereas no responses were obtained after stimulation of the affected hemisphere. Both techniques indicated that motor function was mediated by corticospinal fibers originating from the undamaged (primary) motor cortex and predicted no further loss of motor hand function after surgery. Indeed, subsequent functional hemispherectomy induced no new sensorimotor deficits. Functional MRI was repeated 22 months after surgery and matched preoperative sensorimotor functional MRI findings, confirming reorganization of the primary motor cortex. No additional reorganization was introduced by surgery.     

Desmoplastic melanoma of the lip

BACKGROUND: This retrospective study looks at the prognosis of desmoplastic melanoma of the lip, correlating it with the clinical course, treatment, and patterns of failure. METHOD: Twenty-two patients with desmoplastic melanoma of the lip were seen at the University of Texas M. D. Anderson Cancer Center from 1965 to 1998. RESULTS: Three disease groups: (I) untreated tumor (3 patients), (II) excisional scar (10 patients), and (III) locoregional recurrence (9 patients). Group I had two cures and one failure. In group II six had no recurrences, and there were four failures. In group III, all patients failed. Ten patients (45%) had no evidence of disease, of which three (30%) had an initial misdiagnosis. Twelve patients (55%) died of disease or were living with disease, of which eight (67%) had an initial misdiagnosis. CONCLUSIONS: Desmoplastic melanoma of the lip is often misdiagnosed and, therefore, inappropriately treated with multiple recurrences and poor prognosis. Accurate diagnosis and combined treatment may improve local control and survival.     

Neonatal hearing screening: modelling cost and effectiveness of hospital- and community-based screening

Background  

Children with congenital hearing impairment benefit from early detection and management of their hearing loss. These and related considerations led to the recommendation of universal newborn hearing screening. In 2001 the first phase of a national Newborn Hearing Screening Programme (NHSP) was implemented in England. Objective of this study was to assess costs and effectiveness for hospital and community-based newborn hearing screening systems in England based on data from this first phase with regard to the effects of alterations to parameter values.     

Intra- and inter-investigator variation in the analysis of pressure-flow studies in men with lower urinary tract symptoms

The objective of this study was to assess the intra- and inter-investigator variation in the analysis of pressure-flow studies that were performed in men with lower urinary tract symptoms. Two hospitals were involved in this study. In each hospital 100 PFS were selected. Photocopies of printouts of all PFS were analyzed manually by six experienced investigators, including determination of P(detQmax) and Q(max). Afterward, all 200 PFS were analyzed again in a different order. For each P(detQmax) and accompanying Q(max) the AG-number was calculated. With these AG numbers, the intra-investigator SD, the inter-investigator SD and the intra- and inter-investigator SD combined were calculated. The intra- and inter-investigator SD combined was 10.7. This implies that if one investigator analyzes a PFS once and determines an AG number of 40, another investigator may determine an AG number between 40 +/- 2. 77*10.7 = 10-70, using a 95% confidence interval. The inter-investigator SD was 10.0 and the intra-investigator SD was 3.7. The reproducibility of the manual analysis of urodynamic studies is moderate owing to a considerable intra- and inter- investigator variation. This is mostly caused by the substantial intra-investigator variation.     

Comparison of different computer models of the neural control system of the lower urinary tract

This paper presents a series of five models that were formulated for describing the neural control of the lower urinary tract in humans. A parsimonious formulation of the effect of the sympathetic system, the pre-optic area, and urethral afferents on the simulated behavior are included. In spite of the relative simplicity of the five models studied, behavior that resembles normal lower urinary tract behavior as seen during an urodynamic investigation could be simulated. The models were tested by studying their response to disturbances of the afferent signal from the bladder. It was found that the inhibiting reflex that results from including the sympathetic system or the pre-optic area (PrOA) only counteracts the disturbance in the storage phase. Once micturition has started, these inhibiting reflexes are suppressed. A detrusor contraction that does not result in complete micturition similar to an unstable detrusor contraction could be simulated in a model including urethral afferents. Owing to the number of uncertainties in these models, so far no unambiguous explanation of normal and pathological lower urinary tract behavior can be given. However, these models can be used as an additional tool in studies of the mechanisms of the involved neural control.