Botulinum toxin in the treatment of lingual dystonia induced by speaking.

Primary lingual dystonia is a rare condition, especially when it is only induced by speaking. Trihexyphenidyl failed to improve the symptoms. Several case series have demonstrated the effectiveness of botulinum toxin injection for the management of focal lingual movement disorders. Only 1 case of botulinum toxin injection for primary lingual dystonia induced by speaking has been reported, but this treatment has limited effectiveness. Our patient was treated with botulinum toxin using a superficial approach for injection into the tongue with continuing excellent results. Lingual botulinum toxin injection is a fairly simple, safe and viable treatment option for lingual dystonia induced by speaking.

Serum mannose-binding lectin (MBL) gene polymorphism and low MBL levels are associated with neonatal sepsis and pneumonia.

OBJECTIVE: The aim of this study was to determine the serum mannose-binding lectin (MBL) levels and the frequency of MBL gene polymorphisms in infants with neonatal sepsis. STUDY DESIGN: Between January 2008 and January 2010, a total of 93 infants were included in this study and 53 of them had neonatal sepsis diagnosis as study group and 40 infants who had no sepsis according to clinical and laboratory findings as control group. RESULT: Serum MBL levels were found to be low in 17 of 93 infants. Eleven of them were in the sepsis group and six of them were in the control group. Serum MBL levels were significantly lower in infants with sepsis compared with the control group. Frequencies of genotype AB and BB were also significantly higher in the study group compared with the control group. Most importantly, presence of B allele of MBL exon 1 gene was found to be associated with an increased risk for neonatal sepsis. Additionally, in the study group, the mean serum MBL levels were found to be significantly lower in the premature infants compared with the term infants. Pneumonia, bronchopulmonary dysplasia (BPD) and intraventricular hemorrhage (IVH) were significantly higher in infants with MBL deficiency compared with infants with normal MBL levels. CONCLUSION: Low MBL levels and presence of B allele of MBL exon 1 gene were found to be important risk factors for development of both neonatal sepsis and pneumonia, especially in premature infants. Low MBL levels and MBL gene polymorphisms might also be associated with inflammation-related neonatal morbidities such as BPD and IVH.

Effect of levothyroxine treatment on clinical symptoms and serum cytokine levels in euthyroid patients with chronic idiopathic urticaria and thyroid autoimmunity.

BACKGROUND: Screening for thyroid autoimmunity in patients with chronic idiopathic urticaria (CIU) is generally recommended. However, there are not yet sufficient data as to whether levothyroxine treatment is beneficial for the clinical symptoms of CIU in patients with thyroid autoimmunity. AIM: We investigated the effect of levothyroxine treatment on clinical symptoms and serum tumour necrosis factor (TNF)-alpha, interleukin (IL)-10 and interferon (IFN)-gamma levels in euthyroid patients with CIU and thyroid autoimmunity. METHODS: In total, 15 patients with CIU and positive thyroid autoantibodies were randomized to receive either levothyroxine plus 5 mg/day desloratadine (suppression group, n = 8) or 5 mg/day desloratadine alone (control group, n = 7) for 12 weeks. Clinical symptoms of CIU, thyroid hormone levels, thyroid antibodies and serum cytokine levels were assessed at baseline and after the treatment. RESULTS: There were significant improvements in pruritus score and severity of weals in both groups compared with baseline values, but when the two groups were compared, there was no significant difference in the patients' clinical symptoms. Thyroid antibody titres were not different according to intragroup and intergroup analysis. In the suppression group, serum IFN-gamma and TNF-alpha levels were increased after treatment with levothyroxine compared with baseline values and there was a borderline statistical significance (P = 0.05 for both). CONCLUSIONS: These results suggest that levothyroxine treatment is not a reasonable option in euthyroid patients with CIU and thyroid autoimmunity. Augmentation of cytokine production after levothyroxine treatment seems to be related to the immunomodulatory effects of TSH-suppressive treatment.

Molecular, morphological, and cytological analysis of diverse Brachypodium distachyon inbred lines.

Brachypodium distachyon (brachypodium) is a small grass with the biological and genomic attributes necessary to serve as a model system for all grasses including small grains and grasses being developed as energy crops (e.g., switchgrass and Miscanthus). To add natural variation to the toolkit available to plant biologists using brachypodium as a model system, it is imperative to establish extensive, well-characterized germplasm collections. The objectives of this study were to collect brachypodium accessions from throughout Turkey and then characterize the molecular (nuclear and organelle genome), morphological, and cytological variation within the collection. We collected 164 lines from 45 diverse geographic regions of Turkey and created 146 inbred lines. The majority of this material (116 of 146 inbred lines) was diploid. The similarity matrix for the diploid lines based on AFLP analysis indicated extensive diversity, with genetic distances ranging from 0.05 to 0.78. Organelle genome diversity, on the other hand, was low both among and within the lines used in this study. The geographic distribution of genotypes was not significantly correlated with either nuclear or organelle genome variation for the genotypes studied. Phenotypic characterization of the lines showed extensive variation in flowering time (7-22 weeks), seed production (4-193 seeds/plant), and biomass (15-77 g). Chromosome morphology of the collected brachypodium accessions varied from submetacentric to metacentric, except for chromosome 5, which was acrocentric. The diverse brachypodium lines developed in this study will allow experimental approaches dependent upon natural variation to be applied to this new model grass. These results will also help efforts to have a better understanding of complex large genomes (i.e., wheat, barley, and switchgrass).

Effect of Saccharomyces boulardii in children with acute gastroenteritis and its relationship to the immune response.

We evaluated the effect of Saccharomyces boulardii administration in otherwise healthy children aged between 6 months and 10 years who were admitted for acute diarrhoea (15 males, 12 females). The patients were randomized into two groups: group 1 (n = 16) received 250 mg S. boulardii dissolved in 5 ml of water orally twice daily for 7 days and group 2 (n = 11) received placebo. Clinical and laboratory assessments were performed on admission and on day 7 of follow-up. Both groups experienced reduced daily stool frequency, the decrease being significantly greater in group 1 on days 3 and 4 compared with group 2. Group 1 demonstrated significant increases in serum immunoglobulin A and decreases in C-reactive protein levels on day 7. The percentage of CD8 lymphocytes on day 7 was significantly higher in group 1 than group 2. This study confirmed the efficacy of S. boulardii in paediatric acute gastroenteritis and the findings suggest that S. boulardii treatment enhances the immune response.

Evaluation of serum C-reactive protein, procalcitonin, tumor necrosis factor alpha, and interleukin-10 levels as diagnostic and prognostic parameters in patients with community-acquired sepsis, severe sepsis, and septic shock.

The diagnostic value of procalcitonin, C-reactive protein, tumor necrosis factor-alpha, and interleukin-10 levels in differentiating sepsis from severe sepsis and the prognostic value of these levels in predicting outcome were evaluated and compared in patients with community-acquired sepsis, severe sepsis, and septic shock in the first 72 h of admission to the hospital. Thirty-nine patients were included in the study. The severe sepsis and septic shock cases were combined in a single "severe sepsis" group, and all comparisons were made between the sepsis (n=21 patients) and the severe sepsis (n=18 patients) groups. Procalcitonin levels in the severe sepsis group were found to be significantly higher at all times of measurements within the first 72 h and were significantly higher at the 72nd hour in patients who died. Procalcitonin levels that remain elevated at the 72nd hour indicated a poor prognosis. C-reactive protein levels were not significantly different between the groups, nor were they indicative of prognosis. No significant differences in the levels of tumor necrosis factor-alpha were found between the sepsis and severe sepsis groups; however, levels were higher at the early stages (at admission and the 24th hour) in patients who died. Interleukin-10 levels were also higher in the severe sepsis group and significantly higher at all times of measurement in patients who died. When the diagnostic and prognostic values at admission were evaluated, procalcitonin and interleukin-10 levels were useful in discriminating between sepsis and severe sepsis, whereas tumor necrosis factor-alpha and interleukin-10 levels were useful in predicting which cases were likely to have a fatal outcome.

Nerve conduction study of ulnar nerve in volleyball players.

Ulnar neuropathy at the elbow is a common disorder seen in the throwing athlete. The purpose of our study was to determine whether asymptomatic physically active volleyball players and non-actives demonstrate distinct differences in nerve conduction of the ulnar nerve at the elbow. Nerve conduction studies were performed on both arms of 24 male volleyball players and 24 male non-actives. Nerve conduction velocity at the above to below elbow segment of the ulnar motor nerve were slower in the volleyball players compared with controls and their non-dominant arms. There were no statistical differences in latencies and conduction velocity of the ulnar nerve on the forearm, both in volleyball players and in the controls. In the evoked responses of the ulnar nerve, the amplitude was the same as corresponding normal values in both sensory nerve and muscle action potentials. We conclude that abnormal ulnar (motor) nerve conduction at the elbow segment may suggest a subclinical entrapment neuropathy as a result of strenuous elbow movements in volleyball players.

Comparison of nerve conduction velocities of lower extremities between runners and controls.

Peripheral nerve injuries can occur in runners, but large numbers of nerve injuries remain subclinical. Identification of nerve injuries needs an understanding of common sites of entrapment in running. Fourteen asymptomatic male middle-distance runners and 14 non-active subjects participated in this study. The neurophysiologic study consisted of motor and sensory nerve conduction of medial and lateral plantar nerves, sensory nerve conduction of sural and superficial peroneal nerves, and motor nerve conduction of common peroneal nerve. Active range of motion and muscle strength assessment (dorsi flexion/plantar flexion; inversion/eversion) as measured using a Biodex System3 Dynamometer were observed to be within normal limits for both groups. The medial plantar (sensory) nerve and sural nerve distal latencies were significantly prolonged and sensory conduction velocities were significantly delayed in the runners compared with the control subjects. Many of the asymptomatic runners with abnormal nerve conduction tests in this study may represent presymptomatic or asymptomatic neuropathy similar to the type of subclinical entrapment neuropathy.

Nerve conduction studies of upper extremities in tennis players.

OBJECTIVES: The influence of regular and intense practice of an asymmetric sport such as tennis on nerves in the elbow region was examined. METHODS: The study included 21 male elite tennis players with a mean (SD) age of 27.5 (1.7) years and 21 male non-active controls aged 26.4 (1.9) years. Anthropometric measurements (height, weight, limb length, and perimeters of arm and forearm) were determined for each subject, and range of motion assessment and radiographic examination carried out. Standard nerve conduction techniques using constant measured distances were applied to evaluate the median, ulnar, and radial nerves in the dominant and non-dominant limb of each individual. RESULTS: The sensory and motor conduction velocities of the radial nerve and the sensory conduction velocity of the ulnar nerve were significantly delayed in the dominant arms of tennis players compared with their non-dominant arms and normal subjects. There were no statistical differences in the latencies, conduction velocities, or amplitudes of the median motor and sensory nerves between controls and tennis players in either the dominant or non-dominant arms. However, the range of motion of the upper extremity was significantly increased in tennis players when compared with control subjects. Tennis players were taller and heavier than control subjects and their dominant upper limb lengths were longer, and arm and forearm circumferences greater, than those of the control subjects. CONCLUSIONS: Many of the asymptomatic tennis players with abnormal nerve conduction tests in the present study may have presymptomatic or asymptomatic neuropathy similar to subclinical entrapment nerve neuropathy.

Prevalence and clinical characteristics of an experimental model of 'ice-cream headache' in migraine and episodic tension-type headache patients.

This study was designed to compare the prevalence and clinical characteristics of 'cold-induced headache' between migraine and episodic tension-type headache patients. Seventy-six migraine and 38 episodic tension-type headache patients were included in the study. An experimental model of an 'ice-cream headache' was developed for the study. The pain occurrence period, its location and quality were recorded for each patient who felt pain in their head during the test procedure. Pain in the head occurred in 74% of migraine and 32% of 'tension-type headache' patients. Although the most frequent pain location was the temple in both groups of patients, this rate was greater than twofold in migraine patients when compared with episodic tension-type headache patients. While headache quality was throbbing in 71% of migraine patients, it was so in only 8% of the episodic tension-type headache patients. Considering all the results, it seems that 'cold-stimulus headache' is not only more frequent in migraine patients, but also its location and quality differ from 'tension-type headache'.

Nerve conduction studies of lower extremities in pes planus subjects.

Pes planus is a condition in which the medial longitudinal arch is depressed. Pedoscop, eyeball visualization, ink mat and roentgenography were used in clinical evaluation. We performed nerve conduction studies on both feet of 28 pes planus subjects. Our results demonstrated mild prolongation distal latency of the medial and lateral plantar sensory nerves, and delayed sensory conduction velocity of the medial plantar sensory nerve. The presence of electrodiagnostic abnormalities in this study population helps to substantiate the presence of compression neuropathy of the medial or lateral plantar nerve in pes planus subjects.

Carpal tunnel syndrome in carpet weavers.

We examined the prevalence of abnormal median nerve conduction within the carpal tunnel in carpet weavers. Nerve Conduction Studies (NCS) were performed on both hands of 47 subjects. Subjects had worked previously for an average of 8.4 (range 6-21) years and a mean age of 34.8 (17-55) years. An abnormal median nerve latencies and, symptoms associated with carpal tunnel syndrome (CTS) were found 10 (21.2%) and 2 (4.2%) in the subjects studied, respectively. We conclude that the high prevalence of abnormal median nerve conduction without corresponding symptoms, may suggest a subclinical entity associated with CTS.

T cell epitope-containing hypoallergenic recombinant fragments of the major birch pollen allergen, Bet v 1, induce blocking antibodies.

Allergen-specific immunotherapy represents one of the few curative approaches toward type I allergy. Up to 25% of allergic patients are sensitized against the major birch pollen allergen, Bet v 1. By genetic engineering we produced two recombinant (r) Bet v 1 fragments comprising aa 1-74 and aa 75-160 of Bet v 1, which, due to a loss of their native-like fold, failed to bind IgE Abs and had reduced allergenic activity. Here we show that both fragments covering the full Bet v 1 sequence induced human lymphoproliferative responses similar to rBet v 1 wild type. The C-terminal rBet v 1 fragment induced higher lymphoproliferative responses than the N-terminal fragment and represented a Th1-stimulating segment with high IFN-gamma production, whereas the N-terminal fragment induced higher IL-4, IL-5, and IL-13 secretion. Immunization of mice and rabbits with rBet v 1 fragments induced IgG Abs, which cross-reacted with complete Bet v 1 and Bet v 1-related plant allergens and strongly inhibited the IgE binding of allergic patients to these allergens. Thus, our results demonstrate that hypoallergenic T cell epitope-containing rBet v 1 fragments, despite lacking IgE epitopes, can induce Abs in vivo that prevent the IgE binding of allergic patients to the wild-type allergen. The overall demonstration of the immunogenic features of the hypoallergenic rBet v 1 fragments will now enable clinical studies for safer and more efficient specific immunotherapy.

The F response parameters in Behçet's disease.

The F response parameters may provide a sensitive method for detection of mild neuropathy in patients with otherwise normal nerve conduction studies. We investigated conventional nerve conduction studies and F response parameters in patients with Behçet's disease (BD), but without neurologic involvement. The results indicate that ulnar motor and sensory, tibial motor and sural sensory nerve conduction studies failed to differentiate the patients with BD and controls. In the ulnar nerve, the F response parameters were not significantly different for the populations. In the tibial nerve, the F response latency and chronodispersion were increased while F amplitude, duration, and persistence were all decreased in patients with BD. The results suggests that, (1) peripheral nerve dysfunction occurred especially in lower extremities in patients with Behçet's disease. (2) The F response parameters were considered the most sensitive method for the detection of neuropathy in Behçet's disease.

Primary Angiitis of the Central Nervous System: Unusual Clinical Presentation.

Primary angiitis of the central nervous system (PACNS) is a form of vasculitis restricted to the CNS. Most patients with PACNS present with focal neurologic deficits suggesting stroke or with a syndrome of headache and confusion. Less commonly, seizures, dementia, cranial neuropathies, myelopathies and, rarely, radiculopathies are seen. We report a 14-year-old girl who presented with partial motor seizures complicating focal cerebral vasculitis of superior parietal gyrus on the left. Despite extensive evaluation, no etiology was apparent for the vasculitis. Steroid therapy was administered, and 6 months after the therapy, she remains healthy except for a mild weakness of her right leg. Follow-up brain magnetic resonance images were normal. We conclude that partial motor seizures is uncommon as a presenting complaint in PACNS and steroid therapy is successful for this patient.

Innervation anomalies in upper and lower extremities (an electrophysiological study).

A hundred and eight subjects were studied for the frequent occurrence innervation anomalies in upper and lower extremities. Martin-Gruber Anomalies (MGA) were found in 19 (17.5%) subjects. The anomaly was bilateral in 14 subjects (73.6%). No case of motor ulnar to median nerve anastomosis in the forearm could be found. The 73.1, 20.8 and 14.3% incidences of the neural communication between the ulnar and median nerves in the hand were detected, in the Abductor Pollicis Brevis (APB), in the First Dorsal Interosseus (FDI) and in the Abductor Digiti Minimi (ADM) muscles, respectively. The existence of an accessory deep peroneal nerve was found in 23 (21.3%) subjects and 40 (18.5%) of legs. The anomaly was bilateral in 17 subjects (74%). We observed both MGA and accessory deep peroneal nerve in 9 cases.

Cerebral dural sinus thrombosis. A case report.

Cerebral dural sinus thrombosis is an uncommon syndrome that a patient presents with severe headache, which may be associated with complex physical and neurologic findings. The authors present a thirty-two-year-old woman with severe headache, focal motor seizures, and subsequent hemiparesis when she was ten weeks pregnant, diagnosed as having a dural sinus thrombosis by magnetic resonance imaging and magnetic resonance angiography. In this report, the application of magnetic resonance techniques, including angiography to diagnose and monitor therapy for dural sinus thrombosis, is presented.

The Jervell and Lange-Nielsen syndrome.

The association of prolongation of the QT interval with recurrent attacks of syncope, sudden death, and malignant ventricular arrhythmias is known as the long QT syndrome. The syndrome may be familial with or without congenital deafness, or idiopatic. The syndrome with deafness (Jervell and Lange-Nielsen syndrome) is inherited through an autosomal recessive mechanism. In this study, we attempted to identify patients with the Jervell Lange-Nielsen syndrome amongst 154 deaf mute school children. Two patients had a corrected QT interval of 0.52 and congenital sensorineural hearing loss with the other electrocardiographic changes characteristic of the syndrome, such as inverted or bifid T wave. There was no evidence of electrocardiographic (ECG) abnormality in family members, except only one case of parental deafness. This is the first and preliminary report that analyzed the incidence of the Jervell and Lange-Nielsen syndrome amongst 154 deaf mute school children in Turkey. Our study was conducted to identify patients with this syndrome amongst children of another deaf mute school in Turkey.

Locked-in syndrome: a case report.

A 46-year-old man developed a "locked-in" syndrome due to ventral pontine infarction. MRI showed abnormal signal intensity regions in the ventral pons. The EEG, BAEPs and SEPs were all within normal limits.