Nodding Syndrome (NS) was recently described in children in Northern Uganda. The affected children were in the age group 5 – 15 years. They were stunted, malnourished, dehydrated, mentally retarded and get recurrent seizures. The objective of this study was to describe the cluster distribution of NS cases in Northern Uganda. We conducted a cross-sectional study using available data on the burden of NS in Northern Uganda and used GPS to map the locations of the most affected areas. The results obtained indicate that Nodding Syndrome in Northern Uganda occurs in clusters in the following locations Odek, Atiak, Angagura, Awere, Laguti, Labongo-Amida, Atanga, Pajimo, Palabek Kal and Palabek Gem sub counties and mainly along Aswa and Pager rivers and their tributaries. Nodding Syndrome in Northern Uganda occurs in clusters predominantly along two rivers; perhaps it is an indication for environmental, dietary and common epidemiological exposures for the syndrome.

The seasonal occurrence and habitat of two parasitic copepods, Peniculus minuticaudae (Shiino, 1956) and Peniculus truncatus (Shiino, 1956) (Siphonostomatoida, Pennellidae) infecting the fins of black scraper Thamnaconus modestus and Korean rockfish Sebastes schlegelii, respectively were investigated. The fishes were collected from Tongyeong marine living resources research and conservation center, southern coast of Korea as five per month for two years from July 2011 to June 2013. In total, 391 copepods of P. minuticaudae were collected in two years, in contrast to P. truncatus. Prevalence was 85%, mean intensity was 3.25, and maximum intensity was 33. Season wise, the infestation was observed as the highest in autumn (September-November) season, and the lowest in winter (December- February). It was infested only on fins of black scrapers. Abundance of P. minuticaudae was found on the pectoral fin (43.5%), followed by anal (22.5%), second dorsal (20.5%) and caudal fins (13.5%). Statistically significant interactions were observed between season, infestation and infected regions (P<0.001). It is also reported for the first time in Korea from the fins of wild threadsail filefish Stephanolepis cirrhifer from Busan, Jeju, Tongyeong and Yeosu fish markets. It can be a new record on its host and localities. A total of 51 P. truncatus were collected with the prevalence of 37.5%, mean intensity of 0.37 and maximum intensity of 6. Season wise, infestation was observed as the highest in summer (June-August), and the lowest in winter. Attachment site was the dorsal fin and not found from any other fins of rockfish. Statistically significant interaction was observed between season and infestation (P<0.05). This is the first report on the ecology of these two pennellids.

In recent years, the sural nerve biopsy has become a commonly performed procedure in the diagnostic work-up of patients with peripheral neuropathy. This paper reviews the diagnostic usefulness and limitations of this procedure. Based on 385 sural nerve biopsies, we found clinically helpful or relevant information in 45% of cases. In 24% of cases, specific diagnoses were obtained, among which vasculitic neuropathy was most common.
Biopsy/methods/standards ; Evaluation Studies ; Histological Techniques ; Human ; Peripheral Nervous System Diseases/classification/*diagnosis ; Spinal Nerves/*pathology ; Sural Nerve/*pathology

OBJECTIVES: Periodic leg movements in sleep (PLMS) might be subdivided based upon whether or not they are associated with visible EEG microarousals (MA). MA is considered to be responsible for nonrestorative sleep and daytime fatigue. The American Sleep Disorders Association's (ASDA) scoring rules for MA based on visual analysis of the EEG changes suggest that MA should last more than 3 seconds. However, it has been suggested that visual analysis may not detect some changes in EEG activity. This study is aimed at measuring changes in EEG spectra during PLMS without MA in order to better understand the arousing response of PLMS. METHODS: Ten drug-free patients (three men and seven women) diagnosed with PLMS by polysomnography were studied. Spectral analysis of the EEG was performed in each patient on 30 episodes of PLMS without MA, chosen randomly across the night in stage 2 non-REM sleep. We applied stricter criteria for MA compared to ASDA, by defining it as a return to alpha and theta frequency lasting at least 1 second. RESULTS: The mean PLMS index was 16.7 10.0. The mean PLMS duration was 1.3 0.7 seconds. Comparison of 4-second EEG activity both before and after the onset of PLMS without MA using independent t-test showed that the movements were associated with significant increase of relative activity in the delta band (p=0.000) and significant decrease of activity in the alpha (p=0.01) and sigma (p=0.000) bands. No significant decrease in the theta (p=0.05), beta (p=0.129), or gamma (p=0.062) bands was found. CONCLUSIONS: PLMS without MA was found to be associated with EEG change characterized by increase in the delta frequency band. This finding seems to be compatible with the hypothesis of an integrative hierarchy of arousal responses of Sforza's. Considering that the subjects had lower PLMS index and shorter PLMS duration than those of the previous study, it is suggested that an even less severe form of PLMS without MA could induce neurophysiologic change, which may potentially be of clinical significance.
Arousal* ; Electroencephalography* ; Fatigue ; Humans ; Leg* ; Male ; Polysomnography ; Sleep Wake Disorders

This study assessed whether a current medical school curriculum is adequately preparing medical students to diagnose and treat common dermatologic conditions. A 15-item anonymous multiple choice quiz covering fifteen diseases was developed to test students' ability to diagnose and treat common dermatologic conditions. The quiz also contained five items that assessed students' confidence in their ability to diagnose common dermatologic conditions, their perception of whether they were receiving adequate training in dermatology, and their preferences for additional training in dermatology. The survey was performed in 2014, and was completed by 85 students (79.4%). Many students (87.6%) felt that they received inadequate training in dermatology during medical school. On average, students scored 46.6% on the 15-item quiz. Proficiency at the medical school where the study was performed is considered an overall score of greater than or equal to 70.0%. Students received an average score of 49.9% on the diagnostic items and an average score of 43.2% on the treatment items. The findings of this study suggest that United States medical schools should consider testing their students and assessing whether they are being adequately trained in dermatology. Then schools can decide if they need to re-evaluate the timing and delivery of their current dermatology curriculum, or whether additional curriculum hours or clinical rotations should be assigned for dermatologic training.
Anonyms and Pseudonyms ; Curriculum ; Dermatology* ; Humans ; Schools, Medical ; Students, Medical* ; United States*

BACKGROUND: Lambert-Eaton myasthenic syndrome is well known to be a classical paraneoplastic syndrome of small cell lung carcinoma (SCLC). Three cases of seronegative myasthenia gravis (MG) and SCLC were previously reported. CASE REPORT: A 65-year-old man developed a severe progressive respiratory failure with clinical features of MG. Tests showed a decremental response in the repetitive nerve stimulation test, abnormal single-fiber electromyography, and positive acetylcholine receptor antibody. SCLC was confirmed by the lung biopsy. CONCLUSIONS: This case represents the first case of seropositive MG and SCLC.
Acetylcholine ; Aged ; Electromyography ; Humans ; Lambert-Eaton Myasthenic Syndrome ; Lung ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Respiratory Insufficiency ; Small Cell Lung Carcinoma

We examined the p53 protein overexpression and evaluated its correlation with pathobiological variables, including: (1) patient age, sex, tumor size, histological type and grade, invasion depth, vascular invasion, perineural invasion and lymph node status; (2) the Ki-67 labeling index in 100 gastric carcinomas; and (3) the DNA ploidy pattern, S phase fraction (SPF), and the proliferation index (PI) in 84 cases using flow cytometry. The positive rate of p53 staining was 48% and the p53 immunoreactivity was independent of variable clinicopathologic factors. No correlation was made between the Ki-67 labeling index with p53 immunostaining and DNA ploidy parameters. Aneuploidy rate was slightly higher in the p53 positive group (55.6%) than the p53 negative group (44.4%)(p=0.097). The mean values of SPF and PI were significantly higher in the p53 protein positive group. Aneuploidy was more often observed in the intestinal type (p=0.038), advanced gastric carcinoma (p=0.015) and lymph node positive group(p=0.039). The above results suggest that although the p53 protein overexpression has no significant correlation with pathological factors and the Ki-67 labeling index, it may play an important role in tumor cell proliferation. Since p53 protein overexpression was slightly higher in the aneuploidy group showing significant correlation with poor prognostic parameters, it is thought that re-evaluation of the p53 mutation by molecular biological study is needed.
Aneuploidy ; Cell Proliferation ; DNA* ; Flow Cytometry ; Humans ; Lymph Nodes ; Ploidies* ; S Phase

In 1983, the first successful trial of 3,4-diaminopyridine (3,4-DAP) in Lambert-Eaton myasthenic syndrome (LEMS) was reported. Efficacy of amifampridine (3,4-DAP and 3,4-diaminopyridine phosphate [3,4-DAPP]) for symptomatic treatment in LEMS was proven by seven randomized studies in 3,4-DAP and two randomized studies in 3,4-DAPP. US Food Drug Administration approved 3,4-DAPP usage for adult LEMS in 2018 and for pediatric LEMS in 2022. Nineteen pediatric LEMS cases were identified in the literature. Compared with adult LEMS, the rate of malignancy is low as expected and the rate of dysautonomia is also low in pediatric LEMS. Unexpected finding is two cases of pediatric LEMS following antecedent infection. Amifampridine can be safely used as long the daily dose is less than 80 mg a day for adult LEMS patients and less than 30 mg a day for pediatric LEMS patients. Amifampridines can be supplemented with a liberal amount of pyridostigmine for long term usage. Amifampridine was used as symptomatic treatment in eight (42%) of 19 pediatric LEMS patients: 3,4-DAP in six and 3,4-DAPP in two patients. The most common practice of 3,4-DAP was a combination with pyridostigmine in four patients. With 3,4-DAP, normal activity was reported in 3 cases and mild to moderate-improvement in other 3 cases. In two patients with 3,4-DAPP, significant improvement in one and no improvement in one. Amifampridines are proven to be effective and safe drugs for the symptomatic treatment without serious side reaction in adults as well as in children as long as the dosage is properly adhered.

Myelodysplastic syndromes (MDS) are a group of clonal disorders arising from hematopoietic stem cells generally characterized by inefficient hematopoiesis, dysplasia in one or more myeloid cell lineages, and variable degrees of cytopenias. Most MDS patients are diagnosed in their late 60s to early 70s. The estimated incidence of MDS in the United States and in Europe are 4.3 and 1.8 per 100,000 individuals per year, respectively with lower rates reported in some Asian countries and less well estimated in other parts of the world. Evolution to acute myeloid leukemia can occur in 10-15% of MDS patients. Three drugs are currently approved for the treatment of patients with MDS: immunomodulatory agents (lenalidomide), and hypomethylating therapy [HMT (decitabine and 5-azacytidine)]. All patients will eventually lose their response to therapy, and the survival outcome of MDS patients is poor (median survival of 4.5 months) especially for patients who fail (refractory/relapsed) HMT. The only potential curative treatment for MDS is hematopoietic cell transplantation. Genomic/chromosomal instability and various mechanisms contribute to the pathogenesis and prognosis of the disease. High throughput genetic technologies like single nucleotide polymorphism array analysis and next generation sequencing technologies have uncovered novel genetic alterations and increased our knowledge of MDS pathogenesis. We will review various genetic and non-genetic causes that are involved in the pathogenesis of MDS.
Asian Continental Ancestry Group ; Cell Transplantation ; Europe ; Hematopoiesis ; Hematopoietic Stem Cells ; Humans ; Incidence ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes* ; Myeloid Cells ; Polymorphism, Single Nucleotide ; Prognosis ; Transplants ; United States

Gastroparesis is a clinical disorder characterized by upper gastrointestinal symptoms related with delayed gastric emptying of solids and liquids in the absence of mechanical obstruction. Diabetes mellitus has been the most common cause of gastroparesis and idiopathic gastroparesis also accounts for a third of all chronic cases. The most important mechanisms of gastroparesis, as understood to date, are loss of expression of neuronal nitric oxide synthase and loss of the interstitial cells of Cajal. However, the pathogenesis of gastroparesis is poorly understood. There have been several studies on specific molecules related to the pathogenesis of gastroparesis. Additionally, the Gastroparesis Clinical Research Consortium of the National Institutes of Health has achieved several promising results regarding the pathophysiology of gastroparesis. As the progress in the pathophysiology of gastroparesis has been made, a promising new drug therapy has been found. The pathophysiology and drug therapy of gastroparesis are focused in this review. Until now, the real-world medication options for treatment of gastroparesis are limited. However, it is expected to be substantially improved as the pathophysiology of gastroparesis is elucidated.
Diabetes Mellitus ; Gastric Emptying ; Gastroparesis ; Interstitial Cells of Cajal ; National Institutes of Health (U.S.) ; Nitric Oxide Synthase Type I