Acquired hyperexcitable peripheral nerve disorders: Clinical and laboratory features, therapeutic responses, and long-term follow-up.

INTRODUCTION/AIMS: Hyperexcitable peripheral nerve disorders (HPNDs) are rare. Although their clinical and laboratory features have been well studied, information on treatment and follow-up is limited. The aim of this study is to explore the long-term clinical, investigative, and therapeutic profile of patients with acquired HPNDs. METHODS: This study retrospectively analyzed patients from a single tertiary care center with HPND (January 2012 to January 2022). Patients were recruited according to published inclusion and exclusion criteria. Details of clinical features, diagnostic tests, therapeutic interventions, and follow-up were recorded. This study included patients with follow-up of 2 or more years. RESULTS: A total of 32 patients (M = 26, F = 6) were studied. The common clinical features included myokymia, neuropathic or shock-like pain, cramps, sleep disturbances, encephalopathy, cerebellar ataxia, and seizures. A total of 81.25% of patients responded favorably to corticosteroids and membrane stabilizers. Among the nonresponders, five received intravenous immunoglobulin (IVIG), and one received plasma exchange (PLEX). Two patients required rituximab due to poor responses to the above treatments. The mean duration of response was 6 weeks (4-24 weeks) from the initiation of treatment. All patients had favorable outcomes, reaching clinical remission within 1-5 years from the initiation of treatment. Only two patients had relapses. Immunotherapy could be stopped in 78% of patients within 3 years and 100% by 5 years. DISCUSSION: Chronic immunosuppression starting with corticosteroids is required for satisfactory outcomes of HPNDs. These disorders usually run a monophasic course, and relapses are uncommon.

Alfonso Roque Albanese: pionero de la cirugía cardíaca latinoamericana: homenaje de la Asociación Panamericana de Anatomía / Alfonso Roque Albanese: Latin American pioneer of heart surgery: tribute from the Pan American Association of Anatomy

Alfonso Roque Albanese (1906-2005) es considerado un pionero de la cirugía cardíaca Latinoamericana por sus importantes aportes. Fue miembro fundador de la Asociación Panamericana de Anatomía. Investigador y docente tanto de la anatomía como de la cirugía, uniendo ambas disciplinas.

Alfonso Roque Albanese (1906-2005) is considered a pioneer of Latin American heart surgery because of his important contributions. He was founding member of the Pan American Association of Anatomy. Researcher and teacher of anatomy and surgery, joining both disciplines.

Paracetamol for the treatment of patent ductus arteriosus in preterm neonates: a systematic review and meta-analysis.

OBJECTIVES: We performed a systematic review and meta-analysis of all the available evidence to assess the efficacy and safety of paracetamol for the treatment of patent ductus arteriosus (PDA) in neonates, and to explore the effects of clinical variables on the risk of closure. DATA SOURCE: MEDLINE, Scopus and ISI Web of Knowledge databases, using the following medical subject headings and terms: paracetamol, acetaminophen and patent ductus arteriosus. Electronic and manual screening of conference abstracts from international meetings of relevant organisations. Manual search of the reference lists of all eligible articles. STUDY SELECTION: Studies comparing paracetamol versus ibuprofen, indomethacin, placebo or no intervention for the treatment of PDA. DATA EXTRACTION: Data regarding efficacy and safety were collected and analysed. RESULTS: Sixteen studies were included: 2 randomised controlled trials (RCTs) and 14 uncontrolled studies. Quality of selected studies is poor. A meta-analysis of RCTs does not demonstrate any difference in the risk of ductal closure (Mantel-Haenszel model, RR 1.07, 95% CI 0.87 to 1.33 and RR 1.03, 95% CI 0.92 to 1.16, after 3 and 6 days of treatment, respectively). Proportion meta-analysis of uncontrolled studies demonstrates a pooled ductal closure rate of 49% (95% CI 29% to 69%) and 76% (95% CI 61% to 88%) after 3 and 6 days of treatment with paracetamol, respectively. Safety profiles of paracetamol and ibuprofen are similar. CONCLUSIONS: Efficacy and safety of paracetamol appear to be comparable with those of ibuprofen. These results should be interpreted with caution, taking into account the non-optimal quality of the studies analysed and the limited number of neonates treated with paracetamol so far.

Paracetamol treatment of patent ductus arteriosus in preterm infants.

OBJECTIVE: To determine the effectiveness of paracetamol in closing patent ductus arteriosus (PDA) in preterm infants of our population. STUDY DESIGN: Infants with symptomatic PDA who failed or could not get ibuprofen treatment, and who were candidates for surgical ligation, were administered oral paracetamol 15 mg kg⁻¹ every 6h, for up to 7 days and were monitored for clinical, echocardiographic and laboratorial courses. RESULT: Seven infants, between 24-27 weeks' gestation, were included. In four the DA was closed while treated by paracetamol-in one of them the DA reopened, treated with paracetamol again, and closed. In one infant, the DA almost closed, symptoms disappeared, and the DA subsequently closed spontaneously later. Two needed surgical ligation. There were no hematologic or biochemical abnormalities. CONCLUSION: Although there is the possibility that PDA may have closed spontaneously, it is proposed that paracetamol could contribute to the closure of PDA in preterm infants.

Diagnostic Tests in Neoplastic Meningitis: Lessons Learnt from Three Patients.

Neoplastic meningitis (NM) poses diagnostic challenges and investigations need to be chosen carefully. We present three cases of NM with distinct learning points. In case 1, MRI was diagnostic of melanosis; in case 2, ventricular CSF showed malignant cells when lumbar CSF repeatedly failed to show them; and in the third, whole body PET scan diagnosed the tumour when other tests were negative. A comparative evaluation of various diagnostic modalities used in suspected NM is provided.

An interesting case of headache.

A 35-year-old businessman with a history of migraine with aura developed new neck pain while lying on a sofa in his home. He was given neck massage and physical therapy for a day, and subsequently after two days developed severe generalized headache when sitting or standing. He was hospitalized in his hometown. Investigations revealed venous sinus thrombosis and bilateral thin subdural collections for which he was anticoagulated. Headache improved and then worsened and became severe in all positions. He was then admitted under our care. MRI scan of the brain at our hospital showed left subdural hematoma with midline shift. It required urgent evacuation. His previous first brain MRI was re-evaluated. It showed characteristic features of spontaneous intracranial hypotension (SIH). If not recognized early, SIH results in various complications, some of which require immediate intervention. Any change in the pattern of headache in SIH one must alert the clinician due to the possibility of one of its complications.

Hypertension in the Parsi community of Bombay: a study on prevalence, awareness and compliance to treatment.

BACKGROUND: Uncontrolled hypertension (HT) is an established risk factor for the development of vascular diseases. Prevalence varies in different communities and no such study has been conducted in the Parsi community living in Bombay, India. The objectives of this study were to determine the prevalence, awareness, compliance to medication and control of HT in this community. METHOD: We used a 1 in 4 random selection of subjects who were > or = 20 years of age. A questionnaire was administered and the blood pressure (BP) was measured by a doctor. HT was defined as diastolic blood pressure (DBP) > or = 90 mm Hg +/- systolic pressure (SBP) > or = 140 mm Hg. Isolated systolic hypertension (ISH) was defined as SBP > or = 160 mm Hg with DBP < 90 mm Hg. Subsequently, we reanalysed the data using current definition of ISH as SBP > or = 140 mm Hg with DBP < 90 mm Hg. RESULTS: 2879 subjects > or = 20 years of age were randomly selected of which 2415 (84%) participated in the study. The overall prevalence of HT in the community was 36.4%, of whom 48.5% were unaware of their hypertensive status. Of those aware of having HT, 36.4% were non-compliant with their anti-hypertensive drugs and only 13.6% had optimally controlled HT. Prevalence of ISH using the present criteria was 19.5% and 73% of hypertensives > or = 60 years had ISH. CONCLUSION: This study shows that prevalence of HT in the Parsi community is high and nearly half are unaware of their hypertensive status. ISH is the dominant form of HT in the elderly. Compliance to treatment is poor and optimal BP control is achieved in only a small minority. The study highlights the need for regular screening coupled with educational programs to detect and optimally treat HT in the community.

Visual evoked potentials: a diagnostic test for migraine headache in children.

Visual evoked potentials (VEPs) were performed in a blind fashion in 114 children aged 3 to 17 years with different types of headaches, in order to investigate whether this could be helpful in the diagnosis of migraine versus other types of headaches in children. The study showed that the amplitude between P100 and N2 was significantly larger in children with migraine headaches (mean 19.8 microvol, SD 7.75) compared with other types of headaches (mean 13.1 microvol, SD 7.45). These results suggest that the sensitivity of VEP P100 amplitude to distinguish migraine headaches in children is 67%, the specificity of VEP is 83%, and the predictive positive value is 83%. VEP study might be helpful in work-up of a child with headache, particularly a young child, when signs and symptoms may not be characteristic.

Microsatellite spreading in the human genome: evolutionary mechanisms and structural implications.

Microsatellites are tandem repeat sequences abundant in the genomes of higher eukaryotes and hitherto considered as "junk DNA." Analysis of a human genome representative data base (2.84 Mb) reveals a distinct juxtaposition of A-rich microsatellites and retroposons and suggests their coevolution. The analysis implies that most microsatellites were generated by a 3'-extension of retrotranscripts, similar to mRNA polyadenylylation, and that they serve in turn as "retroposition navigators," directing the retroposons via homology-driven integration into defined sites. Thus, they became instrumental in the preservation and extension of primordial genomic patterns. A role is assigned to these reiterating A-rich loci in the higher-order organization of the chromatin. The disease-associated triplet repeats are mostly found in coding regions and do not show an association with retroposons, constituting a unique set within the family of microsatellite sequences.

Identification and characterization of E.coli ribosomal binding sites by free energy computation.

Sequences upstream from translational initiation sites of different E.coli genes show various degrees of complementarity to the Shine-Dalgarno (SD) sequence at the 3' end of the 16S rRNA. We propose a quantitative measure for the SD region on the mRNA, that reflects its degree of complementarity to the rRNA. This measure is based on the stability of the rRNA-mRNA duplex as established by free energy computations. The free energy calculations are based on the same principles that are used for folding a single RNA molecule, and are executed by similar algorithms. Bulges and internal loops in the rRNA and mRNA are allowed. The mRNA string with maximum free energy gain upon binding to the rRNA is selected as the most favorable SD sequence of a gene. The free energy value that represents the SD region provides a quantitative measure that can be used for comparing SD sequences of different genes. The distribution of this measure in more than 1000 E.coli genes is presented and discussed.