Phenological and productive characteristics of blackberry genotypes grown in an organic production system.

Blackberry is a species which has high potential to grow in an organic production system. However, there is scarce information on the behavior of cultivars and selections of this species conducted in an ecologically based system, a fact that makes it difficult to recommend cultivars for this production system. This study aimed to evaluate and characterize the phenology and productive aspects of six blackberry genotypes grown in an organic system, in Pelotas, Rio Grande do Sul (RS) state, Brazil. Evaluation of the following variables was carried out in the three first production cycles (2015-2016, 2016-2017 and 2017-2018): phenology, number of fruits.pl-¹, mean fruit yield.pl-¹, yield (kg.ha-¹), mean fruit mass (g) and soluble solid content (° Brix). Regarding phenology, genotype Black 112 was later than the others whereas Black 178 was the most precocious one. In the last year under evaluation, genotypes yielded around 10 ton.ha-¹, except Black 128 selection, which yielded approximately 6,767 ton.ha-¹. Genotypes Black 178, Black 112 and Black 145 and both cultivars BRS Xingu and Tupy exhibited potential to grow in an organic production system.

The irrigation effect on nuts' growth and yield of Carya illinoinensis.

Pecan trees require adequate soil moisture conditions to produce nuts with good yield and quality. Irrigation should be an important considered practice in orchards management plan, mainly in regions with water deficit periods. The objective of this research was to evaluate irrigation on pecan nuts growth and yield. This study was conducted in Uruguay, during the fast development phase until the pecan nuts harvest. Three irrigation treatments were used: 0 L (control), 70 L and 140 L plant-1 of water every two days, applied via drip irrigation in seven-year-old pecan trees of the cultivar Success. The number of nuts per cluster and nuts' diameter and length were evaluated during nut growth. After harvesting, peeled nuts' width and length were measured, and after peeling, the kernel length, width, height and filling and the kernel and peel percentage were evaluated. According to the results, it was possible to identify that periods of water deficit during pecan nuts development affected the kernel filling stageand size. Irrigation in pecan plants provides greater dimensions and nuts mass. With the use of 140 L plant-1, there was an increase in the nut mass of more than 100% in relation to the nuts from plants without irrigation.

Sensory ataxia rating scale: Development and validation of a functional scale for patients with sensory neuronopathies.

Sensory neuronopathies (SN) result from dorsal root ganglia damage and manifest with a combination of sensory deficits and proprioceptive ataxia. Characterization of the natural history and development of therapeutic trials are hampered by the lack of clinical scales that capture the whole spectrum of SN-related manifestations. We propose and validate a rating instrument for SN. Three experienced neuromuscular specialists developed items to rate SN. The resultant instrument was later validated by the assessment of the intra-class correlation coefficient, for inter-rater validity in 48 SN patients, and later in a smaller subset of 16 patients to assess its intra-rater validity. Standardized Crombach's alpha and Oblimin rotation analysis were performed to verify internal consistency and items' relationship, respectively. Evaluation of Sensory Ataxia Rating Scale (SEARS)'s external validity was performed by comparison to: scale for the assessment and rating of ataxia (SARA), Beck balance scale (BBS), and INCAT sensory sum score (ISS). A 10-item scale with an intra-class correlation coefficient >0.95 for intra- and inter-rating measurements with a good internal consistency (standardized Cronbach's alpha of 0.83) were observed. There was a normal distribution of the scores without a floor or ceiling effect. A moderate to good correlation between SEARS and SARA, BBS, and ISS was observed. SEARS is a reliable, easy-to-perform and consistent instrument to rate SN. Larger cohorts and multicenter studies are needed to validate its usefulness towards possible treatment trials.

Sensory neuronopathy heralding human T cell lymphotropic virus type I myelopathy.

Neurological phenotypes of human T cell lymphotropic virus type I (HTLV-1) are numerous and rarely may not manifest the classic HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP). We report a case of HTLV-1-related sensory neuronopathy heralding the classic HAM/TSP.

Electrophysiology of Cranial Nerve Testing: Cranial Nerves IX and X.

The cranial nerves IX and X emerge from medulla oblongata and have motor, sensory, and parasympathetic functions. Some of these are amenable to neurophysiological assessment. It is often hard to separate the individual contribution of each nerve; in fact, some of the techniques are indeed a composite functional measure of both nerves. The main methods are the evaluation of the swallowing function (combined IX and X), laryngeal electromyogram (predominant motor vagal function), and heart rate variability (predominant parasympathetic vagal function). This review describes, therefore, the techniques that best evaluate the major symptoms presented in IX and X cranial nerve disturbance: dysphagia, dysphonia, and autonomic parasympathetic dysfunction.

Nonneurological Involvement in Late-Onset Friedreich Ataxia (LOFA): Exploring the Phenotypes.

Friedreich's ataxia (FDRA) is the most common inherited ataxia worldwide, caused by homozygous GAA expansions in the FXN gene. Patients usually have early onset ataxia, areflexia, Babinski sign, scoliosis and pes cavus, but at least 25 % of cases have atypical phenotypes. Disease begins after the age of 25 in occasional patients (late-onset Friedreich ataxia (LOFA)). Little is known about the frequency and clinical profile of LOFA patients. One hundred six patients with molecular confirmation of FDRA and followed in three Brazilian outpatient centers were enrolled. General demographics, GAA expansion size, age at onset, cardiac, endocrine, and skeletal manifestations were evaluated and compared between LOFA and classic FDRA (cFDRA) groups. We used Mann-Whitney and Fisher tests to compare means and proportions between groups; p values <0.05 were considered significant. LOFA accounted for 17 % (18/106) and cFDRA for 83 % (88/106) of the patients. There were 13 and 48 women in each group, respectively. LOFA patients were significantly older and had smaller GAA expansions. Clinically, LOFA group had a tendency toward lower frequency of diabetes/impaired glucose tolerance (5.8 vs. 17 %, p = 0.29) and cardiomyopathy (16.6 vs. 28.4 %, p = 0.38). Skeletal abnormalities were significantly less frequent in LOFA (scoliosis 22 vs. 61 %, p = 0.003, and pes cavus 22 vs.75 %, p < 0.001) as were spasticity and sustained reflexes, found in 22 % of LOFA patients but in none of the cFDRA patients (p = 0.001). LOFA accounts for 17 % of Brazilian FDRA patients evaluated herein. Clinically, orthopedic features and spasticity with retained reflexes are helpful tips to differentiate LOFA from cFDRA patients.

Sleep disorders in Machado-Joseph disease.

PURPOSE OF REVIEW: This article provides a description on clinical features and pathophysiology of the main sleep disorders observed in Machado-Joseph disease (MJD). RECENT FINDINGS: Pathological studies have clearly demonstrated that degenerative process in MJD is widespread in the nervous system, and not restricted to the cerebellum. Nonmotor manifestations are frequent and may include pain, cramps, dysautonomia, cognitive deficits, psychiatric manifestations, olfactory deficits, fatigue, nutritional issues, and sleep disorders. SUMMARY: Sleep disorders are frequent in MJD, and include restless legs syndrome, rapid eye movement sleep behavior disorder, excessive daytime sleepiness, insomnia, sleep apnea, periodic limb movements during sleep, parasomnia, and others. Pathophysiological mechanisms related to sleep disorders in Machado-Joseph are complex and poorly understood. Considering that sleep complaints are a treatable condition, recognizing sleep disorders in MJD is relevant.

Porphyria and anorexia: cause and effect.

Porphyrias are hereditary disorders related to impaired biosynthesis of heme and characterized by multisystemic manifestations. Acute intermittent porphyria (AIP) is the most common acute subtype of the disease, and often associated with psychiatric symptoms. We here report a patient who developed acute flaccid paralysis after remarkable weight loss, which was related to an eating disorder (anorexia nervosa). After an extensive neurologic workup, he was diagnosed with AIP. This case emphasizes a deleterious vicious cycle between AIP and anorexia: porphyria may lead to anorexia and the carbohydrate restriction may lead to recurrent porphyric attacks. Therefore, an interruption of this cycle with psychiatric approaches to the eating disorders is crucial for long-term therapeutic efficacy.