Clinical and neurophysiological findings in patients with hereditary neuropathy with liability to pressure palsy and chromosome 17p11.2 deletion.

INTRODUCTION: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder, typically presenting with recurrent episodes of mononeuropathy in nerves susceptible to compression, with similar neurophysiological characteristics. However, other clinical and neurophysiological presentations have been reported. METHODS: We retrospectively analysed the clinical and neurophysiological characteristics of 20 patients with genetically confirmed HNPP. Sixteen patients were studied in our department between 1996 and 2016. RESULTS: In addition to the typical characteristics of HNPP, we found atypical forms including recurrent positional sensory symptoms in 3 patients, chronic sensorimotor polyneuropathy in one, and non-progressive mononeuropathy in one. Onset was early in 2 patients: one at the age of 7 years, with common peroneal nerve injury, and another at birth, with brachial plexus involvement. By frequency, the main pathological findings in the nerve conduction study were: decreased sensory nerve conduction velocity in the sural (84%) and the median and superficial peroneal nerves (94%); decreased motor nerve conduction velocity in the ulnar nerve through the elbow (97%), and increased motor distal latency of the median and deep peroneal nerves (74%). CONCLUSION: Our results confirm the clinical variability of HNPP, with the most frequent nerve conduction study findings being the generalised decrease in sensory nerve conduction velocity, in addition to motor involvement, mainly in locations susceptible to nerve compression. The nerve conduction study can detect typical, atypical, and asymptomatic cases of HNPP.

Monitoring bovine tick fever on a dairy farm: An economic proposal for rational use of medications.

In this study, we evaluated the monitoring of tick fever (TF) in a Brazilian dairy farm in the Minas Gerais state, Brazil, from July 10 to August 4, 2018. We aimed to identify diagnostic and treatment flaws in the protocol adopted by the farm, and to establish a novel and accurate TF monitoring protocol based on precision dairy farming and rational use of antimicrobials and antiparasitic drugs, while evaluating the economic benefits of the proposed strategy. We monitored TF in 395 heifer calves aged between 3 and 14 mo. According to the farm's standard protocol, all calves with an increase of 0.5°C in rectal temperature compared with the previous week's measurement were treated for Anaplasma spp. and Babesia spp. Blood smears were collected from the tail tip of the treated calves. During the last week of the study, we prepared blood smears of all calves regardless of treatment indication. Economic analysis was performed. The results indicated that at least 56.86% (261/459) of the calves did not require treatment for TF, whereas only 23.09% (106/459) had treatment indications. Negative blood smears (45.97%; 211/459) indicated the possibility of calves being affected by another disease or a condition that was not being adequately treated or those not necessarily sick. These results demonstrate the excessive use of medications, representing a direct economic loss, in addition to potentially favoring the occurrence of resistance to antimicrobials. In contrast, 9.42% (26/276) of calves had no treatment indication based on rectal temperature but had treatment indications based on blood smears. Only 5.73% (42/735) of blood smears had co-infection with hemopathogens, and none had triple co-infection. Therefore, we proposed the monitoring of TF using rectal temperature and microscopic analysis. If implemented, this strategy would result in a direct annual savings of approximately $22,638.96 (77.99%) related to medication for the treatment of TF. Therefore, implementing the proposed protocol would be cheaper than treatment based only on rectal temperatures. The currently implemented TF protocols overestimate the occurrence of TF, resulting in overtreatment. Thus, implementing a TF monitoring protocol based on a microscopy tool is justified, with benefits including rational use of medication, potential to generate savings, and reduced morbidity and mortality rates, in addition to enabling other diagnoses.

An outbreak of compressive myelopathy due to pyogranulomatous reaction to the oily adjuvant of the foot-and-mouth disease vaccine - case report / Surto de mielopatia compressiva associada à reação piogranulomatosa ao adjuvante oleoso da vacina contra Febre Aftosa em Minas Gerais - relato de caso

Cases of compressive myelopathy syndrome associated with post vaccinal pyogranulomas were diagnosed post mortem in three cows from a farm in Minas Gerais state, Brazil. These cows presented ataxia and bilateral paresis of the pelvic limbs, which evolved to paralysis, and sternal recumbence. On necropsy, locally extensive areas of the longissimus dorsi muscle were replaced by pyogranulomas supported by moderate amounts of fibrous connective tissue. On the cut surface, some nodules contained yellowish and viscous fluid (purulent exudate) or whitish fluid (interpreted as the oily adjuvant of a vaccine). In the spinal canal of the subjacent vertebrae, compressing the spinal cord, were pyogranulomas identical to those described in the skeletal muscle. Histologically, the pyogranulomas were composed of a central clear vacuole (consistent with the space left by the oil adjuvant droplets), surrounded by neutrophils and, more externally, by large numbers of epithelioid macrophages and fewer multinucleated giant cells. In the white matter of the spinal cord were numerous well-defined, clear vacuoles (Wallerian degeneration). The association of the clinical history and pathological findings allowed the diagnosis of compressive myelopathy associated with pyogranulomatous reaction to the oily adjuvant of the foot-and-mouth disease vaccine, in this case, due to its inadequate application.(AU)

São descritos casos de síndrome de compressão medular, associada a granulomas pós-vacinais, em bovinos Nelore, provenientes de uma propriedade em Minas Gerais. Esses bovinos apresentavam ataxia e paresia bilateral dos membros pélvicos, que evoluiu para paralisia e decúbito esternal. Na necropsia, áreas focalmente extensas da musculatura na região torácica dorsal (músculo longissimus dorsi) eram substituídas por numerosos piogranulomas, separados por tecido brancacento e firme (tecido conjuntivo fibroso). Ao corte, alguns nódulos continham material amarelado e viscoso (exsudato purulento) ou material esbranquiçado e fluido (sugestivo de adjuvante de vacina). No canal medular das vértebras subjacentes, havia granulomas idênticos aos observados no tecido muscular. Histologicamente, os piogranulomas continham, no centro, vacúolo, bem delimitado e arredondado (consistente com o espaço deixado pela gotícula de lipídio do adjuvante), circundado por variável quantidade de neutrófilos degenerados e íntegros e, mais externamente, por numerosos macrófagos epitelioides e algumas células gigantes multinucleadas. Nas áreas da medula espinhal, circundadas pelos granulomas, numerosos vacúolos, bem definidos, eram observados na substância branca (degeneração walleriana). A associação do histórico clínico e de achados patológicos permitiu o diagnóstico de mielopatia compressiva associada à reação granulomatosa ao adjuvante oleoso da vacina contra febre aftosa, no caso, induzida pela aplicação inadequada da vacina.(AU)

Clinical and neurophysiological findings in patients with hereditary neuropathy with liability to pressure palsy and chromosome 17p11.2 deletion. / Hallazgos clínico-neurofisiológicos en neuropatías hereditarias sensibles a la presión con deleción del cromosoma 17p11.2.

INTRODUCTION: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant disorder, typically presenting with recurrent episodes of mononeuropathy in nerves susceptible to compression, with similar neurophysiological characteristics. However, other clinical and neurophysiological presentations have been reported. METHODS: We retrospectively analysed the clinical and neurophysiological characteristics of 20 patients with genetically confirmed HNPP. Sixteen patients were studied in our department between 1996 and 2016. RESULTS: In addition to the typical characteristics of HNPP, we found atypical forms including recurrent positional sensory symptoms in 3 patients, chronic sensorimotor polyneuropathy in one, and non-progressive mononeuropathy in one. Onset was early in 2 patients: one at the age of 7 years, with common peroneal nerve injury, and another at birth, with brachial plexus involvement. By frequency, the main pathological findings in the nerve conduction study were: decreased sensory nerve conduction velocity in the sural (84%) and the median and superficial peroneal nerves (94%); decreased motor nerve conduction velocity in the ulnar nerve through the elbow (97%), and increased motor distal latency of the median and deep peroneal nerves (74%). CONCLUSION: Our results confirm the clinical variability of HNPP, with the most frequent nerve conduction study findings being the generalised decrease in sensory nerve conduction velocity, in addition to motor involvement, mainly in locations susceptible to nerve compression. The nerve conduction study can detect typical, atypical, and asymptomatic cases of HNPP.

Mineral profile of crossbred F 1 Holstein x Gyr dairy cows during the transition period in summer and winter / Perfil mineral de vacas F1 Holandês x Gir durante o período de transição no verão e inverno

The transition period is often a great challenge for dairy cows and mineral imbalances are frequent. With the aim to better understand the mineral profile of F1 Holstein x Gyr dairy cows and their performances under the different conditions of summer and winter, we collected blood samples to measure calcium, magnesium and phosphorus. Samplings were performed during summer and winter, on 15 and 13 pluriparous F1 Holstein x Gyr dairy cows, respectively. Blood sampling started 4 weeks prior to the expected calving date until 30 days postpartum. The mean concentrations of all three minerals had a different pattern during the transition period in each season, representing the interaction time x season. Calcium concentration was lower in winter and more animals suffered from subclinical hypocalcemia (100%) then in summer (38.46%). Magnesium concentration was also lower in winter and 46.67% of animals had hypomagnesemia, contributing for the higher hypocalcemia frequency observed in the same season. A high proportion of animals had hyperphosphatemia what can represent an environmental problem and more attention should be given to it. The high frequency of animals with subclinical hypocalcemia is alarming once that can lead to greater consequences.(AU)

O período de transição é uma fase de grandes desafios para vacas leiteiras, e desequilíbrios minerais são frequentes. O objetivo ao desenvolver este trabalho foi de entender melhor o perfil mineral de vacas leiteiras F1 Holandês x Gir e suas performances sob as diferentes condições de verão e inverno. Para isso, foram mensurados cálcio, magnésio e fósforo sanguíneo. As coletas de sangue foram realizadas durante os períodos de verão e inverno, e utilizaram-se, respectivamente, 15 e 13 vacas leiteiras pluríparas, todas F1 Holandês x Gir. As coletas de sangue começaram quatro semanas antes da data prevista do parto até 30 dias pós-parto. As concentrações médias e o padrão de variação dos três minerais foram distintos em cada estação do ano, representando a interação tempo de coleta x estação do ano. A concentração de cálcio foi menor no inverno, período em que todos os animais apresentaram hipocalcemia subclínica, enquanto no verão 38,46% apresentaram essa condição. A concentração de magnésio foi maior no verão e nenhum animal teve hipomagnesemia, enquanto no inverno 46,67% dos animais apresentaram hipomagnesemia subclínica, contribuindo para a maior frequência de hipocalcemia observada no inverno. Uma alta proporção de animais teve hiperfosfatemia, o que pode representar um problema ambiental. A alta frequência de animais com hipocalcemia subclínica é alarmante principalmente devido às consequências geradas por essa condição.(AU)

Energetic status of crossbreed dairy cows during transition period in two different seasons / Perfil energético de vacas leiteiras mestiças no período de transição em duas estações do ano

We used 31 crossbreed dairy cows to compare the energetic profile in summer and winter. Blood samples were taken weekly prepartum, at calving and on days 2, 5, 10, 15, 21 and 30 postpartum. All metabolic indicators analyzed were influenced by the physiological status. The glucose concentrations were higher during winter while the triglyceride concentrations and lactate dehydrogenase (LDH) were higher in the summer. The season influenced the concentrations of cholesterol, AST and GGT, showing a different pattern between summer and winter. Non-esterified fatty acids (NEFA) and beta-hidroxibutirate (BHB) were not influenced by the season. Cows that calved during winter had a greater body condition score (BCS) and lost more BCS until calving. During summer, 32.26% of the animals and 29.03% during winter had NEFA concentrations above the optimum level and 22.58% of the animals in summer and 19.35% in the winter had subclinical ketosis at some point during the transition period, making then more susceptible to diseases.

Foram utilizadas 31 vacas leiteiras mestiças para comparar o perfil energético em duas estações, verão e inverno. Amostras de sangue foram colhidas semanalmente durante o pré-parto, no dia do parto e nos dias dois, cinco, 10, 15, 21 e 30 do pós-parto. Todos os metabólitos analisados foram influenciados pelo estádio fisiológico. As concentrações de glicose foram maiores no inverno, enquanto as concentrações de triglicérides e lactato desidrogenase (LDH) foram maiores no verão. As concentrações de colesterol, AST e GGT mostraram um padrão de comportamento diferente entre o verão e o inverno. Ácidos graxos não esterificados (NEFA) e beta-hidroxibutirato (BHB) não foram influenciados pela estação do ano. As vacas que pariram durante o inverno tinham um escore de condição corporal (ECC) maior e perderam mais ECC até o parto. No verão, 32,26% e, no inverno, 29,03% dos animais apresentaram concentrações de NEFA acima do recomendado no pré-parto; 22,58% dos animais no verão e 19,35% no inverno tiveram cetose subclínica em algum momento do período de transição, tornando-os mais suscetíveis a outras doenças.

Fratura de fíbula e lesões de ligamento colateral e menisco lateral em muar: relato de caso / Fibula fracture and injuries to the collateral ligament and lateral meniscus in mules: case report

Relata-se a ocorrência de fratura de fíbula e lesões de ligamento colateral e menisco lateral em um muar, fêmea, de dois anos de idade, provavelmente produzida por um coice na região da articulação femorotibiopatelar do membro pélvico esquerdo. O animal apresentava claudicação de grau 5 desse membro e obteve melhora após bloqueio dos nervos fibular, tibial e safena e da articulação afetada. O exame radiográfico da região acometida revelou fratura do terço proximal da fíbula e, ao exame ultrassonográfico, observaram-se pontos anecoicos no ligamento colateral lateral e menisco.

This is a report of the occurrence of fibula fracture and injuries of the collateral ligament and lateral meniscus in a two year old mule, probably due to a kick in the joint area of the femurtibiopatellar left pelvic limb. The animal presented degree 5 lameness which improved after a nerve block of the affected joint. Radiographic examination of the affected area revealed a fracture on the proximal third of the fibula, and the ultrasound examination showed anechoic points observed in the lateral collateral ligament and meniscus.

Efeitos do polietilenoglicol 3350 e de soluções acidificantes e alcalinizantes sobre o perfil bioquímico de éguas hígidas / Effects of polyethylene glycol 3350 and alkalizing and acidifying solutions on the biochemical profile of healthy mares

Avaliou-se o perfil bioquímico de cinco éguas hígidas tratadas com polietilenoglicol 3350 (PEG); ou com polietilenoglicol 3350+Ringer lactato (PEG+RL); ou com solução isotônica poliônica enteral (SIPE); ou com solução isotônica poliônica enteral associada ao Ringer lactato intravenoso (SIPE+RL); ou com solução de cloreto de sódio a 0,9% (NaCl 0,9%). Os parâmetros avaliados foram: sódio, cloreto, potássio, cálcio ionizado, fósforo, magnésio total, proteínas totais, albumina, creatinina, ureia e osmolaridade calculada no soro de sangue venoso. O tratamento PEG não alterou os parâmetros bioquímicos. O PEG+RL diminuiu o fósforo. O SIPE diminuiu o fósforo e aumentou o cloreto. O SIPE+RL aumentou o sódio, e o NaCl 0,9% ocasionou o aumento do cloreto e a redução da osmolaridade sérica. Concluiu-se que o tratamento PEG associado ao Ringer lactato pode provocar o aparecimento de hipofosfatemia, o SIPE ocasiona hipercloremia, o NaCl 0,9% diminui a osmolaridade sérica e o SIPE+RL provoca hipernatremia.

The biochemical profile of five healthy mares treated with polyethylene glycol 3350 (PEG); or polyethylene glycol 3350+Ringer lactate intravenous (PEG+RL); or enteral polionic isotonic solution (EPIS); or enteral polionic isotonic solution associated with Ringer lactate intravenous (EPIS+RL); or chloride sodium solution 0.9% (NaCl 0.9%) was evaluated. The parameters evaluated were: sodium, chloride, potassium, ionized calcium, phosphorus, total magnesium, total protein, albumin, creatinine, urea, and calculated serum osmolality of venous blood. PEG treatment did not alter biochemical parameters. PEG+RL decreased phosphorus. SIPE decreased phosphorus and increased chloride. The EPIS+RL caused an increase of sodium and NaCl 0.9% caused an increase of chloride and a reduction of serum osmolarity. It is concluded that PEG treatment associated with Ringer lactate solution can cause the appearance of hypophosphatemia, EPIS causes hyperchloremia, NaCl 0.9% decreases the serum osmolality and EPIS+RL causes hypernatremia.

[Pseudo-conduction block in nonsystemic vasculitic neuropathy]. / Pseudobloqueo de conducción en vasculitis aislada del sistema nervioso periférico.

SUMMARY: Introduction. Nonsystemic vasculitic neuropathy (NSVN) is an inflammatory disorder of the vasa nervorum which usually is expressed as a mononeuritis multiplex. We present a patient with NSVN with histological confirmination focused on the neurophysiological findings at the early stages. CASE REPORT: A 36 years-old woman presented with paresthesia and weakness in her right hand followed by left footdrop. The first neurophysiologic examination showed low amplitude of the right median nerve (RMN) CMAP with proximal stimulation. A second examination showed signs of axonal damage in several nerves, including the RMN. CONCLUSIONS: The acute ischemic damage of a nerve can give a pattern of conduction block in the electroneurographic study as in the RMN of the presented case. This phenomenon is referred as "pseudo-conduction block", since it is transient and evolves towards a definite pattern of axonal neuropathy. When a vasculitic neuropathy is suspected, repeated neurophysiologic studies are necessary in order to ensure a proper (appropriate) characterization of the lesional patterns.