Summary: Lectotypification of Amphilophium mutisii (Bignoniaceae) and comments on the original herbarium material. The specimens belonging to the Celestino Mutis collection, kept in the herbarium of the Royal Botanical Garden of Madrid (MA), were studied for the proper lectotypification of Amphilophium mutisii Kunth.

Se estudiaron los ejemplares pertenecientes a la colección de Celestino Mutis, conservada en el herbario del Real Jardín Botánico de Madrid (MA), para la correcta lectotipificación de Amphilophium mutisii Kunth.

In recent years, several devices for the treatment of congenital or acquired structural cardiomyopathies have been designed and introduced in clinical practice. But beyond their formal indications, in situations where conventional surgical treatment has a high risk, off-label use of these devices has been reported with increasing frequency and encouraging results. This work presents four cases of patients with low prevalence disorders who were treated with percutaneous Amplatzer® devices for indications other than those for which they were designed. An aortic paravalvular leak, an acquired Gerbode defect, an aorta to right atrium fistula and a mitral paraprosthetic leak were respectively closed with devices for vascular occlusion, atrial septal defect closure, ductus arteriosus closure and treatment of muscular interventricular communication. In all cases the treatments were effective and patients had a favourable outcome without requiring new surgical interventions. We conclude that, irrespective of the indications for which they were designed, the use of Amplatzer® devices in selected pathologies is a good therapeutic alternative in the hands of experienced teams in this type of treatment.

En los últimos años se han diseñado e introducido en la práctica clínica habitual diversos dispositivos para el tratamiento de cardiopatías estructurales congenitas y adquiridas. Pero más allá de sus indicaciones formales, la utilización off-label de estos dispositivos en situaciones en las que el tratamiento quirúrgico convencional tiene un riesgo elevado se ha comunicado cada vez con más frecuencia con resultados alentadores. En esta comunicación se presentan cuatro casos de pacientes con patologías de baja preva-lencia que fueron tratados por vía endovascular con dispositivos Amplatzer® por fuera de las indicaciones para las que fueron diseñados. Una fuga paravalvular aórtica, un defecto de Gerbode adquirido, una fístula de la aorta hacia la aurícula derecha y una fuga paraprotesica mitral se cerraron con dispositivos para oclusión vascular, para el cierre de comunicación interauricular, para el cierre de conducto arterioso y para el tratamiento de comunicaciones interventriculares musculares, respectivamente. En todos los casos los tratamientos fueron efectivos y los pacientes evolucionaron favorablemente sin requerir nuevas intervenciones. Se concluye que la utilización de dispositivos Amplatzer® en patologías seleccionadas más allá de las indicaciones para las que fueron diseñados es una alternativa terapeutica válida en manos de equipos experimentados en este tipo de tratamiento.

Lesões do nervo espinhal são incomuns e na maioria das vezes iatrogênicas. São mais usualmente encontradas após procedimentos cirúrgicos no triângulo cervical posterior, principalmente biópsias de linfonodos. Apresentamos sete casos de neuropatia do nervo espinhal secundária a cirurgias, estudados mediante eletromiografia (EMG) quantitativa e exames eletrofisiológicos complementares. Em todos os pacientes estava afetado o feixe superior do trapézio, com ombro caído ou limitação para elevação do braço. Dor e queixas sensitivas estavam presentes em quase todos os pacientes e muito provavelmente decorriam de lesão associada dos ramos cutâneos do plexo cervical. O padrão de lesão correpondeu àquele da axonotmese, mas alguns pacientes exibiam sinais de compressão por tecido cicatricial. Salientamos a necessidade de exploração cirúrgica e reparo do nervo nos casos com recuperação lenta.

After innervating the sternocleidomastoid muscle, the spinal accessory nerve emerges and down crosses the posterior cervical triangle to innervate the trapezius. At the posterior triangle, the nerve is closely related to local lymph nodes and may be injured by their enlargement or surgical removal. Injury to this nerve is uncommom. Most cases are iatrogenically due to surgical procedures in the posterior cervical triangle, often following lymph nodes biopsies or benign turmors approaches. We present seven cases of post-surgical related spinal accessory nerve injury and discuss its clinical and electrophysiologycal profiles. The major motor signs were shoulder drop and paresis to raise arm. Pain and sensory complaints were almost universal and most probably due to concomitant injury of cervical plexus cutaneous branches, which are in direct relation to the spinal accessory nerve in the posterior cervical triangle. Lesion type is usually axonotmesis, but varied degrees of compression by cicatricial spurs are observed in some patients. Surgical approach and nerve repair must be considered for pacients with delayed recovery.

Depois de observar há vários anos que o registro eletromiográfico (BMG) do fenômeno de Strümpell (FS) é útil para diagnosticar síndromes piramidais, a partir de março-1987, o autor passou a pesquisar o FS sistematicamente durante a EMG dos músculos tibials anteriores. Realiza esse registro com eletrodo concêntrico de agulha e calibração de 0,05 a 0,2mV e 1s por divisão. Ao paciente, em decúbito dorsal, é solicitado realizar extensão vagarosa e completa da coxa. O FS positivo caracteriza-se por EMG de contração tônica involuntária, de amplitude crescente, do músculo tibial anterior, simultaneamente à extensão da coxa. Com a finalidade de sistematizar a EMG dessa sincinesia e de avaliar sua utilidade no diagnóstico da síndrome piramidal, o autor revisou os 579 relatórios de EMG realizados a partir de março-1987. Foram encontrados 26 (4,49%) relatórios de pacientes com FS positivo, que serviu para estabelecer ou confirmar que esses pacientes apresentavam síndromes piramidais. Em 20 pessoas de idade parecida e sem sinais de liberação piramidal, a pesquisa do FS se acompanhava de silêncio eletromiográfico (90%) ou de algumas contrações clônicas (10%) do tibial anterior. O autor recomenda a inclusão da pesquisa de FS no exame EMG convencional dos tibials anteriores, especialmente em casos de esclerose lateral amiotrófica e frente a síndromes mielorradiculares ou medulares.

After some years of empirical observation which pointed to the possibility of using the Strümpell phenomenon (SPh) electromyographic register (EMG) as a helpful tool in pyramidal syndrome diagnosis, the author began to study the SPh systematically, from March 1987 on, during the EMG examination of the anterior tibial muscles. The EMG was always effected with a needle concentric electrode and a calibration of 0.05 to 0.2mV and ls per division. The patient, lying on the back, is asked to slowly and completely stretch his thigh, and a positive SPh displays an EMG which shows unvoluntary tonic contractions of growing amplitude on the anterior tibial muscle during its extension. In order to establish an EMG systematic schedule of this synkinesia and to be able to evaluate its relevance for pyramidal syndrome diagnosis, the author revised all 579 EMG registers he had established from March-1987 on. 26 reports (4.49%) belonged to SPh-positive patients, and it was possible to detect or to confirm that they presented a pyramidal syndrome. 20 patients of similar age who showed no pyramidal sign presented EMG silence (90%) or some clonic contractions (10%) on the tibial muscle during SPh research. The author suggests, therefore, the inclusion of SPh research into the conventional EMG examination of the anterior tibial muscles, specially in cases of amyotrophic lateral sclerosis and in cases presenting myeloradicular or spinal cord syndromes.

Estudamos um paciente com quadro clínico rebelde, produzido por intoxicação pelo Parathion. Foi observado um fenômeno miotônico transitório, não descrito na literatura revisada. A constatação de cifras de AchE eritrocitária baixas e de colinesterase plasmática normais, indicou que era portador de déficit congênito de AchE, esclarecendo-se a evolução crônica do seu quadro. O fenômeno miotônico associado ao quadro clínico, eletrofisiológico, histológico e bioquímico fazem deste paciente um modelo clínico, de etiologia conhecida, da discutida síndrome de Isaacs.

A case of 38 year old man who worked with organochlorinated and Parathion during 5 years is reported. His follow-up was up to 2 years. The onset of the disease was characterized by cholinergic signs, headache, loss of weight, trembling, miokimias, fasciculations, ataxia, myotonic phenomena (in hands only) and motor sensitive peripheral polyneuropathy (affecting the lower limbs symmetrically). Low concentrations of blood cholinesterases confirmed the etiology. Myotonic phenomena disappeared spontaneuosly 6 months after the initial observation. One year later, the concentration of erytrocyte acetilcholinesterase was found to be low and plasma cholinesterase was normal, suggesting that the patient was carrier of a congenital deficiency of acetilcholinesterase. in literature relationship between myotonia and intoxication due to organophosphorus was not found. The whole clinical picture, cholinergic symptoms, transitory myotonic phenomena and spontaneous motor activity could be explained by an excess of acetilcholine. Eletromyography (EMG) in the first observation showed neuromuscular transmission blocking characterized by deficiency or absence of voluntary activity, unexcitability of fibular nerves, with fibrilations and positive peaks as described previously with Mipafox (another organophosphorus agent). During 2 years of observation numerous end-plates potentials of muscular fibres persisted in the EMG. A progressive increase in voluntary activity showed by unit motor potential of almost normal amplitude and very increased duration was observed. No potentials of reinnervation were noted. The results of EMG were explained as disturbances of neuromuscular transmission associated with moderate signs of denervation The Eaton-Lambert's test and the stimulation of a single unit motor potential confirmed disorder of neuromuscular synapses. The histochemistry of brachial biceps showed scattered atrophic and angulated type I and II fibres. Teased-fibres preparations showed nerve fibres with B, C, and G alterations as defined by Dyck et al. indicating axonal degeneration. These results were according to velocity of sensitive conduction. The conduction velocity of fibular nerves was strongly delayed during all the evolution indicating serious disorders of motor nerves myelin. We think that intoxication by organophosphorus should not be expressed as an identical alteration of both motor and sensitive nerves. The association of myotonic phenomena with such clinical, electrophysiological and histological picture is characteristic of the Isaac's syndrome model with known etiology.

São apresentados os resultados dos exames clínicos, eletrofisiológicos e do estudo do ponto motor, da imunofluorescência, da histoquímica e da ultrami-croscopia da biópsia muscular de um caso de miopatia miotubular. São discutidos estes resultados em relação aos achados de 56 casos desta moléstia consignados na literatura até 1978, sendo dada enfase à etiopatogenia.

A case of myotubular myopathy in a 10 years old girl is reported. Clinically, palpebral ptosis, ocular movements limitation, facial diplegia, posi-tivity of Gower's test, muscular hypotrophy distal, foot drop and deep absent reflexes were found. These signals were described by most of authors, besides symptoms referred, like partial urinary incontinency and frequent vomits. Reflexes H absents and teary in our patient were observed but were not described in the other cases of the literature. Routine laboratory tests were within normal limits; only aldolase was lightly elevated. In all muscles examinated it was noted a spontaneous electromyographic activity with +- of 2,64 ± 1,33 ms, 36,87 ± 30,87 µV and 88,13 ± 24,82 /s of frequency without characteristics of desenervation potentials of myoneural plates. The voluntary electromyographic activity was of myopathic pattern. A curve I/D made in the motor point of braquial biceps muscle was normal. The biopsy was made in the motor point of this muscle for histochemical, electron, immunofluorescence and vital stain microscopy. The biopsy showed 35% of fibras with central nuclei, predominance and hypotrophy of type I fibres, in some of them there were not myofibrils in the central zone, and poor differentiation between the fibre types in oxidative enzymes reactions. The electron microscopy confirmed the histochemical studies. The direct immunofluorescence was positive in some fibres. The vital stain showed beaded subterminal motor fibres. The clinical, electromyographic and principally histological findings suggest a innervation congenital disorder of muscle fibres.