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Tratamento das formas severas de miastenia pelo ACTH por via intravenosa

Treatment of severe forms of myasthenia by intravenous administration of ACTH

Resumos

O autor inicia o trabalho referindo as bases bioquímicas, fisiopatológicas e anátomo-patológicas do tratamento da miastenia pelo ACTH. Na miastenia grave há diminuição da síntese da acetilcolina no organismo, atuando o ACTH no sentido de aumentar esta síntese seja diretamente, por ativação da colinacetilase, seja indiretamente, mediante a redução da massa dos tecidos linfóides, em particular do timo, responsáveis pela elaboração de substâncias que diminuem a síntese da acetilcolina. O autor empregou o ACTH "Armour" e a Cortrofina "Organon", nas doses de 2,5 a 25 mg, sempre pela via intravenosa, diluídos em 250 a 1.000 ml de soluto glicosado a 5%, administrado gota a gôta, na velocidade média de 20 gôtas por minuto, durante 8 horas. Como medicação associada foi administrada a Prostigmina a todos os pacientes, substituída, depois, em alguns casos, pelo Mestinon ou pela Mytelaze. Como adjuvantes foram empregados o cloreto de potássio (2 a 8 g por dia) e o sulfato de efedrina (25 mg 3 vêzes ao dia). Os pacientes foram mantidos em regime hiperprotéico e acloretado, sendo tomados todos os cuidados inerentes ao uso do ACTH. Foram estudados 10 pacientes portadores de miastenia com sintomatologia acentuada (8 casos) e média (2 casos). Todos os doentes vinham sendo tratados com drogas anticolinesterásicas em doses adequadas (Prostigmina, Mestinon, Mytelaze) e a sua sintomatologia respondia cada vez menos a esta terapêutica. Em alguns casos haviam sido tentados outros tratamentos (timectomia, denervação do seio carotídeo, irradiação da região tímica) sem resultado. É de notar que as remissões espontâneas neste grupo de enfermos foram excepcionais e de curta duração. A evolução foi acompanhada do ponto de vista clínico, com a sintomatologia classificada como muito acentuada, acentuada, média e leve. Em todos os casos houve remissão completa ou quase completa da sintomatologia após dosagens variáveis de ACTH; no caso 2, por exemplo, somente na terceira série de ACTH, foi conseguida remissão da sintomatologia acentuada para leve. Dos 10 casos relatados neste trabalho, em 6 houve agravação dos sintomas miastênicos nos primeiros 10 dias de tratamento. O autor considera o ACTH, utilizado por via intravenosa, como importante contribuição na terapêutica da miastenia grave, sendo especialmente indicado nas formas severas que não regridem mediante o emprego dos medicamentos anticolinesterásicos habitualmente usados.


The author presents the biochemical, physiopathological and anatomo-pathological bases of the treatment of myasthenia gravis by ACTH. In this disease there is a reduction of the synthesis of acetylcholine in the body; ACTH stimulates this synthesis, diretly by activation of cholinacetylasis, or indirectly, through reduction of the mass of lymphoid tissues, especially of the thymus, which are responsible for the elaboration of substances which reduce the synthesis of acetylcholine. The author used ACTH "Armour" and Cortrophine "Organon", in the doses of 2.5 to 25 mgm., diluted in 250 to 1,000 ml. of a 5% glycose solution; administration was always by intravenous way, in an average rate of 20 drops/minute, during 8 hours. As associate medication all patients received Prostigmin, which was later replaced, in some cases, by Mestinon or Mytelaze. As accessory medicaments were used potassium chloryde (2 to 8 grams per day) and ephedrine sulfate (25 mgm. 3 times a dayly). Patients were kept in an hyperproteic and achlorated diet and all precautions indicated when using ACTH were employed. Ten patients with myasthenia were studied. All patients had been treated before with anticholinesterasic drugs (Prostigmin, Mestinon, Mytelaze) in proper doses and their reaction to this therapy decreased gradually. In some cases other treatments had been tried thymectomy, denervation of the carotid sinus, radiation of the thymic region) without any result. Spontaneous remission in this group of patients were exceptional and for only short periods. Evolution was followed up from the clinical point of view. In all cases complete or almost complete remission occurred after a variable dosage of ACTH. In case 2, for instance, only after the 3rd series of ACTH remission of a severe symptomatology to a slight one was attained. In 6 of the 10 cases myasthenic symptoms became worse during the first 10 days of treatment. The author considers intravenous administration of ACTH as an important contribution to the therapy of myasthenia gravis, being especially indicated in the severe forms, which does not decrease through the use of anticholinesterasic drugs usually employed.


José Lamartine de Assis

Livre-Docente. Trabalho da Clínica Neurológica da Fac. Med. da Univ. de São Paulo (Prof. A. Tolosa)

RESUMO

O autor inicia o trabalho referindo as bases bioquímicas, fisiopatológicas e anátomo-patológicas do tratamento da miastenia pelo ACTH. Na miastenia grave há diminuição da síntese da acetilcolina no organismo, atuando o ACTH no sentido de aumentar esta síntese seja diretamente, por ativação da colinacetilase, seja indiretamente, mediante a redução da massa dos tecidos linfóides, em particular do timo, responsáveis pela elaboração de substâncias que diminuem a síntese da acetilcolina.

O autor empregou o ACTH "Armour" e a Cortrofina "Organon", nas doses de 2,5 a 25 mg, sempre pela via intravenosa, diluídos em 250 a 1.000 ml de soluto glicosado a 5%, administrado gota a gôta, na velocidade média de 20 gôtas por minuto, durante 8 horas. Como medicação associada foi administrada a Prostigmina a todos os pacientes, substituída, depois, em alguns casos, pelo Mestinon ou pela Mytelaze. Como adjuvantes foram empregados o cloreto de potássio (2 a 8 g por dia) e o sulfato de efedrina (25 mg 3 vêzes ao dia). Os pacientes foram mantidos em regime hiperprotéico e acloretado, sendo tomados todos os cuidados inerentes ao uso do ACTH.

Foram estudados 10 pacientes portadores de miastenia com sintomatologia acentuada (8 casos) e média (2 casos). Todos os doentes vinham sendo tratados com drogas anticolinesterásicas em doses adequadas (Prostigmina, Mestinon, Mytelaze) e a sua sintomatologia respondia cada vez menos a esta terapêutica. Em alguns casos haviam sido tentados outros tratamentos (timectomia, denervação do seio carotídeo, irradiação da região tímica) sem resultado. É de notar que as remissões espontâneas neste grupo de enfermos foram excepcionais e de curta duração. A evolução foi acompanhada do ponto de vista clínico, com a sintomatologia classificada como muito acentuada, acentuada, média e leve. Em todos os casos houve remissão completa ou quase completa da sintomatologia após dosagens variáveis de ACTH; no caso 2, por exemplo, somente na terceira série de ACTH, foi conseguida remissão da sintomatologia acentuada para leve. Dos 10 casos relatados neste trabalho, em 6 houve agravação dos sintomas miastênicos nos primeiros 10 dias de tratamento.

O autor considera o ACTH, utilizado por via intravenosa, como importante contribuição na terapêutica da miastenia grave, sendo especialmente indicado nas formas severas que não regridem mediante o emprego dos medicamentos anticolinesterásicos habitualmente usados.

SUMMARY

The author presents the biochemical, physiopathological and anatomo-pathological bases of the treatment of myasthenia gravis by ACTH. In this disease there is a reduction of the synthesis of acetylcholine in the body; ACTH stimulates this synthesis, diretly by activation of cholinacetylasis, or indirectly, through reduction of the mass of lymphoid tissues, especially of the thymus, which are responsible for the elaboration of substances which reduce the synthesis of acetylcholine.

The author used ACTH "Armour" and Cortrophine "Organon", in the doses of 2.5 to 25 mgm., diluted in 250 to 1,000 ml. of a 5% glycose solution; administration was always by intravenous way, in an average rate of 20 drops/minute, during 8 hours. As associate medication all patients received Prostigmin, which was later replaced, in some cases, by Mestinon or Mytelaze. As accessory medicaments were used potassium chloryde (2 to 8 grams per day) and ephedrine sulfate (25 mgm. 3 times a dayly). Patients were kept in an hyperproteic and achlorated diet and all precautions indicated when using ACTH were employed.

Ten patients with myasthenia were studied. All patients had been treated before with anticholinesterasic drugs (Prostigmin, Mestinon, Mytelaze) in proper doses and their reaction to this therapy decreased gradually. In some cases other treatments had been tried thymectomy, denervation of the carotid sinus, radiation of the thymic region) without any result. Spontaneous remission in this group of patients were exceptional and for only short periods. Evolution was followed up from the clinical point of view. In all cases complete or almost complete remission occurred after a variable dosage of ACTH. In case 2, for instance, only after the 3rd series of ACTH remission of a severe symptomatology to a slight one was attained. In 6 of the 10 cases myasthenic symptoms became worse during the first 10 days of treatment. The author considers intravenous administration of ACTH as an important contribution to the therapy of myasthenia gravis, being especially indicated in the severe forms, which does not decrease through the use of anticholinesterasic drugs usually employed.

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Apresentado à Academia de Medicina de São Paulo

Clínica Neurológica - Hospital das Clínicas da Fac. Med. da Univ. de São Paulo - Caixa Postal 3461 - São Paulo, Brasil.

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  • Tratamento das formas severas de miastenia pelo ACTH por via intravenosa

    Treatment of severe forms of myasthenia by intravenous administration of ACTH
  • Datas de Publicação

    • Publicação nesta coleção
      05 Dez 2013
    • Data do Fascículo
      Dez 1960
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