J. Lamartine de Assis

Subacute sclerosing leucoencephalitis: an epidemiological, clinical and biochemical study of 31 cases

Thirty-one cases of SSLE are reported. The diagnosis was based on the clinical picture, the marked increase of gammaglobulins in the cerebrospinal fluid, the typical electroencephalographic pattern, and the pathological examination. Intracellular inclusion bodies were found at the postmortem examination of 8 among 13 cases and in 1 of 7 cerebral biopsies. The mean age was 11.7 years, with the limits of 3 and 22 years. Males prevailed markedly over females (23:8). Patients from rural regions were the bulk of the series (77.4%). No definite regional predominance was demonstrated by the analysis of the geographic distribution. A seasonal influence was not evidenced. In 16 patients followed up to death, the average duration of the disease was 7 months, with a range from 2 to 20 months. In the clinical picture the constancy of myoclonus or losses of tonus, and psychiatric disorders was evidenced. The first symptom was the psychiatric alteration in 55.6% of the cases, myoclonus in 35.5% and grand mal seizures in 33.3%. Rigidity and pyramidal signs were very frequent, while the cerebellar syndrome was rare. Ophtalmoscopic alterations (edematous or pale disk, choroidits) were found in a third of the cases. In two cases an epizootic of household chicken was coincident with the onset of the disease. The blood copper concentrations determined in 8 cases were always above the normal range, while ceruloplasmin remained within the normal limits. The blood sulfur levels were high in 3 patients studied. The blood magnesium levels were variable in 6 patients studied.

Neurological complication of antirabies vaccination in São Paulo, Brazil. Clinical and therapeutical aspects.

The author studied especially the clinical aspects and therapeutic results in 73 patients with neurological complication of anti-rabies vaccination. The neuroparalytic accidents and the most constant neurologic signs and symptoms were emphasized. The most common clinical syndrome was thoraco-lumbar meningomyelitis and there were CSF alterations in 88% of the cases studied. Eighteen patients presented neurologic complications after Fuenzalida vaccine: only 1 of them had Guillain-Barré syndrome, 1 had meningoradiculitis, 4 had myelitis and in the other 12 cases there was diffuse involvement of the nervous system especially of the spinal cord and meninges (meningomyelitis and meningoencephalomyelitis).

Timectomia na miastenia grave: avaliação dos resultados em 73 pacientes

Foi feita avaliacao dos resultados da timectomia transtoracica em 73 pacientes com miastenia grave generalizada, sendo 64 nao timomatosos e 9 com timomas. Os resultados foram analisados a curto, medio e, em muitos casos, a longo prazo. Foi feito, tambem um estudo comparativo dos resultados em relacao a idade, sexo, tempo de doenca e a presenca ou nao do timoma. A experiencia adquirida neste grupo de pacientes sugere que a timectomia transtoracica e metodo terapeutico util na miastenia grave generalizada nao timomatosa; seu efeito benefico aparece no pos-operatorio mediato e se mantem a longo prazo na maioria dos pacientes. Os resultados da timectomia em pacientes com timoma foram menos favoraveis. A analise estatistica nao mostrou diferencas significantes na evolucao pos-operatoria entre os pacientes nao timomatos selecionados e nao selecionados.An evaluation of results obtained after 73 thymectomies is reported. A transthoracic approach was used on all of the patients. Thymomas were found in 9 out of the 73 patients with myasthenia. All of the 64 patients without thymomas suffered from generalized myasthenia gravis with predominance of severe forms. The results of the early, intermediate and late post-operative course were analysed. A comparative study of results as related to age, sex, duration of disease and presence or absence of thymomas was made. Statistical analysis failed to show any significant difference in the results of thymectomy between selected and randomly chosen patients without thymomas.

Mitochondrial dysfunction in myasthenia gravis: report of a case

The case of an 11-year-old boy with external ophthalmoparesia, tetraparesia and bilateral eyelid ptosis is reported. He was 7-years-old when first symptoms appeared. Anticholinesterasic drugs were used. He was submitted to muscle biopsy. The results of histochemistry analysis showed storage of granulous material at the subsarcolemmal region of muscle fibers by SDH. Increase in the number of mitochondria with electron dense bodies was found at electron microscopy. Anticholinesterasic drugs administration was interrupted and consequently he got worse, and bouts of dyspnea occurred. Due to this worsening anticholinesterasic agents were reintroduced together with prednisone, and he improved. Due to clinical and histological expressions we think it is possible that morphological mitochondrial alterations may occur also in myasthenia gravis.

Immunosupression with corticosteroids and thymectomy in myastenia gravis: an evaluation of immediate and short term results in 20 patients

A comparative study was conducted on two groups of patients with the generalized severe form of myasthenia gravis. The first group of 20 patients received oral daily doses of 60-100 mg of prednisone prior to thymectomy. The control group of 20 were submitted to surgery without prior corticosteroid treatment. The study included statistical analysis of the clinical results and surgical complications for both groups. The authors concluded that the use of steroids preoperatively is beneficial.

Miastenia grave induzida por D-penicilamina em paciente com esclerose sistêmica progressiva

The development of autoimmune diseases in some patients treated with D-penicillamine (DPA) suggests that the reported occurrence of a conduction disorder at the neuromuscular junction and the development of a reversible myasthenia gravis in rheumatoid disease, progressive systemic sclerosis or Wilsons disease after the use of DPA are part of a general predisposition for autoimmune disease related to DPA therapy. The case reported is an example. The DPA- induced myasthenia gravis (MG) is similar to the spontaneous MG clinically and electrophysiologically, though ocular signs prevail in the former. Antibodies to acetylcholine receptor have been demonstrated and thymic hyperplasia also has been formed. Regarding the onset of myasthenic manifestations the duration of the treatment with DPA varies from 6 to 10 months. The action of DPA on the neuromuscular junction is different from that occurring in spontaneous MG. The pathogenesis of the DPA induced MG is still obscure. The chemical properties of DPA permit it to react with many proteins and some alteration of proteins may appear, with structural changes in the composition and antigenicity of the collagen fibers. In vitro DPA causes disorder of acetylcholine receptor bridges to alpha, beta, gamma sub-units with reduction of the S-S bridges in the gamma-subunit. This decreases the linkage of high affinity and abolishes its positive cooperative system, reducing the S-S connection in the alpha-unit near the acetylcholine linkage. The interaction between DPA and receptor may induce antigenic alteration in this latter, starting the autoimmune phenomena. The other possibility is the stimulation of prostaglandin E-1 synthesis by DPA may fill the allosteric place of ACh receptor, interfering on the neuromuscular junction.The development of autoimmune diseases in some patients treated with D-penicillamine (DPA) suggests that the reported occurrence of a conduction disorder at the neuromuscular junction and the development of a reversible myasthenia gravis in rheumatoid disease, progressive systemic sclerosis or Wilsons disease after the use of DPA are part of a general predisposition for autoimmune disease related to DPA therapy. The case reported is an example. The DPA- induced myasthenia gravis (MO) is similar to the spontaneous MG clinically and electrophysiologically, though ocular signs prevail in the former. Antibodies to acetylcholine receptor have been demonstrated and thymic hyperplasia also has been formed. Regarding the onset of myasthenic manifestations the duration of the treatment with DPA varies from 6 to 10 months. The action of DPA on the neuromuscular junction is different from that occurring in spontaneous MG. The pathogenesis of the DPA induced MG is still obscure. The chemical properties of DPA permit it to react with many proteins and some alteration of proteins may appear, with structural changes in the composition and antigenicity of the collagen fibers. In vitro DPA causes disorder of acetylcholine receptor bridges to a,b,g sub-units with reduction of the S-S bridges in the g-subunit. This decreases the linkage of high affinity and abolishes its positive cooperative system, reducing the S-S connection in the a-unit near the acetylcholine linkage. The interation between DPA and receptor may induce antigenic alteration in this latter, starting the autoimmune phenomena. The other possibility is the stimulation of prostaglandin E-l synthesis by DPA may fill the allosteric place of ACh receptor, interfering on the neuromuscular junction.

Parkinsonismo juvenil: considerações a respeito de 10 casos

Ten cases of juvenile parkinsonism are reported. A brief review of the literature is made and the nosologic boundaries of the disease are discussed. Comments are made on some clinical, genetic and therapeutic features of the illness in this series and according to the literature data.Ten cases of juvenile parkinsonism are reported. A brief review of the literature is made and the nosologic boundaires of the disease are discussed. Comments are made on some clinical, genetic and therapeutic features of the illness in this series and according to the literature data.

Síndrome de cauda eqüina produzida por melanoma

Os autores apresentam um caso de melanoma da cauda equina. A evolucao foi de, aproximadamente, dois anos, tendo havido, a principio, dores nos membros inferiores que, depois, adquiriram a forma de ciatalgia bilateral, associada a perturbacoes sensitivo-motoras e esfinctericas. Tratava-se, portanto, de uma sindrome de cauda equina. 0 paciente foi operado, encontrando-se um tumor escuro, infiltrante da cauda, e que foi diagnosticado, ao exame histopatologico, como melanoma meningeo. Ao tratamento cirurgico seguiu-se o radioterapico. Depois de um periodo de melhora sintomatica, o caso evoluiu para a caquexia, tendo-se verificado o falecimento do enfermo 11 meses depois da operacao. Nao foi feita autopsia, mas a ausencia, nos antecedentes, de dados que pudessem sugerir uma possivel me-tastase, o exame clinico e a localizacao do processo fazem pensar no carater primario do tumor.

Gliomas múltiplos do cérebro

Os AA. apresentam um caso de gliomas multiplos do cerebro, com regressao dos sintomas clinicos apos intervencao cirurgica. A localizacao do tumor foi feita exclusivamente pelos dados clinicos. Chamava a atencao o fato de toda area cerebral descoberta pela cranictomia estar ocupada por tres grandes gliomas, que foram facilmente enucleados, e, mais profundamente, tres outros tumores menores. Houve regressao notavel da sintomatologia apos o ato cirurgico. O exame anatomo-patoloico mostrou tratar-se de ependimoma. Os AA. terminam a exposicao do caso tecendo consideracoes de ordem clinica e anatomo-patologica, e admitindo, como ponto de origem dos gliomas no caso em apreco, os germes ependimais deslocados durante o desenvolvimento embrionario, pois os tumores estavam em pleno cortex cerebral, sem conexao com os ventriculos.

Failure of treatment of myasthenia gravis by cyclosporin-A: a case report

Recently, cyclosporin-A (Cy-A) has been used in the treatment of myasthenia gravis (MG). This drug could be employed in some patients refractory to classic treatments or that develop undesirable side effects. It is reported the case of a 22 year-old woman with generalized and severe MG, and diabetes mellitus. She had been submitted to thymectomy and reoperated, to the classic ethiopathogenic methods of therapy, and to total body irradiation. No therapeutical results were observed. Also, she developed transient and slow bone marrow depression, and liver dysfunction. Owing to these limitations and to the absence of response to treatments mentioned. Cy-A use was attempted in this case. Unfortunately, Cy-A did not influence the myasthenic symptomatology. Cy-A also failed in suppressing anti-AChR production, which increased during Cy-A therapy. Results observed in this case are in disagreement with literature data on the subject.