Antonio Alberto Zambon

Treatment of cervical dystonia with botulinum toxin in a patient with myasthenia gravis

We report the case of a 49-year-old woman who has the rare combination of myasthenia gravis and cervical dystonia. She was treated with botulinum toxin type A with good response and no evidence of deterioration of the myasthenic symptoms. We therefore conclude that it is possible to use botulinum toxin in the presence of defective neuromuscular transmission.

Myasthenia gravis and thymoma: evaluation of 41 patients

We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36%) were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.

Tratamento da miastenia grave mediante imunossupressão medicamentosa não esteróide

Fourteen patients (twelve of them were women) with severe myasthenia gravis who had not responded to any treatment at all, were treated by cytostatic drugs. Both azathioprine and cyclophosphamide single or in combination are used. All patients except two were thymectomized and almost all were treated by plasmapheresis and corticosteroid before and during the cytostatic treatment. All patients take cholinesterase inhibitors. No serious complications were observed. The patients received 100-200mg of azathioprine and/or 100-200mg of cyclophosphamide daily by oral route, the first during 20 months and the second one during 6 months. Three patients received cyclophosphamide lg daily by venous rute during 6 months at 15-20 days intervals. The preliminary results were favourable occurring important improvement in 71.4% of the cases. Their performance increased considerably. The progress of the disease was generaly stabilized. Exacerbations and crises did not reccur, except in one case. Only three patients had not responded to the treatment, one responded poorly but better later and one had an exacerbation after the 24o month. This patient returned to azathioprine.

Doenças da tireoide e miastenia grave

In a group of 304 myasthenic patients 15 cases with thyropathies were reported: nine with hyperthyroidism, one with hypothyroidism and five with nontoxic goiter. Four patients presented diffuse simple goiter and one a multinodular goiter with normal thyroid function. No patient came from an endemic goiter region, not even familial goiter. The prevalence and influence of hyperthyroidism on myasthenic symptomatology were studied. Our findings suggest that there is no clinical correlation between both myasthenia symptomatology and thyroid dysfunction, neither significant influence on myasthenic symptoms when the endocrine disorders improve.Os autores estudam o comportamento da tireoide em um grupo de 304 pacientes com miastenia grave, constatando 15 casos de tireopatias, sendo 9 de hipertireoidismo, um de hipotireoidismo e 5 de bocio sem alteracoes da funcao tireoidiana. Nenhum paciente era proveniente de regiao de bocio endemico e nao havia bocio familiar. A tireotoxicose, que foi a disfuncao mais frequente, foi estudada quanto a sua prevalencia em pacientes miastenicos, quanto a sua influencia sobre os sintomas da miastenia grave e quanto a epoca do seu aparecimento.

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.

Bilateral traumatic avulsion of abducens nerve

DISCUSSION Cavernoma is a benign tumor and it is considered a dysplasia of the vessels-forming mesoderm. Cavernous hemangiomas in the vertebral, extradural, intradural extramedullary and intramedullary spaces are responsible for 3 to 16% of spinal vascular anomalies. Extradural cavernous hemangioma represent 4% of all spinal epidural lesions. Modern diagnostic imaging techniques are increasing the number of diagnosis and its frequency may be more than previously reported in the medical literature. There are four clinical syndromes described: slow and progressive spinal cord syndrome, which is the most common form; acute spinal cord syndrome; back pain; and radiculopathy. Imaging diagnostic exams such as spine X-rays, myelography, CT and MRI are important for evaluating the relationship of the lesion with the surrounding anatomic structures. Currently, MRI is the modality of choice. The treatment for these lesions is total removal of the tumor with microsurgical technique. REFERENCES 1. Hatiboglu MA, Iplikcioglu AC, Ozcan D. Epidural spinal cavernous hemangioma-case Report. Neurol Med Chir (Tokyo) 2006;46:455-458. 2. Goyal A, Singh AK, Gupta V, Tatke M. Spinal epidural cavernous haemangioma:a case report and review of literature. Spinal Cord 2002;40: 200-202. 3. Zevgaridis D, Buttner A, Weis S, Hamburger C, Reulen HJ. Spinal epidural cavernous hemangiomas. Report of three cases and review of the literature. J Neurosurg 1988;88:903-908. 4. Saringer W, Nobauer I, Haberler C, Ungersbock K. Extraforaminal, thoracic, epidural cavernous hemangioma:case report with analysis of magnetic resonance imaging characteristics and review of the literature. Acta Neurochir (Wien) 2001;43:1293-1297. 5. Santoro A, Piccirilli M, Bristot R, Norcia V, Salvati M, Delfini R. Extradural spinal cavernous angiomas: report of seven cases. Neurosurg Rev 2005;28: 313-319.

Tratamento conservador e timectomia na miastenia grave: resultados avaliados a curto e longo prazos

A restrospective survey of the records of 33 patients with generalized aquired myasthenia gravis treated at Hospital das Clinicas, Medical School of The University of Sao Paulo, Brazil and in private practice, with and without thymectomy, is reported. Nineteen were thymectomized and 14 non operated. Both groups were demographically homogeneous and were followed from 8 to 24 years. Parameters for evaluating the natural history of disease and other ones for a comparative analysis of the improvement or remissions were established. The influence of the individualized therapy was evaluated by McNemar test (x2); the U-Mann-Whitney was used for evalution of the populational behavior groups; variable parameter with possible influence in the useful life were studied by multivariate analysis. The difference of distance between both groups was not significant: (D2= 0.08894); T2 -7.17 (Hottelling test). There were differences of the isolate clinic parameter responses in both groups, but the global analysis of the parameters did not permit to discriminate them, possibly because of interaction of the parameters influencing the analytic result. Inspite of the fact that superposition of the parameter occured when analysed for a long period, we believe the early thymectomy to be benefic. Our patients who had their operation at a younger age and specially those with recent disease had a maximum improvement.

Cisto neuroenterico: registro de um caso

E relatado caso de doente do sexo masculino, com 15 anos de idade, apresentando compressao da medula espinal em nivel toracico alto (T-2), causada por cisto enterico. A natureza do processo foi determinada na laminectomia de T-1 - T-3 e o diagnostico etiologico foi confirmado pelo exame histologico. Apos a cirurgia o paciente melhorou progressivamente das desordens motoras.The case of 15 years old boy with an enterogenous cyst causing compression of the spinal cord at T-2 is reported. The diagnosis was made by thoracic laminectomy and confirmed by histological examination. The motor disorders improved progressively after surgery.

Doenças e síndromes associadas à miastenia grave em um grupo de 304 pacientes

Epidemiologic and clinic studies of both diseases and syndromes associated in a group of 304 myasthenic patients were made. A comparative projection between the present cases and those of other authors was presented.

Polirradiculoneurite recidivante: relato de dois casos.

Two cases of patients with relapsing polyradiculoneuropathy with high protein level in cerebrospinal fluid are reported. The immunological features and the natural history of the relapsing polyradiculoneuropathy are discussed. This disease is considered a particular auto-immune nosologic condition, independent from acute polyradiculoneuropathy.Two cases of patients with relapsing polyradiculoneuropathy with high protein level in cerebrospinal fluid are reported. The immunological features and the natural history of the relapsing polyradiculoneuropathy are discussed. This disease is considered a particular auto-immune nosologic condition, independent from acute polyradiculoneuropathy.