Abstract：
:The clinical picture, family history, laboratory data, treatment modalities, and outcome in 27 juvenile myasthenics seen over a 25-year period are presented. Onset was after 10 years of age in the majority of patients. Half presented with ocular signs, the other half with generalized-onset myasthenia. Half of those with ocular-onset myasthenia progressed to generalized myasthenia. The female-to-male ratio was 3.5:1. Myasthenia gravis was reported in the mother of one patient. Ptosis was the most common presenting sign. It was unilateral at onset in 33% of patients and remained unilateral in 11%. Pharmacologic tests (neostigmine methylsulfate and edrophonium chloride) were positive in 92% of patients. Serology was positive in 63%, whereas repetitive nerve stimulation was positive in 33% when distal nerves were stimulated and in 66% when proximal and distal nerves were stimulated. Seropositivity tended to increase with generalization of the myasthenic process. No statistically significant difference in seropositivity was noted between males and females. Anti-striated muscle antibodies were detected in two patients, neither of whom had thymoma. The yield of repetitive stimulation increased with generalization of the myasthenic process and when proximal nerves were stimulated. No statistically significant difference was noted in the decremental response between seropositive and seronegative patients. The majority of ocular myasthenics were treated with pyridostigmine bromide monotherapy. With generalization of the myasthenic process, other modes of medical and surgical therapies were needed. All patients given corticosteroids ultimately underwent thymectomy. The mean age at presentation and the duration of symptoms at presentation were longer in thymectomized patients than in those without thymectomy.(ABSTRACT TRUNCATED AT 250 WORDS)
journal_name
J Child Neuroljournal_title
Journal of child neurologyauthors
Afifi AK,Bell WEdoi
10.1177/088307389300800422subject
Has Abstractpub_date
1993-10-01 00:00:00pages
403-11issue
4eissn
0883-0738issn
1708-8283journal_volume
8pub_type
杂志文章,评审abstract：:Using resting state functional magnetic resonance imaging (MRI), we aim to understand the neurologic basis of improved function in children with hemiplegic cerebral palsy treated with constraint-induced movement therapy. Eleven children including 4 untreated comparison subjects diagnosed with hemiplegic cerebral palsy...
journal_title：Journal of child neurology
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journal_title：Journal of child neurology
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journal_title：Journal of child neurology
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journal_title：Journal of child neurology
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journal_title：Journal of child neurology
pub_type： 杂志文章,多中心研究
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journal_title：Journal of child neurology
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journal_title：Journal of child neurology
pub_type： 杂志文章,评审
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journal_title：Journal of child neurology
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pub_type： 临床试验,杂志文章,随机对照试验
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