Revista de neurologia
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Revista de neurologia · Feb 2001
Review[Nemaline congenital myopathy:clinical features and histopathological findings in nine patients].
Nemaline myopathy is a type of congenital myopathy which presents with hypotonia, muscle weakness which is predominantly proximal, lax ligaments, areflexia and skeletal deformities. It is characterized by the presence of intrasarcolemal or intranuclear rods which can be seen with the red color optical microscope using the Gomori technique, and a defect in the Z line of the sarcomere, detected on electron microscopy (EM). ⋯ There are no clinical features which permit distinction from other forms of congenital myopathy, so muscle biopsy is necessary for diagnosis. There is great phenotype and prognostic variety in this disorder.
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Chronic intoxication due to carbon monoxide (CO) is a condition in which the frequency is underestimated since the clinical features are nonspecific. It is important since serious neurological sequelae may result: an extrapyramidal syndrome, dementia or a vegetative state. The diagnosis is established when there are venous blood levels of carboxyhemoglobin greater than 10%. Early treatment with hyperbaric O2 rapidly improves the symptoms and avoids the development of neurological sequelae. ⋯ Occult CO intoxication causes headache which often requires differential diagnosis from psychiatric disorders and episodes of migraine. In patients with refractory nonspecific headache, irregular course and systemic symptoms, usually in winter, CO intoxication should be considered to be a possible cause. Diagnosis is based on finding venous blood levels of carboxyhemoglobin of over 10%. Early treatment avoids lesion of the globus pallidus and irreversible extrapyramidal sequelae.
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To review current concepts regarding the management of severe closed head injury and determine the possible causes of brain damage in these patients. ⋯ All patients with closed head injuries should be evaluated and treated as emergencies, and admitted to a critical care unit to avoid secondary lesions such as hypoxemia, arterial hypotension and cerebral edema. Treatment should be aimed at maintenance of the intracranial pressure and cerebral perfusion within normal limits and also maintenance satisfactory jugular saturation.
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To review the basic concepts and mechanisms of cerebral edema and to analyze current management of this complex clinical problem. ⋯ All patients with acute cerebral edema should be evaluated early and treated with therapy which will solve the underlying pathological problem. Good medical management of these patients is of vital importance to assure a satisfactory clinical outcome. Recently new, effective therapy has been evaluated which may substantially change current concepts of the treatment of patients with acute cerebral edema.