Amyotrophic lateral sclerosis.
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Amyotrophic Lateral Sclerosis↗
Biomedical subjects
R M Pascuzzi
Publications and source records attributed to R M Pascuzzi.
Lymphorrhage localized to the muscle end-plate in myasthenia gravis.
Lymphocytic infiltration of muscle (lymphorrhage) is occasionally observed in myasthenia gravis. We describe a 56-year-old woman with autoimmune myasthenia gravis, whose muscle biopsy specimen demonstrates acute cellular reaction localized to the end-plate region. This observation raises the possibility of a more direct role of the cellular immune system in the pathogenesis of the disease, and provides a morphologic link between the human and the experimental myasthenia gravis.
Biopsy↗
The prevalence of electrophysiological and immunological abnormalities in asymptomatic relatives of patients with myasthenia gravis.
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Adolescent↗
Granulomatous inflammatory myopathy associated with myasthenia gravis. A case report and review of the literature.
A 76-year-old woman with a history of granulomatous pulmonary disease presented with myasthenia gravis involving ocular, pharyngeal, and proximal extremity weakness. She responded to prednisone therapy but three years later developed predominantly distal appendicular weakness. Reinvestigation demonstrated a granulomatous inflammatory myopathy.
Adult↗
Familial autoimmune myasthenia gravis and thymoma: occurrence in two brothers.
At ages 31 and 42 years, two brothers presented with clinical, pharmacologic, electrophysiologic, and immunologic characteristics of autoimmune myasthenia gravis. At thymectomy, both had histologic findings of epithelial thymoma. HLA analysis revealed A2, A3, B7, and B39 antigens in one patient and A3, A24, B7, and B40 antigens in the other. Familial myasthenia gravis with thymoma has not been described previously. Familial thymoma has been rarely reported, but never with myasthenia gravis.
Adult↗
Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients.
One hundred sixteen patients, aged 8 to 82 years, with myasthenia gravis were treated with prednisone, 60 to 80 mg daily, until the onset of improvement, followed by lower-dose alternate-day therapy of several years' duration. Of all patients, 80.2% achieved either remission (27.6%) or marked improvement (52.6%). Moderate improvement occurred in 14.7%, and 5.2% showed no improvement. Increasing age correlated with a favorable outcome, but sex, duration of illness prior to treatment, severity and distribution of weakness at the time of onset of treatment, and presence of thymoma were not factors in the response to therapy.
Adolescent↗
The time course of creatine kinase elevation following concentric needle EMG.
The electromyographic (EMG) exam and creatine kinase (CK) are commonly used to evaluate patients with neuromuscular diseases. Prior studies have demonstrated minimal elevation of CK following monopolar needle examination, indicating that a false positive elevation does not occur in normal individuals. We studied the magnitude and time course of CK change resulting from concentric (CNEMG) and monopolar (MNEMG) needle EMG examination on healthy volunteers. CK increased in all subjects following CNEMG and MNEMG. Statistically significant mean CK elevations over baseline were observed at 4, 8, 12, and 24 hours for CNEMG and at 12 and 24 hours for MNEMG. Maximum elevation occurred at 12 and 24 hours for both CNEMG (mean, 178% of baseline) and MNEMG (mean, 134% of baseline). CK returned to baseline by 48 to 72 hours. In 25-30% of subjects CK elevation significantly exceeded the normal range. The magnitude of CK elevation is greater and the time course more prolonged than previously reported. CK measurements should be avoided 4 to 48 hours following CNEMG because of the potential for false positive results. Measurements immediately after and at 72 hours are not affected by the CNEMG or MNEMG. The time course for CK change following MNEMG is similar to CNEMG, but the magnitude is less.
Adult↗
Subdural hematoma in the setting of internal carotid artery occlusion: CT studies.
A 72-year-old woman with previously symptomatic internal carotid artery occlusion subsequently presented with an acute hemispheric deficit. The patient was initially diagnosed as having an ischemic infarction but eventually was shown to have a subacute subdural hematoma with delay in surgical evacuation and a major permanent deficit. We speculate that internal carotid artery occlusion may predispose the patient to greater morbidity from subdural hematoma. Patchy hemispheric infarction may reflect greater vulnerability to extrinsic hemispheric compression in the setting of carotid occlusion. This case illustrates the necessity for vigorous pursuit of the diagnosis of subdural hematoma in patients presumed to have transient ischemic attacks or acute cerebral infarction.
Aged↗
Chronic focal seizure disorder as a manifestation of intracranial iophendylate.
A 46-year-old woman developed focal seizures 10-15 years following iophendylate myelography. Focal epileptogenic abnormalities on electroencephalogram corresponded to the localization of residual iophendylate in the right sylvian fissure. Intracranial iophendylate may have produced chronic meningeal reaction leading to cortical irritation and a chronic seizure disorder.
Brain↗