Journal of the neurological sciences
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We report a case of motor neuron disease in which fasciculations and cramps progressed generally before the development of muscle wasting. After involvement of the upper and lower motor neurons became clinically manifest, widespread fasciculations and cramps persisted and accompanied pseudotetany. The present case suggests that spinal cord pathology of motor neuron disease can cause the abnormal excitability of the motor neurons, resulting in the development of generalized fasciculations and cramps.
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Comparative Study
Simultaneous bilateral contrast transcranial doppler monitoring in patients with intracardiac and intrapulmonary shunts.
The prevalence of a right-to-left intracardiac shunt, demonstrated by echocardiography and transcranial Doppler sonography has been shown to be higher in stroke patients than in normal controls. The aim of this study was to assess the sensitivity and specificity of contrast transcranial Doppler sonography in comparison to transesophageal echocardiography in the detection and differentiation of intracardiac and intrapulmonary shunts and to correlate the transcranial Doppler findings with clinical outcome and morphological findings. Forty five consecutive stroke patients with suspected paradoxical embolism were entered into the study. ⋯ There was no correlation between the side and numerical distribution of microbubble count and the location and severity of the current clinical symptoms, as well as between microbubble count and presence and hemispherical distribution of brain infarcts. Transcranial Doppler sonography is a highly sensitive method for the detection of right-to-left shunts, whether of cardiac or pulmonary location. However. no correlation was found between the side and number of microbubbles counted and the clinical symptomatology.
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Clinical Trial Controlled Clinical Trial
Hemorheologic profiles of plasma fibrinogen and blood viscosity from silent to acute and chronic cerebral infarctions.
Hemorheologic changes from silent to acute and chronic cerebral infarction have seldom been reported. We evaluated hemorheologic profiles of the whole blood viscosity, plasma viscosity and fibrinogen level in stroke at-risk patients with silent cerebral infarction, patients with acute or chronic cerebral lacunar infarction, and subjects at low risk for stroke. Hemorheologic profiles were measured in 88 subjects: (1) 36 patients with silent cerebral infarction (mean 64.7 years), who provided no clinical history of having had definitive stroke but showing > 5 mm lesions of cerebral infarction or periventricular hyperintensity (PVH) observed in magnetic resonance imaging (MRI) T2-weighted images; (2) 12 patients with acute cerebral lacunar infarction (mean 69.1 years), measured within 3 days and repeated 1 month after onset; (3) 25 patients with chronic cerebral lacunar infarction (mean 66.2 years), measured 12.5 months after onset; and (4) 15 subjects at low risk for stroke (mean 65.8 years) without cardiovascular risk factors or lesions on MRI. ⋯ Plasma fibrinogen level persisted to be elevated up to 1 month after onset, which continued as well in patients with chronic cerebral infarction. Advanced grades of silent cerebral infarction in stroke at-risk patients are accompanied by elevations of plasma fibrinogen level, which increases further after onset of cerebral infarction; such abnormalities persist up to the chronic stage. Elevated plasma fibrinogen level might reflect progression of atherogenesis in patients with advanced grades of silent cerebral infarction, resulted in an increased probability as to be a risk factor for cerebral infarction.
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Comparative Study Clinical Trial
Pattern reversal visual evoked potentials in classic and common migraine.
Pattern reversal visual evoked potentials (PVEPs) to transient checkerboard were recorded in 19 patients with migraine with visual aura (i.e., classic migraine), 14 patients with migraine without aura (i.e., common migraine) in the interictal period and 43 normal subjects. Latencies and amplitudes of PVEPs in each group were analyzed. In classic migraine patients, P100 amplitude was significantly higher than in normal subjects (p < 0.01), whereas latencies of PVEPs did not significantly differ. ⋯ Four patients with classic migraine underwent PVEPs during or 1-2 h immediately after their migraine attacks. Two of these patients who underwent PVEPs 1.5-2 h after their attacks showed abnormally increased PVEP amplitudes. These results suggest that there are different pathophysiologies in the visual pathway between classic and common migraine and furthermore, classic migraine patients in interictal periods may have hyperexcitability in the visual pathway and that the increased amplitude of PVEPs after attacks may be due to cortical spreading depression.
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We investigated the immunohistochemical distribution of cytoskeletal proteins in smooth muscles of 15 patients with Duchenne muscular dystrophy (DMD), 8 patients with Becker muscular dystrophy (BMD), 28 patients with various neuromuscular diseases, and 2 normal controls, performing skin and muscle biopsies. Dystrophin immunostaining confirmed absent reaction in the arrector pili muscles of DMD patients, faint positive reaction in BMD patients, and strong dystrophin reaction in patients with other neuromuscular diseases and normal controls. Immunostaining of utrophin was positive with variable intensity in the arrector pili muscles in all DMD patients. ⋯ Vinculin, vimentin and desmin were expressed both in arrector pili smooth muscles and in vessel walls of patients with dystrophinopathy and other neuromuscular diseases, as well as in normal controls. Thus utrophin is normally expressed in the smooth muscle of the vessels and its expression does not vary in neuromuscular diseases. On the contrary, in the arrector pili smooth muscle utrophin is not expressed in normal controls but it is in dystrophinopathies, paralleling the findings in striated muscle, which expresses utrophin in a reciprocal manner with respect to dystrophin.