We describe three patients with chronic progressive polyneuropathy associated with IgA monoclonal gammopathy. Two patients had a prominent sensory neuropathy and one had a prominent motor neuropathy. ...Sural nerve biopsies showed axonal degeneration in all cases. In immunocytochemical studies patients' IgG immunostained axons. By Western immunoblot a band of IgG reactivity with an axonal protein of 66 kDa was found. No band of IgA and IgM were found. We suggest the possibility that the IgA monoclonal protein may act as a stimulating factor of preexisting B cell clones eliciting an immune reaction against nerve antigens.
In order to evaluate whether the results of ultrasound examination may be associated with 30-day outcome, 76 consecutive patients (43 men and 33 women; mean age 68.1 +/- 8.9 years) underwent duplex ...scanning of the carotid bifurcations and transcranial doppler investigation of the basal skull arteries within the first few hours of the onset of an acute carotid stroke. Forty-three patients (56.6%) had appropriate arterial occlusion at ultrasounds examination. On day 30, 22 patients (28.9%) were self-sufficient, 41 (53.9%) were disabled and 13 (17.1%) were dead. The chi-squared test showed that the ultrasound results were significantly related to 30-day outcome (p = .0003). After logistic regression analysis, the ultrasound results remained independent predictors of 30-days outcome (p = .0129), together with neurological impairment 24 hours after stroke onset and lesion size at control computed tomography. Our study suggests that the results of ultrasound examination may be useful in the management of acute carotid stroke as an early indicator of patients with a worse prognosis.
Somatosensory evoked potentials from median nerve stimulation were recorded in 12 patients with newly diagnosed epilepsy, before and after 1 year of treatment with carbamazepine. The plasma ...concentrations of the drug were consistently within therapeutic range. We assessed the latencies of the early components at the level of the cervical spine (N9 and N13) and on the parietal scalp (P14, N20, P25) and the interpeak latencies (N9-N13, N13-N20, P14-N20). None of the patients presented anomalies in any of the parameters, and there was no significant difference between the patient and control means or between the patient means before and after 1 year of treatment.
We sought to detect prognostic factors related to functional outcome during the first 6 hours after a first-ever stroke in the carotid artery territory.
All patients with these characteristics seen ...during a 3-year period were included. Outcome was evaluated according to a modified Rankin scale. The following variables were examined at univariate analysis: sex, age, severity of deficit at entry and at day 7, level of consciousness at entry, time after symptom onset, history of smoking, history of hypertension, diabetes, myocardial infarction, atrial fibrillation, rheumatic heart disease, dilated cardiomyopathy, all potential cardioembolic sources, presence of a consistent lesion on computed tomography at entry and at days 5-9, and the size of such lesion.
All entry criteria were met by 172 patients. Age > or = 70 years, a Canadian Neurological Scale score < 6.5 at entry and at day 7, atrial fibrillation, presence of a potential cardioembolic source, and a "large" lesion (involving more than half the cerebral lobe) on computed tomography at days 5-9 were associated with a significantly worse outcome both at 30 days and at 6 months. After multivariate analysis, a Canadian Scale score < 6.5 at entry (p < 0.0001) and atrial fibrillation (p = 0.005) were associated with a significant handicap or death at 30 days, whereas only a Canadian Scale score < 6.5 (p < 0.0001) was associated with a worse prognosis at 6 months. An association of age > or = 70 years with a worse outcome at 6 months was of borderline significance (p = 0.054).
Some prognostic indicators are available during the first few hours after onset of a carotid ischemic stroke and may be useful in the stratification of patients in clinical trials. Severity of deficit is the most important indicator, whereas the presence of atrial fibrillation worsens the prognostic outlook with respect to early handicap but not mortality.
We have observed 9 patients (8 men and 1 woman), 58 to 77 years of age with neuropathy with only sensory symptoms and insidious onset. Five of them (4 men and 1 woman) aged 65 to 77 years, had normal ...serum electrophoretic profiles, while the others (all men), 58 to 74 years, had IgM monoclonal gammopathy of undetermined significance (MGUS). Clinical data were consistent with a sensory neuropathy affecting predominantly the kinesthetic sense (position and vibration sensation). The electrophysiological data indicated predominant sensory axonal neuropathy. Morphological data confirmed the primary axonal damage. Western immunoblot showed that the IgG from a patient without MGUS reacted with a 55 kD protein of dorsal root ganglion homogenate. Three of four patients with IgM MGUS were serum reactive against chondroitin sulfate C (ChS-C) in double immunodiffusion. After absorption with ChS-C the monoclonal peak completely disappeared from two patients and was decreased in the third patient. Our data indicate that immunological abnormalities are part of the pathogenesis for a subgroup of chronic neuropathy with only sensory symptoms.
Essential thrombocytemia (ET) is a clonal myeloproliferative disorder of unknown cause, characterized by an increased number of apparently normal platelets. It has been related to a large number of ...mild ischemic complications, but rarely to acute stroke associated with documented large vessel thrombosis. We report the cases of 6 patients with acute ischemic stroke associated with ET (4 men and 2 women; mean age 61.7: range 49-78 years). The diagnosis of ET followed the onset of the stroke. The number of platelets was never greatly increased at the time of the stroke, and two patients presented no major risk factors for stroke. The involvement of the posterior circulation was observed in three patients, that of the anterior circulation in two patient and that of the border territory in one. The obstruction of large intracranial or extracranial vessels was detected in all of the patients, and all of them were treated with antiplatelet agents (aspirin or ticlopidine). The outcome was good in 3 patients, fair in one and bad in two. We retain that ET might be a cause of acute ischemic stroke as a result of large vessel obstruction.
A deficit of nigrostriatal, mesocortical and mesolimbic dopamine systems in Parkinson's disease is well known. We know less about the involvement of tuberoinfundibular dopamine (TID) systems. In ...untreated (naive or wash-out) men with Parkinson's disease, we studied TID function through basal and stimulated plasma levels of growth hormone, prolactin and thyrotropin. Only minor abnormalities in prolactin responses to thyrotropin-releasing hormone were found, probably reflecting denervation hypersensitivity. TID function is preserved in men with Parkinson's disease.
To elicit possible variations in the CSF concentrations of copper, iron and manganese due to Parkinson disease (PD) or to the stage reached, we tested 11 patients with idiopathic PD, 6 untreated and ...5 on long term L-dopa, versus 22 age and sex matched patients with other neurological disorders (control group-CG). The CSF levels of the three metals, measured by electrothermal atomization, did not differ significantly between the PD group and CG or between either of the PD subgroups and CG. Our findings therefore do not support the hypothesis that CSF Cu is a marker of PD.
Somatosensory evoked potentials (SEPs) following median nerve stimulation were used to monitor cerebral function during 26 carotid endarterectomies. The patients with minor SEP variations had no ...neurological deficits on regaining consciousness while the one with more serious SEP variations had a transient deficit. The method thus seems useful in the early detection of ischemic brain impairment.
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