Eleven patients with demyelinating polyneuropathy associated with monoclonal IgM antibodies were randomized to receive IVIg or placebo, monthly, for 3 months in a double-blind study. After a washout period, they crossed over to the alternate therapy. Response was gauged by evaluating muscle strength, sensation, and neuromuscular symptoms at baseline, after 3 months, and at treatment's end. After IVIg therapy, the strength improved in only 2 of 11 patients, by 28 and 38.5 points from baseline, and declined after placebo. In 1 other patient, the sensory score improved by 13 points. Antibody titers to MAG/SGPG or gangliosides did not appreciably change. We conclude that IVIg has only a modest benefit to not more than 18% of patients with IgM paraproteinemic demyelinating neuropathy.
Neurological complications occurred in 4 (19%) of 21 consecutive patients (Group II) undergoing orthotopic liver transplantation, compared with a 47% (9/19) incidence in our initial series (Group I). In Group II, the neurological problems included new recurrent headaches and delayed intracerebral hemorrhage (1 patient), partial third nerve palsy and brachial plexopathy (1 patient), and ataxic dysarthria with encephalopathy (2 patients). Seizures, noted in 8 of 9 neurologically affected Group I patients, were not encountered in Group II. Of the 4 patients in the entire series with the cerebrocerebellar syndrome, 2 had partial recovery after stopping treatment with cyclosporine, 1 stabilized when cyclosporine was discontinued but later worsened when rechallenged, and 1 had full recovery but died following a second transplantation. Brain magnetic resonance images appeared normal in 3 of the 4 patients. Complications affecting vision included cortical blindness in 2 patients and occipital lobe hemorrhage in 1. All completely recovered. Survival was comparable for patients with and those without neurological complications (69% and 63%, respectively). Immediate withdrawal of cyclosporine at the onset of a change in mental status or dysarthria and improvement in intra- and postoperative management may have contributed to the decreased incidence of neurological complications.
Because amantadine has been shown to reduce fatigue in patients with multiple sclerosis, we performed a double-blind, placebo-controlled study to assess its efficacy in the disabling symptom of post-polio fatigue. Twenty-three patients completed six weeks of therapy. Fatigue was measured by the patients using visual analogue scales (twice per day) and numerical fatigue severity scales (once per week) and by overall impression (at end of therapy). Formal neuropsychological testing and serum drug levels were performed to assess compliance. On all measures, no significant difference was found between treatment and placebo groups. Fifty-four percent of patients given amantadine and 43% given placebo reported a decrease in fatigue; however, the visual analogue scales and fatigue severity scales failed to reflect any improvement. Several patients in the treatment group elected to continue amantadine therapy after the study was completed. Our findings suggest that amantadine is not significantly better than placebo in reducing the sensation of fatigue in post-polio syndrome, and that the measures we employed were insensitive to capture the subjective response experienced by a few patients.
The sparse literature concerning the psychology of PPS is easy to summarize. Some psychopathology can be observed in PPS, but it is not clear whether it is a reaction to PPS or a by‐product of a concurrent but unrelated disorder. Psychopathology does not seem to be responsible for the onset or intensity of new PPS symptoms. A relationship may exist between the perception of stress, an inability to handle stress, and perceived fatigue in PPS. Neuropsychological studies have been equivocal with patients performing well on some tests and more poorly on others compared to controls. An attempt has been made to link reticular formation and hypothalamic involvement in PPS to decreased attention and arousal and increased fatigue. Higher levels of fatigue have been associated with the presence of hyperintensities on MRI scanning. Unfortunately, a variety of methodological problems are present with most of these studies and indicate that caution should be used in their interpretation (see section on Methodological Considerations).
Future studies should adapt more stringent controls in subject selection so that non‐PPS factors that might affect behavior or cognition can be factored out (e.g., hypertension or exogenous depression). Tests specifically designed to evaluate components of attention and motor control can be used to test hypotheses regarding fatigue, arousal, and attention. Our experience is that patients carefully selected to have pure PPS without concurrent medical disease have few complaints about cognition or altered mood. Before we pronounce neuropsychological deficits in PPS patients, more carefully designed studies are required.
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