This paper describes effects of therapeutic electrical stimulation(TES) on progressively deteriorating muscles in 28 patients with amyotrophic lateral sclerosis (ALS). A multichannel percutaneous FES system which we developed was used in order to apply cyclic stimulation to the affected muscles automatically. Within one or two weeks TES therapy, many patients showed an improvement of voluntary motion due to afferent effects of TES. Long-term TES therapy more than 6 months resulted in the increment or maintenance of muscle strength in some of these patients. CT findings of the TES treated muscles in these patients showed an increase in muscle density and a decrease in moth-eaten image. Thus it is likely that TES is effective for the treatment of ALS.

This paper describes the effects of therapeutic electrical stimulation (TES) on the wasting muscles in a patient with amyotrophic lateral sclerosis. The patient is a 47-year-old male, and he has a history of progressive muscle weakness and atrophy, affected more in the right side. Percutaneously indwelling intramuscular electrodes were implanted to the affected muscles in the right upper and lower extremities but no electrode in the corresponding left region. Within a month of TES therapy, a rapid improvement of extremity motion appeared in the TES treated side. Long-term application of TES more than 3 months increased the strength of the muscle which had been evidently weaker than the non-treated side. CT findings of both the upper and lower extremities with TES therapy showed an increase in the density and a reduction in the moth-eaten image. An increase in the thickness of the muscles was also observed in the TES treated side while deterioration was observed in the muscles on the non-treated side.

The EEG was evaluated in three cases of hydranencephaly and two cases of maximal hydrocephalus, and the visual evoked potential was studied in one case each of hydranencephaly and maximal hydrocephalus. The EEG tracing of hydranencephaly typically showed a flat pattern in most bipolar derivations because of the differential amplification of the same activity from remote generators. However, the EEG of maximal hydrocephalus did not demonstrate a flat pattern in bipolar as well as referential derivation, and the activities were different from various electrodes in referential derivation. The visual evoked potential of maximal hydrocephalus showed a normal pattern, while that of hydranencephaly showed no response. Electrophysiological examinations (such as EEG and visual evoked potential) are useful for differentiation of hydranencephaly and maximal hydrocephalus in cases whose computed tomographic scans do not provide clear differentiation. ( J Child Neurol 1989;4:114-117).

An 8 year-old-boy with adrenoleukodystrophy (ALD) was subject to an x-ray computed tomography (CT) scan, magnetic resonance imaging (MRI), positron emission tomography (PET) using 18F-fluorodeoxy glucose and evoked potential studies, about 3 months after the onset of neurologic symptoms. A CT showed decreased radiodensity over the white matter of both occipital lobes, and high density areas on the periventricular space. No contrast enhancement was observed around the low dense white matter, while occipital grey matter seemed to be almost normal. MRI demonstrated a larger and more obvious lesion of prolonged T2 relaxation time which was 170-250 msec than that detected by CT scan. The PET demonstrated wide and severe hypometabolism for glucose over the grey and the white matter of bilateral occipital lobes. The visual evoked potential (VEP) was scarcely elicitable, while the auditory brainstem response (ABR) and short latency somatosensory evoked potential (SSEP) was normal. These imaging studies suggest that extensive and profound demyelination exists in the occipital white matter. For detection of demyelinizing process, MRI may be more effective than CT in this disorder. The PET study revealed that there may have been an abnormality of the grey matter as well as the white matter in the occipital lobe. The VEP disappeared in the relatively early stage. The brainstem may not be involved at such early stage in light of normal ABR and SSEP.

We have developed a motion analyzing system with eight force plates for upper extremities in standing FES and measured the efforts of upper extremities of three complete paraplegics with FES using percutaneous electrodes. At vertical direction, there were small reaction forces of upper extremities, but, at anterior and posterior direction there were efforts for correction of body balance. The reason why patients couldn't leave their hand off the parallel bar in FES standing is that small horizontal force are needed. .