Short latency inhibition of human hand motor cortex by somatosensory input from the hand.

1. EMG responses evoked in hand muscles by transcranial stimulation over the motor cortex were conditioned by a single motor threshold electrical stimulus to the median nerve at the wrist in a total of ten healthy subjects and in five patients who had electrodes implanted chronically into the cervical epidural space. 2. The median nerve stimulus suppressed responses evoked by transcranial magnetic stimulation (TMS) in relaxed or active muscle. The minimum interval between the stimuli at which this occurred was 19 ms. A similar effect was seen if electrical stimulation was applied to the digital nerves of the first two fingers. 3. Median or digital nerve stimulation could suppress the responses evoked in active muscle by transcranial electrical stimulation over the motor cortex, but the effect was much less than with magnetic stimulation. 4. During contraction without TMS, both types of conditioning stimuli evoked a cutaneomuscular reflex that began with a short period of inhibition. This started about 5 ms after the inhibition of responses evoked by TMS. 5. Recordings in the patients showed that median nerve stimulation reduced the size and number of descending corticospinal volleys evoked by magnetic stimulation. 6. We conclude that mixed or cutaneous input from the hand can suppress the excitability of the motor cortex at short latency. This suppression may contribute to the initial inhibition of the cutaneomuscular reflex. Reduced spinal excitability in this period could account for the mild inhibition of responses to electrical brain stimulation.

[Acute isolated levator palpebral myositis].

A 17-year old man developed acute onset blepharoptosis of the right upper eyelid with swelling. On examination, the patient exhibited right blepharoptosis without limitation of eye movement. MRI revealed swelling of right levator palpebral muscle. Oral steroid administration led to complete resolution of the blepharoptosis within 3 weeks. Isolated levator palpebral myositis is a rare disease, but, it is important for differential diagnosis on patients with blepharoptosis.

Speech-induced changes in corticospinal excitability.

The aim of the experiments was to investigate the effects of speech on the excitability of corticospinal pathways to human hand muscles. Single transcranial magnetic stimuli were given randomly over the hand area of either the left or right motor cortex of 10 right-handed and 3 left-handed normal volunteers. Electromyographic responses were recorded in the relaxed first dorsal interosseous muscle while the subjects (a) read aloud a piece of text, (b) read silently, (c) spoke spontaneously, or (d) made sounds without speaking. The only consistent effect across subjects occurred during task a, which significantly increased the size of responses evoked in the dominant hand of all subjects, but had either no effect (8 subjects) or a smaller effect in the nondominant hand. Tasks b and d had no reliable effect, whereas task c tended to increase response size in both hands. Control measurements suggest that the effects in task a were caused by changes in cortical rather than spinal excitability. This is the first demonstration of lateralized speech effects on the excitability of cortical arm areas. The results provide a useful adjunct to other tests of cerebral dominance, using only single- rather than repetitive-pulse cortical stimulation.

Short latency facilitation between pairs of threshold magnetic stimuli applied to human motor cortex.

Pairs of threshold magnetic stimuli were applied over the motor cortex at interstimulus intervals of 1-6 ms, and EMG responses recorded from the relaxed or active first dorsal interosseous muscle of 7 normal subjects. In relaxed subjects, when the interval between the stimuli was around 1.0-1.5 ms, 2.5-3.0 ms or 4.5 ms or later, the size of the response to the pair of stimuli was much greater than the algebraic sum of the response to each stimulus alone. During contraction, fewer peaks of facilitation were observed. Facilitation was evident if the stimuli were 0.9-1.1 times threshold in the relaxed state, and 1.0-1.1 times threshold during voluntary contraction. Experiments using either magnetic followed by anodal electric stimulation, or pairs of anodal electric stimuli, suggested that the facilitation most likely occurred within the cerebral motor cortex. Given the timings at which facilitation is prominent, it seems likely that it reflects interactions between circuits normally responsible for production of I-waves.

Study of lower limb somatosensory evoked potentials in 96 cases of HTLV-I-associated myelopathy/tropical spastic paraparesis.

Lower limb somatosensory evoked potentials (LSEPs) were performed along with neurological evaluation in 96 HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients. The N20 latencies were abnormal in 5 cases. Central sensory conduction time (CSCT) was abnormal in 42 cases. A highly significant correlation (p < 0.001, r = 0.418) was found between CSCT and disability score. Such a correlation was not found between CSCT and other clinical findings, onset of illness, illness duration, serum and CSF antibody titer to HTLV-I and vibratory sensation. There was no difference of the mean of CSCT between the cases with sensory impairment and those without it. This shows that LSEPs are useful to estimate the disability of HAM/TSP. Also the cases with delayed CSCT and normal sensation suggest that LSEPs are capable of detecting subclinical lesions of the spinal cord in HAM/TSP.

Treatment of cerebrotendinous xanthomatosis: effects of chenodeoxycholic acid, pravastatin, and combined use.

Treatments by oral administration of chenodeoxycholic acid (CDCA) alone, 3-hydroxy-3-methylglutaryl (HMG) CoA reductase inhibitor (pravastatin) alone, and combination of the two drugs were attempted for 7 patients with cerebrotendinous xanthomatosis (CTX). CDCA treatment at a dose of 300 mg/day reduced serum cholestanol (67.3% reduction), lathosterol (50.8%), campesterol (61.7%) and sitosterol (12.7%). However, the sera of the patients changed to be "atherogenic"; total cholesterol, triglyceride and low-density lipoprotein (LDL)-cholesterol were increased, while high-density lipoprotein (HDL)-cholesterol was decreased. Contrarily, pravastatin at a dose of 10 mg/day improved the sera of the patients to be markedly "anti-atherogenic", but the reductions of cholestanol (30.4%), lathosterol (44.0%), campesterol (22.9%) and sitosterol (9.6%) were inadequate. Combined treatment with CDCA and pravastatin showed good overlapping of the effects of each drug alone. The sera of the patients were apparently more "anti-atherogenic" than those after CDCA treatment. Serum cholestanol concentration was still 2.7 times higher than in controls, but the serum lathosterol level was within the normal range, indicating that the enhancement of overall cholesterol synthesis in the patients was sufficiently suppressed. Plant sterol levels were also within the normal range. The combination of CDCA and pravastatin was a good treatment for CTX, based on the improvement of serum lipoprotein metabolism, the suppression of cholesterol synthesis, and reductions of cholestanol and plant sterol levels. In all of 7 patients, the progression of disease was arrested, but dramatic effects on clinical manifestations, xanthoma, and electrophysiological findings could not be found after the treatment of these drugs.

Transcranial magnetic stimulation excites the root exit zone of the facial nerve.

The actual site of excitation of the facial nerve by transcranial magnetic stimulation was investigated in five patients with hemifacial spasm who underwent microvascular decompression. The facial nerve was stimulated preoperatively and intraoperatively by transcranial magnetic stimulation and intraoperatively by electrical stimulation at its root exit zone with a minimum of surgical invasion of the facial nerves. The onset latency of compound muscle action potentials recorded from the nasalis muscle was 5.06 +/- 0.44 ms by magnetic stimulation and 5.08 +/- 0.43 ms by electrical stimulation. The latency difference was 0.06 +/- 0.08 ms. Therefore, transcranial magnetic stimulation was basically the same as electrical stimulation in onset latency. From this study, it appears that the root exit zone of the facial nerves is stimulated by transcranial magnetic stimulation.

Treatment of cerebrotendinous xanthomatosis with low-density lipoprotein (LDL)-apheresis.

We studied the effects of LDL-apheresis on the biochemical and clinical abnormalities of 5 patients with cerebrotendinous xanthomatosis (CTX). Levels of both cholestanol and cholesterol decreased to approximately 60% of those of pretreatment after one perfusion and gradually returned to their initial levels within 2 weeks. Improvement of clinical manifestations and regression of Achilles tendon xanthomas were detected after several perfusions, though dramatic changes could not be recognized. EEG abnormalities were improved immediately after LDL-apheresis in one patient. We conclude that LDL-apheresis may affect the serum cholestanol level and clinical manifestations in patients with CTX.

Electrophysiological studies in cerebrotendinous xanthomatosis.

Seven patients with cerebrotendinous xanthomatosis (CTX) were studied by electrophysiological techniques. The percentages of abnormalities detected in nerve conduction studies and electroencephalograms were 28.6% (two patients) and 100%, respectively. All patients showed prolonged central conduction times in short latency somatosensory evoked potentials (SSEPs) by tibial nerve stimulation but normal SSEPs by median nerve stimulation. Brain stem auditory evoked potentials and visual evoked potentials were abnormal in three (42.9%) and four patients (57.1%), respectively. These electrophysiological parameters were correlated with the ratio of serum cholestanol to cholesterol concentration. The results of SSEPs suggest that the polyneuropathy in CTX is caused by distal axonopathy affecting longer axons before shorter axons (central-peripheral distal axonopathy).

[Direct surgical management of aneurysms in the cavernous sinus: a report of 5 cases].

Five patients with aneurysms in the cavernous sinus were treated by direct surgical approach. Two small aneurysms were located in the C3 segment, one small aneurysm was on the primitive trigeminal artery (PTA), and the last two aneurysms were giant ones. The three small aneurysms were discovered incidentally by angiography and the two giant aneurysms presented oculomotor palsy. After ordinary fronto-temporal craniotomy, the two C3 aneurysms were treated through an intradural approach, and the PTA aneurysm and the two giant aneurysms were treated through combined epi- and subdural approach. The three small aneurysms were clipped and the two giant aneurysms were trapped with the reconstruction of ICA by saphenous veins. In all cases the aneurysms were excluded postoperatively, but in one giant-aneurysm case (Case 5) the bypass was occluded postoperatively, but the patient had no permanent neurological deficit. The four patients developed third nerve palsy and one developed fourth nerve palsy which resolved within 3 to 6 months. In one C3-aneurysm case (case i) the patient had loss of vision due to much packing in the medial side of the ICA. All patients were discharged and returned to their usual life. Three cases (case 2, 4, 6) are presented here and the problems of direct surgical management of aneurysms in the cavernous sinus are discussed with the related literature.

[Intraoperative ABR monitoring during cerebello-pontine angle surgery].

Intraoperative auditory brainstem response (ABR) monitoring was performed in 27 patients undergoing C-P angle surgery (12: hemifacial spasm, 10: trigeminal neuralgia, 2: glossopharyngeal neuralgia, 3: C-P angle tumor, 2 epidermoid, 1 meningioma). Because of the introduction of this method, no patient suffered from postoperative hearing disturbance in this series. During C-P angle surgery, the wave V of ABR changes according to the retraction of the cerebellum and the manipulation of the eighth cranial nerve. Many authors have discussed this change, however the timing and the mechanism of disappearance of wave V is unclear. Accordingly, the authors discussed the correlation between the prolongation of wave V latency and its amplitude. The wave V amplitude was measured from the positive peak of wave V to the next negative peak. Then, the correlation between the prolongation of wave V latency and its reduction ratio (%) of amplitude was represented as a parabola. The wave V reduces its amplitude when the prolongation of the latency is from 1.5 ms to 2.0 ms. Once the prolongation of the latency is over 1.5 ms, the amplitude of wave V seems to be reduced suddenly, because it takes over 1'30'' to finish each record. But the authors demonstrated the gradual reduction of the amplitude of wave V in Figure 3. As mentioned above, the prolongation of wave V latency must be less than 1.5 ms, and the neurosurgeon must recognize this turning point during C-P angle surgery.