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We present a variant of a split cord malformation with coexisting segmental spinal dysgenesis. CT myelography showed the left hemicord with a small remnant of subarachnoid space running through an intravertebral cleft in a spine anomaly. The left hemicord had no apparent intradual connection to the upper cord on any radiologic examination, though functional electrical stimulation studies revealed an intact efferent pathway that connected the left hemicord to the main spinal cord.

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OBJECTIVES: To examine high frequency oscillations (HFOs) of somatosensory evoked potentials (SEPs) recorded directly from subdural electrodes to investigate the relationship between the primary somatosensory cortex and HFOs. METHODS: SEPs were recorded directly from subdural electrodes previously implanted in 3 patients for clinical evaluation prior to surgical treatment of intractable epilepsy. RESULTS: The primary sensory cortex (area 3b) was proposed as the source of somatosensory HFOs, because the distribution of HFOs recorded from the subdural electrodes agreed with the distribution of the N20-P20 components of the somatosensory evoked potential. The somatosensory HFOs showed a strictly somatotopic source arrangement. There was a polarity inversion of the prophase component and also the N20-P20 component of HFOs across the central sulcus. However, the phase was synchronized in the latter part of the HFOs. CONCLUSIONS: We propose that the origins of the early and latter parts of HFOs are different, and that there was a clear somatotopy.

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A 16-year-old boy presented with delayed, transient neurological deterioration 18 days after mild head injury. Left hemiparesis and left homonymous hemianopsia appeared after right frontal contusional and mild subdural hematomas subsided. Neuroimaging examinations including cerebral angiography, magnetic resonance imaging, and single photon emission computed tomography showed vasodilation and hyperemia in the right cerebral hemisphere. The present case is not typical of acute "juvenile head trauma syndrome," but may represent a possible pathophysiology of the delayed type of transient neurological deterioration after mild head injury.

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A 14-year-old Japanese boy presented with myelopathy due to atlas hypoplasia with complete posterior arch. Decompressive laminectomy of the atlas produced good neurological recovery, and follow-up T2-weighted magnetic resonance imaging showed disappearance of spinal cord edema. Congenital atlas stenosis may be symptomatic even in children, with no accompanying cervical spondylotic change. Such cases have previously occurred only in Asian adults. A radiological study of the patient's brother showed median cleft formation of the posterior arch of atlas, indicative of a wide spectrum of atlas anomalies and a possible genetic relationship between these anomalies.

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The authors present a case in which a cavernous sinus (CS) hemangioma was totally removed following intratumoral injection of a plastic fixation material. This unique method is extremely useful for the removal of CS hemangiomas, which often feature massive intraoperative bleeding as an unsolved problem.

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Here we report a case of bitemporal epilepsy that was well controlled after unilateral temporal lobectomy. The patient was a 31-year-old woman, who was born in an asphyxia state and had a history of a febrile convulsion. Complex partial seizure(CPS) preceded by abdominal aura appeared at the age of three. Despite anticonvulsant medication, the seizures gradually increased in frequency and sometimes developed into secondary generalized convulsions. Her scalp electroencephalogram(EEG) showed interictal spikes and seizure activities arising from the bilateral temporal lobes. No apparent lateralities in regard to the size or blood flow at the hippocampus were detected by MRI and SPECT studies. Intracranial EEG monitoring revealed that 80% of the clinical seizures originated from the left mesial temporal lobe, which was the non-dominant side for speech and memory functions as demonstrated by Wada test. Therefore, we performed a left temporal lobectomy that resulted in the complete disappearance of CPS. The present case exemplified the usefulness of intracranial EEG monitoring to clarify the dominant epileptic focus for surgery.

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A 17-year-old male presented with paroxysmal kinesigenic choreoathetosis (PKC) associated with frontotemporal arachnoid cyst. Xenon-133 single photon emission computed tomography detected a slight but equivocal decrease in regional cerebral blood flow in the vicinity of basal ganglia associated with the PKC episodes. PKC continued after surgical removal of the cyst but was well controlled by oral administration of carbamazepine. Whether the pathogenesis of symptomatic PKC was associated with the cortical lesion could not be determined in the present case.

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A 54-year-old female, who had been treated for aplastic anemia by metenolone acetate since 1981, developed a sudden unconsciousness in September 1995. On admission, she was drowny, CT showed a subarachnoid hemorrhage (SAH) in the right Sylvian fissure. Angiography demonstrated a complete occlusion of the superior sagittal sinus. The SAH was assumed to be originated from rupture of the right Sylvian vein, which was irregularly dilated on angiography. The dural sinus thrombosis was thought to be caused by a long term use of metenolone acetate, and it was discontinued. But her platelet count dropped due to the aggravation of aplastic anemia, and she developed repeated hemorrhagic infarction. An active anticoagulant therapy for the dural sinus thrombosis was thought to be inappropriate because she had the aplastic anemia and the hemorrhagic infarction recurred. We have successfully treated this case by mild anticoagulant therapy with nafamostat mesilate (Futhan).

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The effect of transcutaneous electrical nerve stimulation (TENS) on the central nervous system amplification process was investigated focusing on the dorsal column-medial lemniscal pathway, because the dorsal column nucleus was recently shown to receive multiple sources of sensory information, including pain. Short latency somatosensory evoked potentials (SSEPs) were recorded in ten healthy normal volunteers. Amplitude changes in each SSEP component (the N9 brachial plexus potential, the P14 potential that originates from the cervicomedullary junction, spinal N13/P13 generated by the cervical dorsal horn and the cortical N20/P25 potential) were studied at stimulus strengths ranging from the threshold (40% maximum stimulus) to 2.5 times the threshold (maximum). The findings suggest that sensory amplification begins at the P14 generator source near the cuneate nucleus. There was no statistically significant difference in sensory amplification between P14 and cortical N20/P25, indicating that the cuneate nucleus is the main site of the central amplifying process. When TENS was applied to the palm distal to the median nerve stimulation used for SSEP, cortical N20/P25 amplification disappeared, evidence that TENS suppresses the central amplification phenomenon, most probably at the level of the cuneate nucleus.

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We present the first report of primary spinal intramedullary lymphoma of the T-cell type associated with human T-cell lymphotropic virus Type I infection in a 52-year-old woman who had undergone a renal transplant. The pathogenesis of spinal cord T-cell lymphoma is discussed.

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To estimate the effects of night work on the human cognitive function, P300 event related potentials (ERPs) evoked with an auditory "oddball" paradigm were recorded for 17 nurses (mean age 27.4 +/- 6.1 years; range 21-41 years) for 3 epochs; after a night of work, after a day of work and on a holiday, and for 12 elderly security guards (mean age 62.8 +/- 2.2 years; range 60-67 years) for 2 epochs; after a night of work and on a holiday. The Stress Arousal Check List (SACL) was used for all the subjects just prior to the P300 ERP recording to determine the extent of stress and the arousal grades. Fourteen of the nurses were in their twenties (mean age 24.9 +/- 2.6 years; range 21-29 years), and 3 (39.0 +/- 2.6) were older (36, 40 and 41 years). The 14 nurses were classed as the young group. All the security guards were classed as the elderly group. In the young group, the stress grade scores increased significantly (P < 0.05) and the arousal grades decreased significantly (P < 0.01) after night work as compared to the holiday values. Although statistically not significant, the amplitude of the P300 component tended to decrease after night work, whereas the latency was very stable for these 2 epochs. The P300 latencies of the 3 older nurses were as stable as those of the young group, but their amplitudes were significantly reduced after night work as compared with the holiday amplitudes (P < 0.05). In contrast, the 12 elderly security guards showed no statistically significant changes in the scores for the stress and arousal grades between the 2 epochs, after night work and holiday. Latency prolongation however, was statistically significant (P < 0.01) after night work. The P300 amplitudes for many of the elderly security guards also tended to decrease after night work, but were not statistically significant. The P300 amplitude is considered to reflect the amount of attentional resources and the latency to reflect the time needed for the cognitive process, indicating that the elderly security guards experiences slowing of the cognitive process in night work. Our results suggest that the effect of night work on the cognitive function is greater for elderly than for young workers. We conclude that P300 can be used to evaluate changes in the human cognitive function produced by night or rotating shift work and that the results provide useful information with which to plan shift schedules on the basis of worker age.

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The intracranial spatiotemporal distributions of brainstem auditory evoked potentials were analyzed in two patients with epilepsy to study the dipole directions of waves III and V, previously found to have a horizontal and a vertical dipole, respectively. Depth electrodes were implanted bilaterally into the frontal and temporal lobes of these patients, the targets including the amygdala and hippocampus which are close to the upper pons and midbrain. Recordings around the upper pons and from the subcortical area downwards along the dorsal aspect of the upper pons indicated that waves III and V are constructed of mixed dipoles, wave III also having a vertical dipole segment and wave V also a horizontal one. These results suggest that several neural activities from the upper pons to midbrain contribute to the generation of waves III and V.

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A family with central nervous system (CNS) arteriovenous malformations (AVMs) and hereditary hemorrhagic telangiectasia (HHT) is reported. A 46-year-old man had an intracerebral hemorrhage. Cerebral angiography showed one AVM and two angiomas. The HHT was diagnosed because of the concomitant existence of cutaneous telangiectasia. The patient's brother had HHT and paraplegia since the age of 21. Magnetic resonance imaging revealed an old spinal cord hemorrhage. The patient's son with HHT had an intracerebral hemorrhage at age 6. Angiograms showed two AVMs and one angioma. Familial CNS AVMs with HHT are extremely rare. The loci for human leukocyte antigen of the affected cases with HHT were evaluated, and the management of CNS AVMs with HHT is discussed.

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To evaluate the clinical utility of somatosensory evoked potentials elicited by stimulation of the paraspinal region (PS-SEPs), waveform characteristics and scalp topography were studied in 23 normal controls. PS-SEPs were recorded in 22 patients with spinal lesions, and the relation between the abnormal PS-SEPs and clinico-radiological findings was investigated. 1) The normal control study showed clear waveforms on the scalp elicited by stimulation of the paraspinal region from C7 to the L2 spinous process level, with both bilateral and unilateral stimulation. The latency of PS-SEPs was gradually decreased when stimulation was moved in the caudo-rostral direction. 2) The normal range of spinal conduction time and conduction velocity was wide. The conduction velocity resulting from stimulation of the lumbar region tended to be slower than that from stimulation of the thoracic region, perhaps due to the difference in the length of the peripheral cutaneous nerves. 3) There was no correlation between the height and the latency of PS-SEPs. 4) Amplitudes of PS-SEPs after unilateral stimulation of the C7 and Th5 spinous levels were larger on the scalp contralateral to the side of stimulation. These findings were similar to SEPs elicited by median nerve stimulation. There was no significant amplitude laterality of PS-SEPs when the Th10 and L2 spinous levels were stimulated. Posterior tibial nerve SEPs showed a larger amplitude at the scalp ipsilateral to the side of stimulation than at the contralateral side. All these findings suggest that the cortical generator sites of PS-SEPs elicited by C7 and Th5 stimulation are located near the hand area, and those from Th10 and L2 stimulation are between the hand and the foot area. 5) Unilateral stimulation disclosed a clear laterality of sensory disturbance that was obscure when only bilateral stimulation was employed. PS-SEPs showed a high degree of abnormality when the patient had deep sensory disturbance. Generally, abnormal PS-SEPs were found caudal to the clinical sensory level, and a few cases showed abnormal PS-SEPs rostral to the sensory level. The latter might indicate that PS-SEPs detected subclinical sensory disturbance. It was therefore concluded that PS-SEPs are a useful tool for the objective evaluation of sensory disturbance, especially in cases of thoracic lesion, because conventional SEP studies, utilizing non-cephalic references do not provide clear identification of abnormal sensory levels along the thoracic spinal cord.

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Motor and sensory cortical tongue representations were examined in 40 patients with intractable seizures who underwent chronic subdural electrode grid implantation. Tongue responses were observed in a wide area 4.5 cm anterior and 3 cm posterior to the central sulcus. The distribution of the responses was not influenced by whether the responses were unilateral or bilateral. In patients with fronto-parietal lesions, the tongue motor area was located significantly more superior to the Sylvian fissure and more anterior to the central sulcus than was the tongue motor area of patients without organic lesion. Both motor and sensory responses were found outside of the classic precentral or postcentral area on the lateral surface of the cortex. Motor responses ('parietal motor responses') could occur posterior to the central sulcus and, rarely, sensory responses ('frontal sensory responses') were identified anterior to the central sulcus. These paradoxical parietal motor and frontal sensory responses were seen in 17 out of 40 (42.5%) patients. Nine of these 17 patients had no organic brain lesion on MRI. Clinical factors, such as patient's age, duration of seizures and cognitive functions (IQ, word fluency score), did not influence the frequency of the paradoxical responses. However, patients with brain lesions showed a tendency to have associated paradoxical responses (P < 0.05). In conclusion, paradoxical responses are not uncommon in epilepsy patients, particularly in those with organic lesions. The physiological and clinical implications of the paradoxical responses are discussed.

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A 52-year-old female admitted for subarachnoid hemorrhage and preoperative angiography revealed bilateral symmetrical middle cerebral artery aneurysms, which were successfully clipped. Post-operative angiography revealed bilateral symmetrical aneurysms in the distal anterior cerebral arteries, which were not identified on the preoperative angiograms, and were also clipped successfully. Such multiple aneurysms should not be treated by bilateral approaches in a one-stage operation.

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Generator sources of far-field P30 and N33 components produced by posterior tibial nerve stimulation were compared with those of the P14 and N18 components of median nerve stimulated somatosensory evoked potentials. Intracranial spatio-temporal distributions of P30 and N33 were similar to those of the P14 and N18 obtained by median nerve stimulation. In clinical cases, the changes in P30 and N33 were correlated with those in P14 and N18, indicative that P30 and N33 are derived from activities similar to those that produce P14 and N18.

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To identify the origin of short latency somatosensory evoked potentials (SSEPs) to posterior tibial nerve stimulation, direct recordings were made from the cervical cord, the ventricular system and the frontal subcortex during 8 neurosurgical operations. The origin of each component of SSEPs was also studied in 7 selected patients with various lesions in the central nervous system. In addition, SSEPs to median nerve stimulation were investigated in 4 of 8 surgical cases and all 7 cases of the lesion study group. Bilateral posterior tibial nerve stimulation in 10 normal subjects showed spinal N28 on the skin of the posterior neck and far-field P30 and N33 components followed by a cortical P38 component at the scalp. Direct recordings made to the mid-brain through the medulla oblongata showed a negative potential with gradually increasing latency. The peak of the negativity in the vicinity of the dorsal column nucleus showed almost the same latency as that of the scalp far-field P30, and positivity with a stationary peak was found above the dorsal column nucleus. Above the mid-pons, there was a stationary negativity with no latency shift, showing the same peak latency as that of scalp N33. The spatiotemporal distributions of P30 and N33 to posterior tibial nerve stimulation were analogous to those of P14 and N18 by median nerve stimulation. Transesophageal and direct cervical cord recordings showed that the spinal N13 phase to median nerve stimulation was reversed between the dorsal and ventral sides of the cervical cord. No such reversal occurred for the spinal N28 potential. Clinical lesion studies showed that changes in P30 and P14, and in N33 and N18 correlated with one another: that is, 1) prolongation of latency of N33 was also observed for N18; 2) absence of P30 was paralleled by the absence of P14. These data suggest that spinal N28 originates from ascending activity such as a dorsal column volley, and scalp P30 comes from activity near the dorsal column nucleus, which is similar to the P14 component of median nerve stimulation. The origin of N33 is thought to be similar to N18 from median nerve stimulation, which originates from brainstem activity below the thalamus.

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