Clinical improvement of adrenoleukodystrophy following intravenous gammaglobulin therapy.

A patient with adrenoleukodystrophy was successfully treated by means of intravenous gammaglobulin injections. The clinical symptoms, especially visual loss, were apparently relieved and no neurological deterioration was observed during a 18-month period following the start of the gammaglobulin treatment.

Quantitative analysis of reversible dysfunction of brain-stem midline structures caused by disturbance of basilar artery blood flow with the auditory brain-stem responses.

To clarify the effects of disturbances in basilar artery blood flow, basilar artery angiospasm was induced in 2 cats and 4 guinea pigs and auditory brain-stem responses (ABRs) were continuously recorded preceding, during and following the angiospasm. The angiospasm caused specific ABR changes in that waves II (P2-N2) and III (P3-N3) were attenuated without any corresponding amplitude reduction of P4. Those changes were equivalent following stimulation of either ear. Moreover, the ABR changes gradually recovered within 5 h. On the basis of the animal experiments, 52 patients with subarachnoid hemorrhage, supratentorial tumor showing increased intracranial pressure or hydrocephalus were selected for repeated ABR examinations. ABR abnormalities similar to those observed in the animal experiment were obtained especially from the patients exhibiting grade 3 or 4 symptomatology with subarachnoid hemorrhage. In these cases, the wave III to wave IV-V amplitude ratio was significantly decreased. In some cases the ABR abnormalities and the wave III to wave IV-V amplitude ratio recovered as the clinical course improved. These results support the conclusion that specific ABR changes (wave III to wave IV-V amplitude ratio) reflect transient ischemic dysfunction of the midline portion of the brain-stem caused by disturbances of basilar artery blood flow.

[Frontal SEPs and parietal SEPs following median nerve stimulation--clinical correlation and consideration of their generation sites].

In order to know the characteristics of frontal and parietal SEP components following median nerve stimulation, 25 patients with unilateral cerebral lesions above the thalamus were examined, and their SSEPs were carefully compared with the clinical and radiological findings. In 10 normal subjects, there were three cortical components of the frontal SEPs (P 20-N 28-P 44) and four those components of the parietal SEPs (N 18-P 22-N 26-P 42). In patient's group, central conduction times (CCTs) between components P 13 and each cortical component were measured and the latency differences between normal side and affected side were calculated. When the latency differences increased over 3 S.D. from the mean of the control values or the some cortical components disappeared, they were regarded as abnormal. According to the combination of the abnormalities in frontal and parietal SEPs, three groups were classified as follows: group 1; frontal and parietal SEPs were normal (n = 10), group 2; frontal and parietal SEPs were both affected (n = 10), group 3; parietal SEPs were affected but frontal components were preserved in normal range (n = 5). CT scan showed that the region from internal capsule to cortex around the central sulcus remained intact in the patients of group 1, while this region was involved in various degrees in all cases of the group 2. In patients of group 3, frontal or parietal regions were variously affected. Both the motor and sensory functions were mainly intact in group 1, and disturbed in group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

[Hydrocephalus due to membranous obstruction of the fourth ventricle aperture].

Three cases of hydrocephalus due to membranous obstruction of the fourth ventricle aperture are reported. Patient's age was 4, 34 and 68 years old and they complained of slowly progressive cerebellar ataxia, gait disturbance and other hydrocephalic symptoms. CT scan revealed symmetrical dilatation of lateral ventricles, and, especially, the fourth ventricle aperture was confirmed by ventriculography or metrizamide CT cisternography. Suboccipital craniectomy was performed in all cases and cerebellum was of normal shape and semilucent membrane was found obliterating the foramen of Magendie. Histological finding of the membrane was chiefly composed of fibroblast, but inflammatory evidence suggesting any particular etiological process. The cause of membranous obstruction was undetermined and obsolete infectious or inflammatory change was suspected.

[Origin of the early components of somatosensory evoked potentials by the posterior tibial nerve stimulation--a comparative study of median nerve stimulation in clinical cases].

In order to determine the generation sites of short latency somatosensory evoked potentials to the posterior tibial nerve stimulation, scalp topography was performed on 10 normal subjects in the two different band-pass recordings, i.e., wide band-pass filter (5-3000 Hz) and narrow band-pass filter (100-1000 Hz). Furthermore, comparative study of the changes of evoked potentials between posterior tibial nerve stimulation and median nerve stimulation was carried out in 22 cases with well localized lesion of the central nervous system in the same wide band-pass filter setting. The early components of somatosensory evoked potentials elicited by the posterior tibial nerve stimulation were obtained as P 30, N 34, and P 38 in the wide band-pass filter, and P 29, N 32, P 36 in the narrow band-pass filter. Components P 30, N 34 and components P 29, N 32 were widely distributed on the scalp, but were disappeared on the scalp-scalp recording. These results suggested all those components were generated from the deep subcortical structures. In the case with high cervical lesion, component P 30 at the posterior tibial nerve stimulation was remarkably prolonged in latency, and component P 13 at the median nerve stimulation was disappeared. P 30-N 34 interpeak latency at the posterior tibial nerve stimulation was prolonged in the case with pontine lesion, while P 13-N 16 interpeak latency at the median nerve stimulation was also prolonged. In the cases with thalamic and internal capsular lesion, P 30 and N 34 at the posterior tibial nerve stimulation and P 13 and N 16 at the median nerve stimulation were all preserved in normal range. These results revealed that components P 30 and N 34 were almost identical to components P 13 and N 16, respectively. On the other hand, component P 38 at the posterior tibial nerve stimulation was suppressed or disappeared in the cases with well localized lesion at the midcentro parietal region, that includes the primary foot sensory area.(ABSTRACT TRUNCATED AT 400 WORDS)

[Origin of component N16 in short latency somatosensory evoked potentials (SSEP) to median nerve stimulation--correlation between component N16 and thalamus].

Short latency somatosensory evoked potentials (SSEP) to median nerve stimulation consists of four main subcortical components, namely P 9, P 11, P 13 and N 16 which appears before cortial N 18. However, the origin of component N 16 is a subject of controversy. In an attempt to learn about the generator source(s) of component N 16, SSEP was recorded from 25 patients with various focal lesions of the brain stem and/or thalamus, and abnormalities of the each potential was correlated to the clinically and radiologically defined site of the lesions. Furthermore, the effects of the different frequency in stimulation were also investigated in 6 normal subjects, because latency changes of each component might contribute to the understanding of the generation. Recordings were obtained from 13 patients with brain stem lesion which included 3 cases with pontine hemorrhage, 3 cases with pontine tumor, 3 cases with cerebello-pontine angle tumor, one case of pontine angioma, one case of chordoma, one case of tentorial tumor and one case of MLF syndrome. SSEP changes in these cases were classified into four types as follows: type 1: no response over the base line was recorded, type 2; some responses over the base line were recorded but N 16 was uncertain, type 3; component N 16 was clearly identified but its latency was significantly prolonged, type 4; component N 16 was divided into two peaks. Bilateral abnormality on SSEP with splitted combination of these four types in various degree was observed. Furthermore, these SSEP abnormalities were seen even in the some cases without sensory disturbance. On the other hand, component N 16 was clearly identified in all 12 patients with thalamic lesion which included 11 cases with thalamic hemorrhage and one case with thalamic tumor on the effected side. Comparison of latency and amplitude between normal side and affected side statistically showed no laterality of components P 9, P 11 and P 13, but a tendency of delay in latency of component N 16 on the affected side. Different stimulus repetition rate revealed some other characteristics of each component. Electrical stimuli to median nerve at the wrist were delivered at rates of 3, 6, 9, 12, 15, 18, 21, 24 and 27 Hz. Latencies of components P 9, P 11, P 13, N 16 in Fro.-Cv 7 lead and component N 18 in Par.-Erb lead were measured and all latency changes were calculated relative to the 3 Hz stimulation.(ABSTRACT TRUNCATED AT 400 WORDS)

[Origin of components P 11 and P 13 in short latency somato-sensory evoked potentials (SSEP): correlative study of SSEP and intraoperative evoked potentials].

In 15 patients with cervical or posterior fossa lesion, SSEPs were recorded between the skull and the non-cephalic reference electrodes during the surgical operation and compared with the evoked potentials directly recorded at the same time from the surface of the cervical spinal cord and the brain stem. The directly recorded evoked potential consisted of three main components appeared within about 25 ms., they were a small negative spike wave, a large positive spike wave and a subsequent slow potential. The positive spike wave of the evoked potentials recorded from the surface of the dorsal column was not only coincided in latency with component P 11 of SSEP, but also showed the greatest amplitude at the lower cervical level. Moreover, the positive spike wave gradually delayed in latency and reduced in amplitude from lower to upper cervical segments. The amplitude of the positive spike wave was greater at the surface of the dorsal column ipsilateral to the stimulated median nerve than that of contra-lateral recording. No polarity change was observed between the anterior and posterior surface of the spinal cord. Similarly, the positive spike wave of the evoked potentials, recorded from the surface of the brain stem, showed fairly same latency with P 13 and a maximal amplitude at the surface of the cuneate tuberculum ipsilateral to the median nerve stimulated, and those positive spike wave traveled to contra-lateral ventral surface of the pons, presumably from ipsilateral cuneate nucleus to the contra-lateral medial lemniscus.(ABSTRACT TRUNCATED AT 250 WORDS)