Yoshigoro Kuroiwa

The Crow‐Fukase syndrome A study of 102 cases in Japan

Clinical manifestations of 102 cases with the Crow-Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castlemans disease.

Nationwide survey of multiple sclerosis in Japan Clinical analysis of 1,084 cases

Between October 1972 and October 1973, the first nationwide survey of the multiple sclerosis group of diseases in Japan was performed by the Multiple Sclerosis Research Committee of Japan, supported by the Japan Ministry of Health and Welfare. Reports on 1,084 patients with the multiple sclerosis group were collected: 509 patients with multiple sclerosis, 82 with Devics disease, 357 with “multiple sclerosis possible,” and 136 with “other or unclassified demyelinating diseases.” The natural history in the present nationwide series of multiple sclerosis patients showed considerable similarity to that of patients in Western countries, suggesting that multiple sclerosis in Japan is essentially the same as that in the Western countries. However, the previously reported special characteristics of Japanese multiple sclerosis patients, namely, a higher rate of visual impairment at onset, a higher rate of optic nerve involvement during the course of illness, and a higher rate of Devics disease, were reconfirmed in the present series.

Clinical studies of the movement-related cortical potential (MP) and the relationship between the dentatorubrothalamic pathway and readiness potential (RP).

SummaryIn order to investigate the influence of basal ganglia and cerebellar involvement on the preparatory state of the cerebral cortex for voluntary movement, the cortical potential preceding finger movement was studied in 20 patients with Parkinsons disease and 20 patients with cerebellar ataxia. Readiness potential (RP) was abnormal in 90% of the Parkinson group and in 55% of the cerebellar ataxia group. The most frequent abnormality was a depressed amplitude and earlier onset of RP in both groups. The most remarkable finding in the present study was the complete absence of RP with dyssynergia cerebellaris myoclonica (presumed Ramsay Hunt syndrome) whereas normal RP was obtained with cerebellar cortical degeneration. In addition, RP was absent or severely depressed in patients with a unilateral vascular lesion of the midbrain (Benedikts syndrome) and in patients with Parkinsons disease who underwent unilateral intermedioventral (Vim) thalamotomy. These facts suggest a possible important role of the dentatorubro-thalamic or dentatothalamic pathway in the physiogenesis of RP.ZusammenfassungKortikale Potentiale vor der willkürlichen Fingerbewegung wurden bei 20 Patienten mit Parkinsonscher Krankheit und bei 20 mit cerebellärer Ataxie studiert, um den Effekt der Stammganglien- und Kleinhirnläsion auf den Bereitschaftszustand der Großhirnrinde für Willkürbewegungen zu untersuchen. Das Bereitschaftspotential war abnorm bei 90% der Parkinsonpatienten und bei 55% der Patienten mit Ataxie. Die häufigste Abnormität war eine Depression und ein früherer Anstieg des Bereitschaftspotentials bei beiden Patientengruppen. Der bemerkenswerteste Befund war ein vollständiges Fehlen des Bereitschaftspotentials bei Patienten mit wahrscheinlicher Dyssynergia cerebellaris myoclonica (Ramsay Hunt Syndrom), während ein normales Bereitschaftspotential bei Patienten mit cerebellärer kortikaler Degeneration gefunden wurde. Das Bereitschaftspotential fehlte oder war auffallend niedrig bei Patienten mit einseitiger vaskulärer Mittelhirnläsion (Benedikt Syndrom) und bei Patienten mit Parkinsonscher Krankheit nach einseitiger Thalamotomie (Nukleus ventralis intermedius). Diese Befunde weisen auf eine wichtige Rolle der dentato-rubro-thalamischen oder dentato-thalamischen Bahn in der Pathogenese des Bereitschaftspotentials hin.

Racial modification of clinical picture of multiple sclerosis

Two hundred and four consecutive British cases and 60 consecutive Japanese cases of probable (clinically definite) multiple sclerosis, seen at The National Hospital for Nervous Disease, Maida Vale, London and at Kyushu University Hospital, Fukuoka, Japan, respectively, over a defined period of time, were reviewed retrospectively by the same neurologist, using the same diagnostic criteria and the same method of analysis. The clinical pictures in the present 2 series were in many respects similar to each other and to that of other reported series. Some significant differences were found, however: there was (1) a greater incidence of visual loss at the onset of illness, (2) more frequent and severe involvement of spinal cord and brainstem during the course of illness, as judged clinically, and (3) more severe visual disability in the Japanese series as compared with the British series.

Electroencephalographic correlates of myoclonus

In order to overcome various drawbacks of the conventional polygraphic study of a relationship between myoclonus and EEG, the EEG preceding and following the myoclonic jerk was simultaneously averaged by the CNV program. The subjects were 7 patients presenting with myoclonus of various kinds. The conventional polygraphs showed various paroxysmal EEG activities in 4 patients, but none of those paroxysmal activities was temporally related to myoclonus except for one case. As a result of the present averaging technique, 2 patients with cerebellar ataxia with intention myoclonus showed myoclonus-related EEG spikes or spike-and-slow-waves in the contralateral central or centroparietal region. These myoclonus-related spikes preceded the myoclonus by 10-17 msec, suggesting the presence of a discharging focus in the deep cerebral structures, rather than in the cerebral cortex, in these cases. Two other patients, one with resting myoclonus and the other with postural myoclonus, showed myoclonus-related slow waves on the contralateral hemisphere. This previously undescribed method of averaged polygraphic recording will be very useful in detecting an EEG correlate of spontaneously occurring myoclonus.

Toxic polyneuropathy due to glue sniffing

Four cases of toxic polyneuropathy due to glue-sniffing were reported. Neurological examination revealed motor predominant mixed type polyneuropathy. The cause of polyneuropathies in these cases was considered to be due to the inhalation of the vaporized elements of the adhesive agent, which contain mainly n-hexane and toluene. N-hexane is considered to be chiefly responsible for the polyneuropathy, though toluene also could have some influence on the illness.

Polyneuropathy, skin hyperpigmentation, edema, and hypertrichosis in localized osteosclerotic myeloma.

A 61-year-old man had osteosclerotic myeloma that was localized in the eleventh thoracic vertebral body and associated with sensorimotor polyneuropathy, skin hyperpigmentation, edema, hypertrichosis, gynecomastia, and white nails. Cases of osteosclerotic myeloma with and without polyneuropathy in the literature were reviewed with special reference to accompanying dermatologic and endocrinologic signs and symptoms. We assume that the polyneuropathy, cutaneous hyperpigmentation, edema, hypertrichosis, gynecomastia, and white nails are causally related to each other and are a remote effect of osteosclerotic myeloma. Quantitative histologic analysis of two sural nerves biopsied within 2 years of each other during the course of the disease indicated that both large and small myelinated fibers degenerated progressively, with relative preservation of unmyelinated fibers.

Short latency somatosensory evoked spinal and scalp-recorded potentials following posterior tibial nerve stimulation in man

Short latency somatosensory evoked potentials (SEPs) following posterior tibial nerve stimulation at the ankle were studied by using surface electrodes placed on the spine and scalp with a non-cephalic reference (unipolar recording) in normal young adults. In order to improve recording technique, ECG was used to trigger the stimulator, and bilateral simultaneous stimulation was employed. A triphasic (positive-negative-positive) or biphasic (negative-positive) potential was recorded at each spinal electrode. Latency of this potential was longer in the more rostral channels. Conduction velocity of the spinal cord from the 12th thoracic to the 2nd cervical spine was 73.4 +/- 7.53 m/sec. At scalp electrodes, 4 components (P25, N27, P28 and N31) were identified preceding the major positive peak (P36). These 4 components showed similar latency and amplitude at all 4 electrodes investigated (Fs, Fz, C3 and C4) and are considered to be generated in the deep structure. Especially, P25 is estimated to be generated in the ascending sensory pathway of the upper cervical cord, but generator sources of other components remain to be determined. P36 appears to be generated in the somatosensory foot area.

Giant axonal neuropathy: A clinical entity affecting the central as well as the peripheral nervous system

An 8-year-old girl had progressive muscle weakness and a unique posture of the lower limbs, areflexia, distal sensory impairment, and remarkably kinky hair. Histologic examination of the sural nerve showed giant axons filled with neurofilamentous masses. The clinical and histologic findings resembled those of recent cases reported as “giant axonal neuropathy.” Our patients precocious puberty, Babinskis sign, and electroencephalographic abnormalities suggested central nervous system involvement. Two cases previously reported and the present one appear to represent a new clinical entity that affects the central and the peripheral nervous system.

Multiple sclerosis in Asia

Cases of multiple sclerosis were collected by Asian neurologists from recognized medical centers, using standardized formats similar to those used for the Japan nationwide multiple sclerosis survey. As a result, information on a total of 61 cases was collected from six neurologic centers in five Asian countries excluding Japan. Multiple sclerosis appears to be a rare disease throughout Asia. Certain clinical characteristics that had been previously noted in Japanese and other individual Asian series were again observed in the present Asian series. When compared with Western multiple sclerosis, the Asian multiple sclerosis cases showed a relatively higherfrequency of optic nerve involvement, frequently bilateral and severe, at the onset, as well as during the course of illness, and no rare occurrence of Devics syndrome.