Yasuo Toyokura

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.

Preservation of a certain motoneurone group of the sacral cord in amyotrophic lateral sclerosis: its clinical significance.

Histopathological description of a special nerve cell group of the sacral spinal cord in five cases of amyotrophic lateral sclerosis is presented. Remarkable preservation of this group of neurones (the X group of Onuf) was noted in each case, and this is discussed with particular reference to vesicorectal function, since it is well known that the function of bladder and rectal sphincters is often intact until the latest stage of the illness.

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.

Juvenile muscular atrophy of unilateral upper extremity

IN 1959 WE SUGGESTED a new clinical entity of juvenile muscular atrophy of unilateral upper extremity,l based on our experience of I2 cases. The present communication deals with more detailed observation and laboratory tests on 11 of the 12 patients whose cases were previously reported and 9 additional patients examined during the past three years. One of the 12 patients was excluded in view of the further clinical course. Muscle biopsy specimens taken from 3 patients enabled us to differentiate this syndrome definitely from such myopathies as myositis and muscular dystrophy. Juvenile onset, localized muscular atrophy with unique distribution in the arm of one side, and a nonprogressive course in later stages characterize this disorder. This muscular atrophy is distinctly different from any hitherto described entities of muscular atrophy.

Quantitative changes with age in normal sural nerves

SummaryQuantitative changes with age of the myelinated fibre density, nuclear density, the thickness of the perineurium, and the vasa nervorum were studied histologically in the human sural nerve. Materials were obtained from 79 necropsies of acute death without any accompanying peripheral nerve diseases, ranging from 1 week to 88 years of age.The average small myelinated fibre density decreased rapidly from the age of 1 week (26300/mm2) to the second decade (9560/mm2), and continued to decrease gradually with age, reaching an average of 9730/mm2 for the eighth decade, 74% of that for the second decade.Large myelinated fibres appeared first in a 3-month-old infant. The average large myelinated fibre density increased rapidly, attaining the level of a young adult at 3 years. The average was maximum at the third decade (6480/mm2) and thereafter decreased with age, reaching an average of 3480/mm2 for the ninth decade, 54% of that of the third decade.Nuclear density decreased rapidly from 1 week of age (9800/mm2) to the second decade (3750/mm2). Subsequently, it increased gradually with age up to the eighth decade (6090/mm2), at which time it measured 163% of the average of the second decade.The decrease of large myelinated fibres could not be related to changes of vasa nervorum due to aging before 60 years, while after 60 years there was a greater reduction of large myelinated fibres when the stenosis of vasa nervorum was more pronounced.A linear relationship was found between the thickness of the perineurium and the diameter of the fascicle. The perincurial index (the thickness of perineurium/diameter of a fascicle×100) showed wide variation among individuals though it showed a tendency to increase with age.

The Onuf's nucleus and the external anal sphincter muscles in amyotrophic lateral sclerosis and Shy-Drager syndrome

SummaryThe anterior sacral horns and external anal sphincter muscle were examined histologically with special reference to Onufs nucleus in amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome. Onufs nucleus and the external anal sphincter muscles were well preserved in ALS, but in Shy-Drager syndrome both structures showed marked degenerative changes. These findings suggest that Onufs nucleus does not belong to the autonomic neurons, but to the motoneurons in anterior sacral horn, and it innervates the external sphincter muscles of the anus and urethra.

The initial positive component of the scalp-recorded somatosensory evoked potential in normal subjects and in patients with neurological disorders

The initial positive component of the scalp-recorded somatosensory evoked potential to median nerve stimulation was studied in 12 patients with sensory impairment for all modalities of the unilateral extremities due to lesions at or above or below the thalamic level, taking the potentials obtained from eight normal subjects as control. In normal subjects, this potential could be easily obtained in ear reference recordings with a peak latency of 12--15 msec. This finding and positive polarity of this potential indicate that the earlobe could be active for this potential. The wide distribution of this potential was different from the subsequent negative-positive diphasic components. In patients with lesions at or above the thalamic level the p15 potential was of normal configuration and latency to stimulation of the affected side, whilst in patients with lesions in the brain stem or in the cervical cord it could not be obtained to stimualtion of the affected side. These findings and short latency of the p15 potential suggest that it may be the result of activity of the medial lemniscal systems from the medulla to the thalamus.

Corticofugal projections to the motor nuclei of the brainstem and spinal cord in humans

We studied corticofugal projections to the motoneurons with Nauta-Gygaxs technique in a patient with cerebral infarction of both hemispheres. Motoneurons in the brainstem motor nuclei and spinal anterior horns seem to receive direct cortical projections, except for the oculomotor and abducens nuclei and Onuf s nucleus in the sacral cord.

Spinocerebellar degenerations in Japan: a nationwide epidemiological and clinical study

A nationwide survey of patients in Japan with spinocerebellar degenerations (SCD), including SDS and SND, was conducted from 1988 to 1989. The survey consisted of two parts. The first revealed that the estimated total number of patients with SCD in Japan was 5,050 (range: 4,100–6,000) with an estimated prevalence of 4.53 per 100,000 in 1987. The second part investigated the neurological and functional status of patients with SCD. The percentages of those belonging to each subtype of SCD were: OPCA; 34.4%, LCCA; 15.2%, MHCA; 12.6%, HHCA; 7.5%, SDS; 7.0%, HSP; 3.9%, DRPLA; 2.5%, FA; 2.4%, MJD; 2.0% and SND; 1.5%. Compared with European epidemiological studies Japan had a higher proportion of non‐hereditary types of SCD. Various clinical features of SCD subtypes were compared grouped by pathological lesion and heredity. HHCA and LCCA: cerebellar ataxia predominated in all stages, and neurological signs other than cerebellar ataxia were rare. MHCA, DRPLA and MJD: inthe early phase ataxia was the most common symptom in MHCA, the AC form of DRPLA and MJD, but ataxia was less common and chorea or epilepsy were often observed in ME and PH forms of DRPLA. Other frequently observed clinical features were parkinsonian rigidty in MHCA, abnormal movements and posture in DRPLA and MJD, and disturbances of eye movements in MHCA, the AC form of DRPLA and MJD. OPCA, SDS and SND: dominant clinical features were cerebellar ataxia in OPCA, autonomic disturbance in SDS, and parkinsonian rigidity in SND. FA and HSP: both were rare in Japan. Clinical features related to supra‐supinal lesions were frequently observed in FA. Functional status of SCD: the severity of illness was significantly associated with the level of independence in each item of ADL. Activities not requiring dynamic balance were performed independently for a longer period than those requiring dynamic balance. Among SCD subtypes, functional prognosis was poorest in non‐hereditary, multi‐systemic types (OPCA, SDS and SND) followed by hereditary multi‐systemic types (MHCA, DRPLA and MJD), and better in spinal types (FA and HSP) and cerebellar types (HHCA and LCCA).

Postural hypotension and low R‐R interval variability in parkinsonism, spino‐cerebellar degeneration, and Shy‐Drager syndrome

Systolic blood pressure. mean R-R interval, and R-R interval variance were studied in patients with Parkinsons disease, spinocerebellar degeneration, and Shy-Drager syndrome. Postural hypotension correlated with anhidrosis (p < 0.05), indicating sympathetic vasomotor dysfunction. Reduction of R-R interval variance while resting supine correlated with bladder-bowel dysfunction (p < 0.05), indicating parasympathetic impairment. Reduction of R-R interval variance after postural changes correlated with constipation (p < 0.005), postural hypotension (p < 0.05), and anhidrosis (p < 0.05), indicating both parasympathetic and sympathetic involvement. Dynamic study of the R-R interval provides objective information about autonomic function in neurologic disease.