Takao Nakanishi

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.

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.

Anal muscle electromygrams differ in amyotrophic lateral sclerosis and Shy‐Drager syndrome

Electromyography (EMG) of anal sphincter muscles was different in patients with amyotrophic lateral sclerosis (ALS) and Shy-Drager syndrome. In 30 patients with ALS, EMG of the external sphincter muscle was essentially normal, with no signs of denervation. In eight cases of Shy-Drager syndrome, however, motor unit potentials of the anal sphincter had highly polyphasic forms of long duration and high amplitude. In the Shy-Drager syndrome, there seems to be specific damage of lower motor neurons that innervate the external sphincter muscle of the anus.

Somatosensory evoked responses to mechanical stimulation in normal subjects and in patients with neurological disorders

Abstract The contralateral somatosensory evoked responses to mechanical stimuli, such as pin-prick, touch and tactile tap, applied to the middle finger and to the big toe were studied in 33 normal young volunteers and in 61 patients with neurological disorders, with emphasis on the early components of the response. In normal subjects, the well-defined cortical responses to mechanical stimuli could be obtained by the method used in this study, but the consistency with which the responses could be recorded was a little lower than in the case of electrical stimulation. In each subject, the bilateral peak latencies and wave forms were almost equal, whereas the amplitudes were greatly changed over the two hemispheres. The results obtained in this study with regard to a correlation between sensory deficit and alterations in the somatosensory evoked responses to mechanical stimulation in patients with peripheral neuropathy and with cerebral lesions are in general agreement with previous investigations of the responses evoked by electrical stimulation. The findings in patients with dissociated sensory loss due to the spinal cord lesions suggest that the afferent impulses responsible for the somatosensory evoked responses to mechanical stimulation travel by the ventro-lateral tracts.

Myoclonus, cerebellar disorder, neuropathy, mitochondria1 myopathy, and ACTH deficiency

A 50-year-old Japanese woman with action myoclonus, cerebellar signs, neuropathy with axonal degeneration and onion-bulb formation, muscle atrophy with mitochondrial abnormalities, and isolated ACTH deficiency was reported. Her daughter had myoclonus epilepsy and cerebellar ataxia. Neuropathologic findings included atrophy of the dentate and inferior olivary nuclei, Purkinjes cell loss, and demyelination of the posterior columns and spinocerebellar and pyramidal tracts of the spinal cord, besides severe respirator changes. Laforas bodies were absent. The present case should be included in the entity “myoclonus epilepsy associated with mitochondrial myopathy.”

Somatosensory evoked responses to tactile tap in man

Abstract The average somatosensory evoked response to tactile tap was studied in 16 normal young volunteers, with emphasis on the early components of the response. Most evoked responses consisted of the reversed W shaped wave form, in which the peak latencies were 23.8±2.5 msec for N 1 , 31.0±2.8 msec for P 1 , and the amplitude of the first positive deflection (N 1 −P 1 ) 2.0±1.0 μ V. For each individual, the bilateral peak latencies were almost equal, whereas the amplitude was greatly changed in the two hemispheres. The amplitude of the first positive deflection to the test stimulation exhibitted considerable recovery as early as 64 msec. The latency of the first positive deflection (N 1 −P 1 ) evoked by mechanical stimulation to the middle finger was longer than that evoked by electrical stimulation at the wrist. Three possible explanations for this longer latency are discussed.

Ethylene oxide polyneuropathy

Sensorimotor polyneuropathy developed in two workers who had been exposed to ethylene oxide gas repeatedly for several months. Sural nerve biopsies revealed axonal degeneration with mild changes of the myelin sheath. Unmyelinated fibers were also involved. Muscle biopsies showed typical denervation atrophy. Symptoms improved after exposure to ethylene oxide terminated.

Symptomatic ossification of the posterior longitudinal ligament of the cervical spine CIinical findings

Article abstract Eight patients with ossification of the posterior longitudinal ligament of the cervical spine but without cervical spondylosis are described. Seven of them showed clinical signs of long tract involvement of the spinal cord. Fifty-nine male patients with ossification associated with cervical spondylosis were compared syrnptornatologic;ally and roentgenologically with 52 male patients with cervical spondylosis alone. A significantly higher incidence of cord signs and smaller average sagittal diameters of the cervical bony spinal canal were obtained in the group with ossification associated with cervical spondylosis.

Asymptomatic ossification of the posterior longitudinal ligament of the cervical spine: Incidence and roentgenographic findings

Abstract The cervical spines of 698 individuals without neurological symptoms, 20–80 years old, were studied roentgenographically and the incidence of ossification of the posterior longitudinal ligament of the cervical spine was determined. The incidence of asymptomatic ossification showed a roughly linear progression with advancing age. Eleven percent of the normal individuals in the sixth decade of life exhibited roentgenologic changes suggestive of ossification. There was no significant difference in incidence in the two sexes. Ossification of the posterior longitudinal ligament was seen to be widely distributed throughout the cervical vertebrae with the highest incidence at the level of C4. The sagittal diameters of the cervical bony spinal canal at the affected level were significantly smaller in the asymptomatic group of cases with ossification than in normal subjects without such changes. Sixty-seven percent of the ossified ligaments in this survey were independent of spondylotic changes such as posterior osteophytes or narrowing of the intervertebral disc spaces.

Nation-wide collaborative study on the long-term effects of bromocriptine in the treatment of parkinsonian patients. Final report.

Final results of the 5-year multicentric collaborative study on the long-term effects of bromocriptine in the patients with Parkinsons disease are reported. This prospective study started in May 1985 in order to see whether the early combination therapy with bromocriptine and levodopa is really superior to the levodopa monotherapy with regard to the late side effects of levodopa in the treatment of parkinsonian patients. Another project of the study was to see the therapeutic efficacy of bromocriptine monotherapy without concomitant use of levodopa. For these purposes, a total of 702 patients with Parkinsons disease were enrolled into three groups: Group 1 (n = 286) with bromocriptine monotherapy, Group 2A (n = 216) with early combination of bromocriptine and levodopa, and Group 2B (n = 200) with levodopa alone. At the end of the 5-year study, 48 patients in Group 1 (16.8%) were still continuing bromocriptine monotherapy with satisfactorily good therapeutic effects. About half (49.1%) of the Group 2A patients remained on the combined therapy, and the comparable number of the Group 2B patients (46.0%) were also kept on the initial mode of therapy, while 13.5% of the latter group with levodopa monotherapy needed bromocriptine to be added in order to assure the good therapeutic effects. Moreover, significant differences were seen between group 2A and Group 2B with regard to the incidence of wearing-off phenomenon and dyskinesias. Disappearance rate of dyskinesias which were present at the time of enrollment was significantly higher in Group 2A than in Group 2B. No significant difference was noted as to the incidence of untoward symptoms and the death rate among all three therapeutic groups. These results support the view that the early combination of bromocriptine with levodopa is superior to levodopa alone in the treatment of Parkinsons disease.