[Endocrinological investigation in patients with progressive proximal spinal and bulbar muscular atrophy of late onset (Kennedy-Alter-Sung type) (author's transl)].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to H Narabayashi.
Explore the source record for details and available documents.
In 1959, this disorder was first reported by one of authors (K.H.) (6). Since then, many Japanese cases have been reported and recently some cases are also reported in other countries. Its cause and pathology are still unknown, because no autopsy case was obtained. In this report, some electromyographic findings are described and discussed. This series contain 26 cases (male 23, female 3). Age of onset: 13-33 years old (mean 19.4 years old). Clinical duration: 2-32 years old (mean 9.0 years). Only 6 cases (23%) showed trauma as the past history (Table 2). We classified 26 cases in three groups (UL: unilateral, BP: bilateral, one-side predominant, BS: bilateral symmetrical) based on the laterality of physical findings (amyotrophy, weakness, finger tremor, etc.) (Table 3). Conventional needle EMG were done in all cases and these findings were judged by the criteria which we defined as Fig. 2. In some cases, conduction velocity (MCV & SCV) were measured. Needle EMG showed more selective and localized distribution of NGP (neurogenic pattern) in the hand muscles, the forearm muscles except for M. brachioradialis, and M. triceps brachii on the atrophied sides of the arm in all clinical groups (UL, BP, BS) (Table 4.5). On the normal side of bilaterally examined cases of UL group, NGP was shown in 9/11 cases, buts its distribution seemed to be slightly restricted in the more distal part. Thus, in this disorder, 22/26 cases (77%) were electromyographically affected on the bilateral sides, although the clinical feature was mostly unilateral. The clinical features and pathological process seemed to be established in a few years after the onset without the apparent tendency of further progression. Afterwards, no apparent extension of NGP distribution and increment of grading were observed in the Clinical course. No delay was observed on MCV and SCV. The pathological lesions responsible for these electromyographic changes are discussed, either on spinal segmental origin or on brachial plexus origin, but some ambiguous portions are still remained.
Explore the source record for details and available documents.
Echocardiographic studies of a 19-year-old female with Ebstein's anomaly and type B Wolff-Parkinson-White syndrome showed an earlier closure of the anterior tricuspid leaflet (ATL) than of the anterior mitral leaflet (AML), contrary to the previous reports. The interval between the closure of the ATL and the AML was 20 msec and 30 msec, respectively, before and after administration of edrophonium chloride. However, closure of the AML preceded that of the ATL after administration of atropine sulfate and during supraventricular tachycardia by 10 msec and 60 msec, respectively, concomitant with the shortening of the duration of the QRS complex. We conclude that early closure of the ATL may depend on preexcitation of the right ventricle.
Unitary or a group of unitary responses recorded extracellularly in the rostral part of the thalamic sensory nucleus during the course of thalamotomy for parkinsonian and other kinds of tremor were studied. They were the first sensory neurons encountered in the frontocaudal approach of the stereotaxic needle to the therapeutic target at the lower border of the ventralis intermedius nucleus. The spike discharges, isolated from high neural noise background, responded to passive and/or active movement of joint or muscle on the contralateral extremity but never to light tactile stimuli. In five cases, electrical stimulation of the corresponding peripheral nerves elicited the same spike discharge at short and fixed latency of about 12 msec. Weak electrical stimulation given to each thalamic point produced paresthetic or electric sensation around the receptive field, and stronger stimulation induced tremor-like movement in the area involving the receptive field. Finally, a small coagulation lesion in this thalamic point resulted in immediate relief of tremor without any sensory deficit. From these findings, the possibility is considered that in the human, this particular thalamic zone could be the ventralis intermedius nucleus, which is separable from the surrounding nuclei and is related to tremor mechanism.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Pathological changes of the fusimotor endings in parkinsonism, motor neuron disease and myasthenia were examined by the acetylcholinesterase technic on serial sections. In parkinsonism, the diffuse endings, which are thought to be supplied by the static gamma nerve fibers, showed remarkable enlargement, while en plaque and en grappe endings were atrophic. In motor neuron disease, en plaque and en grappe endings, which are thought to be innervated by the beta nerve fibers and dynamic gamma nerve fibers respectively, revealed marked atrophy. However the diffuse endings were normal. In myasthenia gravis and myasthenic syndrome (Eaton-Lambert syndrome), en plaque and en grappe endings were atrophic, though only the diffuse endings were spared. The significance of these changes in the fusimotor endings is discussed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A tapping test in which the tasks required a synchronous response to a periodic sound signal by the finger tapping was performed by 27 normal subjects and 146 patients with Parkinson's disease. Some patients could not maintain a synchronous response at a critical frequency of 2.5 or 5 Hz (taps per second) and showed a hastened tapping of 5 6 Hz, independent of the input signal frequencies, i.e., the 'hastening phenomenon.' The other patients and normal subjects could respond synchronously up to 5 7 Hz. Autocorrelational analysis of the sequential tapping intervals suggested that the synchronized response was performed by a feedback control with the input signal as target except at 2.5 and 5 Hz, whereas the hastened tapping of patients was a random process. The frequency-dependence of the error in the tapping was interpreted as representing an intrinsic oscillation in the central nervous system, which would be masked in normal subjects but released in patients with Parkinson's disease. Around 2.5 or 5 Hz the error or response becomes so large that some patients can no longer maintain the synchronized response and show a hastened tapping due to this intrinsic oscillation.
Normal muscle spindles of human skeletal muscle were studied histochemically. 1) Four histochemical types of intrafusal muscle fibers were classified by ATPase stain: Bag I fiber, Bag II fiber, Chain I fiber and Chain II fiber. Moreover, two types of nuclear bag fibers were classified by NADH Tetrazolium Reductase stain and PAS stain: Bag I fiber and Bag II fiber. 2) Three kinds of fusimotor endings were verified by the cholinesterase technic: en plaque, en grappe and diffuse endings. 3) Two kinds of fusisensory endings were verified by NADH TR stain and also electron-microscopically: primary and secondary sensory endings.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.