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Biomedical subjects

R Shiozawa

Publications and source records attributed to R Shiozawa.

At least 19 recordsLinked to original sources
PubMed1992

Amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators.

We describe two patients with sporadic amyotrophic lateral sclerosis (ALS), who had developed progressive external ophthalmoplegia of a predominantly supranuclear type while they survived on respirators, and displayed histopathological abnormalities both typical and atypical of ALS. Patient 1 was a 43-year-old man with ALS of 5-year duration, who had initially exhibited fulminant ALS, and remained on a respirator for 4 years. Patient 2 was a 51-year-old man with ALS of 13-year duration, who remained on a respirator for 8 years. Both patients died in a "totally locked-in state". Autopsy of both patients revealed not only histopathological abnormalities consistent with ALS, but also multisystem degeneration which involved the pontine tegmentum, substantia nigra, Clarke's dorsal nuclei and spinocerebellar tracts. In addition, Patient 2 displayed intracytoplasmic neuronal basophilic inclusion bodies which exhibited marked immunoreactivity to anti-ubiquitin antibodies. Our case reports indicate that the longer survival which is possible through the use of respirators may make one subgroup of ALS patients prone to develop atypical clinical and neuropathological features which are not observed during the natural course of ALS.

Adult↗
PubMed1991

[Electrophysiological studies of a case of startle disease].

A 53-year-old female with startle disease (major form) was reported. An abnormal startle response was the most prominent clinical feature. Physical examination revealed left lateral gaze palsy and left extensor plantar response. The caloric test evoked no responses bilaterally. Blood examinations including lysozomal enzymes and radiological examinations including MRI of the brain were all normal. A pathological startle reflex was elicited by the tap on the upper lip, causing the marked extension of the head with the elbow, hip, and knee joints slightly flexing. The earliest reflex activity in a surface-EMG study was recorded in the masseter muscle and the reflex then spread down the brain stem and the spinal cord. The duration of the discharge varied from 16 to 30 ms. The onset latencies of these responses from the tap were 11.2 ms, 12.7 ms, 14.5 ms, 25.7 ms, 38.5 ms, and 47.5 ms in the masseter, sternocleidomastoid, posterior-neck, biceps brachii, quadriceps femoris, and tibialis anterior muscle, respectively. An averaged electroencephalogram triggered by the taps showed no abnormal EEG activity preceding the pathological startle response, although a negative peak, which was thought as a normal early component of the trigeminal somatosensory evoked potentials, was followed by the reflex. High amplitude SEPs and long loop reflexes were observed following stimulation of the posterior tibial nerve but not of the median nerve. Blink reflexes and auditory evoked potentials were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Electrophysiology↗
PubMed1990

Unilateral asterixis.

We describe three patients with unilateral asterixis. One diabetic patient with a moderately-sized haematoma in the left putamen initially developed bilateral and symmetrical asterixis, which became confined to the right side as his diabetes mellitus was controlled. Two patients showed unilateral asterixis due to haematomas in the contralateral basal ganglia and thalamus, respectively. The present findings indicate that not only lesions of the thalamus but also those of the basal ganglia can cause unilateral asterixis, and bilateral and symmetrical asterixis does not necessarily exclude the presence of a focal mass lesion in the cerebrum.

Aged↗
PubMed1990

Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators.

Patients with amyotrophic lateral sclerosis (ALS), who survive longer on a life-support system, exceeding the natural course of this disease, show new features of ALS. We report here a clinico-pathologic study of a 51-year-old patient with sporadic ALS who developed progressive external ophthalmoplegia 3 years after he remained on a respirator and died 5 years later, 13 years after the onset of his illness. The external ophthalmoplegia was initially accompanied by preserved doll's eye phenomenon, which later became absent. Autopsy revealed not only degeneration of the upper and lower motor neuron systems typical of ALS, but also degeneration of the Clarke's dorsal nuclei, spinocerebellar tracts, substantia nigra and inferior olives in addition to intracytoplasmic neuronal inclusion bodies in various areas. The oculomotor and abducens nuclei were variably involved, accompanied by neurogenic atrophy of the extraocular muscles. Our case report is consistent with the idea that ALS comprises a heterogeneous group of disorders, and also indicates that long-term use of respirators may make some patients with this illness prone to developing atypical clinical and neuropathologic features which are not observed during the natural course of ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1989

[Muscle fiber type abnormalities in bulbospinal muscular atrophy. Comparison with amyotrophic lateral sclerosis].

Bulbospinal muscular atrophy (BSA) is a distinct clinical type of chronic spinal muscular atrophy of adult onset, which was first reported by Kennedy et al. Histochemical findings of involved muscles are poorly described. In the present study, the muscle fiber type was studied on biopsied specimens by the histochemical method. The results were compared with those of amyotrophic lateral sclerosis (ALS). Muscle specimens were obtained mainly from biceps brachii muscles of 7 cases of BSA and 10 cases of ALS. Both group of patients were matched in respect to age, sex and stage of muscle weakness. The following results were obtained. Histopathological findings of BSA are classed as stage 4 abnormalities of Jennekins. Both fiber type grouping and type 2B deficiency are more frequent in BSA than in ALS. Type 2C fiber seems to be more in ALS than in BSA. Atrophy factor of Dubowitz is the same in BSA and in ALS. Hypertrophy factor is more prominent in BSA than in ALS. Significant correlation between fiber type grouping and type 2B deficiency was confirmed in muscles from other neurogenic atrophy. Since there is a significant association of the type 2B deficiency with fiber type grouping, type 2B fiber might be transformed to type 2A during the reinnervation process. The adaptation to the increased activities of the survived muscles and motoneurons might be contributory to the transformation, which is already known to occur in normal subjects during the endurance training.

Adult↗
PubMed1987

Cerebral infarction due to systemic necrotizing vasculitis in a patient with rheumatic heart disease, subacute bacterial endocarditis and status epilepticus.

Systemic necrotizing vasculitis involving cerebral blood vessels is described in a 30-year-old man with rheumatic heart disease and subacute bacterial endocarditis. Fever, anaemia, splenomegaly and positive blood cultures for Gram-negative bacteria were found on admission. The fever resolved with antibiotic therapy on the third hospital day but he then developed hemiplegia and multifocal seizures. The seizures progressed to uncontrollable status epilepticus accompanied by congestive heart failure and the patient died 20 d after admission. At autopsy, exudative and necrotizing vasculitis involving medium- to small-sized arteries was seen in the brain, the heart and the skeletal muscles. Rheumatic myocarditis and endocarditis and old rheumatic mitral valve deformities were also present. In addition, verrucous endocarditis in the mitral valve and Löhlein's focal glomerulonephritis were noted. We discuss the possible mechanism of the systemic necrotizing vasculitis in relation to rheumatic fever.

Adult↗
PubMed1985

[Anti-platelet therapy in ischemic cerebrovascular disorder--clinical and hematological study].

Anti-platelet therapy (Aspirin 250 mg/every other day approximately 1000 mg/day + Dipyridamole 150 mg/day) was performed on 51 patients with ischemic cerebrovascular disorders (ICVD). Among these patients, 41 cases showed no recurrence of ICVD attack, whereas the remaining 10 cases had re-attacks. These 2 groups were compared from the viewpoint of various risk factors and other clinical findings: e.g. CT scan and angiographic appearance. But there was no statistically significant difference between them. Platelet aggregability and platelet survival time were examined in twenty-eight patients out of cases. We used a simple nonradioisotope technic for the determination of platelet survival time. Platelet survival time was shortened in 22 of 28 (79%) patients. On the other hand, platelet aggregability was found to have no definite tendency. In 9 of 10 cases, platelet survival time was revealed to be prolonged several months after administration of anti-platelet drugs. There was no re-attack in 7 of these 9 patients. Some reports also showed that platelet survival time was shortened in various thromboembolism and recovered by anti-platelet drugs. Measurement of platelet survival time is thought to be an important method as to the indication and the monitoring of anti-platelet therapy.

Adult↗