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Biomedical subjects

M Sakuta

Publications and source records attributed to M Sakuta.

At least 55 records · Page 3Linked to original sources

[A case of Crow-Fukase syndrome with increased serum interleukin-6].

We experienced a 47-year-old Japanese female with polyneuropathy, edema, hypertrichosis, hyperpigmentation, and white nail, which were diagnostic as having Crow-Fukase syndrome. Laboratory and radiological evaluation showed neither plasma cell dyscrasia nor monoclonal gammopathy. Increased factor VIII activity and thrombocytosis, which suggested thrombotic tendency, were observed at the exacerbation of clinical symptoms. In her third exacerbation, she presented marked cyanosis in her right foot, and angiography confirmed narrowing of arteries at the ankle. Increased serum interleukin-6 was also observed, and the production of interleukin-6 by endothelial cells of cutaneous angioma was shown. Possible role of interleukin-6 in Crow-Fukase syndrome was discussed.

Factor VIII↗

[A case of idiopathic, symmetrical non-arteriosclerotic, intracerebral calcification (Fahr's disease) associated with M-proteinemia, followed by multiple myeloma].

We described a 41-year-old woman with idiopathic, symmetrical, non-arteriosclerotic, intracerebral calcification (Fahr's disease), associated with multiple myeloma. CT scans revealed severe calcification in the basal ganglia, floors of cortices, subcortical white matter, brainstem and cerebellum without calcification in the spinal cord. Cerebral angiography showed no evidence of arteriosclerosis. The cerebral blood flow measured by SPECT, parathyroid function and calcium metabolism were within normal range. The initial symptom was dystonia and spasticity in the left leg, when she was 30 years old, followed by gait disturbance, speech impairment, micrographia and dementia. M-proteinemia was pointed out when she 32 years old. M-proteinemia, which was due to primary benign monoclonal immunoglobulinemia (PBMI), made progress slowly, followed by multiple myeloma when she was 40 years old. Periodical CT scans revealed that the intracerebral calcification had worsened gradually through 8 years. Neurological abnormality had also progressed slowly. In literature, there has not been any report about Fahr's disease associated with PBMI and multiple myeloma. Our present study is the first to radiologically prove that the intracerebral calcification in Fahr's disease progresses gradually through its course.

Adult↗

[A case of progressive systemic sclerosis and Sjögren's syndrome complicated by parkinsonism with special reference to the beneficial effect of corticosteroid].

A 79-year-old woman was consulted by us because of 2 years' history of difficulty in neck flexion and frozen gait. On admission, the neurological examination disclosed findings compatible with parkinsonism such as bradykinesia and muscle rigidity. In addition, she felt thirsty and hard skin, cyanosis and Raynaud phenomenon in the limbs. These were consistent with diagnosis of progressive systemic sclerosis and Sjögren's syndrome, which was then confirmed by high titers of anti-nuclear antibody and anti-ENA antibody and abnormal sialogram; marked decrease of saliva and tear secretion was demonstrated. Brain CT and angiography showed no evidence of ischemic stroke. Parkinson symptoms were not relieved by a month's treatment with L-dopa, but was improved by 3 weeks' administration of corticosteroids; the latter also provided a benefit to the poor circulation in her finger tips. Parkinsonism in the present case is likely related to Sjögren's syndrome. Our report proposes a possibility of autoimmune disease as one of underlying causes for drug-resistant parkinsonism.

Aged↗

[A child case of atypical chronic fasciitis--diagnostic usefulness of muscle CT scan].

Eosinophilic fasciitis is an uncommon disorder characterized by peripheral blood eosinophilia, hypergamma-globulinemia, and inflammation of fascia and skin. Its typical symptom in the acute stage includes muscle pain and swelling, followed by slowly progressive joint contractures. We report an 11-year-old girl with pathological diagnosis of chronic fasciitis, probable eosinophillic fasciitis. Her first symptom was chronic symmetrical joint contractures, which developed when she was 4 years old. She had atypical course as compared with patients described in literature, lack of acute symptom and very early onset of the disease. Her muscle CT scan revealed very fibrotic fascia, which might be responsible for joint contractures. Muscle and fascia biopsies were performed to confirm the diagnosis. The fascia was very fibrotic containing mononuclear cell infiltration, compatible with a chronic stage of eosinophilic fasciitis. In conclusion muscle CT scan is useful for differential diagnosis of this disorder in patients presenting with chronic symmetrical joint contractures, but without acute inflammatory symptom.

Age Factors↗

[Chronic dementia in Parkinson disease treated by long-term administration of anticholinergic drug--evaluation of neuropsychological test, PET, and SPECT].

It is known that the anticholinergic drug causes acute psychosis and memory disturbance, which may be due to cholinergic dysfunction. But it has not been well described that the anticholinergic drug causes chronic dementia after long administration. We describe six cases of chronic dementia, which is thought to be induced by anticholinergic drug. We conducted detailed neuropsychological and neuroradiological examination in these six cases. All of them were Parkinson's disease, and had used anticholinergic drug for more than six months without any side effect, and then their initial symptom of dementia appeared, followed by chronic dementia. All cases recovered reversibly from dementia within a few weeks after stoppage of taking anticholinergic drug. And any of our cases had no possibility in the cause of chronic dementia other than the side effect of anticholinergic drug. Detailed neuropsychological examinations (WAIS, WMSR, and so on) were conducted in all cases, SPECT was in two cases, and PET was in one case, before and after stoppage of the anticholinergic drug. And in all cases, brain MRI was conducted. The indices of attention, verbal memory, visual memory, and delayed recall were significantly elevated after stoppage of drug. And both verbal IQ and performance IQ were increased after stoppage of drug, too. SPECT and PET revealed significant improvement, which showed diffuse and generalized change, not localized change.

Aged↗

[Muscle contraction headache and posture--with special reference to ischemic contraction of the posterior neck muscles].

Cause of muscle contraction headache (MCH) results from sustained contraction of scalp and posterior neck muscles. Recently, we published an effect of posture on the etiology of MCH. According to our data, head bending posture seems to be one of the main causes of sustained contraction of the posterior neck muscles. Wolff presented a hypothesis of ischemic contraction of these muscles as a cause of pain. However, about the blood flow of scalp or posterior neck muscles, only two reports were published so far. These two papers failed to demonstrate a reduction of blood flow in MCH patients, and neglected the effect of posture. The purpose of this report is to examine a change of blood flow of posterior neck muscles with the change of posture. A total of 40 patients with MCH were studied using laser doppler blood flow meter. Needle shaped probe with a diameter of 0.55 mm was inserted 15 mm into the posterior neck muscle. The angle between orbito-meatal line and horizontal plane were measured using a light helmet with goniometer. Surface EMG of the posterior neck muscles was recorded at the same time. In the case of controls who do not experience headache, the amplitude of EMG increases slightly with the bending posture (40 microV with OM line 20 degrees upward from the horizontal plane, 46 microV with OM line horizontal, and 52 microV when 30 degrees downward). In reverse to the increase of the EMG activity, blood flow of the neck muscles decreases (12 ml/100 g/min with OM line 20 degrees upward, 10.8 with OM line horizontal, 7.6 at 10 degrees down, 4.6 at 20 degrees down, and 4.1 at 30 degrees down).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Remarkable effect of class Ib Na channel blocker on painful alcoholic neuropathy].

Two cases of painful alcoholic neuropathy are reported. The pain in both cases were incurable by using pain killing drugs, but administration of class Ib Na channel blocker, mexiletine, was remarkably effective. In the first case, suspension of drug administration produced the recurrence in the pain. Objective findings, for example, deep tendon reflex, vibration sense, and nerve conduction velocity, did not change significantly, after medication. And the only side effect was hot sensation in the lower extremities. We report the pain killing effect of class Ib Na channel blocker on painful alcoholic neuropathy, that has not yet reported.

Alcoholism↗

[A case of flexion myelopathy presenting juvenile segmental muscular atrophy of upper extremities--a successful treatment by cervical spine immobilization].

A 24-year-old man was well until 1982, when he noticed weakness and atrophy of right arm especially those of biceps muscles. These symptoms did not progress until 1988. In 1988 weakness of left elbow flexion appeared and he was admitted to our hospital. On examination, he had weakness and atrophy of both upper extremities. Fasciculation was noticed in the proximal part of right arm. When his head was bent forward, dysesthesia appeared around radial side of his arm. There were no long tract sign or objective sensory loss. EMG showed neurogenic change over both his upper extremities. In plain cervical roentgenogram there existed abnormal kyphosis of cervical vertebrae. In MRI imaged with his neck flexed, cervical cord was compressed by the posterior surface of 4th cervical vertebral body, which corresponded to the top of cervical kyphosis. Based on these signs and examinations, a diagnosis of flexion myelopathy was made. Spinal immobilization by wiring and bone graft implantation ranging from C2 to C6 was performed to limit the range of anterior nuchal flexion. After this operation, improvement of muscle strength along with the disappearance of dysesthesia on neck flexion was observed. Clinically, this case closely resembles to juvenile type of distal and segmental muscular atrophy of upper extremities (Hirayama type). From our study, pathomechanism of this case seems to be a flexion myelopathy at C4 level. This view is supported also by the good recovery after the operation. This case suggests that nonprogressive atrophy of upper extremities due to flexion myelopathy can be successfully treated by cervical spine immobilization.

Adult↗

[A case of long thoracic nerve palsy, with winged scapula, as a result of prolonged exertion on practicing archery].

Reports of isolated long thoracic nerve palsy are rare in Japan. We reported a case of isolated long thoracic nerve palsy, resulted from recurrent injury to the nerve. Muscle CT and electrodiagnostic study were useful for confirming diagnosis of this cases. This patient was a student aged 20 years, with nothing of importance in his family or past history. After he started practicing archery, winging of left scapula was gradually developed. Physical examination revealed weakness and atrophy of left serratus anterior muscle. There was no wasting and weakness of other should girdle muscles. Hematochemical tests were normal, except slight hyperthyroidism. Radiography and myelography of the cervical spine were normal. Muscle CT of upper thoracic level demonstrated atrophy of left serratus anterior muscle, and no abnormality were found in other muscles. Electromyogram of the left serratus anterior revealed discrete activity of reduced amplitude, and fibrillation potentials and positive sharp waves. Conduction time for left long thoracic nerve was prolonged, and amplitude of the evoked response was small and there were temporal dispersion. Muscle CT and electrodiagnostic studies were suggestive of neuroapraxia of left long thoracic nerve. Over stretching or compression during exercises may be responsible for the damage to the long thoracic nerve.

Adult↗

[Neurosarcoidosis].

It is easy to identify patients with neurosarcoidosis who have a lesion the neural lesion. However, it is be difficult if the patient has a preceding neural lesion or isolated CNS sarcoidosis in which the lesion is localized in CNS and the lesion in another organ hem regressed. The finding of generalized or localized cerebral edema and the shadow of tumor on CT, and the result of CSF examination which indicates an increase in cell count, protein or ACE activity (especially in meningitis type) are helpful for the diagnosis of the above disorder. Epidemiological studies on the basis of 21 patients with neurosarcoidosis in our center and 166 patients in our country indicated that this disorder was more common in females than in males (1.6/1). The age of patients showed predominance in the twenties in males, and the twenties, forties and fifties in females. Common pathological finding in our postmortem examinations (n = 2) and intracranial biopsy (n = 1) was lymphocytic phlebitis in central and peripheral neurons and in neurons with no clinical symptom.

Adult↗

[Significance of flexed posture and neck instability as a cause of chronic muscle contraction headache].

Cause of a muscle contraction headache (MCH) is due to sustained contraction of the neck and head muscles. This is proved by the effect of local anesthesia and EMG findings. However, the pathophysiology of abnormal muscle contraction is still unclear. Also, there is no explanation about female's greater susceptibility to MCH. The purpose of our report is to study the mechanism of abnormal muscle contraction and to find out a way to prevent it. We have examined 826 (572 female & 254 male) patients with MCH using EMG and dynamic X-ray. Results are most of MCH patients have a tendency to bend their head downward at the onset of headache. EMG shows continuous discharge of the posterior neck muscles so long as they keep this posture. Once they look up, however, EMG discharge usually subsides. Therefore, these muscle contraction are not involuntary, but passive as the results of drooping head. In 12 patients, severe attack of MCH were temporarily alleviated by the local anesthesia to the suboccipital tender point. In patients complaining MCH of one side, they often have a tendency to bend their neck toward the opposite direction, thus contracting the painful side of the neck muscles. It is also found that looking up to make orbito-meatal line more than 10 degrees from horizontal plane is enough to minimize muscle contraction, and to prevent or alleviate headache. Not all people experience headache, though.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Physiological and clinical correlates of cardiorespiratory reflexes in diabetes mellitus.

Cardiorespiratory reflexes (CRRs) were studied by measuring heart-rate (HR) variation during 6 breaths/min respiration (delta R6) and Valsalva maneuver (VR) in 145 healthy and 417 type I (insulin-dependent) diabetic subjects. HR variation with breathing at 12 breaths/min and ventilatory response to hypercapnia/hypoxia were measured in fewer subjects. CRR results were compared with symptoms of autonomic dysfunction, the neurological examination, nerve conduction studies, and quantitative sweat testing. The objective was to compare the sensitivity of various methods of characterizing diabetic patients and to use this information when staging patients for clinical therapeutic trials. CRR responses were age dependent in both populations. Either delta R6 or VR was abnormal in 74% of diabetic patients, delta R6 being more sensitive. CRRs correlated well with the presence of symptoms of autonomic dysfunction, abnormalities on the neurological examination, results of nerve conduction studies, and sweating activity in the feet of the same patients. However, both CRRs and sweating were abnormal in a high proportion of patients without any clinical manifestations of neuropathy. The ventilatory reflex response to moderate hypercapnia/hypoxia was also measured. It was normal in most of the diabetic patients tested, including many with severe reduction of CRRs. We conclude from the combined results of CRR, ventilatory response, and other studies that the causative factors for abnormal CRR may not be confined to the vagus nerves, and that in most instances, the depressed CRR may be due to a decrease in the efficacy of sensorimotor nerve conduction around the reflex arc.

Adolescent↗

[Paralytic ileus and atonic bladder in a case of mitochondrial encephalomyopathy--electrophysiological, chemical and pathological study with evidence of the peripheral nerve involvement].

A 41-year-old female of mitochondrial myopathy characterized by recurrent paralytic ileus and atonic bladder with the evidence of peripheral nerve involvement was described. This patient was admitted to our hospital because of the episode of paralytic ileus and atonic bladder at the age of 40 and 41 (1987). She had noticed sporadic headache from 1967, constipation from 1977, tinnitus and hearing disturbance from 1984. One month after her second admission in 1987, her symptoms of paralytic ileus and atonic bladder gradually disappeared. She was then transferred to the department of neurology for the evaluation of underlining neurological disorders. Neurological examination revealed dementia, oro-lingual dyskinesia, and proximal muscular weakness. However, none of the following signs or symptoms were observed; Ophthalmoplegia, blepharoptosis, retinitis pigmentosa, myoclonus, cerebellar ataxia, sensory disturbance, and orthostatic hypotension. Deep tendon reflexes were normal. Planter responses were flexor. Pyruvate and lactate were elevated in both serum and cerebrospinal fluid. Brain CT scan displayed moderate cerebral atrophy and basal ganglia calcifications. EMG was normal except for the external anal sphincter muscles which showed a denervation pattern. Motor nerve conduction velocity was normal in the right median and the right peroneal nerves. Sensory nerve conduction velocity was also normal in the right median and the right sural nerves. However, the amplitude of sensory potential was low in both these nerves. Atonic type of neurogenic bladder was noted on cystometry. There was a lack of voiding desire. The number of active sweat glands iontophoretically stimulated by pilocarpine was reduced. The most prominent feature of the muscle biopsy (the left biceps brachii) was myopathic changes with ragged-red fibers.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Recurrent episodic unilateral mydriasis with pleocytosis in the cerebrospinal fluid--a case report].

A 24-year-old female was admitted to our hospital on Aug. 20 in 1986 because of blurred vision and right pupillary dilatation. She had sometimes noticed headache later than 1976, and blurred vision without headache several times a year later than 1983. She had been told her right pupil dilated when she had complained of blurred vision. Neurological examination revealed abnormal findings as follows; diminished sense of smell in the right side, anisocoria (R 8 mm, L 5 mm), bilateral hippus, hypesthesioalgesia in her right face, left trunk and left arm. The pupils were round and contracted promptly to light. Accommodation reflex and ciliospinal reflexes were normal. Neither blepharoptosis nor external ocular muscle paresis were observed. Deep tendon reflexes were normal. Planter responses were flexor. There was no meningeal irritative sign. No abnormal findings were obtained in blood and urine, chest X-p, brain enhanced CT scan, EEG, and cerebral angiography except for slight degree of anemia. Serum TPHA was negative. However, the cell count of cerebrospinal fluid (CSF) was 18/mm3 (Ly 100%) and decreased to 9/mm3 (Ly 100%) in nine days. Protein content and glucose level of CSF were normal. Pupils were not constricted by 0.125% pilocarpine instillation. Loss of smell and sensory disturbance disappeared within three days and her pupils became isocoric by five days after admission. The patients of episodic unilateral mydriasis without apparent cause had relatively same clinical features as "unilateral springing pupil" proposed by Hallett et al. (1970). Except for mydriasis, they had no abnormal findings of neurological and laboratory examinations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Readiness potential associated with an athetotic movement].

A 28-year-old man was admitted to our hospital because of focal seizures probably generated by the right sensorimotor cortex with secondary generalization for 3 months. He had 5 years history of keratoconjunctivitis and iriditis. Physical examination revealed minimal hemiparesis and hyperreflexia in the left side and left papillitis. Cerebrospinal fluid analysis showed 75 cells per cubic-millimeter (all lymphocytes) and 88 mg/dl protein. Computerized tomography of the brain revealed elimination of the sulcus and vague enhancement of the cortex in the right cerebral hemisphere. The symptoms and the laboratory data improved spontaneously in a few months. Slow, athetotic movements were observed in the left forearm extensor muscles for about 10 days period. Electromyography (EMG) of these movements showed bursts of 300-800 sec in duration which repeated almost regularly at a varying frequency of 0.3-0.5 msec. The electroencephalographic (EEG) studies revealed slow background activities over the right hemisphere. The simultaneous recording of EEGs and EMGs demonstrated no EEG activity correlated with the athetotic movements. In the jerk-locked averaging (JLA) recorded with the time constant of 0.3 sec, no spiky or sharp EEG activities were detected before the EMG discharges of the left forearm extensor muscles. However, JLA with the time constant of 3.2 sec disclosed a slow negative shift which preceded the athetotic movement by about 500 msec. This negative potential was fairly localized in the right central area, which was thought to be consistent with the hand area of the motor cortex contralateral to the muscles showing the athetotic movements. The present case had a wide-spread, mainly unilateral lesion in the cerebral hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)

Action Potentials↗

[Chronic inflammatory demyelinating polyradiculoneuropathy of acute onset: relapse after temporary improvement by plasma exchange and longstanding remission by corticosteroid therapy].

A 59-year-old woman became tetraparetic and unable to stand or walk acutely in several days after she had first developed weakness of her legs and dysesthesia of both hands. Neurological examination revealed facial weakness, proximal dominant tetraparesis, areflexia and acrodysesthesia. The cerebrospinal fluid protein was elevated without pleocytosis, and the nerve conduction velocity was markedly slowed. The sural nerve biopsy indicated demyelination. The tentative diagnosis was Guillain-Barré syndrome (GBS). The initial and second courses of plasma exchanges temporarily improved the weakness, but relapses occurred within a few weeks. The third course of plasma exchange was, therefore, immediately followed by corticosteroid therapy, which successfully improved the tetraparesis. However, weakness again worsened during the rapid reduction of corticosteroid. A high dose of corticosteroid was again started and maintained for two months. The patient recovered gradually and relapses had not occurred so far after gradual reduction of the dose of corticosteroid. Her disease was finally diagnosed as corticosteroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy, although the onset had been as acute as that of GBS. The present case suggests that corticosteroid-dependent chronic inflammatory demyelinating polyradiculoneuropathy may present as a case of acute onset polyradiculoneuropathy resembling GBS, and that corticosteroid therapy must be tried in such a case when plasma exchanges are not effective or relapses occur.

Acute Disease↗

Free functional musculocutaneous transfer: electrophysiological studies.

Transplanted autogenous muscles were electrophysiologically studied in 2 patients with forearm crush injury. Only fibrillation action potentials and positive sharp waves were seen at 1 month after the operation. Low-amplitude and short-duration muscular unit potentials (MUPs) appeared at 3 months and high-amplitude, long-duration and polyphasic MUPs were frequently seen at 6-7 months after the transplantation. The distal latencies of the evoked motor responses were gradually shortened from time to time and eventually became normal at about 1 year after the grafting. The mean consecutive difference became normal at about 6 months after the normalization of the distal latencies. We conclude that the motor endplate is functionally completed at about half a year after the completion of the myelination of the grafted nerve.

Action Potentials↗