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R Okiyama

Publications and source records attributed to R Okiyama.

At least 19 recordsLinked to original sources
PubMed1996

[An unusual autopsy case of corticobasal degeneration--with special reference to clinicopathological differentiation from progressive supranuclear palsy and slowly progressive aphasia].

We report an unusual autopsy case of corticobasal degeneration. The patient was male who was 67 years old at the time of his death. He developed clumsiness of his right hand at age 65. Neurological examination at age 66 revealed supranuclear ophthalmoplegia of upward gaze and parkinsonism. Progressive supranuclear palsy was suspected. The patient's clinical course was rapidly progressive. Dementia developed nine months after the onset of the disease, and the patient manifested apallic syndrome at 21 months and died of pneumonia at about 25 months. At autopsy the brain weighed 1370g. Macroscopic examination revealed prominent atrophy of the anterior and parietal lobes, particularly of the pars opercularis in the left inferior frontal gyrus, and there was marked depigmentation of the substantia nigra. Histological examination showed neuronal loss, glial proliferation, spongy state, and ballooned neurons in the frontal and parietal lobes. Neuronal loss with glial proliferation was conspicuous in the striatum, pallidum, thalamus, and substantia nigra. There was slight neuronal loss in the dentate nucleus and locus ceruleus. Staining by Gallyas-Braak method revealed massive appearance of argyrophilic threads. This case is important in terms of the clinical differentiation from progressive supranuclear palsy, and is also interesting in regard to the pathological correlation with slowly progressive aphasia.

Aged↗
PubMed1995

Effects of cueing on visuospatial processing in unilateral spatial neglect.

Patients with typical left unilateral spatial neglect bisected lines after cueing to the left end-point, the fixation point being monitored with an eye camera. They persisted with the point of initial fixation made after cueing and placed the mark there without searching leftwards again. The rightward shift of fixation to the initial point of fixation thus determined the location of the subjective midpoint. We consider that rightward attentional bias increased the amplitude of this shift that was planned on the basis of the perception of the whole line while cueing. This hypothesis may explain smaller but obvious rightward bisection errors found in the cueing condition.

Aged↗
PubMed1995

A new familial disorder presenting with amyotrophic lateral sclerosis-like manifestation: a clinicopathological study.

We studied a family in which three siblings had an identical clinical feature indistinguishable from familial amyotrophic lateral sclerosis (ALS), consisting of progressive generalized neurogenic muscular atrophy with hyporeflexia and normal sensations beginning in the fourth decade. The duration of illness was about 4 years in all affected members. Autopsy of one patient revealed multiple foci of spongy degeneration in the white matter of the spinal cord, brain stem, cerebellum, and the thalamus, characterized by vacuoles of various size, foamy macrophages and degenerating swollen axons. These changes were most marked in the spinal cord, where there was neither pyramidal tract involvement nor neuronal loss in the anterior horn. The pathological findings were different from those of ALS. A similar disease affected the siblings' mother, suggesting an autosomal dominant inheritance. The disease in the kindred, therefore, appears to be a unique hereditary disorder.

Amyotrophic Lateral Sclerosis↗
PubMed1995

[Chronic inflammatory demyelinating polyneuropathy associated with chronic liver disease due to type B and type C hepatitis virus].

A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) associated with type B and type C hepatitis virus infection is reported. A 54-year-old female who had a blood transfusion at the age of 31 years was diagnosed as a carrier of hepatitis B virus at the age of 43. Liver dysfunction was first noted in 1987 and gradually grew worse year by year. Beginning in early June 1992, the patients general fatigue became worse, her serum GOT and GPT levels became elevated, and she complained of a tingling sensation in her arms and legs. Neurological examination revealed moderate sensory disturbance of the glove-and-stocking type in all of her extremities. Deep tendon reflexes were all diminished. Hepatitis C antibody was detected in the serum at this time. On June 12, 1993, progression of her sensory disturbance was found to be associated with generalized muscle weakness. Cerebrospinal fluid studies showed increased protein without pleocytosis. Motor nerve conduction studies revealed marked prolongation of terminal latencies, reduction of conduction velocities, and abnormal temporal dispersion of the motor potentials. No sensory potentials could be evoked at any of the sites stimulated. Sural nerve biopsy showed segmental demyelination and severe loss of large myelinated fibers as well as some onion bulb formation. A diagnosis of CIDP was made. Treatment with corticosteroids was started, but there was little improvement in neurological function. The liver dysfunction progressed further and ultimately the patient died of hepatic failure. An autopsy demonstrated liver cirrhosis, but no malignant tumors were evident.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic Disease↗
PubMed1994

[Acute encephalopathy with symmetrical lesions of the thalamus, the putamen and the cerebellum on magnetic resonance imaging].

A 19-year-old boy was admitted to our hospital because of impaired consciousness after experiencing fever and headaches for 7 days. The patient was in a deep coma or generalized convulsions, with retrocollis and decorticate posture. He never regained consciousness, remaining in an apallic state. The initial CSF pressure was 290 mm H2O, CSF cell count 18/3 mm3, protein 75 mg/100 ml, and sugar 74 mg/100 ml. There was no significant elevation in the viral titer in the serum or CSF. Plain CT on admission showed marked brain edema. MRI three months thereafter demonstrated symmetrical high intensity in the thalamus, the putamen and the cerebellum on both T1- and T2-weighted images, probably representing petechial bleeding. The lesions in the medial portion of the occipital lobes showed iso intensity on T1-weighted images and high intensity on T2-weighted images. These lesions were consistent with infarction of both posterior cerebral arteries, which may have been due to severe brain edema. The clinical and MRI findings were similar to those of patients with acute encephalopathy with low-density areas in the thalamus or bilateral striatal necrosis in childhood. To the best of our knowledge, there have been no reports of cases like ours in adults.

Acute Disease↗
PubMed1993

Mononeuropathy multiplex with ulcerative colitis.

We describe acute mononeuropathy multiplex in a patient with chronic ulcerative colitis. The symptoms of neuropathy were well correlated with the disease activity of colitis. Both electrophysiological study and sural nerve biopsy revealed axonal degeneration. Mononeuropathy multiplex may be an extraintestinal manifestation of ulcerative colitis.

Adult↗
PubMed1993

[A case of chronic thinner intoxication developing hyperkinésie volitionnelle three years after stopping thinner abuse].

We reported a 25-year-old male with chronic thinner intoxication. He had been sniffing thinner from the age of 15 to 20. Since the age of 20, he developed gait disturbance, weakness of lower extremities, and scanning speech. At the age of 23, three years after he stopped sniffing thinner, he had hyperkinésie volitionnelle (HV) of bilateral upper extremities. MRI study revealed diffuse high intensity areas in the cerebral white matter, thinning of the corpus callosum, and cerebellar and brainstem atrophy on T1 weighted images. On T2 weighted images, low intensity areas in the thalamus, the striatum, the anterior limb of the internal capsule, the tegmentum of midbrain, high intensity areas in middle cerebellar peduncle, the posterior limb of the internal capsule, the lateral part of the cerebral peduncle, and the cerebral white matter were noted. HV almost disappeared with 2.5 mg of clonazepam daily administered. The disappearance of HV by ischemic compression of his arm suggested that input from the periphery might be relevant to the etiology of HV. The time interval between exposure to thinner and the recognition of HV in our case was in agreement with that between the occurrence of cerebrovascular disease and the recognition of HV in the previous reports. We postulated that HV in this case was the manifestation of the denervation hypersensitivity in the dentatorubroolivary system.

Adult↗
PubMed1993

[Oculopharyngeal myopathy with autoimmune disease].

A 34-year-old woman had suffered from systemic lupus erythematosus (SLE) and autoimmune hemolytic anemia (AIHA) in the teen age. She developed progressive ptosis of the eyelids, and difficulty in swallowing and speaking for several years. Endocrinological studies showed primary hypothyroidism. A serum IgG level was elevated (1,973 mg/dl), and antinuclear antibody, thyroid test and microsome test were positive. A muscle biopsy showed massive inflammatory cell infiltrates in the perivascular area in addition to some myopathic change; some variation in fiber size. Immunological staining demonstrated most of these inflammatory cell infiltrates were CD3+ cells and CD4+ cells were counted more than CD8+ cells (CD4/CD8 = 2.3). The diagnoses were confirmed as oculopharyngeal myopathy and Hashimoto's disease. In addition, she had suffered from SLE and AIHA. Therefore we conclude that manifestation of this myopathy may be associated with some autoimmune process.

Adult↗
PubMed1993

[Saccadic eye movement related scalp potentials--scalp distribution of presaccadic slow negative potential].

We studied scalp distribution of presaccadic slow negative potential (PSN) among saccadic eye movement related scalp potentials in 17 normal subjects performing self-paced voluntary saccades in light (VS). There was a widespread PSN beginning at 1400 msec prior to saccades, maximum at the vertex. Amplitudes of PSN were greater over frontocentral electrodes contralateral to the direction of the saccades. These findings are consistent with reflection of activation of the supplementary motor area, and the frontal eye field contralateral to the direction of the saccades. On the other hand, amplitudes of PSN were relatively greater over occipital electrodes ipsilateral to the direction of the saccades. In addition, we studied PSN in 6 normal subjects performing self-paced voluntary saccades in darkness (VSD). PSN with VSD did not extend to occipital electrodes in comparison with PSN with VS. Occipital component of PSN with VS may reflect activation of other cerebral cortices which relate to visual inputs.

Adult↗
PubMed1992

[A case of periodic ataxia].

We report a sporadic case of periodic ataxia characterized by recurrent attacks of vertigo and ataxia. A 62-year-old male was known to have nystagmus at the age of 18. He has had recurrent episodes of vertigo and ataxia since the age of 48. During an attack remarkable downbeat nystagmus, limb ataxia predominant in the lower extremities and ataxic gait were present. MRI demonstrated an atrophy of the anterosuperior region of the cerebellar vermis. Vertical nystagmus, dysesthesia of gloves and stocking type and deep sensory disorder persisted during interictal intervals. There is no finding which supports this case to be vascular disorder, congenital anomaly, tumor, infection or demyelinating disease. We thought this case to be periodic ataxia and to belong to vestibulocerebellar ataxia reported by Farmer and his colleagues.

Cerebellar Ataxia↗
PubMed1991

Sympathetic skin response in patients with multiple sclerosis compared with patients with spinal cord transection and normal controls.

In 28 patients with definite multiple sclerosis (MS) and 21 patients with a functionally complete transection of the spinal cord sympathetic skin responses (SSR) from both the palms and soles were studied. In the patients with a complete transection at T3 or 4, SSRs were impaired in the palms as well as the soles, and most patients with a transection at T11 or T12 had normal SSRs in the soles. These findings indicate that the central pathway mediating the SSR descends to the upper thoracic cord where connections are made with the sympathetic distribution to the palms, and then to the lower thoracic cord to reach the sympathetic outflow to the soles. This is compatible with the anatomical knowledge of the sympathetic system. Of the MS patients, 75% had abnormal SSR results. The SSR for the soles was more sensitive than that for the palms. The incidence of SSR abnormality was as high as those of somatosensory evoked potential (SEP) (79%) and visual evoked potential (VEP) (75%) abnormalities. Of the patients with normal SEPs 14% had impaired SSRs and 21% had abnormal SSR with normal VEPs. When the results of these 3 tests were combined, the abnormality increased to 100%. From our study, the SSR probably reflects not only postganglionic sympathetic activity but also preganglionic function. The SSR is therefore useful in evaluating myelopathy, providing information different from that given by assessment of the somatic nervous system. Since the SSR detected a few MS lesions which were not demonstrated by other evoked potentials, it is potentially a new tool for the detection of MS lesions in addition to conventional evoked potential studies.

Adult↗
PubMed1991

Optokinetic response and adaptation of the vestibulo-ocular reflex (VOR) in a patient with chronic cortical blindness.

Optokinetic response and adaptation of the VOR were investigated in a patient with chronic cortical blindness. Our results suggest that: 1) optokinetic response was present in a patient with cortical blindness. This optokinetic response may relate to the extrastriate pathways: 2) the occurrence of VOR adaptation was shown in a patient with cortical blindness, which may indicate preservation of the pathways of the visual-vestibular interaction in the brain stem and cerebellum. The result is in agreement with previous reports in experimental animals; 3) less adaptation of the VOR in this patient than in normal adults may relate to a chronic loss of visual feed back. To the best of our knowledge, this is the first report of adaptation of the VOR in a patient with cortical blindness.

Adaptation, Physiological↗
PubMed1989

[An autopsy case of panencephalopathic type of Creutzfeldt-Jakob disease: an early clinical sign documented by magnetic resonance imaging].

We reported an autopsy case of panencephalopathic type of Creutzfeldt-Jakob disease (CJD), one of whose early clinical signs, visual loss, had been documented by magnetic resonance imaging (MRI). The patient was a 59-year-old woman, who showed disturbance of visual acuity, memory and calculation at the early stage. About 2 months later she developed apallic syndrome and revealed myoclonic jerks in her eye balls, face, neck and extremities. Electroencephalography showed periodic synchronous discharge. Three months later after her first symptoms MR showed bilateral occipital atrophy, although we could find no brain atrophy on CT scan. She died of the suffocation caused by bronchopneumonia at the age of 60 years. The total duration of her illness was about 20 months. Neuropathological examination revealed a pronounced diffuse atrophy of the cerebral cortex and white matter. In addition to severe cortical neuronal loss and astrocytosis, spongiform changes were evident. The hippocampus was relatively well preserved. In the cerebral white matter both myelin sheaths and axons disappeared almost completely. The cerebellum showed the severe degeneration of granule cells. These neuropathological findings are consistent with those of panencephalopathic type of CJD. It is important to clarify the abnormalities of MR findings in relation to such early clinical signs of CJD. To the best of our knowledge such reports as ours have not been published previously.

Atrophy↗