Biomedical subjects

S Hitoshi

Publications and source records attributed to S Hitoshi.

32 records · Page 2Linked to original sources

PubMed1994

Cerebellar ataxia and polyneuropathy in a patient with IgM M-protein specific to the Gal(beta 1-3)GalNAc epitope.

A 79-year-old man with sensory dominant polyneuropathy, cerebellar ataxia, and palatal myoclonus had serum IgM M-protein that specifically bound to GM1, GD1b, and asialo-GM1. IgM with the same specificity was detected in his cerebrospinal fluid. Results of immunohistochemical studies showed specific binding of this monoclonal IgM to the cerebellar granular layer, dentate nucleus, inferior olive, and gray matter of the cerebrum and spinal cord. Monoclonal antibody GGR12, monospecific to GD1b, had an immunostaining distribution similar to that of the patient's IgM M-protein. The binding of M-protein may be associated with the development of cerebellar ataxia and palatal myoclonus in this patient.

Aged↗

PubMed1994

Elevated serum interleukin-6 in POEMS syndrome reflects the activity of the disease.

In patients with POEMS syndrome, which often accompanies plasma cell dyscrasia, the roles of interleukin-6 (IL-6) and other acute phase cytokines are unknown. Serum IL-6, interleukin-1 beta (IL-1 beta) and tumor necrosis factor-alpha (TNF-alpha) were measured by the enzyme-linked immunosorbent assay technique in 16 patients with POEMS syndrome and in patients with other neurological diseases (OND) as control. Serum IL-6 was more frequently detected and higher in active POEMS than in stable POEMS or in OND patients. No sample was positive for IL-1 beta. TNF-alpha was positive in 3 out of 7 active POEMS patients, but its significance was not definite. Serum IL-6 levels in POEMS patients reflected the disease activity but not the severity of accompanying plasma cell dyscrasia.

Adult↗

PubMed1994

[Adult-onset dementia with abundant neurofibrillary tangles resembling progressive supranuclear palsy].

We described an autopsy case of 79-year-old woman with clinically unclassified senile dementia. The patient developed forgetfulness at the age of 73, and later, persecution mania, apathy and episodic stupor, but no extrapyramidal symptoms. Neuropathological examination revealed severe neuronal loss and gliosis of substantia nigra, moderate neuronal loss and marked grumose degeneration of dentate nucleus, and mild astrocytosis of subthalamic nucleus. Abundant neurofibrillary tangles (NFT) were observed in subthalamic nucleus, globus pallidus, substantia nigra, locus ceruleus, tegmentum of brain stem, pontine nucleus, inferior olive, and dentate nucleus. Gallyas silver impregnation method showed a wide distribution of argyrophilic grains and threads in cerebrum, brain stem and cerebellum. Although absence of clinical and neuropathological hallmarks excluded the diagnosis of progressive supranuclear palsy (PSP), the distribution of NFT and argyrophilic grains in this patient resembled PSP.

Age of Onset↗

PubMed1993

Double mutations at codon 180 and codon 232 of the PRNP gene in an apparently sporadic case of Creutzfeldt-Jakob disease.

Several polymorphisms of the prion protein gene are associated with the occurrence of familial Creutzfeldt-Jakob disease. We described a 84-year-old Japanese man with neuropathologically verified Creutzfeldt-Jakob disease of apparently sporadic type. His clinical presentation was atypical in point of a very late age at onset and absence of periodic synchronous discharge on electroencephalography. The patient carried double hitherto undescribed mutations of the prion protein gene; at codon 180 on one allele and at codon 232 on another. The mutation at codon 180 abolishes the Tth111I cutting site, which may be misunderstood to represent codon 178 mutation on routine restriction fragment length polymorphism study.

Aged↗

PubMed1993

Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome.

We found 3 novel missense variants in the open reading frame of the prion protein (PrP) gene. The codon 105 point mutation (proline to leucine) was found on a codon 129 (Valine) PrP allele in 4 patients from 3 different Japanese families with Gerstmann-Sträussler syndrome. The codon 180 variant PrP (valine to isoleucine) was found in Creutzfeldt-Jakob disease (CJD) patients with a similar clinical course to that of codon 178 mutation. The codon 232 variant PrP (methionine to arginine) was documented in the CJD patients with typical clinical and pathological findings. These variant PrP molecules were not detected in 200 normal Japanese PrP alleles. PrP has a large repertoire of variant forms, and each primary structure of PrP corresponds to the distinct phenotype of prion diseases.

Base Sequence↗

PubMed1993

Portal-systemic encephalopathy and hypothalamic hypothyroidism: effect of thyroid hormone on ammonia metabolism.

We describe a 53-year-old woman with portal-systemic encephalopathy and altered thyroid function. Endocrinological studies revealed low levels of free thyroid hormone with an inappropriately low level of thyroid-stimulating hormone that responded to bolus injection of thyrotropin-releasing hormone with a normal but somewhat delayed pattern. On the diagnosis of hypothalamic hypothyroidism, she was treated with levothyroxine sodium. Thyroid hormone replacement improved not only the symptoms of hypothyroidism but the hyperammonemia and consciousness disturbance, which suggested a hitherto undescribed possibility that hypothyroidism may be an exacerbation factor of hyperammonemia and portal-systemic encephalopathy.

Ammonia↗

PubMed1992

[The effect of anticholinergic drugs on 123I-IMP SPECT in Parkinson's disease].

Several reports have suggested that anticholinergics have some associations with mental deterioration in Parkinson's disease (PD). We investigated the effect of anticholinergics on regional cerebral uptake of tracer in PD patients using N-isopropyl p-[I-123] iodoamphetamine SPECT. Sixteen pairs of region of interest (ROI) were located in the cortex, a pair in the basal ganglia, thalamus, and cerebellum. The size of each ROI was about 16 mm x 16 mm. Regional cerebral uptake ratio (rCUR) was calculated by the next equation: rCUR = (total count in an ORI)/(mean of total count in the cerebellar ROIs). The comparison consisted of two parts; (1) 7 PD patients (age 59-76 (65.4 +/- 6.7 mean +/- S.D.)) who had been on chronic anticholinergic therapy underwent SPECT and Wechsler Adult Intelligence Scale (WAIS) twice, for the first time when they were on anticholinergics and second a month after discontinuation of anticholinergics. All but two patients performed significantly better on WAIS after discontinuation than when they were on anticholinergics. The improvement was about 10 points in total IQ. (2) 11 PD patients (age 52-79 (64.5 +/- 8.6 mean +/- S.D.)) on chronic anticholinergic therapy including all but two patients mentioned above (group A) and 25 PD patients (age 52-88 (66.7 +/- 9.8 mean +/- S.D.)) not receiving anticholinergics (group B) also underwent SEPCT. In the comparison (1), at all but two ROIs was the mean rCUR higher after discontinuation than when they were on anticholinergics and the difference was significant at 10 ROIs out of 32 ROIs in the cortex.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1992

[The role of interleukin-6 in Crow-Fukase syndrome].

We measured serum interleukin-6 (IL-6) levels in 14 patients with Crow-Fukase syndrome. Five out of 14 patients with Crow-Fukase syndrome showed high serum IL-6 levels above 10 pg/ml, which was statistically significant in comparison with control subjects with other neurological diseases. Serial studies of serum IL-6 levels in two patients revealed the increase before the exacerbation of clinical symptoms of edema, and pleural or cardiac effusion, and the fall after the treatment by high dose pulsed methylprednisolone. We suggest that serum IL-6 level appears to be a useful marker to predict its exacerbation. Also we performed immunohistochemical study on cutaneous angioma from three Crow-Fukase syndrome patients using anti-IL-6 antibody. The cytoplasm of endothelial cells of cutaneous angioma from two patients was positively stained, which might imply the abnormality of endothelial cells in Crow-Fukase syndrome.

Endothelium, Vascular↗

PubMed1992

[Gerstmann-Sträussler-Scheinker disease with heterozygous codon change at prion protein codon 129].

A 53-year-old male was admitted to our hospital for progressive dementia and gait disturbance which had started at the age of 48. Examination indicated dementia, dysarthria, dysphagia, bilateral pyramidal signs, apraxia of the limbs, and extrapyramidal signs such as fine finger tremors, and rigidity of limbs. There were no cerebellar signs or myoclonus. His mother and elder brother showed similar symptoms and died at the ages of 53 and 50, respectively. EEG was normal. CT and MRI showed mild brain atrophy, but no cerebellar atrophy. T2 weighted image indicated low intensity areas covering bilateral caudate nuclei and putamina. A heterozygous amino acid change from methionine to valine was noted at codon 129 of the prion protein of the patient as well as in one of his son. The most likely diagnosis was Gerstmann-Sträussler-Scheinker (GSS) disease without cerebellar atrophy. GSS may include a broad spectrum of brain pathology. Whether the codon change is associated with pathology without cerebellar atrophy is a problem that awaits further investigation.

Atrophy↗

PubMed1992

[A case of portal-systemic encephalopathy presenting characteristic MR images in globus pallidus, hypothalamus, corpus callosum, pontine base, and middle cerebellar peduncle].

A 53-year-old woman developed symptoms of slow speech, mild dementia, increased deep tendon reflex, ataxic gait, flapping tremor, and dystonic posture during two years. She had liver cirrhosis and hyperammonemia, which suggested a diagnosis of portal-systemic encephalopathy. MR T1-weighted images showed increased signal intensity in globus pallidus, internal capsule, substantia innominata, and a part of hypothalamus. T2-weighted images revealed abnormal findings as follows: high intense area in middle cerebellar peduncle, and low signal intensity of corpus callosum and pontine base. This is the first report about MRI abnormalities in substantia innominata, corpus callosum, and pontine base in a patient with portal-systemic encephalopathy.

Brain↗

PubMed1991

Mid-brain pathology of Wilson's disease: MRI analysis of three cases.

MRI scans were obtained from three patients with Wilson's disease, all of whom showed rigidity and dysarthria; two also showed tremor and dystonia. Two had been treated with D-penicillamine for seven and 14 years, respectively and their neurological abnormalities had improved, but the third patient had not been treated. T2-weighted MRI of the mid-brain in all three revealed the characteristic "face of the giant panda" sign, consisting of high signal intensity in the tegmentum except for red nucleus, preservation of signal intensity of the lateral portion of the pars reticulata of the substantia nigra and hypo-intensity of the superior colliculus. The clinical significance of these MRI abnormalities is discussed.

Adolescent↗

PubMed1991

[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↗

PubMed1990

[High field magnetic resonance imaging in Wilson's disease].

Magnetic resonance imaging studies on 3 cases with Wilson's disease were performed, using high field magnetic resonance system of 1.5 tesla. All patients had neurological findings of tremor, rigidity, dystonia or dysarthria at onset. Two patients had been treated with D-penicillamine for 14 years and 7 years respectively, and one patient was not treated then. T2-weighted images revealed abnormalities of signal intensity in lenticular nucleus, thalamus, pulvinar, superior colliculus, lateral portion of substantia nigra, midbrain and pontine tegmentum, and cerebral and cerebellar white-matter. Especially noted were following three hitherto undescribed abnormalities; high signal intensity of globus pallidus which normally shows very low signal intensity, restoration of signal intensity of lateral portion of substantia nigra, and marked low signal intensity of pulvinar and superior colliculus.

Adolescent↗

PubMedDate not supplied

A case of malignant hypertension and scleroderma after cosmetic surgery.

A 44-year-old woman with scleroderma-like skin lesions and malignant hypertension following mammoplasty is reported. Sclerotic change is an unusual finding for ordinary finding progressive systemic sclerosis. On admission, she had severe high-renin hypertension and progressive renal failure, suggesting scleroderma renal crisis. With intensive treatment for hypertension including angiotensin-converting enzyme inhibitor, the blood pressure was well controlled. It was then suggested that she had malignant hypertension due to scleroderma after silicone injection, or the so-called human adjuvant disease after cosmetic surgery.

Adjuvants, Immunologic↗