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Biomedical subjects

Shoichi Ito

Publications and source records attributed to Shoichi Ito.

At least 19 recordsLinked to original sources

Mechanism of constipation in familial amyloid polyneuropathy: a case report.

Previously no functional study has been available for the mechanism of constipation in familial amyloid polyneuropathy (FAP). We performed a gut function test in a 78-year-old woman with transthyretin-type FAP who had severe constipation. The gut function test showed a prolonged colonic transit time, a low anal pressure at rest and upon squeezing, loss of the spontaneous phasic rectal contractions, and weak abdominal strain. All these abnormalities suggested a peripheral autonomic dysfunction, corresponding to the enteric neuropathy that was confirmed upon autopsy.

Aged↗

Urinary dysfunction and autonomic control in amyloid neuropathy.

Uro-neurological assessment was performed in four patients with small-fiber neuropathy due to amyloidosis (2 transthyretin-type/2 immunoglobulin light-chain-type). Voiding difficulties were due to detrusor weakness and impaired bladder sensation. In two patients cholinesterase inhibition treatment caused urge incontinence, indicating detrusor denervation supersensitivity. The underlying mechanisms of urinary dysfunction seem to involve postganglionic cholinergic and afferent somatic nerves.

Aged↗

The importance of bacterial superantigens produced by Staphylococcus aureus in the treatment of atopic dermatitis using povidone-iodine.

Atopic dermatitis (AD) is frequently associated with intestinal and cervical lesions. Staphylococcus aureus produces many kinds of toxins, the bacterial superantigens. The detection rate of toxins was 80.1% from 196 S. aureus strains. Neurological examinations revealed abnormalities in 59 out of 81 AD patients. Cervical magnetic resonance imaging (MRI) was performed in 46 patients randomly and showed abnormal findings in 38 of these patients. In 23 patients who underwent MRI and duodenal biopsy, 3 were found to be normal neurologically and 2 patients showed normal duodenal tissue. However, 18 patients had abnormal findings both on neurological examination and in duodenal tissue. Serial duodenal biopsy tests were performed in 10 AD patients. In 5 patients, the findings of chronic duodenitis disappeared after the therapy with povidone-iodine. These data indicate that the therapy was effective not only for the skin lesions, but improved gastrointestinal tract lesions and cervical myelopathy, by eradicating bacterial superantigens.

Adolescent↗

[A patient with coccidioidal meningoencephalitis].

A 37-year-old man presented with coccidioidal meningoencephalitis (CM) 1 month after a preceding case of pneumonia. Initially, he could not be definitely diagnosed with CM because of nonspecific features of the clinical, laboratory, and radiological findings. However, we began to suspect CM because the patient had lived in endemic area of coccidioidomycosis, and our subsequent analysis provided evidence of complement-fixing antibodies for Coccidioides immitis in serum and CSF, leading us to a final diagnosis. The CM was intractable, despite intensive administration of fluconazole and amphotericin B. Although the patient's CM gradually and mildly improved, he also suffered from bacterial meningoencephalitis and left putaminal hemorrhage with intraventricular hematoma, which caused persistent right hemiparesis and dementia. The incidence of coccidioidomycosis in Japan is rapidly increasing. The initial clinical manifestation of coccidioidomycosis is usually pneumonia, which in most cases heals spontaneously. Coccidioidomycosis rarely presents with meningoencephalitis, which is thought to be fatal. Immediate and adequate initiation of anti-fugal treatment is necessary to obtain a better prognosis for CM. Careful history-taking after a foreign trip is helpful when there is a suspicion of coccidioidomycosis.

Adult↗

Combined treatment with LDL-apheresis, chenodeoxycholic acid and HMG-CoA reductase inhibitor for cerebrotendinous xanthomatosis.

The effects of combined treatment with low-density lipoprotein (LDL)-apheresis, chenodeoxycholic acid (CDCA) and 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase inhibitor were studied in 2 patients with cerebrotendinous xanthomatosis. Patient 1 was initially treated with LDL-apheresis alone: serum cholestanol levels decreased by 50% after each apheresis, but returned to their initial levels within 2 weeks. After an addition of CDCA administration, the serum cholestanol levels steadily decreased, resulting in slight improvement of neurological symptoms. Patient 2 received a combined treatment with LDL-apheresis, CDCA and HMG-CoA reductase inhibitor. This combination showed less LDL-apheresis-dependent fluctuation and more rapid decrease of serum cholestanol levels than those in Patient 1, resulting in improvement and stabilization of the symptoms. Our results suggest that LDL-apheresis in combination with CDCA and HMG-CoA reductase inhibitor may have beneficial effects and can be one of the treatment options.

Adult↗

von Hippel-Lindau disease type 2A in a family with a duplicated 21-base-pair in-frame insertion mutation in the VHL gene.

BACKGROUND: von Hippel-Lindau disease (VHL), also called angiomatosis retinae, is inherited as an autosomal dominant trait. It is frequently associated with other tumors in the central nervous system, kidneys, or adrenal glands. In order to investigate the relationship between genotype and corresponding phenotypes, we performed molecular genetic analysis in a Japanese patient with VHL type 2A. METHODS: After informed consent had been obtained, the three exons of the VHL gene were PCR-amplified and sequenced either directly or after subcloning. Clinical features were also examined. RESULTS: A novel in-frame duplication of the 21 base pairs at nucleotide 806 (the position of codon 198) of the VHL gene was found in our patient. The clinical phenotype of the patient included retinal hemangiomas associated with vitreous hemorrhage and traction retinal detachment, pheochromocytoma, and hemangioma-like lesions in the cerebellum which corresponded to those of VHL type 2A. Abnormal diffuse vascular leakage was observed in the apparently intact retina by fluorescein angiography. CONCLUSION: An insertion mutation of the VHL gene is a rare association with VHL type 2. This insertion mutation may interfere the binding between the VHL gene and elongins. Abnormal retinal vascular leakage suggests the possible effects of overexpressed vascular permeability factors such as vascular endothelial growth factor from hemangiomas associated with defective VHL gene.

Adrenal Gland Neoplasms↗

Is atopic dermatitis a risk factor for intervertebral disc degeneration? A preliminary clinical and MRI study.

Atopic dermatitis (AD) is a common allergic disease that has recently been reported to be complicated with acute myelitis. To clinically evaluate the occurrence of myelitis in AD, 65 consecutive AD patients were neurologically examined. Of these, 37 underwent cervical MRI scans. Unexpectedly, the neurologic and MRI findings did not suggest myelitis, but rather, in most cases, cervical spondylosis. Therefore, we assessed the relationship between AD and cervical spondylosis. In addition, cervical MRI findings in 26 patients with multiple sclerosis (MS) and in 12 normal controls were also evaluated. The neurologic examinations in the AD patients frequently showed hyperreflexia in the legs, and sensory and motor disturbances were often present in the limbs. Cervical MRI findings suggestive of spondylosis, such as intervertebral disc degeneration and bulging/protrusion, were found more frequently in AD patients than in MS patients, with statistical significance. Posterior spondylolisthesis was observed with higher frequency in AD and MS patients than in normal controls. We concluded that AD might be a risk factor for intervertebral disc degeneration. As far as we know, this is the first paper describing the potential association between disc degeneration and AD.

Adolescent↗

Severe personality changes after unilateral left paramedian thalamic infarct.

Personality changes are not uncommon after paramedian thalamic infarction, but usually bilateral or relatively large lesions, often complicated by other neurological or neuropsychological deficits, are present. 'Pure' cases of unilateral lesions are extremely rare. We report that a right-handed, 48-year-old man, who was hypertensive and diabetic but had no prior psychiatric history, developed severe personality changes and a frontal-like syndrome after recovery from acute-onset impairment of consciousness at the age of 43. Other neurological and neuropsychological disturbances, especially verbal and visual amnesia, were unremarkable. MRI showed a very small infarct in the left paramedian area of the thalamus, mainly involving the dorsomedial nucleus.

Adult↗

The importance of disinfection therapy using povidone-iodine solution in atopic dermatitis.

The combination of the local disinfection therapy against Staphylococcus aureus with the conventional therapy for atopic dermatitis has been widely used, and the improvement in skin lesions has been reported to be associated with a remarkable decrease in IgE levels and reagin antibody titers. We have already reported that affected organs were not only the skin but also the gastrointestinal tract in a case with atopic dermatitis. In the present study, the duodenal tissues were examined by biopsy in 32 patients with atopic dermatitis, and mild or chronic duodenitis was observed in all samples. Toxins were examined by PCR from 180 Staphylococcus aureus strains obtained from our patients. The detection rate of toxins was 82.8%. In many patients, antitoxin IgE antibody titers corresponding to their types of toxin and IgE levels were decreased in a parallel manner as time passed. We found 1 patient who complained of paresthesia in all four limbs, and her neurological and radiological examinations showed moderate cervical spondylosis. Neurological examinations revealed some abnormalities in 43 out of 50 patients with atopic dermatitis, such as hyperreflexia of the legs. Cervical MRI was carried out randomly and showed abnormal findings in 21 of 25 patients, in whom 18 duodenal tissues were examined by biopsies.

Adolescent↗