Biomedical subjects

J Kira

Publications and source records attributed to J Kira.

At least 37 records · Page 2Linked to original sources

PubMed2001

Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy.

Juvenile muscular atrophy of the distal upper limb (Hirayama disease) is a rare disease predominantly affecting the anterior horn cells of the cervical spinal cord in young men. Although the disease is considered to be a type of cervical myelopathy, the mechanism remains unknown. An immunological study of five consecutive patients with this disorder who were examined in the neurology clinic at Kyushu University Hospital during the past 2 years were performed. All developed distal muscular atrophy and weakness of one or both upper limbs in the second decade, and showed forward displacement of the dural sac and passive dilatation of the posterior venus plexus at the lower cervical portion on MRI during neck flexion. Four of the five patients had one or more coexistent airway allergies, such as allergic rhinitis, pollinosis, and asthma, and all five patients had a family history of atopic or allergic disorders in close relatives. Four of the five patients had mild eosinophilia. All five patients commonly had IgE specific to two mite antigens, Dermatophagoides pteronyssinus and Dermatophagoides farinae, whereas three of them also showed a raised total serum IgE concentration. The frequency of mite antigen specific IgE was significantly higher in the present patients with Hirayama disease than in 82 healthy controls (26/82, p<0.005). These findings suggest that atopy may be one of the contributing factors for Hirayama disease.

Adolescent↗

PubMed2001

Myelitis associated with atopic disorders in Japan: a retrospective clinical study of the past 20 years.

OBJECTIVE: To clarify the clinical features of myelitis associated with atopic disorders in Japanese patients. SUBJECTS AND METHODS: We retrospectively studied the clinical, immunological and electrophysiological features of 68 consecutive patients with myelitis of acute or subacute onset diagnosed at Kyushu University Hospital during the past 20 years. RESULTS: While only 2 of 28 (7%) patients with myelitis diagnosed between 1979 and 1993 had either atopic dermatitis (AD) or bronchial asthma (BA), 19 of 40 (48%) patients with myelitis diagnosed between 1994 and 1998 did. Among the 40 patients with myelitis diagnosed between 1994 and 1998, 19 patients with either AD or BA as well as 21 patients without either disease showed a significantly higher level of serum total IgE, higher frequency of hyperIgEaemia and higher frequency of mite antigen-specific IgE than 82 healthy controls. Myelitis patients with AD presenting as persistent paresthesia/dysesthesia in all four limbs showed cervical cord lesions on MRI and abnormalities in upper limb motor evoked potentials but no abnormalities in the cerebrospinal fluid (CSF), while myelitis patients with BA showed preferential involvement of the lower motor neurons clinically and electromyographically. In addition, 12 patients with myelitis who had hyperIgEaemia and mite antigen-specific IgE but neither AD nor BA showed incomplete transverse myelitis with mild motor disability and few CSF abnormalities. CONCLUSION: The clinical features of myelitis associated with atopic disorders were in part distinguished by the type of preceding atopic disorder, and also were different from those of hyperIgEaemic myelitis with no preceding atopic disorders.

Adult↗

PubMed2001

High-dose vitamin C therapy for inclusion body myositis.

OBJECTIVES: The efficiency of high-dose vitamin C therapy for inclusion body myositis (IBM) was assessed. SUBJECTS & METHODS: The subjects were five patients with IBM confirmed pathologically. After the intravenous administration of 40 mg/kg vitamin C five times/week for four weeks, muscle weakness was found to improve in three cases. The average muscle score improved from 8.1 to 8.8, from 7.0 to 8.1 and from 6.2 to 6.8. Magnetic resonance imaging (MRI) demonstrated a reduction in the size of T2 high lesions and gadolinium enhancement in the thigh muscles in one case. Based on our findings, high-dose vitamin C therapy is considered to be effective in some cases of IBM.

Aged↗

PubMed2001

[Multiple sclerosis: the recent progress of research and treatment].

In this review, we summarized the recent progress of research and treatment of multiple sclerosis (MS). The immunogenetic study including analyses of HLA polymorphism revealed that there are two distinct subtypes of MS: Asian-type and Western-type. Since MS is a polygenic disease, there must be several susceptible genes other than HLA. A large number of candidate genes are being searched and analyzed for polymorphism. Autoantigens responsible for MS are of great interest. Screening peptide library which bind to MS susceptible genes are expected to provide with new autoantigens. Advance in treatment is also remarkable. Since interferon (IFN)-beta 1b became available, great deal of data concerning IFN beta are accumulated. IFN beta not only reduced clinical exacerbation and MRI brain lesions but also slowed the development of brain atrophy. New treatments include altered peptide ligand therapy and oral tolerization with myelin antigens, which are on the clinical trial. Vaccination with myelin reactive T cells or T cell receptor peptide also attracts a great deal of attention.

Autoantigens↗

PubMed2001

[A case of paraneoplastic cerebellar degeneration with resting tremor].

A 65-year-old woman was operated for gastric adenocarcinoma in 1989. Six years later, peritonitis carcinomatosa, swelling of periaortic lymphnodes and high serum CA-125 were discovered. She received chemotherapy with 5-FU and cisplatin resulting in reduction of ascites. In September, 1998, the swelling of left supraclavicular lymphnodes and the elevation of serum CA-125 reappeared. Pathological diagnosis of supraclavicular lymphnodes was adenocarcinoma. Serum CA-125 was normalized by chemotherapy using cisplatin, farumorubicin and endoxan. However, unsteadiness appeared since December 10, 1998 followed by dysarthria and involuntary movement of neck and upper limbs. These symptoms progressed subacutely. The physical examination on admission revealed swelling of left suraclavicular lymphnodes, nystagmus on lateral gaze, saccadic eye movement on smooth pursuit and severe cerebellar ataxia. In addition, resting tremor of 3-4 Hz was observed at right hand, left wrist and neck which tended to increase amplitude by calculation. Similar movements were seen in the left first toe, though the frequency was lower. Brain MRI revealed mild cerebellar atrophy. She was diagnosed as paraneoplastic cerebellar degeneration (PCD) by serum anti Yo antibody and clinical course. The study of HLA showed positive link to A4 without A24. The primary focus of adenocarcinoma in cervical lymphnodes was suggested to be ovary rather than stomach due to the pattern of immunostaining for cytokeratin, CEA and CA125, although no carcinoma was found in ovarium clinically. The feature of this case is a PCD with resting tremor of frequency of 3-4 Hz and negative link to HLA-A24 in Japanese.

Adenocarcinoma↗

PubMed2001

Sporadic amyotrophic lateral sclerosis showing abnormal somatosensory evoked potentials: a report of three cases.

OBJECTIVE: Although dysfunction of the sensory systems in sporadic amyotrophic lateral sclerosis (ALS) has been reported, the clinical characteristics of such cases still remain unknown. We therefore performed a clinico-electrophysiological analysis of sporadic ALS patients. MATERIAL AND METHODS: Twelve ALS patients (aged 36-66 years), who had their somatosensory evoked potentials (SEPs) evaluated, were reviewed and their clinical characteristics were delineated. In addition, needle EMG, sensory nerve conduction velocities, motor evoked potentials (MEPs) and cervical MRI or plain X-ray of the neck were also recorded. RESULTS: Three cases were segregated from the other 9 patients because of predominant upper motor neuron signs with pseudobulbar palsy and abnormal posterior tibial nerve and/or median nerve SEPs. The MEPs were also abnormal in these 3 patients and the brainstem auditory evoked potentials were abnormal in one patient. EMG revealed less involvement in the lower motor neurons. CONCLUSION: Sporadic ALS with a predominant upper motor neuron sign and also demonstrating pseudobulbar palsy with abnormal SEPs, may therefore form a clinical subtype of ALS.

Adult↗

PubMed2001