Triphasic waves in a patient with tuberculous meningitis.

We report on the case of a 32-year-old woman with tuberculous meningitis (TBM) with electroencephalogram (EEG) output displaying triphasic waves (TWs). The EEG on day 8 revealed generalized slowing, frontal bilateral TWs, a background of 2Hz delta waves, and no epileptiform activity. The patient's condition improved slowly with antituberculosis chemotherapy treatment.

Autoimmune polyglandular syndrome type 2 with myasthenia gravis crisis.

We describe a rare case of autoimmune polyglandular syndrome type 2 initially presenting as Addison disease and autoimmune thyroid disease, with subsequent development of autoimmune hepatitis and myasthenia gravis (MG) crisis in a Japanese woman. MG improved with oral prednisolone followed by plasmapheresis for immunoadsorption; thymectomy was not performed. Conventional treatment for MG was effective and safe in this case, in which there was positivity for human leukocyte antigen A23, B52, B62, DR11, and DR15.

Suppressive potencies of calcineurin inhibitors against the mitogen-induced blastogenesis of peripheral-blood mononuclear cells of myasthenia gravis patients.

The calcineurin inhibitors, tacrolimus and ciclosporin, are two useful immunosuppressive drugs for the treatment of myasthenia gravis (MG), for patients who have low responses to glucocorticoids. We have studied the suppressive potencies of tacrolimus and ciclosporin on concanavalin A-induced blastogenesis of peripheral-blood mononuclear cells (PBMCs) obtained from 38 MG patients and 26 healthy volunteers. Differences in the IC50 values of the two calcineurin inhibitors between the patients and the healthy subjects were evaluated.

Familial Creutzfeldt-Jakob Disease with a codon 200 mutation presenting as thalamic syndrome: diagnosis by single photon emission computed tomography using (99m)Tc-ethyl cysteinate dimer.

The clinical features of familial Creutzfeldt-Jakob disease with a codon 200 point mutation [fCJD (E200K)] are similar to those of sporadic CJD (sCJD). MRI diffusion-weighted imaging (MRI-DWI) has been reported to be useful for the early diagnosis of CJD. We describe a Japanese fCJD (E200K) case in which thalamic symptoms were the initial manifestations.

Myasthenia gravis with concomitant severe paraspinal muscle degeneration and mitochondrial DNA4977 deletion.

Recent reports have discussed the many causes of dropped head syndrome and bent spine syndrome. We described a case of myasthenia gravis with concomitant severe degeneration of spinal muscle, mitochondrial DNA4977 deletion and sensorineural deafness. These associations were thought to be independent, however this is an important case to consider the etiology of bent spine syndrome.

Myasthenia gravis and diabetes mellitus: a 35-year retrospective study.

Background: The most common treatment of myasthenia gravis is high-dose prednisolone administration and thymectomy. A well-known adverse effect of prednisolone is hyperglycemia, however, to date there is no such detailed report.

Patients And Methods: We treated 325 myasthenia gravis patients in a recent 35 years period, and found 11 patients with diabetes mellitus.

P-glycoprotein function in peripheral blood mononuclear cells of myasthenia gravis patients treated with tacrolimus.

Tacrolimus hydrate (FK506) reduces the symptoms of myasthenia gravis (MG) due to its immunosuppressive properties. A drug efflux pump P-glycoprotein (P-gp) actively transports FK506 out of target cells, thereby reducing their efficacy. We investigated the influence of FK506 therapy on the P-gp function of peripheral-blood mononuclear cells (PBMCs) in MG patients.

[A case of primary HIV infection presenting as mononeuritis multiplex].

A 34-year-old man presented with gait disturbance. He had a low grade fever and diarrhea for a few days prior to admission. Two weeks after he started to have diarrhea, he developed gait disturbance with a left foot drop.

Subacute combined degeneration of the spinal cord concomitant with gastric cancer.

We report a rare case of subacute combined degeneration of the spinal cord concomitant with gastric cancer. A 67-year-old man was admitted because of posterior column symptoms, pyramidal tract sign and peripheral neuropathy with severe hyperchromic anemia. He was treated with mecobalamin 1 mg IM, after which his anemia and neurological signs recovered.

Autoimmune thrombocytopenic purpura, autoimmune hemolytic anemia and gastric cancer appeared in a patient with myasthenia gravis.

We report a case of myasthenia gravis (MG) associated with autoimmune thrombocytopenic purpura (AITP) and autoimmune hemolytic anemia (AIHA), and after that gastric cancer appeared. A 51-year-old man began to suffer from fluctuated muscle weakness in 1985. Muscle weaknesses became exacerbated, and he was admitted to our hospital in 1989.

Extrathymic tumors in patients with myasthenia gravis: a 35-year retrospective study.

Objective: Autoimmune diseases are frequently associated with malignant tumor. In addition, prolonged immunosuppression may favor the development of malignancy. While the coincidence of myasthenia gravis and extrathymic tumor has been reported, the risk and features of these tumors are not well understood.

[A case of Brown-Vialetto-van Laere (BVVL) syndrome in Japan].

Here we report a sixty-year-old woman of Brown-Vialetto-van Laere (BVVL) syndrome in Japan. She had sensorineural deafness, weakness and atrophy of her extremities from 15 years of age. Her neurological symptoms slowly progressed.

Autosomal dominant childhood onset slowly progressive leukodystrophy--a Japanese family with spastic paraparesis, ataxia, mental deterioration, and skeletal abnormality.

Autosomal dominant leukodystrophy is an extremely rare disease. Here we report on a dominantly inherited disease in a Japanese family with slowly progressive clinical course. Their symptoms and signs started in early childhood and very slowly progressed.

Ulcerative colitis presenting as sensorineural deafness, brainstem encephalopathy, and white matter lesions.

Background: Several rare neurologic complications of ulcerative colitis have been reported.

Review Summary: We report a 69-year-old Japanese woman who developed bilateral sensorineural deafness, 2 attacks of bilateral ophthalmoplegia, and bilateral facial nerve palsy in association with ulcerative colitis. Laboratory data showed elevated cerebrospinal fluid (CSF) protein without pleocytosis, abnormal auditory brainstem evoked potentials, and multiple high signal lesions on magnetic resonance imaging of the brainstem and cerebral deep white matter.

Bone density in myasthenia gravis patients receiving long-term prednisolone therapy.

Osteoporosis is an adverse effect of prednisolone therapy, although no study has been conducted on myasthenia gravis patients receiving high-dose prednisolone. We measured bone density in 36 patients (26 females and 10 males) who had undergone long-term prednisolone administration, and found a decrease in bone density in 31% of female patients and osteoporosis in only 11.5% (three cases).

Tacrolimus hydrate (FK506): therapeutic effects and selection of responders in the treatment of myasthenia gravis.

Tacrolimus hydrate (FK506) reduces myasthenic symptoms due to its immunosuppressive properties. We studied the therapeutic effects of FK506 and noted improvement in 7 of 13 myasthenic patients on the clinical muscle test (myasthenia gravis, MG score). Two other patients with relapsing ocular symptoms improved.

[Dimethyl sulphoxide suppresses hydroxyl radical formation of muscle fiber necrosis induced by bupivacaine hydrochloride].

We induced acute skeletal muscle necrosis by using bupivacaine hydrochloride in Wistar rats and found that both 2,5- and 2, 3-dihydroxybenzoic acid significantly increased in the skeletal muscle. Dimethyl sulphoxide, a free radical scavenger, was administrated for 5 days, and resulted in significant lowering of the concentrations of 2, 5- and 2, 3-dihydroxybenzoic acid for four days. Histologically, the diameter of the regenerated muscle fiber seemed to grow by using dimethyl sulphoxide.

[A case of Fahr's disease presenting "diffuse neurofibrillary tangles with calcification"].

77-year-old woman presented memory disturbance, hallucination, and personality change since the summer of 1988. Laboratory findings revealed normal serum Ca, P, HS-PTH levels and Ellsworth-Howard test was intact. Neuroradiological studies disclosed calcification in the dentate nucleus, putamen, globus pallidus and thalamus.

Relapse of ocular symptoms after remission of myasthenia gravis--a comparison of relapsed and complete remission cases.

Extended thymectomy and high-dose alternate-day prednisolone administration may increase the chance of remission in myasthenia gravis (MG) patients. In cases of remission, ocular symptoms sometimes reappear after a gradual decrease or discontinuation of prednisolone administration. We compared relapsed patients with those who experienced complete remission.

Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study.

Background: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear.

Patients And Method: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer.