No abstract

No abstract

Rheumatoid vasculitis (RV) usually occurs in patients with refractory rheumatoid arthritis (RA). An 80year-old woman was transferred to our hospital because of muscle weakness and paresthesia in all 4 limbs. She had been diagnosed with RA 30 years ago and achieved sustained clinical remission. At presentation, polyarthritis and drop foot were observed, and rheumatoid factor was prominently elevated. A peripheral nerve conduction test revealed mononeuritis multiplex in her limbs. We suspected that RV had developed rapidly despite RA having been stable for many years and started immunosuppression therapy with steroids combined with azathioprine. The treatment prevented worsening of muscle weakness and paresthesia.

Vogt-Koyanagi-Harada syndrome (VKH) is a disease with a bilateral uveitis, dermatologic findings, and neurologic deficits. Highdose corticosteroid therapy frequently improves visual involvement in VKH.' Recently, Helveston and G:ilmore2 reported treatment of VKH with intravenous immunoglobulin, although prednisone (80 mg/d) treatment had not ameliorated neurologic signs in their patient. We report a patient who had successful treatment with high-dose oral betamethasone after having failed with standard oral prednisolone. We discuss initial treatment of neurologic signs in VKH with high-dose corticosteroids.Case report. A 37-year-old Japanese man noticed dysesthesia of the feet late December 1995. Gait disturbance, dysuria, and impotence developed early January 1996. He was not able to walk and developed bilateral blurred vision and ocular pain mid-February 1996. Ophthalmologic examination showed anterior uveitis and retinal detachments. Physical examination was normal. Neurologic examination indicated spastic paraplegia and loss of all sensation to the level of T9. He did not have deafness, meningeal signs, or cerebellar ataxia. Brain and spinal cord MRI were normal. CSF study revealed only mild lymphocytosis (16/mm3). We diagnosed uveitis and transverse myelitis associated with VKH. Oral prednisolone therapy (100 mg/d) for 1 week did not improve his neurologic signs and caused liver dysfunction. Lymphocytestimulating test using prednisolone was abnormal, suggesting prednisolone-induced liver dysfunction. We changed to oral betamethasone treatment (20 mg/d, corresponding to 133.3 mg of prednisone). From 1 week after betamethasone administration, many of the ocular signs, the paraplegia, and dysesthesia in the legs had markedly improved. Betamethasone was slowly tapered.

Amyotrophic lateral sclerosis (ALS) due to a fused in sarcoma (FUS) P525L mutation is characterized by a rapidly progressive course. Multifocal motor neuropathy (MMN) may resemble ALS in early stage and is associated with anti-ganglioside antibodies. A 38-year-old woman was admitted to our hospital because of progressive muscle weakness in the right limbs. She had mild mental retardation and minor deformities. Initially, we suspected MMN given the asymmetric muscle weakness and detection of anti-ganglioside antibodies. However, physical and electrophysiological tests did not support MMN, instead suggesting ALS. We confirmed a heterozygous P525L mutation and finally diagnosed this case as ALS due to an FUS mutation.