[Orbital apex syndrome without MRI lesion caused by ANCA-associated vasculitis].

A 73-year-old man developed double vision and a progressive loss of visual acuity of the left eye over one week. Examination showed disturbances of the left II, III, IV, and VI cranial nerves, that is, an orbital apex syndrome. A brain MRI showed abnormal T2-high signals in the right maxillary sinus and the left mastoid cells without abnormalities in the left orbital apex and the surroundings. Laboratory examination showed an elevated erythrocyte sedimentation rate and a positive perinuclear anti-neutrophil cytoplasmic antibody (MPO-ANCA). After two courses of methyl-prednisolone pulse treatment, his external ophthalmoplegia fully recovered and he regained his left eye's sight. MPO-ANCA was negative and MRI abnormalities were disappeared after treatment. Two years later, the patient developed upper respiratory symptoms associated with an elevation of MPO-ANCA titer, and rapidly progressive renal failure. Renal biopsy specimen showed fibrinoid necrosis with periarteriolar neutrophil infiltration, which suggested that the patient suffered from ANCA-associated vasculitis probably of Wegener's granulomatosis or microscopic polyarteritis. ANCA-associated vasculitis may present with a focal neurological syndrome such as the orbital apex syndrome without a lesion detectable with MRI.

[Intramedullary spinal cord metastasis presenting with acute onset longitudinally extensive spinal cord lesion].

A 34-year-old previously healthy man presented with acute transverse lumbar myelopathy and MRI evidence of a longitudinally extensive spinal cord lesion (LESCL) from the upper thoracic cord extending down to the conus medullaris. Gadolinium-DTPA enhancement revealed a clearly demarcated enhanced nodule confined to the level of the 11th thoracic vertebral body (T11), which might have caused longitudinally extensive edema in the spinal cord. Histopathological appearance of adenocarcinoma of the nodule led to the ultimate diagnosis of lung cancer. Intramedullary spinal cord metastasis in a young patient without previously-diagnosed malignancy is a rare disorder, but should be listed up as a cause of LESCL.

Ocular myasthenia gravis associated with euthyroid ophthalmopathy.

We report a 71-year-old woman with concomitant ocular myasthenia gravis and euthyroid Graves' ophthalmopathy. Unilateral ophthalmoplegia, including ptosis, initially was responsive to edrophonium and corticosteroids, except for diplopia on upward gaze, but refractory swelling of the inferior rectus muscle and proptosis followed. Autoantibodies to acetylcholine and thyrotropin receptors were detected. Her ophthalmopathy abated after orbital irradiation in combination with systemic steroids. There may be an immunological basis for the association of ocular myasthenia gravis with euthyroid Graves' ophthalmopathy.

[Prospects of the therapeutic approaches to Creutzfeldt-Jakob disease: a clinical trial of antimalarial, quinacrine].

Prion diseases are lethal transmissible neurodegenerative illnesses that affect humans and many other animals. Since the accumulation of the pathogenic form of prion protein is a pivotal event in prion diseases, most of the therapeutic strategies are designed to prevent the conformational change of normal prion protein to that of the pathogenic form or to remove the accumulated prion protein. Quinacrine is one of the compounds that can inhibit the accumulation of pathogenic prion protein in cultured neuroblastoma cells. Here we report the results of clinical trials of quinacrine administration to the patients of Creutzfeldt-Jakob disease. In some patients, response to visual and auditory stimulations improved transiently. Lemon-yellow discolorization of skin and liver dysfunction were common side effects by quinacrine.