Search

We here report a case of Miller Fisher syndrome (MFS) in which serum anti-cerebellar antibody was detected by Western blot analysis. The 32-year-old male studied suffered from diplopia, gait ataxia and sensory disturbance in the distal portion of the upper limbs preceded by cold-like symptoms. Neurological examination on admission revealed that he had external ophthalmoplegia with bilateral ptosis, cerebellar ataxia and areflexia. A cerebrospinal fluid examination showed albuminocytologic dissociation with a protein concentration of 60 mg/dl. Brain CT and MRI showed no significant abnormalities. The patient was diagnosed as MFS, and treated it with two sessions of immunoadsorption plasmapheresis (IAPP). After receiving IAPP therapy, the patient's neurological symptoms and signs were improved. Western blot analysis showed the existence of antibody directed against mouse cerebellum but not against mouse cerebrum, brain stem, and spinal cord in his serum, the level of which was decreased after the IAPP therapy. Serum anti-GQ1b antibody was also elevated. As far as we are aware, there have been no reports showing the existence of anti-cerebellar antibodies detected by Western blot analysis. Though the pathogenesis of MFS remains unclear, our findings suggest that anti-cerebellar antibody detected by Western blot analysis may be caused by cerebellar ataxia in MFS.

Mononuclear cells (MNCs) infiltrating in spinal cords (SCs) of Lewis rats with EAE were isolated in order to permit flow cytometric (FCM) analysis of these cell populations, using monoclonal antibody to T cell, CD4, CD8. MHC class II antigen (Ia), intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function associated antigen-1 (LFA-1). The number of MNCs isolated from SC varied from 5 to 620 x 10(4). They were increased and reached a peak on day 2 post clinical onset, and subsequently declined through the clinical course. The increase of infiltrating cells in SC paralleled the severity of the disease development. The surface phenotypes of MNCs from rats on the day of clinical onset were determined by flow cytometry. The phenotypes found in these rats were as follows: T cells (70.3%); CD4 + (58.3%); CD8 + (40.3%); Ia (51.9%); ICAM-1 (62.6%); LFA-1 (75.8%). These findings suggest that high proportion of ICMA-1 and LFA-1 expression may be important in antigen presentation in the inflammatory lesions of SC and in promoting lymphocyte extravasation across the blood-brain barrier during the disease. The approach we employed offered more sophisticated and quantitative analysis of CNS inflammatory cells, which is unobtainable by tissue section staining.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is thought to be an inflammatory autoimmune disease against peripheral myelin. Recently several animal models have suggested that preferential activation of Th1 cell response is central to the pathogenesis of this disease. We studied the function of CD4 positive T cell subsets, Th1 cells and Th2 cells by analyzing their representative secreting cytokines. Th1 cells secrete Interferon gamma (IFH gamma) and Th2 cells secrete Interleukin 4 (IL4). Using enzyme linked immunospot (ELISPOT) assay, a very sensitive single cell analysis system, we enumerated IFN gamma and IL4 secreting peripheral blood lymphocytes in patients with CIDP. Also we examined these cytokines in supernatants of cultured peripheral blood lymphocytes by a very sensitive enzyme linked immunosorbent assay (ELISA), Immunodot assay and westernblot assay. In culture supernatants of CIDP patients' peripheral blood lymphocytes, the levels of both IFN gamma and IL4 were higher during the exacerbation stage than the remission stage. In the remission period, though the levels of IFN gamma and IL4 were low in the supernatants, the ELISPOT assay revealed that the number of IL4 secreting cells was elevated compared with that of IFN gamma secreting cells. Our results suggest that the switch from Th1 to Th2 might play an important role in inducing the remission stage in patients with CIDP.

We immunologically examined the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy in a patient with HB hepatitis. A 41-year-old male clerk has been suffered from muscle weakness, tingling and numbness in the distal portion of all limbs. All symptoms were compatible with the typical patterns of chronic inflammatory demyelinating polyradiculoneuropathy. We examined the patient's serum and biopsied sural nerve, using histochemical and immunological techniques. We detected the band that reacted with anti-HBs antibody in the sural nerve in western blotting. The result indicated that HBs antigen was expressed on the peripheral nerves in the patient. There were no anti-peripheral nerve antibodies neither in the sural nerve or serum. There was no increase of immune complex in the serum. No deposition of immunoglobulins and complements were detected in the sural nerve. Immunoadsorption therapy had no effect on this patient, but administration of prednisolone improved his symptoms drastically. These findings suggest that a cytotoxic T cell may had played a more important role than humoral factors in this patient's nerve injury. Though the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy remains unclear, our findings seem to be very interesting in that they go some way toward clarifying the pathogenesis of this disease.