References 1. Freedman MS, Ruijs TCG, Selin LK,Antel JP. Peripheral blood y-8 T cells lyse fresh human brain-derived oligodendrocytes. Ann Neurol 1991;30:794-800 2. Salvetti M, Buttinelli C, Fiorilli M, et al. Proliferative response to heat shock proteins and gammddelta T cell receptor expression of PPD specific T lymphocytes in multiple sclerosis. J Neuroimmunol 1991:(Suppl 1):29 3. Spits H. Human T cell receptor $5' T cells. Semin Immunology 1991;3:119- 129
PeriDheral NeuroDathv in Ch&-Strauss Syidrome Associated with I&C3 Deposits
Immunofuorescence of artery in deltoid muscle biopsy showing segmental disruption of internal elastic lamina with granular deposits of IgA, and perivascular injltrate of I g A /ymphogtes and plasma cells.
Cormac A. ODonovan, MD," Mary Keogan, MB,t Hugh Staunton, FRCP,? Orla Browne, FFPath,? and Michael A. Farrell. FRCPCt We note with interest the study of Kissel and associates 111, who analyzed the immune characteristics of nerve biopsy specimens of patients with peripheral neuropathy associated with necrotizing vasculitis, including patients with Wegener's granulomatosis, polyarteritis nodosa, rheumatoid arthritis, systemic lupus erythematosus, and isolated peripheral neuropathy. We wish to report our findings in a patient with Churg-Strauss syndrome, another condition in which vasculitis is associated with peripheral neuropathy. A 49-year-old steroid-dependent, asthmatic woman was hospitalized because of progressive bilateral visual loss and painful flaccid weakness of all four limbs. On examination, the patient was cushingoid and had a low-grade fever, bilateral retinal artery occlusions, and distal symmetrical sensorimotor neuropathy. Investigations showed a peripheral and CSF eosinophilic leukocytosis, with an erythrocyte sedimentation rate of 32 d h r . Routine biochemical studies and urinalysis were normal. Creatinine phosphokinase was elevated at 132 1 IU/L. Serological tests for rheumatoid factor, hepatitis B antigen, and autoantibody screen were negative. Protein electrophoresis and C3 complement levels were normal. Elevated levels of circulating IgA immune complexes were found. Deltoid muscle biopsy showed an eosinophilic vasculitis of medium-sized arteries with mild denervation atrophy. Immunofluorescence showed C3 complement and IgA deposits present in the vessel wall (Fig). Surd nerve biopsy showed an eosinophilic infiltrate in the epineurium, but no evidence of axonal degeneration or demyelination. Immunofluorescent findings in nerve and muscle biopsy specimens of patients with Churg-Strauss have not been characterized adequately to our knowledge. It has been suggested that immune complexes may play a role in the pathogenesis of this condition, and that IgM is a prominent component of this complex [ 2 ] . The association of raised IgE levels and asthma in this condition has been used to suggest a probable allergic basis for the disorder. However, the presence of other immunological abnormalities, such as IgA and C3 com-
plement seen histologically in our patient, may point to other possible mechanisms of local tissue injury. 'Department of NeuroLogy Cleveland Clinic Foundation Cleveland, OH tThe Richmond Institute of Neurology and Neurosurgery Beaumont Hospital Dublin, Ireland
References 1. Kissel JT, Riethman JL, Omena J, et al. Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol 1989;25:291-297 2. Lanham JG, Elkon KE3, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg Strauss syndrome. Medicine 1984;63:65-81
Modification of Muscle Activity after BOTOX Injections in Spasmodic Torticohs C. Marin, MD,' M. J. Marti, MD,' E. Tolosa, MD,' R. Alvarez, MD," L. L. Montserrat, MD,t and J. Santamaria, MD' Gelb and associates [11 recently presented the results of an electromyographic (EMG) study in patients with spasmodic torticollis (ST) treated with botulinum toxin (BOTOX). The goal of their study was to evaluate whether an abnormal mo-
Annals of Neurology Vol 32 No 3 September 1992 411
PeriDheral NeuroDathv in Ch&-Strauss Syidrome Associated with I&C3 Deposits
Immunofuorescence of artery in deltoid muscle biopsy showing segmental disruption of internal elastic lamina with granular deposits of IgA, and perivascular injltrate of I g A /ymphogtes and plasma cells.
Cormac A. ODonovan, MD," Mary Keogan, MB,t Hugh Staunton, FRCP,? Orla Browne, FFPath,? and Michael A. Farrell. FRCPCt We note with interest the study of Kissel and associates 111, who analyzed the immune characteristics of nerve biopsy specimens of patients with peripheral neuropathy associated with necrotizing vasculitis, including patients with Wegener's granulomatosis, polyarteritis nodosa, rheumatoid arthritis, systemic lupus erythematosus, and isolated peripheral neuropathy. We wish to report our findings in a patient with Churg-Strauss syndrome, another condition in which vasculitis is associated with peripheral neuropathy. A 49-year-old steroid-dependent, asthmatic woman was hospitalized because of progressive bilateral visual loss and painful flaccid weakness of all four limbs. On examination, the patient was cushingoid and had a low-grade fever, bilateral retinal artery occlusions, and distal symmetrical sensorimotor neuropathy. Investigations showed a peripheral and CSF eosinophilic leukocytosis, with an erythrocyte sedimentation rate of 32 d h r . Routine biochemical studies and urinalysis were normal. Creatinine phosphokinase was elevated at 132 1 IU/L. Serological tests for rheumatoid factor, hepatitis B antigen, and autoantibody screen were negative. Protein electrophoresis and C3 complement levels were normal. Elevated levels of circulating IgA immune complexes were found. Deltoid muscle biopsy showed an eosinophilic vasculitis of medium-sized arteries with mild denervation atrophy. Immunofluorescence showed C3 complement and IgA deposits present in the vessel wall (Fig). Surd nerve biopsy showed an eosinophilic infiltrate in the epineurium, but no evidence of axonal degeneration or demyelination. Immunofluorescent findings in nerve and muscle biopsy specimens of patients with Churg-Strauss have not been characterized adequately to our knowledge. It has been suggested that immune complexes may play a role in the pathogenesis of this condition, and that IgM is a prominent component of this complex [ 2 ] . The association of raised IgE levels and asthma in this condition has been used to suggest a probable allergic basis for the disorder. However, the presence of other immunological abnormalities, such as IgA and C3 com-
plement seen histologically in our patient, may point to other possible mechanisms of local tissue injury. 'Department of NeuroLogy Cleveland Clinic Foundation Cleveland, OH tThe Richmond Institute of Neurology and Neurosurgery Beaumont Hospital Dublin, Ireland
References 1. Kissel JT, Riethman JL, Omena J, et al. Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol 1989;25:291-297 2. Lanham JG, Elkon KE3, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg Strauss syndrome. Medicine 1984;63:65-81
Modification of Muscle Activity after BOTOX Injections in Spasmodic Torticohs C. Marin, MD,' M. J. Marti, MD,' E. Tolosa, MD,' R. Alvarez, MD," L. L. Montserrat, MD,t and J. Santamaria, MD' Gelb and associates [11 recently presented the results of an electromyographic (EMG) study in patients with spasmodic torticollis (ST) treated with botulinum toxin (BOTOX). The goal of their study was to evaluate whether an abnormal mo-
Annals of Neurology Vol 32 No 3 September 1992 411
