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Protein gene product (PGP) 9.5 is a new brain-specific protein originally detected by high-resolution two-dimensional electrophoresis of the soluble proteins of human brain and other organs. We have purified this protein from human brain and raised a rabbit antihuman PGP 9.5 antiserum. The protein has a monomer molecular weight of approximately 27,000 and is present in brain at concentrations at least 50 times greater than in other organs. Immunoperoxidase labelling has localised PGP 9.5 to neurones in the human cerebral cortex with no evidence of staining of glial elements. PGP 9.5 is estimated to be present in brain at concentrations of 200-500 micrograms/g wet weight and represents a major protein component of neuronal cytoplasm. This new neurone-specific cytoplasmic marker may prove useful in studies of neuronal development and in the detection of neuronal damage in disease of the nervous system.

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Objective. Myelitis causes pain, weakness, and sphincteric deficits, and is 1,000-fold more prevalent in patients with systemic lupus erythematosus (SLE) than in the general population. For the last century, descriptions of SLE myelitis have been primarily limited to case reports. In contrast, larger-scale cohort studies have revealed that myelitis occurring in the idiopathic demyelinating diseases (i.e., multiple sclerosis versus neuromyelitis optica) represents distinct syndromes. This study was undertaken to determine whether SLE myelitis similarly encapsulates distinct syndromes.Methods. We analyzed a cohort of 22 patients with SLE and myelitis. Patients were assessed for neurologic variables related to myelitis and for clinical and serologic features of SLE. Magnetic resonance images of the spine, cerebrospinal fluid profiles, and autoantibody profiles were obtained.Results. Eleven patients presented with signs of gray matter dysfunction (i.e., flaccidity and hyporeflexia), whereas 11 patients presented with signs of white matter dysfunction (i.e., spasticity and hyperreflexia). Patients with gray matter dysfunction were more likely to have irreversible paraplegia (P < 0.01), despite presenting with a monophasic versus polyphasic course (P ‫؍‬ 0.01), higher levels of SLE activity (mean SLE Disease Activity Index 9.8 versus 2.0; P ‫؍‬ 0.01), and a cerebrospinal fluid profile indistinguishable from bacterial meningitis. Prior to irreversible paraplegia, these patients presented with prodromes of fever and urinary retention, but were misdiagnosed by physicians of different specialties as having urinary tract infections. Patients with white matter dysfunction were more likely to meet criteria for neuromyelitis optica (P ‫؍‬ 0.04) and were also more likely to have antiphospholipid antibodies (lupus anticoagulant) (P ‫؍‬ 0.01). Conclusion. Our findings indicate that SLE myelitis encapsulates 2 distinct and previously unrecognized syndromes that can be distinguished clinically by gray matter versus white matter findings. Recognition of fever and urinary retention as prodromes of irreversible paraplegia may allow earlier diagnosis and treatment in SLE patients presenting with gray matter findings.Myelitis is a devastating inflammatory syndrome of the spinal cord, causing weakness, numbness, and sphincteric deficits. Myelitis is estimated to affect 1-2% of patients with systemic lupus erythematosus (SLE) (1), more than 1,000 times greater than the prevalence of idiopathic myelitis in the general population (2). Despite this disproportionate morbidity affecting SLE patients, the last century has witnessed mostly isolated case reports of myelitis in SLE patients.SLE myelitis is traditionally regarded as a single diagnostic entity (3-5). However, subsuming all cases of myelitis in SLE patients under a unifying diagnostic rubric may be incorrect. Forms of SLE myelitis may have distinct clinical patterns, relationships to SLE activity, cerebrospinal fluid (CSF) profiles, and mechanisms of action. Such heterogeneity is di...

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