Abstract
This third issue of Neurology® Neuroimmunology & Neuroinflammation incorporates a number of fascinating and informative clinical reports, including a presentation of prion disease as hyperacusis from Merkler et al.1 as well as a case of NMDA receptor antibody disease associated with bilateral sensorineural hearing loss from Taraschenko et al.2 In the case reported by Merkler et al., central auditory processing is the suspected pathophysiology, while the data from Taraschenko et al. suggest that the cochlear apparatus was directly targeted by pathogenic autoantibodies. Together the 2 cases illustrate how much neurophysiology can be gleaned from thoughtful study of these unusual patients. We also present a transient episode of autoimmune autonomic ganglionopathy (AAG) from Baker et al.3 This case illustrates the clinical and scientific importance of case reports. Individuals with AAG harbor autoantibodies to ganglionic acetylcholine receptors, producing (usually) modest autonomic insufficiency syndromes. This case of transient symptoms in a neonate born to a patient with AAG confirms that the antibodies are directly pathogenic and also alerts clinicians to the potential for this complication of the disorder. Fernandez-Fournier et al.4 report a careful study of cervical myelitis in a young girl, with onset only 3 days after human papillomavirus immunization. The extremely short interval between immunization and neuroinflammation, along with the presence of CSF oligoclonal immunoglobulin M, are consistent with the possibility that a preexisting autoimmune diathesis was present. This challenges the research community to identify markers of the few individuals (insufficient numbers to exceed background in surveys) who may be at risk for expressing inflammatory complications of prophylactic immunization.
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