Vertical gaze palsy and saccadic intrusions due to anti-Ri from head and neck carcinoma

A 55-yo- woman was admitted for imbalance and double vision. Three weeks prior to presentation she first noticed swelling on the right side of her face and neck. CT of the head and neck showed right-sided cervical adenopathy and enlarged left retropharyngeal node. Ultrasound- guided biopsy of the neck mass showed a poorly differentiated, non-small cell carcinoma with focal squamous features. ; MRI brain was initially normal, but T2 changes within the midbrain were seen as her condition worsened. Work-up demonstrated lymphocytic pleocytosis on lumbar puncture and serum paraneoplastic panel was positive for anti-Ri antibody (ANNA-2). Over weeks, she developed complete ophthalmoplegia, dysphagia and respiratory failure. ; On exam, she could not make upward or downward saccades or smooth pursuit movements. However, there was mild preservation of the vertical VOR (i.e., vertical defects were overcome partially), suggestive of at least some degree of "supranuclear" involvement. There was also a left hypertropia, and because she was unable to generate vertical refixation saccades with cover-uncover testing, her misalignment could not be accurately measured. This could have been related to partial 3rd(s) nerve palsy on the basis of nuclear or fascicular damage (although no clear ptosis, mydriasis, adduction paresis, and vertical VOR wouldn't be expected to overcome vertical motility deficits related to a 3rd); however, involvement of the otolith-ocular motor pathways in or around the interstitial nucleus of Cajal (INC - either left sided or left>right sided) causing a skew deviation seemed the most likely explanation. In her case, vertical gaze palsy was probably due to a combination of deficits involving the fascicles of the 3rd nerve, INC, and riMLF. ; The saccadic intrusions seen in this video are most consistent with square wave jerks, although occasionally there appeared to be back-to-back horizontal saccades with no intersaccadic interval, suggestive of ocular flutter. Eye movement recordings could not be completed. Of note, anti-Ri has been associated with ocular flutter and opsoclonus

Acute flaccid myelitis: cause, diagnosis, and management

Acute flaccid myelitis (AFM) is a disabling, polio-like illness mainly affecting children. Outbreaks of AFM have occurred across multiple global regions since 2012, and the disease appears to be caused by non-polio enterovirus infection, posing a major public health challenge. The clinical presentation of flaccid and often profound muscle weakness (which can invoke respiratory failure and other critical complications) can mimic several other acute neurological illnesses. There is no single sensitive and specific test for AFM, and the diagnosis relies on identification of several important clinical, neuroimaging, and cerebrospinal fluid characteristics. Following the acute phase of AFM, patients typically have substantial residual disability and unique long-term rehabilitation needs. In this Review we describe the epidemiology, clinical features, course, and outcomes of AFM to help to guide diagnosis, management, and rehabilitation. Future research directions include further studies evaluating host and pathogen factors, including investigations into genetic, viral, and immunological features of affected patients, host-virus interactions, and investigations of targeted therapeutic approaches to improve the long-term outcomes in this population