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Diplopia may be the first or only sign of orbital disease including thyroid eye disease, idiopathic orbital inflammation, trauma, infection, neoplasm and congenital myopathies. Presentations may be difficult to differentiate from neurological causes including cranial neuropathies, but orbital pain, proptosis, peri-orbital edema and resistance to retropulsion are all suggestive of an orbital process. In many cases, limitation of movement reflects restriction of an antagonist muscle rather than weakness of the agonist, a phenomenon that may be confirmed with attempted forced ductions of the eye. Lack of pupil or lid involvement helps differentiate adduction defects localizing to the medial rectus from partial third nerve palsies. Traditional brain CT or MRI may miss orbital disease, leading to significant delays in diagnosis and treatment. Therefore, a careful history and exam aimed at uncovering myopathic causes of diplopia is essential and dedicated orbital imaging (with fat saturation) when disease affecting the extraocular muscles is suspected. Orbital inflammation may be idiopathic or related to a systemic disease such sarcoidosis, granulomatosis polyangiitis, temporal arteritis and IgG4 disease. Systemic corticosteroids are typically effective. Thyroid eye disease (TED) tends to present with less pain and spares the muscle tendons. Orbital neoplasm may cause hypoglobus or enophthalmos and tend to be diffusion positive on MRI. CT of the orbit is preferred in cases of trauma because of its ability to show bony fractures and defects. In this course, through several cases, we will review the exam skills needed to differentiate orbital disease from neurological.

Abstract Body

Progressive Supranuclear Palsy (PSP) is a rapidly progressive neurodegenerative tauopathy. It is a rare neurodegenerative condition characterised by a range of motor and cognitive symptoms. Unfortunately, despite several promising pharmacological research approaches, including reducing levels of the toxic tau protein or alleviating the loss of tau function, there is currently no approved disease-modifying treatment option for this condition. To aid in the development of such treatments, there is a real need for the development of objective clinical tools for the support of diagnosis and for monitoring disease progression.

In this talk, I will describe how to spot supranuclear gaze disorders and will go through some patient videos from our work here in Oxford.

Very little is known about the longitudinal change in these symptoms over time. Moreover, the effectiveness of clinical scales to detect early changes in PSP is still a matter of debate. I will go through some of the data indicating the longitudinal changes in motor and cognitive features from our work through the Oxford Quantification in Parkinsonism study (OxQUIP) using multiple closely spaced follow-up timepoints over a period of 2 years. In addition, I will show some of the digital technology we have been using and the updated news on the use of telemedicine and home monitoring especially in the era of Covid 19.

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Nystagmus is defined as to-and-fro oscillations of the eyes initiated by a slow drift of the eyes away from desired position and followed by either quick phases (jerk nystagmus) or slow drifts in the opposite direction (pendular nystagmus). There are a multitude of nystagmus types, etiologies, and mechanisms – each with different clinical implications and pharmacological treatments; thus, proper pattern identification of nystagmus is critical to accurate neurological diagnosis and effective therapeutic intervention. The most common acquired nystagmus types seen clinically that occur in central gaze and thereby may cause disabling oscillopsia (i.e., a subjective sense of visual motion) are acquired pendular nystagmus (APN) and pure vertical jerk nystagmus (upbeat = UBN, downbeat = DBN). The two most common forms of APN are horizontal/elliptical APN in multiple sclerosis and vertical/torsional APN with simultaneous spontaneous palatal movements in oculopalatal tremor. Oculopalatal tremor typically begins weeks to months after a brainstem stroke and is associated with hypertrophic degeneration of the inferior olive. UBN is most common with medullary lesions from multiple sclerosis or in the setting of Wernicke encephalopathy. DBN occurs with many pathologies affecting the cerebellum, especially the cerebellar flocculus/paraflocculus or nodulus/uvula.

Recent updates to be highlighted include a slowly progressive form of oculopalatal tremor called progressive ataxia and palatal tremor (PAPT) and diagnostic insights to be gained from careful examination of the behavior of UBN and DBN in different gaze positions and following provocative maneuvers (e.g., head shaking, supine position).