Objective: To determine the prevalence of ocular motor disorders among X-Linked Dystonia Parkinsonism (XDP) patients seen in Jose R Reyes Memorial Medical Center, and at a collaborative movement disorders clinic in Roxas City, Philippines.

Background: For most people with X-Linked dystonia parkinsonism, the most obvious concern is the involuntary movement: dystonia, poor mobility and impaired balance. The study of eye movement abnormalities as well as the possible neurophysiologic correlates using the blink reflex studies will pave the way to interaction of basic science and clinical studies to this rather, under-recognized, solely Filipino movement disorder. No report on ocular abnormalities in XDP patients has been made as of the time of this writing. Knowing how prevalent the eye movement abnormalities are in this cohort of patients will increase our understanding of the pathophysiology of the disease and perhaps will eventually lead to the discovery of future diagnostic/prognostic markers in this extremely disabling neurodegenerative condition.

Methods: Twenty-three XDP patients from our IRB-approved registries were evaluated of eye movement abnormalities.  Demographic data such as age, sex, place of origin, onset and duration of illness were obtained.  Staging of XDP was also assessed.   

Results: The mean age of patients was 45 years.  All were male, about 90% came from the Island of Panay, mostly married with children, and with a duration of illness ranging from 8 months to 20 years.  All patients were noted to have ocular motor abnormalities with 95% (n=22) having impaired convergence movements, regardless of the stage of XDP.  This was followed by reduced range of saccadic movement particularly on horizontal plane in 35% (n=8) of patients. Additionally, blink reflex studies were done on four patients which all revealed normal results.

Conclusions: In our XDP patients, like the other progressive, neurodegenerative disorders such as Parkinon’s disease, Progressive Supranuclear Palsy and Huntington’s disease, we have observed similar ocular movement abnormalities, particularly abnormal convergence, slow smooth pursuit, and hypometric saccades.  However, due to small data, we could not establish correlation between the stage of illness and type of ocular movement abnormality.  

References:

1. Lee, L. V., Kupke, Rivera, C., Teleg, R. A., Dantes, M.B., Pasco, M.D., Jamora, R.G., Arancillo, J., Jordan, R.V., Rosales, R. L., Demaisip, C., Maranon, E., Peralta, O., Borres, R., Tolentino, C., Monding, J.M. and Sarcia, S. (2011). The Unique Phenomenology of Sex-Linked Dystonia Parkinsonism (XDP, DYT3, “Lubag”).International Journal of Neuroscience, 121, 3–11.
2. Esmaili D, Shubin R and Waters C. (2004) Eye Movement Abnormalities in a Case of X-Linked Dystonia Parkinsonism (Lubag). Journal of Neuro-Ophthalmology Vol 24, 188-189

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MDS Abstracts - https://www.mdsabstracts.org/abstract/ocular-motor-disorders-among-filipino-lubag-or-x-linked-dystonia-parkinsonism-xdp-patients/