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Phonic Tic as a New Presentation of Progressive Supranuclear Palsy

Paria Arfa Fatollahkhani, Seyed Amir Hasan Habibi, Ghazal Haeri


Progressive supranuclear palsy (PSP) is the most common neurodegenerative form of atypical Parkinsonism characterized by sudden falls, vertical supranuclear gaze palsy, axial rigidity and a frontotemporal behavioral syndrome. Despite decades of research, a clear understanding of the pathophysiology of PSP disease is still unknown, nonetheless, protein tau-related neurodegeneration is suggested to have the main role in pathogenesis. Interestingly, we herein report a 72-year old woman with a 3-year history of phonic tic as an unusual initial manifestation of PSP. These unpleasant sounds were disappeared during sleep. Notably, whenever she tried to resist and stop the sounds, she got a strong feeling of agitation and discomfort, which is phenomenologically compatible with tic manifestations. Noticeably, she developed multiple forward falling since last year. In her neurological examinations, we detected a masked face with up and downward gaze limitations and an axial rigidity with a frontalis muscle dystonia. The pulling test examination also revealed a marked postural instability. Besides these clinical presentations, a hummingbird sign (HBS) was observed in her brain magnetic resonance image (MRI). Based on the examinations and imaging results, she has prescribed Levodopa (90 mg daily for the first week, then incremented to 180 mg daily) and Sertraline (150 mg daily). In conclusion, this report underlines that tics not only should be considered as a symptom of Progressive supranuclear palsy but also may be the phenotypic variability of PSP at presentation. Moreover, this case provides insight into the importance of exact evaluation for adult-onset hyperkinetic involuntary movements such as tics.

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