Session Information
Date: Monday, June 20, 2016
Session Title: Parkinsonism, MSA, PSP (secondary and parkinsonism-plus)
Session Time: 12:30pm-2:00pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: We report a patient with apraxia of speech as the initial symptom who finally developed a progressive supranuclear palsy.
Background: Progressive supranuclear palsy (PSP) is a rare brain disorder which typically presents as an akinetic-rigid syndrome with early falls, axial rigidity, vertical supranuclear gaze palsy and levodopa resistance. It also causes severe alterations in the control of gait and balance. The hallmark of PSP is a visual disturbance, which results from a progressive inability to coordinate eye movements. Nevertheless, some patients might show an atypical presentation.
Methods: A 57-year-old patient complained of language problems. The most notable symptom was finding difficulty in putting sounds and syllables together in the correct order to form words. Longer or more complex words were usually tougher to produce than shorter or simpler words. The patient tended to make inconsistent mistakes when speaking. Another characteristic of the speech was the incorrect use of ‘prosody’ — that is, the varying rhythms, stresses, and inflections of speech that are used to help express meaning. Brain magnetic resonance imaging (MRI) was normal so a primary apraxia of speech (AOS) was diagnosed.
Results: After 4 years the patient developed loss of balance and gait instability, slowness and stiffness of movements and dysphagia, although ophtalmoplegia was not present. Marked atrophy of the midbrain tegmentum and a distinct ‘hummingbird sign’ were observed in the T1-weighted midsagittal MRI sections of the rostral midbrain and pons. Therefore, the final diagnosis was PSP.
Conclusions: We report a patient with symptoms initially consistent with AOS who finally developed the typical phenotype of PSP-Parkinsonism. This case, like another one reported in the literature, demonstrates that atypical PSP can present as AOS without the classic features of PSP.
To cite this abstract in AMA style:
J.J. Bravo, M.J. Gallardo, J.P. Cabello, R.E. Ibañez, J. Vaamonde. Apraxia of speech as the initial manifestation of progressive supranuclear palsy [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/apraxia-of-speech-as-the-initial-manifestation-of-progressive-supranuclear-palsy/. Accessed November 22, 2024.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/apraxia-of-speech-as-the-initial-manifestation-of-progressive-supranuclear-palsy/