Long lasting pure freezing of gait preceding progressive supranuclear palsy: A clinicopathological study

• Published in: Movement disorders Vol. 22; no. 13; pp. 1954 - 1958
• Main Authors: Compta, Yaroslau, Valldeoriola, Francesc, Tolosa, Eduardo, Rey, María Jesús, Martí, María José, Valls-Solé, Josep
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 15.10.2007; Wiley
• Subjects: Accidental Falls; Aged; Astrocytes - pathology; Biological and medical sciences; Brain - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Diagnosis, Differential; Disease Progression; Dopamine Plasma Membrane Transport Proteins - physiology; Female; gait disorders; Gait Disorders, Neurologic - diagnosis; Gait Disorders, Neurologic - pathology; Hormones. Endocrine system; Humans; Inclusion Bodies - pathology; Iodine Radioisotopes; Magnetic Resonance Imaging; Medical sciences; Neurofibrillary Tangles - pathology; Neurologic Examination; Neurology; Neuropil - pathology; Nortropanes; Oligodendroglia - pathology; Pharmacology. Drug treatments; progressive supranuclear palsy; pure freezing of gait; Supranuclear Palsy, Progressive - diagnosis; Supranuclear Palsy, Progressive - pathology; Tauopathies - diagnosis; Tauopathies - pathology; Tomography, Emission-Computed, Single-Photon; Gait disorder; Nervous system diseases; pure freezing of gait; gait disorders; Long lasting; Freezing; progressive supranuclear palsy
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/mds.21612

Primary progressive freezing of gait (PPFG) is the term used to designate an uncommon condition featuring freezing of gait with frequent falls, without bradykinesia, rigidity or tremor, and unresponsive to levodopa. There are very few pathological reports of patients with PPFG in the literature. We report on 2 patients (one with pathological confirmation) diagnosed initially as PPFG and evolving into clinically defined progressive supranuclear palsy (PSP) more than 10 years after onset of symptoms. These 2 cases suggest that PPFG can represent the initial manifestation of a neurodegenerative disease, such as PSP, rather than a differentiated nosological entity. © 2007 Movement Disorder Society