Higher-level gait disorder as a presenting manifestation of progressive supranuclear palsy: a video case report
*Corresponding author: Dr. Julián Benito León. Servicio de Neurología. Hospital Universitario 12 de Octubre. Avda. Córdoba, s/n. E-28041 Madrid.
E-mail: jbenitol67@gmail.com
Introduction: Frontal gait disorder/gait apraxia is a higher-order motor deficit with various causes, characterized by difficulties with gait initiation, such as freezing or ignition failure. We aimed to report a patient who presented with progressive higher-level gait disorder and fall episodes as the initial manifestations of progressive supranuclear palsy (PSP). Patient data were obtained from medical records from the Department of General Medicine, Burdwan Medical College and Hospital (Burdwan, West Bengal, India).
Case report: A 58-year-old previously healthy woman presented with a gait disorder and fall episodes. Detailed neurological examination highlighted characteristic facial appearance (wide-eyed staring, furrowing of the forehead with a frowning expression, and fixed expression of the lower face). She was hypokinetic-rigid with symmetrical signs and predominant axial rigidity with retrocolic trunk and neck posture. Gait examination revealed a higher-level gait pattern characterized by an exhibition of profound start hesitation requiring assistance from nearby objects/persons. Once walking was underway, steps became relatively better, but ineffective gait re-emerged when she attempted turning. She had short strides, freezing, broad stance base, disequilibrium, slow leg movement, shuffling, and loss of normal fluidity of trunk and limbs. Postural reflexes were impaired. Brain magnetic resonance imaging revealed atrophy of the midbrain, dilated aqueduct of Sylvius and third ventricle, atrophy of frontal lobes and typical hummingbird sign. Diagnosis of probable PSP was finally made.
Conclusions: Several etiologies, including PSP, should be considered in appropriate clinical contexts if gait examination demonstrates a higher-order gait disorder.
Caso clínico Mujer de 58 años sana que consultó por un trastorno de la marcha con caídas. La exploración neurológica mostró una apariencia facial característica (mirada fija, ojos muy abiertos, ceño fruncido y expresión fija hemifacial inferior), e hipocinesia-rigidez simétrica de predominio axial (postura retrocólica del tronco y el cuello). La exploración de la marcha reveló un trastorno de la marcha del nivel superior, caracterizado por una significativa vacilación inicial, que precisaba ayuda de objetos/personas cercanos. Al iniciar la marcha, los pasos mejoraban relativamente, pero reaparecía una deambulación inefectiva al girar. Presentaba zancadas cortas, congelación, base amplia de sustentación, desequilibrio, movimiento lento de las piernas, arrastre de los pies, y pérdida de la cadencia normal del tronco y las extremidades. Los reflejos posturales estaban alterados. La resonancia magnética cerebral desveló atrofia mesencefálica, dilatación de acueducto de Silvio y III ventrículo, atrofia frontal bilateral y el signo típico del colibrí. Finalmente, la paciente fue diagnosticada de una PSP probable.
Conclusiones Varias etiologías, incluida la PSP, deben considerarse, en el contexto clínico apropiado, si la exploración de la deambulación demuestra un trastorno de la marcha del nivel superior.