Intraventricular tumor presenting as progressive supranuclear palsy–like phenotype
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A 70-year-old woman presented with a 2-year history of progressive difficulty in walking with frequent falls. Neurologic examination showed postural instability with backward falls, vertical supranuclear gaze palsy with normal vestibular-ocular reflex, rigidity, and pyramidal signs in the right limbs. There was no clinical response to levodopa. Laboratory serologic tests had normal results. MRI displayed midbrain compression and dislocation caused by a large tumor in the left lateral ventricle (figure). Dopamine transporter SPECT showed normal striatal binding. The patient died before neurosurgery could be performed; there was no autopsy. Brain tumors should be considered in the diagnostic workup1 of progressive supranuclear palsy–like phenotypes.
Sagittal T1-weighted images (A, B): midbrain compression caused by a globular tumor. Axial fluid-attenuated inversion recovery (FLAIR) image (C): left lateral ventricle enlargement; vascular pedicle connecting the tumor to the choroidal plexus (arrow). Axial-GE-T2* image (D): tumor hypointensity by mineral accumulation. Coronal FLAIR image (E): left cerebral peduncle dislocation and transependymal CSF diffusion.
Footnotes
Author contributions: Dr. Morelli: drafting the manuscript, acquisition of data, analysis and interpretation of data, accepts responsibility for conduct of research and final approval. Dr. Fera: acquisition of data, analysis and interpretation of data, accepts responsibility for conduct of research and final approval. Francesco Bono: acquisition of data, accepts responsibility for conduct of research and final approval. Dr. Fratto: acquisition of data, accepts responsibility for conduct of research and final approval. Dr. Arabia: acquisition of data, accepts responsibility for conduct of research and final approval. Dr. Quattrone: corresponding author; study concept and design, drafting/revising the manuscript, study supervision, accepts responsibility for conduct of research and final approval.
Study funding: No targeted funding reported.
Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
- © 2014 American Academy of Neurology
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Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)Report of the NINDS-SPSP International Workshop*I. Litvan, Y. Agid, D. Calne et al.Neurology, July 01, 1996