Pathologically Verified Corticobasal Degeneration Mimicking Richardson's Syndrome Coexisting with Clinically and Radiologically Shunt-Responsive Normal Pressure Hydrocephalus

Yuji Saitoh; Masaki Iwasaki; Masashi Mizutani; Yukio Kimura; Masato Hasegawa; Noriko Sato; Masaki Takao; Yuji Takahashi

doi:10.1002/mdc3.13442

Pathologically Verified Corticobasal Degeneration Mimicking Richardson's Syndrome Coexisting with Clinically and Radiologically Shunt-Responsive Normal Pressure Hydrocephalus

Mov Disord Clin Pract. 2022 Apr 12;9(4):508-515. doi: 10.1002/mdc3.13442. eCollection 2022 May.

Authors

Yuji Saitoh¹, Masaki Iwasaki², Masashi Mizutani³, Yukio Kimura⁴, Masato Hasegawa⁵, Noriko Sato⁴, Masaki Takao³, Yuji Takahashi¹

Affiliations

¹ Department of Neurology National Center Hospital, National Center of Neurology and Psychiatry Tokyo Japan.
² Department of Neurosurgery National Center Hospital, National Center of Neurology and Psychiatry Tokyo Japan.
³ Department of Laboratory Medicine National Center Hospital, National Center of Neurology and Psychiatry Tokyo Japan.
⁴ Department of Radiology National Center Hospital, National Center of Neurology and Psychiatry Tokyo Japan.
⁵ Dementia Research Project Tokyo Metropolitan Institute of Medical Science Tokyo Japan.

Abstract

Background: Normal pressure hydrocephalus (NPH) manifests as gait instability, cognitive impairment, and urinary incontinence. This clinical triad of NPH sometimes occurs with ventriculomegaly in patients with neurodegenerative disease. Patients with pathologically verified neurodegenerative diseases, such as progressive supranuclear palsy (PSP), have received antemortem diagnoses of NPH.

Objectives: This study presents clinical and pathological features of a patient with pathologically verified corticobasal degeneration (CBD) coexisting with clinically shunt-responsive NPH.

Methods: We performed clinical, radiological, and pathological evaluations in a patient with CBD whose antemortem diagnosis was PSP Richardson's syndrome (PSP-RS) coexisting with shunt-responsive NPH.

Results: A 59-year-old woman developed bradykinesia and gait instability and then frequent falls, urinary incontinence, and supranuclear vertical gaze palsy followed. At 63 years of age, her gait disturbance and urinary incontinence had deteriorated rapidly, and cognitive impairment was disclosed. There were typical findings of NPH with ventriculomegaly and disproportionately enlarged subarachnoid space hydrocephalus as well as a 2-layer appearance with decreased and increased cerebral blood perfusion. Shunt placement ameliorated gait instability for more than 1 year and improved radiological indicators of NPH. However, atrophy of the midbrain progressed with time after transient increases in size. Although the antemortem diagnosis was probable PSP-RS, pathological evaluation verified CBD. There were severe discontinuities of the ependymal lining of the lateral ventricles and subependymal rarefaction and gliosis with tau-positive deposition.

Conclusions: Shunt surgery could ameliorate NPH symptoms in patients with 4-repeat tauopathies. Careful assessments of clinical findings are necessary to predict the benefits of shunts as a therapeutic option for patients with neurodegenerative diseases coexisting with NPH.

Keywords: corticobasal degeneration; neurodegenerative NPH; normal pressure hydrocephalus; progressive supranuclear palsy; shunt surgery.