Objective: .

Background: Normal pressure hydrocephalus (NPH) is characterized by a triad of symptoms: gait abnormality, urinary incontinence (UI) and cognitive decline.  Though clinical findings distinguish it from atypical parkinsonism, we present a case of atypical parkinsonian features in a patient diagnosed and treated for NPH, followed by improvement after shunting.

Method: .

Results: An 80yo male presented with 4 years of gait and balance difficulty, with two years of UI and cognitive change. Family history of NPH in sister. Gait exam showed mild shuffling, en bloc turns and reduced gait stride. He had severe supranuclear palsy, without hypomimia, hypophonia, tremor or rigidity, and no gait response to 400mg LEDD. His DAT scan was negative, and brain MRI showed cerebral ventriculomegaly out of proportion to cerebral atrophy and absence of midbrain atrophy. Fluoro-guided lumbar puncture (LP) was performed, with opening pressure of 17 cmH2O. Pre-LP physical therapy (PT) assessment, he was able to walk 640ft with festination, but post-LP was able to walk 1440ft, except for severe postural instability. He underwent right parietal ventriculoperitoneal shunt placement, and during follow-up, he denied shuffling steps or cane use. In routine 6- month follow-up, he denied any balance complaints or falls, and wife thought his cognition had improved. Exam demonstrated moderate supranuclear palsy, which was improved from prior exam, and normal gait. He also had recent improvement in intraocular pressures (IOP). IOP improved from 22 OD and 20 OS to 13 OD and 12 OS six months following shunting, though optic nerve tomography continued to show abnormal cupping OU and nerve fiber layer loss OS.  He was taking travoprost 0.004% eye drops nightly OU.

Conclusion: Increased IOP is associated with NPH, but this is the first known case report of documented improvement in supranuclear palsy and increased IOP after shunt. Post-shunting optic nerve tomography is associated with normal tension glaucoma, but it is unclear if changes reflect prolonged increase in ICP, post-operative changes, or an entirely different pathogenesis. It is unclear from this case report if glaucoma and NPH are two separate pathologic conditions, but the association reflects a need to characterize neurologic and ophthalmologic features in NPH as an effort to further understand pathogenesis and improvement in symptomatology following therapeutic shunting.

« Back to 2023 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/case-report-supranuclear-palsy-and-increased-intraocular-pressure-in-normal-pressure-hydrocephalus/