Objective: To review a case of a patient diagnosed with both NPH and MSA.

Background: Multiple system atrophy (MSA) is a progressive neurodegenerative disease that presents with autonomic failure, parkinsonism and cerebellar symptoms​[1]​. Included in the differential diagnosis for MSA is normal pressure hydrocephalus (NPH) which is a potentially reversible neurologic condition characterized by ventriculomegaly, gait dysfunction, cognitive impairment, and urinary incontinence ​[2]​.

Method: Case Report

Results: A 62 year old right-handed woman was referred to neurology clinic for cognitive symptoms and gait disorder. She experienced progressive gait changes over the last 4 years with shuffling steps and difficulty picking up her feet, eventually requiring a rollator. She then developed unexplained urinary incontinence and cognitive slowing. She denied fluctuating cognition, anosmia or visual hallucinations.  Initial workup with head CT scan showed diffuse severe ventriculomegaly and she was referred to neurosurgery for NPH evaluation. She underwent high-volume lumbar puncture with improvement in gait and cognitive measures following CSF removal and underwent VP shunt placement. She continued to have urinary incontinence and significant gait difficulties after VP shunt placement. Neurological exam in movement disorder clinic was notable for asymmetric bradykinesia, increased muscle tone, left laterocolis and truncal lateral flexion.  She was unable to stand without assistance and her hands were dusky and cold. There was no supranuclear gaze palsy and her MoCA was 26/30. DaTscan showed diminished uptake within the caudate and putamen on the right compared to the left.  History, exam, and imaging was most consistent with MSA. The patient was recently seen for follow-up in neurology clinic and started on carbidopa levodopa and onabotulinumtoxinA injections for cranial dystonia.

Conclusion: We report a case of a patient initially diagnosed with NPH with partial response to CSF drainage and shunt who subsequently presented to our clinic for further evaluation and was diagnosed with MSA. We considered the possibility that there was a misdiagnosis of NPH given the lack of clear clinical benefit following VPS implantation with recurrent adjustments, however her disproportionate ventriculomegaly strongly suggestive of NPH and neuropsychological testing showing subcortical cognitive dysfunction would not be expected in MSA, but it doesn’t rule it out.

References: ​​[1] G. K. Wenning et al., “The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy,” Movement Disorders, vol. 37, no. 6, pp. 1131–1148, Jun. 2022, doi: 10.1002/MDS.29005.

​[2] B. Bluett et al., “Clinical Features of Idiopathic Normal Pressure Hydrocephalus: Critical Review of Objective Findings,” Mov Disord Clin Pract, vol. 10, no. 1, pp. 9–16, Jan. 2023, doi: 10.1002/MDC3.13608.

​ ​

« Back to 2024 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/multiple-system-atrophy-in-a-patient-with-normal-pressure-hydrocephalus/