Case
A 51-year-old woman presented with apraxia of eyelid opening, followed by slowly progressive masked facies, tongue tremor, dysphagia, neck and upper extremity rigidity, and bradykinesia 6 months after lumboperitoneal shunt placement for hydrocephalus after subarachnoid hemorrhage. An MRI examination of the brain showed midbrain compression, brainstem displacement inferiorly, and cistern effacement, consistent with infratentorial hypotension. 123I-ioflupane SPECT imaging showed reduced striatal dopamine transporter binding bilaterally. All symptoms and findings ameliorated after increasing shunt pressure (Figures 1 and 2 and Video 1). UPDRS Part III score improved from 24 to 5. Intracranial hypotension with midbrain sagging can cause reversible parkinsonism1,2 when displacement shear forces impair the nigrostriatal dopamine pathway.
Initial axial T2-weighted imaging (A) and sagittal T1-weighted imaging (B) show marked midbrain compression (dashed arrows), brainstem sagging (arrowhead), inferior drooping of the splenium (arrow), narrowing of the fourth ventricle (yellow arrowhead), and cerebellar tonsillar herniation (yellow arrow). (C, D) After shunt pressure adjustment, structures around the brain stem recovered.
Initial SPECT (A) demonstrates impaired dopamine transporter binding in the bilateral striata, which recovered after shunt pressure adjustment (B). The SBR (specific binding ratio) values were obtained using the Tossici-Bolt method (SBRBolt). Red lines represent the mean SBR by age and purple and green lines the 95% upper and lower prediction intervals.
Video 1
Video demonstrating apraxia of eyelid opening and reduced arm swing during walking before shunt pressure adjustment. After shunt pressure adjustment, these findings were almost resolved.Download Supplementary Video 1 via http://dx.doi.org/10.1212/200994_Video_1
Study Funding
This study was funded by Grants-in Aid from the Research Committee of CNS Degenerative Diseases, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health, Labour and Welfare Sciences Research Grants, the Ministry of Health, Labour and Welfare, Japan (Grant Number 20FC1049).
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Acknowledgment
The authors thank Rinako Shimada at Kyushu University and Edanz (jp.edanz.com/ac) for editing a draft of this article.
Appendix Authors
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, Med, MS.
Teaching slides links.lww.com/WNL/C179
- Received February 24, 2022.
- Accepted in final form June 8, 2022.
- © 2022 American Academy of Neurology
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