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A case of spinal anesthesia in a patient with progressive supranuclear palsy.

ABSTRACT: Progressive supranuclear palsy (PSP) is one of the rare diseases. PSP is characterized by oculomotor dysfunction, postural instability, akinesia, dysarthria, and dysphagia. The major cause of death in patients with PSP is aspiration pneumonia. Considering these complications, spinal anesthesia is useful in patients with PSP. However, the potential harmful effects of spinal anesthesia including neurotoxicity of local anesthetics and neurologic complications for patients with PSP are unclear, because there has been no report. Here, we present spinal anesthesia for a patient with PSP. An 80-year-old man with progressive oculomotor dysfunction, dysphagia, and history of repeated aspiration pneumonia was scheduled for inguinal hernia surgery. Acutely concerning about perioperative pulmonary complications, we performed spinal anesthesia. Fortunately, there was no complication associated with respiration or neural system during perioperative period. We hope our experience and case report will be helpful in specific perioperative anesthetic care for patients with PSP.

SUBMITTER: Tonan M

PROVIDER: S-EPMC5804689 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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A case of spinal anesthesia in a patient with progressive supranuclear palsy.

Tonan Momoka M Egi Moritoki M Furushima Nana N Mizobuchi Satoshi S

JA clinical reports 20180125 1

Progressive supranuclear palsy (PSP) is one of the rare diseases. PSP is characterized by oculomotor dysfunction, postural instability, akinesia, dysarthria, and dysphagia. The major cause of death in patients with PSP is aspiration pneumonia. Considering these complications, spinal anesthesia is useful in patients with PSP. However, the potential harmful effects of spinal anesthesia including neurotoxicity of local anesthetics and neurologic complications for patients with PSP are unclear, beca ...[more]

PMID: 29457122

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Project description:The most frequent neurodegenerative proteinopathies include diseases with deposition of misfolded tau or α-synuclein in the brain. Pathological protein aggregates in the PNS are well-recognized in α-synucleinopathies and have recently attracted attention as a diagnostic biomarker. However, there is a paucity of observations in tauopathies. To characterize the involvement of the PNS in tauopathies, we investigated tau pathology in cranial and spinal nerves (PNS-tau) in 54 tauopathy cases [progressive supranuclear palsy (PSP), n = 15; Alzheimer's disease (AD), n = 18; chronic traumatic encephalopathy (CTE), n = 5; and corticobasal degeneration (CBD), n = 6; Pick's disease, n = 9; limbic-predominant neuronal inclusion body 4-repeat tauopathy (LNT), n = 1] using immunohistochemistry, Gallyas silver staining, biochemistry, and seeding assays. Most PSP cases revealed phosphorylated and 4-repeat tau immunoreactive tau deposits in the PNS as follows: (number of tau-positive cases/available cases) cranial nerves III: 7/8 (88%); IX/X: 10/11 (91%); and XII: 6/6 (100%); anterior spinal roots: 10/10 (100%). The tau-positive inclusions in PSP often showed structures with fibrillary (neurofibrillary tangle-like) morphology in the axon that were also recognized with Gallyas silver staining. CBD cases rarely showed fine granular non-argyrophilic tau deposits. In contrast, tau pathology in the PNS was not evident in AD, CTE and Pick's disease cases. The single LNT case also showed tau pathology in the PNS. In PSP, the severity of PNS-tau involvement correlated with that of the corresponding nuclei, although, occasionally, p-tau deposits were present in the cranial nerves but not in the related brainstem nuclei. Not surprisingly, most of the PSP cases presented with eye movement disorder and bulbar symptoms, and some cases also showed lower-motor neuron signs. Using tau biosensor cells, for the first time we demonstrated seeding capacity of tau in the PNS. In conclusion, prominent PNS-tau distinguishes PSP from other tauopathies. The morphological differences of PNS-tau between PSP and CBD suggest that the tau pathology in PNS could reflect that in the central nervous system. The high frequency and early presence of tau lesions in PSP suggest that PNS-tau may have clinical and biomarker relevance.

Dataset Information

A case of spinal anesthesia in a patient with progressive supranuclear palsy.

Publications

A case of spinal anesthesia in a patient with progressive supranuclear palsy.

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