Scheie syndrome: Enzyme replacement therapy does not prevent progression of cervical myelopathy due to spinal cord compression

Abstract Background Persistent first intersegmental artery (PFIA) is a rare anatomical variation of vertebral arteries and is an asymptomatic finding in most cases. Here we report a rare case of cervical myelopathy caused by spinal cord compression by the PFIA. Case presentation The patient was a 52-year-old man who complained of numbness and burning sensation around the neck and left shoulder area, partial weakness in the left deltoid muscle, right side thermal hypoalgesia, and disturbance of deep sensation since the past 1 year, and the symptoms had gradually worsened. Magnetic resonance imaging (MRI) and computed tomography (CT) showed spinal cord compression by the left PFIA at the C1/C2 level. Because conservative treatment was ineffective, microvascular decompression (MVD) of the PFIA was performed. The left PFIA was laterally transposed using polytetrafluoroethylene (PTFE) bands and anchored to the dura mater using three PTFE bands. To achieve adequate transposition, the small blood vessels bridging the spinal cord and PFIA and the dorsal root nerve had to be sacrificed. Postoperative T2-weighted MRI showed a small hyperintense region in the lateral funiculus of the spinal cord, but no new neurological deficits were identified. In the early postoperative stage, the patient’s deep sensory impairment and motor dysfunction were improved. His numbness and burning sensation almost disappeared, but slight thermal hypoalgesia remained in the lower limb. Conclusion MVD is an effective treatment for spinal cord compression caused by the PFIA, but further studies are necessary to help address technical difficulties and avoid complications.

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Human recombinant erythropoietin improves motor function in rats with spinal cord compression-induced cervical myelopathy

PLoS ONE ◽

10.1371/journal.pone.0214351 ◽

2019 ◽

Vol 14 (12) ◽

pp. e0214351

Author(s):

Takahiro Tanaka ◽

Hidetoshi Murata ◽

Ryohei Miyazaki ◽

Tetsuya Yoshizumi ◽

Mitsuru Sato ◽

...

Keyword(s):

Spinal Cord ◽

Motor Function ◽

Spinal Cord Compression ◽

Cervical Myelopathy ◽

Cord Compression ◽

Recombinant Erythropoietin ◽

Human Recombinant Erythropoietin

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Study protocol for an observational study of cerebrospinal fluid pressure in patients with degenerative cervical myelopathy undergoing surgical deCOMPression of the spinal CORD: the COMP-CORD study

BMJ Open ◽

10.1136/bmjopen-2020-037332 ◽

2020 ◽

Vol 10 (9) ◽

pp. e037332

Author(s):

Carl Moritz Zipser ◽

Nikolai Pfender ◽

Jose Miguel Spirig ◽

Michael Betz ◽

Jose Aguirre ◽

...

Keyword(s):

Blood Pressure ◽

Spinal Cord ◽

Cerebrospinal Fluid ◽

Spinal Cord Compression ◽

Study Protocol ◽

Cervical Myelopathy ◽

Fluid Pressure ◽

Cord Compression ◽

Surgical Decompression ◽

Degenerative Cervical Myelopathy

IntroductionDegenerative cervical myelopathy (DCM) is a disabling spinal disorder characterised by sensorimotor deficits of upper and lower limbs, neurogenic bladder dysfunction and neuropathic pain. When suspected, cervical MRI helps to reveal spinal cord compression and rules out alternative diagnoses. However, the correlation between radiological findings and symptoms is weak. Cerebrospinal fluid pressure (CSFP) analysis may complement the appreciation of cord compression and be used for intraoperative and postoperative monitorings in patients undergoing surgical decompression.Methods and analysisTwenty patients diagnosed with DCM undergoing surgical decompression will receive standardised lumbar CSFP monitoring immediately before, during and 24 hours after operation. Rest (ie, opening pressure, CSF pulsation) and stimulated (ie, Valsalva, Queckenstedt’s) CSFP—findings in DCM will be compared with 20 controls and results from CSFP monitoring will be related to clinical and neurophysiological findings. Arterial blood pressure will be recorded perioperatively and postoperatively to calculate spinal cord perfusion pressure and spinal vascular reactivity index. Furthermore, measures of CSFP will be compared with markers of spinal cord compression by means of MR imaging.Ethics and disseminationThe study protocol conformed to the latest revision of the Declaration of Helsinki and was approved by the local Ethics Committee of the University Hospital of Zurich (KEK-ZH number PB-2016-00623). The main publications from this study will cover the CSFP fluid dynamics and pressure analysis preoperative, perioperative and postoperative correlated with imaging, clinical scores and neurophysiology. Other publications will deal with preoperative and postoperative spinal perfusion. Furthermore, we will disseminate an analysis on waveform morphology and the correlation with blood pressure and ECG. Parts of the data will be used for computational modelling of cervical stenosis.Trial registration numberClinicalTrials.gov Registry (NCT02170155).

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