Spinal cord compressive syndromes and other rаrе myelopathies: clinical presentations, classification and diagnostic difficulties. Part I

Abstract

Background The diagnosis of different thoracic spinal cord compressions (SCC) and some rare myelopathies has been and still remains a challenge for the current clinical practice in comparison with MS, disk herniation or spondylogenic lesions.
aimTo describe and analyse clinical and imaging data, etiology and topical distribution of the lesions, reasons for misdiagnosis and results from surgical treatment. Additional part two literature review on the topic gives information for some rare diseases, differential diagnosis and therapeutic recommendations.
Methods: In a single-center study from 2017 through 2020, we describe the data of 25 patients with SCS and some rare myelopathies, 17 women and 8 men, mean age 61.2 ±13 years. Etiology, course of the disease evolution, duration of symptoms, delay of the diagnosis, clinical and imaging characteristics, and treatment results were descriptively analyzed.
Results: Spinal meningioma was found in seven patients. Spondylogenic or discogenic compressive myelopathies had other 7 patients; spinal dural AVM fistula 1, iatrogenic epidural hematoma 1; syringomyelia 2; seropositve neuromyelitis optica 2; multiple lesion metastasis 1, post-radiologic myelopathy 1, paraspinal B-cell lymphoma 1; another thoracic-pleural tumor 1 and chronic relapsing demyelinating disorder 1. Of all patients, 76% presented with chronic symptoms, mean duration 8,2 ±7,9 months, and diagnostic delay of 3,9 ±3,8 months (range 3 days to 13 months). The thoracic segment was involved by pathology in 52%, severe motor deficit had 48%, Babinski sign 68%, sensory level 56%, pain on percussion 44% and autonomic disturbance 48%. MRI before the final diagnosis of SCC was carried out in 48%, whereas incorrectly or at another spinal level in 32%. Dissociation between clinical, topical diagnosis, and MRI finding was defined in 56%, however similarity in 32%. Eleven from 12 (96.2%) patients with thoracic meningioma despite diagnostic delay had significant improvement after neurosurgery treatment on follow up.
Conclusion: The most common reasons for diagnostic difficulties were: poor discerning of the initial symptoms; a non-comprehensive neurological examination, poor verification of the sensory level of involvement in the higher thoracic levels; inaccurate working diagnosis, that was not revised later on; absence of systematic followup; not using MRI as a golden diagnostic standard or irrelevant imaging, non-sufficient patients’ compliance and accepting the initial diagnosis as a final one.
The diagnosis of SCC could be established easier within one month with the following constellation: pain, leg weakness, Babinski sign, sensory level deficit, percussion-exaggerated segmental pain and autonomic disturbances. Neurosurgical treatment of spinal meningeomas, despite of some diagnostic delays, leads to significant clinical improvement.