Long-term course of anterior spinal cord herniation presenting with an upper motor neuron syndrome: case report illustra
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CASE REPORT
Open Access
Long-term course of anterior spinal cord herniation presenting with an upper motor neuron syndrome: case report illustrating diagnostic and therapeutic implications Martin Regensburger1,2,3* , Johannes C. M. Schlachetzki1, Jörg Klekamp4, Arnd Doerfler5 and Jürgen Winkler1
Abstract Background: Anterior spinal cord herniation (aSCH) is a rare cause of myelopathy which may present as pure motor syndrome and mimic other degenerative diseases of the spinal cord. In slowly progressive cases, diagnosis may be impeded by equivocal imaging results and mistaken for evolving upper motor neuron disease. As early imaging studies are lacking, we aimed to provide a detailed description of imaging and neurophysiology findings in a patient with aSCH, focusing on the early symptomatic stages. Case presentation: We here present the case of a 51-year old male patient with an episode of pain in the right trunk and a normal spinal MRI. After a symptom-free interval of 8 years, spasticity and paresis evolved in the right leg. There was subtle ventral displacement and posterior indentation of the thoracic spinal cord on MRI which, in retrospect, was missed as an early sign of aSCH. After another 3 years, symptoms spread to the left leg and a sensory deficit of the trunk became evident. Follow-up MRI now clearly showed an aSCH. Neurosurgical intervention consisted of remobilization of the herniated spinal cord and patch closure of the dura defect. Over the following years, motor and sensory symptoms partially improved. Conclusions: The history of this patient with aSCH illustrates the importance of careful longitudinal clinical follow-up with repeated imaging studies in progressive upper motor neuron syndromes. Specific attention should be paid to a history of truncal pain and to MRI findings of a ventrally displaced spinal cord. Neurosurgical intervention may halt the progression of herniation. Keywords: Spinal cord herniation, Evoked potentials, Spasticity, Case Report
Background In early stages of slowly progressive spasticity of the legs, establishing a diagnosis may be challenging as symptoms are often limited to just one region. The diagnosis of motor neuron diseases like amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia and primary lateral sclerosis * Correspondence: [email protected] 1 Department of Molecular Neurology, University Hospital Erlangen, Schwabachanlage 6, 91054 Erlangen, Germany 2 Center for Rare Diseases Erlangen (ZSEER), University Hospital Erlangen, Erlangen, Germany Full list of author information is available at the end of the article
relies on the exclusion of spinal stenosis and inflammatory diseases of the central nervous system. This clinical scenario is reflected by the category “clinically possible ALS” according to the El Escorial criteria, defined by clinical evidence of upper and lower motor neuron dysfunction in one region only [1]. Patterns and time course of progression of motor neuron diseases show a considerable variation and cannot be su
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