Snake-Eyes Appearance on MRI Occurs during the Late Stage of Hirayama Disease and Indicates Poor Prognosis

Abstract

Purpose Because Hirayama disease is stereotyped as a self-limited disease in the absence of a definite pathology, we investigated the potential relationship between snake-eyes appearance (SEA) and Hirayama disease to bring a new perspective in the pathological process of Hirayama disease based on relevant radiological and clinical evidence. Methods A total of 30 cases observed SEA were selected from 293 patients with Hirayama disease to constitute the SEA group, and an equal number of cases were randomly selected from the remaining patients to form the non-SEA group. Cervical magnetic resonance imaging (MRI) was performed and subsequently used to measure the anteroposterior diameter and anterior shifting of the spinal cord. Additionally, clinical data, such as age, sex, duration of symptoms, symptoms, and signs, were collected and analyzed. Results Of 293 patients, 10.6% appeared with the SEA, which was mainly multisegmental (86.7%), particularly at the C5-6 segment (73.3%), and intense with a well-defined border (70.0%). The SEA group was an older population (p < 0.0001) with a longer duration (p < 0.0001) and a higher incidence of Hoffmann signs and knee hyperreflexia (p < 0.0001, p = 0.0038, respectively). The degree of spinal cord atrophy demonstrated a close association with the SEA, as it was significantly worse in the SEA group and SEA segment (p = 0.0008, p < 0.0001, respectively). The degree of spinal cord atrophy was positively related to both age and duration (p = 0.0095, p = 0.0176, respectively). Conclusions Confirmed as an irreversible lesion and an indication of poor prognosis, SEA appears during the late stage of Hirayama disease and is closely related to pyramidal signs and spinal cord atrophy.

Magnetic resonance imaging and dynamic X-ray’s correlations with dynamic electrophysiological findings in cervical spondylotic myelopathy: a retrospective cohort study

“…In the disease, forward displacement of the cervical dural sac and compressive flattening of the lower cervical cord occurs during neck flexion [41]. This causes occlusion of the anterior spinal artery in the lower cervical spinal cord and subsequent ischemia in the anterior horn cells [41,42]. If left untreated, Hirayama disease will result in lower cervical spinal cord atrophy and "snake-eye appearance" in MRI, which are considered negative prognostic indicators [42].…”

“…This causes occlusion of the anterior spinal artery in the lower cervical spinal cord and subsequent ischemia in the anterior horn cells [41,42]. If left untreated, Hirayama disease will result in lower cervical spinal cord atrophy and "snake-eye appearance" in MRI, which are considered negative prognostic indicators [42]. The exact pathophysiology of the cervical cords with different patterns of intramedullary T2 hyperintensity during motion still needs to be further studied.…”

Hirayama Disease in an Adolescent Male With Right Hand Weakness and Muscle Wasting

“…12 Hirayama disease often has an insidious onset of progressive weakness and muscular wasting of the upper extremity. 6,9,13 The disease appears to be self-limiting, reaching a stable state approximately 3 to 5 years after its onset, 4,14 a characteristic that differentiates this entity from other known motor neuron diseases. 8 Although it is often unilateral, affecting the left side more than the right, 15 Hirayama disease also presents bilaterally (symmetrically or asymmetrically) in 10% of cases.…”

Magnetic Resonance Imaging and Dynamic X-ray's correlations with Dynamic Electrophysiological Findings in Cervical Spondylotic Myelopathy: a retrospective cohort study.

“…In the disease, forward displacement of the cervical dural sac and compressive attening of the lower cervical cord occurs during neck exion [41]. This causes occlusion of the anterior spinal artery in the lower cervical spinal cord and subsequent ischemia in the anterior horn cells [41,42]. If left untreated, Hirayama disease will result in lower cervical spinal cord atrophy and "snake-eye appearance" in MRI, which are considered negative prognostic indicators [42].…”