Exploring the frequency and clinical background of the “zebra sign” in amyotrophic lateral sclerosis and multiple system atrophy

• Published in: Journal of the neurological sciences Vol. 401; pp. 90 - 94
• Main Authors: Sugiyama, Atsuhiko, Sato, Noriko, Kimura, Yukio, Shigemoto, Yoko, Suzuki, Fumio, Morimoto, Emiko, Takahashi, Yuji, Matsuda, Hiroshi, Kuwabara, Satoshi
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 15.06.2019
• Subjects: ALS; Iron; MSA; Myelin; PADRE; Upper motor neuron; ALS; MSA; Iron; PADRE; Upper motor neuron; Myelin
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2019.04.032

In amyotrophic lateral sclerosis (ALS), the “zebra sign” in the precentral gyrus on phase difference enhanced magnetic resonance imaging (PADRE) recently has been reported as a possible imaging biomarker for upper motor neuron (UMN) involvement. A previous study has shown that the “zebra sign” allowed us to differentiate patients with ALS from healthy subjects with excellent accuracy. We validated the usefulness of the sign for differentiating patients with ALS from healthy subjects and investigated whether the “zebra sign” can be observed other neurodegenerative disorders with UMN involvement. The “zebra sign” on PADRE was assessed in 26 patients with ALS, 26 with multiple system atrophy (MSA) and 26 healthy controls, and the sign was observed in 50%, 23%, and no subjects, respectively. ALS patients with the “zebra sign” demonstrated a higher UMN burden score than those without the sign. The “zebra sign” on PADRE is not specific to ALS, also present in MSA, but might reflect the degeneration of the UMN within the motor cortex in neurodegenerative disorders. •In half of ALS patients, the “zebra sign” on PADRE images was observed.•The “zebra sign” has high specificity for differentiating ALS from healthy subjects.•The “zebra sign” might reflect degeneration of the UMN within the motor cortex.•The “zebra sign” was observed in some MSA patients and not specific to ALS.