Sulcal Artery Syndrome in a 10-Year-Old Boy

Occlusion of the anterior spinal artery (ASA) usually affects both anterior horns. Patients present with paraparesis and dissociated sensory deficits secondary to involvement of cortical spinal and spinal thalamic tracts, while vibration and proprioception testing are normal, since there is sparing of the posterior columns. Our patient complained about the sudden onset of neck pain after abrupt turning of the head during gymnastics at school. Within 1 hour, he developed left-sided hemiparesis and Horner syndrome, along with contralateral sensory loss to nociception, while tactile perception remained unaffected on both sides ([Video 1]). Diffusion-weighted magnetic resonance imaging (MRI) performed 6 hours after onset of symptoms disclosed a signal hyperintensity of the upper cervical spinal cord ([Fig. 1A]). The patient's hemiparesis slightly improved during the course, and MRI 2 weeks later showed demarcation of a wedge-shaped cervical infarction ([Fig. 1B, C]) The boy's unusual clinical symptoms are explained by occlusion of a sulcal artery, the terminal branch of the ASA, which solely supplies the territory of one anterior cervical horn ([Fig. 1D]). A careful workup including MR angiography ruled out vertebral dissection or occlusion as well as other rare causes of spinal cord ischemia. Therefore, the etiology of this very rare event remained elusive in our patient.

Video 1

Neurological examination of the patient showing left-sided central Horner syndrome and hemiparesis due to the affection of the ipsilateral descending sympathetic pathway and corticospinal tract. Notice also perceived bilateral tactile perception, but lacking right-sided reaction to pain caused by the affection of the contralateral ascending spinothalamic tract. Online content including video sequences viewable at: www.thieme-connect.com/products/ejournals/html/10.1055/s-0036-1579634.

Fig. 1 (A) Diffusion-weighted MRI 6 hours after onset of symptoms depicting signal hyperintensity of the upper spinal cord. (B and C) T2-weighted axial and sagittal MRI 2 weeks after onset of symptoms demonstrating a left-sided infarct at the level of C2/C3. (D) Sketch of the blood supply, location of major tracts in the cervical spinal cord, and area of infarction in the patient. The area contralateral to the infarct is supplied by the sulcal artery above or below the depicted segment.

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