Locked-in syndrome caused by extracranial and intracranial takayasu arteritis: A rare case report and radiological findings

Abstract

Locked-in syndrome (LiS) is a condition of motor paralysis involving all the voluntary muscles of 4 extremities with retained consciousness and alertness. Meanwhile, Takayasu arteritis (TA) is a systemic inflammatory disease affecting large arteries, including the aorta and the branches. This disease often affects the extracranial arteries but rarely occurs in the intracranial arteries. An 18-year-old male presented with a sudden onset of decreased consciousness and bilateral weakness. The patient exhibited normal horizontal and vertical eye movements but presented with impairment of the trigeminal nerve, peripheral-type facial palsy, absence of gag reflex and tongue movement, and neck weakness. Physiological reflexes were increased bilaterally, while pathological reflexes were present on both sides. Autonomic function was impaired, and communication was impossible due to paralysis. Additionally, the patient displayed varying blood pressure readings between the right and left arms. The antinuclear antibody (ANA) and antismooth muscle (ASMA) antibody tests yielded negative results, while the electroencephalogram (EEG) showed normal readings. The cerebral angiogram indicated multiple internal and external stenoses, with total stenosis evident in the basilar artery. The patient was diagnosed with multiple extracranial and intracranial stenoses due to TA. Total stenosis of the basilar artery resulted in brainstem infarction, which led to the development of LiS. Meanwhile, the disturbance of the motor tracts in the ventral brainstem was the underlying cause of the LiS. This case report demonstrated a variety of atypical presentations of TA. The involvement of multiple extracranial and intracranial arterial was attributed to LiS.

Keywords

• Takayasu's arteritis
• Locked-in Syndrome
• Stroke
• Brainstem
• Cardiovascular disease