A case of vertebral artery compression syndrome mimicking primary lateral sclerosis

Vertebral artery compression syndrome (VACS) is an under-recognised condition that may be misdiagnosed as motor neuron disease. We report a case presenting features initially suggestive of primary lateral sclerosis (PLS) but later found to be VACS.

A 65-year-old man with hypertension was referred to our neurology department in the suspect of PLS. He presented with a 10-year history of involuntary jerk of the left lower limb, which ascended to the left upper limb 9 years later. He also developed intermittent painful spasms with a tendency to drag his left leg. His symptoms fluctuated with blood pressure. Neurological examination revealed upper motor neuron signs without lower motor neuron or sensory involvement. Electrophysiology studies were unremarkable. Brain MRI disclosed the left side of medulla oblongata was compressed by the tortuous left vertebral artery. Diffusion tensor tractography confirmed the corresponding corticospinal tract disruption. He was diagnosed with VACS and treated with antispasmodic medications and antihypertensive drugs.

VACS should be considered into the differential diagnoses of PLS. A thorough clinical assessment and careful interpretation of brain MRI with advanced diffusion neuroimages can help confirm the diagnosis.