Port-Wine Stain and Monomelic Weakness Associated With Perineurioma: A Neuromuscular Image

Abstract

CASEA 31-year-old woman presented with progressive weakness, numbness, and atrophy of the left lower extremity that started manifesting at age 14 years. She also had a port-wine stain in the affected leg since 6 months of age. The medical history was notable for gestational diabetes mellitus and recurrent nephrolithiasis. The family history was unremarkable. On examination, she had a large port-wine stain over the left lower leg extending to her buttock, hip, and trunk (Fig. 1). There was marked atrophy in the entire left lower extremity. On manual muscle strength testing, left hip flexion, knee extension, and knee flexion were 4/5, ankle dorsiflexion and plantar flexion were 3/5, and inversion was 0/5. Deep tendon reflexes were absent at the left knee and ankle. Sensation was impaired to pain and temperature below the left knee.