Klippel–Feil syndrome associated with a craniocervico-thoracic dermoid cyst
A 47-year-old female in good health was referred for progressive gait disturbance. At birth she had been operated for a palatal fissure and had been diagnosed with KFS at 1 year of age. Two months prior to presentation, the patient developed bilateral lower extremity paresthesias associated with progressive paraparesis.\nOn physical evaluation, the patient had a short webbed neck, a low occipital hairline, and restricted neck mobility. She did not report any symptoms and did not present any neurological deficits in her upper extremities. On examination she exhibited bilateral proximal paresis (4/5) in the iliopsoas and quadriceps muscles. Her motor deficit was accompanied by marked patellar hyperreflexia (4+), but without hyperactive Achilles responses (no clonus) or Babinski responses. Her sensory deficit included a loss of position and vibration appreciation in the distal lower extremities involving the ankles but not the knees, decreased superficial touch sensation in the L1-L3 distributions, and decreased pin-prick sensation on the left hemibody from the clavicle down and on the right hemibody from the lower thorax down. She also exhibited difficulty with tandem gait testing, falling to either side, while finger-nose-finger remained intact.