This patient, to our knowledge, represents the longest surviving patient with hypertonic muscular dystrophy (HMD), originally reported in 1994 in a Canadian Cree Indian population.1 Her rigidity progressed until she could only move her eyebrows, and she became too stiff to ventilate. She died of respiratory failure at age 10¾ years. She had continued to be active in school and extracurricular activities until that time. Case 8 of a recent report1 showed no progression beyond age 18 months. That patient died at age 3½ years (Elizabeth Gibbings, MD, oral communication, June 1991). As in previous cases, our patient's rigidity became evident after the neonatal period.1- 3 The muscles remained rigid despite sequential motor unit blockade with curare, pancuronium, and total anesthetic nerve block. The fibrillation potentials on EMG resemble that seen in previously reported cases. Normal cognitive development and nerve conduction velocities further support the concept that the central and peripheral nervous systems are normal in HMD. Autopsy studies on previous cases support these findings as well.1 Creek and Cree Indians are from geographically distinct regions. However, Creek Indians traded in regions that may have been inhabited by the Cree. We cannot, therefore, rule out a genetic transmission similar to those reported.