Movement Disorders Vol. 6, No. 4, 1991, pp. 384 0 1991 Movement Disorder Society
Letter to the Editor veloped slight tics in the face. At the last visit in the clinic 1 month ago, we did not observe any of these symptoms, but his parents and brother confirmed them. He is living at home like twin A, but we do not suppose his symptoms are imitations of twin A, as the mother tells us that he grunts when he is alone too. The monozygosity of these twins was confirmed by the following tests: BMNS + Rh C-D-E-c + K, Hp 2-2 Gc 2-15 Tf 2-2, Gm (a - x - b +) Inv - , PGM a 2-a 4 ACP B GPT 2-1, EsD 1-1 GLO 2-2 AK 1-1, and ADA 2-3 for both brothers. These tests were performed by The Forensic Institute of the University of Copenhagen. The chance of monozygosity is 99.9%. We think the diagnosis is correct, as there is no Huntington’s disease in the family, and twin A is not in any way demented in spite of 11 years of symptoms. The diagnosis of twin B is not completely safe, but as it is well known that GTS can begin as late as the age of 21 years, we find the GTS diagnosis to be most probable. We suppose, like Goetz and Tanner, that the predisposition to GTS is inherited, but other factors are important for the development of the disease. In the present case, the heavily affected twin A had a higher birth weight than twin B. Why the clinical manifestations of the disease were manifested so late in twin B is unknown.
Tourette’s Syndrome in Twins To the Editor: Goetz and Tanner (Mov Disord 1990;5:173-175) presented findings in monozygotic twins with Gilles de la Tourette’s syndrome (GTS) of great difference in severity. We have followed a pair of monozygotic twins with GTS for 4 years where the difference in symptoms is as great as possible. The twins are now 21 years old. They have no siblings. No one in the family has movement disorders of any type. The birth weight of twin A was 2,200 g. His development was normal, he was walking at 15 months of age, and he was healthy until the age of 10 years. At that time, grunting and slight tics in the face started. During the next 5 or 6 years, the symptoms increased slowly, but when I saw him 4 years ago, when he was 17 years old, he had the most violent tics we have seen in our clinic (among 130 GTS patients). He was screaming and grunting every second to fourth second and could hardly sit in a chair. He is now working in a workshop as an instrument maker(!). He can manage to handle the most delicate tools, but he needs more time than others to finish a task. The birth weight of twin B was 1,750 g. He had a normal development during childhood, and until the age of 20 years, 1 year ago, did not have the slightest symptom. He was not hyperactive as a child, and he is now a blacksmith. One year ago, he started grunting and stuttering for short periods, and during the last few months he has de-
H. Pakkenberg B. Pakkenberg Department of Neurology Hvidovre University Hospital Hvidovre, Denmark
384
Letter to the Editor veloped slight tics in the face. At the last visit in the clinic 1 month ago, we did not observe any of these symptoms, but his parents and brother confirmed them. He is living at home like twin A, but we do not suppose his symptoms are imitations of twin A, as the mother tells us that he grunts when he is alone too. The monozygosity of these twins was confirmed by the following tests: BMNS + Rh C-D-E-c + K, Hp 2-2 Gc 2-15 Tf 2-2, Gm (a - x - b +) Inv - , PGM a 2-a 4 ACP B GPT 2-1, EsD 1-1 GLO 2-2 AK 1-1, and ADA 2-3 for both brothers. These tests were performed by The Forensic Institute of the University of Copenhagen. The chance of monozygosity is 99.9%. We think the diagnosis is correct, as there is no Huntington’s disease in the family, and twin A is not in any way demented in spite of 11 years of symptoms. The diagnosis of twin B is not completely safe, but as it is well known that GTS can begin as late as the age of 21 years, we find the GTS diagnosis to be most probable. We suppose, like Goetz and Tanner, that the predisposition to GTS is inherited, but other factors are important for the development of the disease. In the present case, the heavily affected twin A had a higher birth weight than twin B. Why the clinical manifestations of the disease were manifested so late in twin B is unknown.
Tourette’s Syndrome in Twins To the Editor: Goetz and Tanner (Mov Disord 1990;5:173-175) presented findings in monozygotic twins with Gilles de la Tourette’s syndrome (GTS) of great difference in severity. We have followed a pair of monozygotic twins with GTS for 4 years where the difference in symptoms is as great as possible. The twins are now 21 years old. They have no siblings. No one in the family has movement disorders of any type. The birth weight of twin A was 2,200 g. His development was normal, he was walking at 15 months of age, and he was healthy until the age of 10 years. At that time, grunting and slight tics in the face started. During the next 5 or 6 years, the symptoms increased slowly, but when I saw him 4 years ago, when he was 17 years old, he had the most violent tics we have seen in our clinic (among 130 GTS patients). He was screaming and grunting every second to fourth second and could hardly sit in a chair. He is now working in a workshop as an instrument maker(!). He can manage to handle the most delicate tools, but he needs more time than others to finish a task. The birth weight of twin B was 1,750 g. He had a normal development during childhood, and until the age of 20 years, 1 year ago, did not have the slightest symptom. He was not hyperactive as a child, and he is now a blacksmith. One year ago, he started grunting and stuttering for short periods, and during the last few months he has de-
H. Pakkenberg B. Pakkenberg Department of Neurology Hvidovre University Hospital Hvidovre, Denmark
384
