Tesi etd-07272018-123038 |
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Tipo di tesi
Tesi di specializzazione (5 anni)
Autore
DEL GAMBA, CLAUDIA
URN
etd-07272018-123038
Titolo
THE ASSOCIATION OF PRIMARY DYSTONIA WITH TICS: RESULTS FROM A CLINICAL AND ELECTROPHYSIOLOGICAL STUDY
Dipartimento
MEDICINA CLINICA E SPERIMENTALE
Corso di studi
NEUROLOGIA
Relatori
relatore Prof. Ceravolo, Roberto
relatore Prof. Siciliano, Gabriele
relatore Prof. Siciliano, Gabriele
Parole chiave
- bot
- dystonia
- Gilles de la Tourette syndrome
- tic
Data inizio appello
28/08/2018
Consultabilità
Non consultabile
Data di rilascio
28/08/2088
Riassunto
This study aimed to evaluate the association between dystonia and tics as a primary clinical entity.
Primary cranio-cervical dystonia (PCCD) is an idiopathic condition, which typically occurs in late adulthood and in women more than men. Primary tics (PT) generally start during childhood/adolescence, and a later age of onset or other associated movement disorders are “red flags” to suspect secondary causes. Primary dystonia and PT are distinct entities, but nevertheless, a clinical syndrome with these two features has been described. We report a series of patients presenting both PCCD and PT.
248 patients with PCCD attending our Botulinum toxin clinic at the National Hospital of Neurology and Neurosurgery - Queen Square, were examined for PT over a period of 4 months. Secondary tic causes were excluded by clinical interview and appropriate investigations (brain MRI, extensive blood tests, acanthocytes, genetics).
We have found 16 patients (6,5%) with PCCD, also presenting PT. Thirteen patients are males while three are females. In eleven dystonia started below the age of 40. Eleven had a focal involvement, while 5 a segmental. Dystonia was found to affect neck (13), vocal cords (4), jaw (1) eyes (1) and arms (4). Six patients displayed a sensory trick. The association with tics can be stratified as follows: 3/16 patients presented tics before 21 years old, fitting Tourette’s syndrome (GTS) criteria (also presenting >1 motor tic and ≥1 vocal tic); for the other patients, we were not able to recall the onset, which was therefore more likely to fall into the Adult Primary Tics (APT) category. Most of our patients also showed psychiatric issues (hyperactivity, anxiety, depression or obsessive-compulsive features), which were generally mild.
We described a primary clinical entity of PCCD associated with PT. This syndrome differs from pure cranio-cervical dystonia by a higher prevalence in males and a lower age of onset. It presents a tic disorder, which, in a minority of cases resembles GTS, whereas more frequently falls into APT category.
Primary cranio-cervical dystonia (PCCD) is an idiopathic condition, which typically occurs in late adulthood and in women more than men. Primary tics (PT) generally start during childhood/adolescence, and a later age of onset or other associated movement disorders are “red flags” to suspect secondary causes. Primary dystonia and PT are distinct entities, but nevertheless, a clinical syndrome with these two features has been described. We report a series of patients presenting both PCCD and PT.
248 patients with PCCD attending our Botulinum toxin clinic at the National Hospital of Neurology and Neurosurgery - Queen Square, were examined for PT over a period of 4 months. Secondary tic causes were excluded by clinical interview and appropriate investigations (brain MRI, extensive blood tests, acanthocytes, genetics).
We have found 16 patients (6,5%) with PCCD, also presenting PT. Thirteen patients are males while three are females. In eleven dystonia started below the age of 40. Eleven had a focal involvement, while 5 a segmental. Dystonia was found to affect neck (13), vocal cords (4), jaw (1) eyes (1) and arms (4). Six patients displayed a sensory trick. The association with tics can be stratified as follows: 3/16 patients presented tics before 21 years old, fitting Tourette’s syndrome (GTS) criteria (also presenting >1 motor tic and ≥1 vocal tic); for the other patients, we were not able to recall the onset, which was therefore more likely to fall into the Adult Primary Tics (APT) category. Most of our patients also showed psychiatric issues (hyperactivity, anxiety, depression or obsessive-compulsive features), which were generally mild.
We described a primary clinical entity of PCCD associated with PT. This syndrome differs from pure cranio-cervical dystonia by a higher prevalence in males and a lower age of onset. It presents a tic disorder, which, in a minority of cases resembles GTS, whereas more frequently falls into APT category.
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