Dystonia Dystonia is a movement disorder in which your muscles contract involuntarily, causing repetitive or twisting movements. The condition can affect one part of your body (focal dystonia), two or more adjacent parts (segmental dystonia) or all parts of your body (general dystonia). The muscle spasms can range from mild to severe. They may be painful, and they can interfere with your performance of day-to-day tasks. Dystonia: Causes, Types, Symptoms, and Treatments

1. Amr Hassan, MD, FEBN
Professor of Neurology- Cairo University
DYSTONIA

2. Clinical assessment of dystonia
• Describe the movement
• Differentiate from other MD
• Dystonia or dystonia plus
• Distribution
• Decreased by , Increased by
• Diurnal variation
• Duration
• Distinguished phenomenon
Sensory tricks
Overflow
mirroring
Dr Amr Hassan 2017

3. Describe the movement

4. Hyperkinetic Dystonia

5. Distribution of dystonia
Alberto Albanese, 2017

6. Alberto Albanese, 2017

7. Cervical dystonia

8. Cervical dystonia

9. Cervical dystonia

10. Cervical dystonia
Laterocaput Laterocollis Torticaput
Anterocaput Anterocollis Retrocaput Retrocollis Sagittal shift
forward
Torticollis Lateral shift

11. Dystonic tremor
• A spontaneous oscillatory, rhythmical,
although often inconstant, patterned
movement produced by contractions of
dystonic muscles
• Often exacerbated by an attempt to maintain
primary (normal) posture.
• Dystonic tremor may be difficult to distinguish
from essential-type tremor.

12. Meigs' syndrome
Triad of
Ascites
Pleural effusion
Benign ovarian tumor
Meige syndrome

13. • 2 groups : Choreo-
athetotic and dystonic
• Often severe hypoxia in
term baby; previously
kernicterus
• History of insult + UMN
signs
• Incidence of MR 30%
Dyskinetic CP

14. Decreased by , increased by
• Sleep
• Sensory tricks
• Rest
(Task specific, Action)
• Stress
• Fatigue

15. • AD
• Mutation of the enzyme GTP
cyclohydrolase, which is the rate-
limiting step in the production of
tetrahydrobiopterin, a cofactor in the
metabolism of dopamine.
• Limb dystonia that typically affects
walking.
Dopa-responsive dystonia

16. • Diurnal fluctuation.
• Spastic gait, parkinsonism, or cerebral
palsy.
• Focal dystonia or parkinsonism in
adults.
• Patients respond to low dosages: 300
mg per day.
Dopa-responsive dystonia

17. Sensory tricks
• Alleviating maneuvers (gestes antagonistes)
• Voluntary actions that specifically correct the
abnormal
• posture or alleviate the dystonic movements.
These are
• usually simple movements (“gestes”) involving, or
directed to, the body region affected by dystonia,
but not consisting in a forceful opposition to the
phenomenology of dystonia.

18. Overflow
• Commonly found in dystonia.
• Unintentional muscle contraction which
accompanies, but is anatomically distinct from
the primary dystonic movement.
• It commonly occurs at the peak of dystonic
movements.

19. Mirror dystonia
• Mirror dystonia is a unilateral posture or
movement that is the same or similar in
character to a dystonic feature that can be
elicited, usually in the more severely affected
side, when contralateral movements or
actions are performed.

20. Clinical assessment of dystonia
• Describe the movement
• Differentiate from other MD
• Dystonia or dystonia plus
• Distribution
• Decreased by , Increased by
• Diurnal variation
• Distinguished phenomenon
Sensory tricks
Overflow
mirroring
Dr Amr Hassan 2017

21. THANK YOU