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Dystonia

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Amr Hassan
Amr Hassan

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

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Amr Hassan, MD, FEBN Professor of Neurology- Cairo University DYSTONIA
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
Describe the movement
Hyperkinetic Dystonia
Distribution of dystonia Alberto Albanese, 2017
Alberto Albanese, 2017
Cervical dystonia
Cervical dystonia
Cervical dystonia
Cervical dystonia Laterocaput Laterocollis Torticaput Anterocaput Anterocollis Retrocaput Retrocollis Sagittal shift forward Torticollis Lateral shift
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.
Meigs' syndrome Triad of Ascites Pleural effusion Benign ovarian tumor Meige syndrome
• 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
Decreased by , increased by • Sleep • Sensory tricks • Rest (Task specific, Action) • Stress • Fatigue
• 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
• 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
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.
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.
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.
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
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Dystonia

  • 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
  • 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