Dystonia

Clinic

• Dystonia is a disease, characterized with uncontrolled and sometimes painful spasms.
• Although it is a disease, It is a categorized below hyperkinetic movement disorders.
• Sustained or repetitive muscle contractions of dystonia result in twisting and repetitive movements or abnormal fixed postures.
• The movements may resemble a tremor.
• Dystonia is often agg by physical activity, and may progress into adjacent muscles.
• It can affect your whole body or just a single part. It can start at any age.

Symptoms

• Uncontrolled muscle Cramps / Spasms
• Twisting into unusual positions
• Tremors
• Uncontrolled blinking

Entities / Miasms

	CJD	WNE
Rigidity	+++	+++
Spasm	+++	+++

Pathophysiology

• Dystonia is a disorder of motor programs controlling semiautomatic movements or postures, with clinical features such as sensory trick, which suggests sensorimotor mismatch as the basis.
• Dystonia was originally classified as a basal ganglia disease
• Rare autopsy studies have found pathology both in the striatum and the cerebellum, and functional disorganisation was reported in the somatosensory cortex in patients.
• We review clinical evidence in light of this new functional interaction between the cerebellum and basal ganglia, and put forward a hypothesis that dystonia is a basal ganglia disorder that can be induced by aberrant afferent inputs from the cerebellum

Types

Generalized dystonias

• Normal birth history and milestones
• Autosomal dominant, Childhood onset
• Starts in lower limbs and spreads upwards
• Also known as torsion dystonia or idiopathic torsion dystonia or dystonia musculorum deformans.

Focal dystonias

Anismus

• It causes painful defecation, constipation; may be complicated by encopresis.

Cervical dystonia

• Alos called Spasmodic Torticollis
• It causes the head to rotate to one side, to pull down towards the chest, or back, or a combination of these postures.

Blepharospasm

• The patient experiences rapid blinking of the eyes or even their forced closure causing functional blindness.

Oculogyric crisis

• An extreme and sustained (usually) upward deviation of the eyes often with convergence causing diplopia (double vision).
• It is frequently associated with backward and lateral flexion of the neck and either widely opened mouth or jaw clenching.

Oromandibular dystonia

• Causes distortions of the mouth and tongue.

Spasmodic dysphonia

• Also called Laryngeal dystonia

• Causes the voice to sound broken, become hoarse, sometimes reducing it to a whisper.

Focal hand dystonia

• Also known as musician's or writer's cramp
• It interferes with activities such as writing or playing a musical instrument by causing involuntary muscular contractions. The condition is sometimes "task-specific," meaning that it is generally apparent during only certain activities. Focal hand dystonia is neurological in origin and is not due to normal fatigue. The loss of precise muscle control and continuous unintentional movement results in painful cramping and abnormal positioning that makes continued use of the affected body parts impossible.

• The combination of blepharospasmodic contractions and oromandibular dystonia is called cranial dystonia or Meige's syndrome.

Segmental dystonias

Segmental dystonias affect two adjoining parts of the body:

• Hemidystonia affects an arm and foot on one side of the body.
• Multifocal dystonia affects many different parts of the body.
• Generalized dystonia affects most of the body, frequently involving the legs and back.