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CHOREA
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The word "chorea" comes from the Greek word (χορεία ) for dance
The word "chorea" comes from the Greek word (χορεία ) for dance. The jerky movements of the feet or hands are similar to dancing or piano playing. Characterized by involuntary, rapid, irregular & jerky movements involving multiple joints. Affects both Distal & Proximal muscles. Caused by damage to Basal Ganglia. Excess of Dopamine, the main neurotransmitter used in basal ganglia, prevents the basal ganglia from functioning normally.
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EXAMINATION Systemic clinical observation : describe parts affected.
Observe under which conditions symptoms aggravate or decrease. Neurological examination.
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TREATMENT Patient education. Maintain ROM. Wt bearing exercises.
Relaxation techniques. Proximal stabilization and distal fixation. Vestibular stimulation. Reduce stress.
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Rehabilitaton foccuses on : Compensatory movements.
Eg: Performing functional movements which reduce effort, Grasping objects to reduce tremor, Keeping hands in pocket, Reducing the types of movements that trigger or worsen dystonic symptoms provides some relief. Hypotonia – weighted jackets, weights. Wt bearing, Approximation – to increase joint stability. Distal fixation – to control involuntary movements. -Provide external holds on wheelchairs, lap boards, desks.
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ATHETOSIS
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Characterized by slow, involuntary, writhing, twisting, wormlike movements.
Purposeless movements. Caused by damage to the Corpus Striatum specifically to the Putamen. Movements are slower than that of chorea. Exaggerated by voluntary control.
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Clinical Features Alternating abduction, adduction at shoulder.
Flexion ,extension at distal joints. Wrist flexion but fingers extension. Hands unable to grasp due to constant fluctuation in tonic contraction of postural muscles. Flexion response in UL & Extension in LL (striatal position). Co-exists with chorea. Other areas like Neck, Face, Tongue, Feet & Trunk may also be involved.
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DYSTONIA
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Dystonia is a neurological movement disorder which causes sustained involuntary muscle contraction.
These contractions result in twisting and repetitive movements or abnormal postures. Common in trunk and extremities but may affect neck, face & vocal cord.
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2 Dystonic postures: Flexed dystonic posture – upper & lower limbs are flexed.(pallidal position). d/t damage to globus pallidus. 2. Flexion of upper limb & extension of lower limb known striatal position. Damage to Putamen.
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Types Generalized - affects most or all of the body.
Focal - is localized to a specific part of the body. e.g Cervical dystonia. Multifocal - involves two or more different body parts. Segmental - affects two or more adjacent parts of the body. Hemidystonia - affects the arm and leg on the same side of the body.
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Etiology Full body dystonia - genetic involving DYT gene.
Focal dystonia – unknown. - Clinical Feature: Excessive co-activation of agonist & antagonist interfering with timing, execution & loss of independent joint motion.
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Symptoms Loss of precision muscle coordination.
Cramping pain(minor exertions like holding a book and turning pages). Deterioration in handwriting after writing few lines. Draging / pulling up of foot(after running or walking some distance). Tremor. Voice or speech difficulties.
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Spasmodic torticollis/ cervical dystonia
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It is a painful condition in which, neck muscles contract involuntarily, causing head to twist or turn to one side and to uncontrollably tilt forward or backward. A rare disorder that can occur at any age, even infancy. Most often occurs in middle-aged people. Women more than Men. Symptoms generally begin gradually and then reach a point where they don't get substantially worse. The disorder sometimes resolves without treatment. Injecting botulinum toxin into the affected muscles often reduces the signs and symptoms of cervical dystonia. Surgery may be appropriate in a few cases.
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Symptoms The muscle contractions involved in cervical dystonia can cause head to twist in a variety of directions, including: - Chin toward shoulder. - Ear toward shoulder. - Chin straight up. - Chin straight down. Abnormal head postures. Jerking motion of the head also may occur. Neck pain radiating into the shoulders. Headaches.
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Causes Some cases, however, appear to be linked to:
Head, neck or shoulder injuries. Certain drugs, notably specific antipsychotic or anti-nausea agents.
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Risk factors Risk factors for cervical dystonia include:
Age : Most commonly begins between the ages of 40 and 70. Sex : Women are nearly twice as likely to develop cervical dystonia than Men. Positive Family history.
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Complications Some people who start out with cervical dystonia eventually develop similar symptoms in neighboring regions, such as the shoulder or face. However, cervical dystonia in middle age does not expand to wide areas of the body, as may occur in children. The disability and pain that can be caused by cervical dystonia may result in depression.
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Diagnosis and investigations
Blood or urine tests. These may reveal the presence of toxins. Magnetic resonance imaging (MRI). to identify and visualize tumors or evidence of stroke. Electromyography (EMG).
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Treatment Medications:
Botulinum toxin. This paralyzing agent, often used to smooth facial wrinkles, can be injected directly into the neck muscles affected by cervical dystonia. Most people with cervical dystonia see an immediate improvement with this treatment, which usually must be repeated every three to four months. Parkinson's drugs. Medications used to combat the tremors associated with Parkinson's disease, including trihexyphenidyl and benztropine (Cogentin), may be used in combination with botulinum toxin injections. Muscle relaxants : Diazepam (Valium, Diastat), lorazepam (Ativan), clonazepam (Klonopin) and baclofen (Lioresal). Pain medications: NSAIDS, Analgesics.
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The signs and symptoms of cervical dystonia are sometimes eased by:
Physical Therapy: The signs and symptoms of cervical dystonia are sometimes eased by: Exercises that improve neck strength and flexibility. Use of a neck brace. Training in stress management techniques. Surgical and other procedures: Cutting muscles or nerves. Deep brain stimulation (DBS): Used only in the most difficult of cervical dystonia cases.
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