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Hyperkinetic movement disorders
Essential tremor is a common condition characterized by bilateral, fast, low amplitude tremors mainly in the upper limbs that are often inherited and worsened by anxiety. It causes postural tremors when holding objects. Huntington's disease is a cause of chorea that presents in middle life with initial subtle movements and later psychiatric and cognitive symptoms due to a CAG trinucleotide repeat expansion. Tourette's syndrome is the most common cause of tics, characterized by multiple motor and at least one vocal tic starting in childhood and persisting over a year, affecting boys more than girls and associated with behavioral problems and disorders like ADHD and OCD.
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Hyperkinetic movement disorders
- 1. JMJ Movement DisordersMJNF HYPERKINETIC MOVEMENT DISORDERS ESSENTIAL TREMOR 1) Common condition 2) Often inherited as an autosomal dominant 3) Causes bilateral, fast, low amplitude tremor mainly in the upper limbs 4) Head and voice – occasionally involved 5) Tremor is postural –when holding a glass 6) There may be a cerebellar type action tremor component 7) Anxiety exacerbates the tremor 8) Tx – unnecessary and unsatisfactory 9) Salbutamol can worse the condition ESSENTIAL TREMOR CHOREA MYOCLONUS TICS DYSTONIA Rhythmic sinusoidal oscillation of a body part Excessive, irregular movements flitting from one body part to another Brief electric shock like jerk Stereotyped movements or vocalization (may be temporarily suppressed) Sustained muscle spasms causing twisting movements and abnormal postures DRUGS TX – small amounts of alcohol - Beta blockers (propranolol) - Primidone - Gabapentin SURGICAL TX – stereotactic thalamotomy - thalamic DBS CHOREA – causes SYSTEMIC DISEASES - Thyrotoxicosis - SLE - ALP - 1ry Polychythemia GENETIC DISORDRES - Huntington’s disease - neuroacanthocytosis - benign heriditory chorea STRUCTURAL AND VASCULAR DISORDERS AFFECTING BASAL GANGLIA DRUGS - Levodopa - OC pills POST INFECTIOUS – Sydenham’s chorea - rheumatic fever PREGNANCY Tx- dopamine blocking drugs (phenothiazines) dopamine depleting drugs (tetrabenazine)
- 2. JMJ Movement DisordersMJNF HUNTINGTON’S DISEASE (HD) 1) Cause of chorea 2) Present in middle life 3) Initially with subtle ‘fidgetiness’ followed by development of progressive psychiatric and cognitive symptoms 4) Due CAG trinucleotide repeat expansion 5) Inherited as autosomal dominant 6) Progressive neurodegenerative leads to dementia and ultimately death after 10-20 years HEMIBALLISMUS 1) Violent swinging movement of one side 2) Caused usually by infarction or hemorrhage in the contralateral sub thalamic nucleus MYOCLONUS PRIMARY MYOCLONUS Physiological myoclonus o Nocturnal myoclonus o Consisting a sudden jerks on dropping off to sleep or walking Myoclonic dystonia (DYT11) o ‘Lightning jerks’ often with dystonia o Autosomal dominant MYOCLONUS IN EPILEPSY EPILEPSY-ATAXIA SYNDROMES SECONDARY MYOCLONUS Metabolic diseases o Hepatic and renal diseases TICS 1) Common 2) Brief stereotyped movements usually affecting the face or neck 3) May affect any part of the body (vocal tics) 4) Simple transient tics common in childhood a. Blinking b. Sniffing c. Facial grimacing TOURETTE’S SYNDROME - Commonest cause of tics - characterized by multiple motor tics and at least one vocal tic, starting in childhood and persisting longer than a year - Boys affect more than girls - behavioral problems - Attention deficit hyperactivity disorder (ADHD) - obsessive – compulsive disorders (OCD)
- 3. JMJ Movement DisordersMJNF DYSTONIA PRIMARY DYSTONIAS SECONDARY DYSTONIA HEREDO- DEGENERATIVE DYSTONIA PAROXYSMAL DYSTONIAS Where dystonia is the only, or main clinical menifestation Due to brain injury, cerebral palsy or drugs As part of a wider neurodegenerative disorder Rare, mostly genetic, attacks of sudden involuntary movements with elements of dystonia and chorea