Hyperkinetic Movement Disorders Flashcards
what is the pathophysiology of a tremor?
Rhythmic sinusoidal oscillation of a body part. Usually due to alternate activation of agonist and antagonist muscles.
how are tremors classified?
Position - Postural, Kinetic, Task specific, Isometric tremor
Distribution
Frequency
Amplitude
what are common types of tremor?
cerebellar dystonic essential orthostatic parkinsonian physiological psychogenic rubral
what are the features of cerebellar tremors?
low, broad tremor of the extremities that occurs at the end of a purposeful movement
what are the features of dystonic tremors?
effects those with dystonia (involuntary muscle contractions cause twisting and repetitive motions and/or painful and abnormal postures or position)
what are the features of essential tremors?
hands are most often affected but the head, voice, tongue, legs, and trunk may also be involved. Head tremor may be seen as a vertical or horizontal motion. May be accompanied by mild gait disturbance, may be mild and non-progressive – or progressive
what are the features of orthostatic tremors?
characterized by fast (>12 Hz) rhythmic muscle contractions that occur in the legs and trunk immediately after standing.
what are the features of Parkinsonian tremors?
caused by damage to structures within the brain that control movement. Resting tremor = pill rolling
what are the features of physiological tremors?
tremor occurs in every normal individual and has no clinical significance
what are the features of psychogenic tremors?
occur at rest or during postural or kinetic movement. The characteristics of this kind of tremor may vary but generally include sudden onset and remission, increased incidence with stress,
what are the features of rubral tremors?
coarse slow tremor which is present at rest, at posture and with intention. Associated with conditions that effect red nucleus
what are the causes of resting tremor?
Parkinson’s disease
Drug-induced parkinsonism
what are the causes of psychogenic tremors?
POSTURAL TREMOR
Essential tremor
Enhanced physiological tremor
Tremor associated with neuropathy
what are the causes of kinetic tremors?
Cerebellar disease (demyelination, haemorrhage, degenrative, toxic) Wilson’s disease
what are the causes of head tremor?
Dystonia
Cerebellar disease
what are the causes of jaw tremor?
Dystonia
Parkinson’s disease
what are the causes of palatal tremor?
With ataxia
Symptomatic
Essential tremor
what is the pharmacological management of tremors?
Betablockers – propranolol
Anti-seizure medications – gabapentin and topiramate
Benzodiazepines – alprazolam and clonazepam
Parkinson’s – levodopa, carbidopa
Botulinum toxin
what is the core criteria of essential tremors?
Bilateral action tremor of the hands and forearms (not rest tremor)
Absence of other neurologial signs (except for cogwheel phenomenon)
May have isolated head tremor with no signs of dystonia
what is the secondary criteria of essential tremors?
Longy duration (> 3 years)
Positive family history
Beneficial response to alcohol
what are the first line treatments of essential tremors?
propanolol and primidone
what is a tic?
“un”-voluntary, stereotyped movements, or vocalizations.
what are the different types of tics?
Motor Vocal Stereotypies Mannerisms Primary and Secondary
Motor and vocal tics can be…
simple or complex
what are examples of simple motor tics?
one discrete movement) – eye blinking, head jerking, shoulder shrugging
what are examples of complex motor tics?
(involve a cluster of movements and appear coordinated) – pulling at clothes, touching objects, touching people, echopraxia, copropraxia
what are examples of simple vocal tics?
(single unarticulated sounds) – throat clearing sniffing or grunting
what are examples of complex vocal tics?
(stereotyped words or phrases) – echolalia, palilalia, coprolalia
what are examples of primary tic disorder?
Simple transient tics of childhood
Chronic tics of childhood
Gilles de la Tourette’s syndrome
Adult onset Tourettism
what are the neurodegenerative causes of secondary tics?
Huntington’s disease, Wilson’s disease, Neuroacanthocythosis, Neuronal brain iron accumulation syndrome, Rett’s syndromes, Lesch-Nyhan syndrome
what are the developmental causes of secondary tics?
Down syndrome and other chromosomal abnormalities, Fragile X syndrome, Autism, non-specific mental retardation
what are the structural causes of secondary tics?
Post-encephalopathy, Basal ganglia lesions (usually caudate nucleus)
what are the infective causes of secondary tics?
Sydenham’s chorea, PANDAS
what are the drugs and toxins causes of secondary tics?
Carbon monoxide poisoning, cocaine, amphetamines, anticonvulsants
what are the causes of Gilles de la Tourette Syndrome?
Autosomal dominant inheritance
what is the diagnostic criteria for Tourettes?
Both multiple motor tics and one or more vocal tics must be present
The tics must occur many times a day, nearly every day, or intermittently for more than 1 year with no longer than 3 months interval of tic-freeness
Age of onset < 18
Exclusion of obvious secondary causes
what is the treatment of tourette?
Symptomatic treatment (clonidine, tetrabenazine) CBT
what are chorea?
brief irregular purposeless movements that flit and flow from one body part to another.
generalised or local
what are the inherited/degenerative disorders that cause chorea?
Huntington’s disease and HD-like syndromes Wilson’s disease Neuroacanthocythosis Benign hereditary chorea Ataxia telangiectasia Spinocerebellar ataxia Typ 17
what are the autoimmune disorders that cause chorea?
SLE APS Bechet syndrome Coeliac disease Sydenham’s chorea Hashimotos
what are the infectious causes chorea?
HIV
what are the drugs that cause chorea?
Dopamine-receptor blocking drugs, levodopa, stimulants, oral contraceptive pill, anticonvulsants
what are the disorders that cause paroxysmal chorea?
Parkinson’s disease
what are the metabolic causes of chorea?
Chorea gravidarum, glucose, thyroid, parathyroid, sodium, magnesium metabolism
what is the symptomatic management of chorea?
terabenazine or dopamine receptor blocking drugs.
what is myoclonus?
Brief electric-shock like jerks
what is a negative myoclonus?
a temporary cessation of muscle activity e.g. liver flap
what is the pathophysiology of myoclonus?
Activation from the cortex, subcortical structures, spinal cord, or nerve root and plexus.
Imbalance of neurotransmitters controlling communication of feedback loops in motor pathways - serotonin, GABA
what are the causes of primary myoclonus?
Hypnic jerks, hiccup Benign infantile myoclonus with feeding / sleep Essential myoclonus Myoclonus dystonia Cortical tremor
what are the causes of myoclonus with epilepsy?
Benign myoclonic epilepsy of infancy Juvenile myoclonic epilepsy Early infantile myoclonic encephalopathy West’s syndrome Lennox-Gastaut syndrome Epilepsia partialis continua
what are the causes of progressive myoclonic epilepsy and ataxia?
Lafora body disease Unverricht-Lindborg disease Neuronal ceroid lipofuscinosis Sialidosis Myoclonic epilepsy with ragged red fibres Dentatorubropallidoluysian atrophy
Symptomatic myoclonus occurs in what conditions?
With encephalopathy Liver failure Renal failure Drug intoxication (alcohol, lithium) Toxins (lead) Post hypoxia Progressive encephalomyelitis with rigidity
what is dystonia?
o involuntary muscle spasm which leads to sustained abnormal postures of the affected body part.
what are the clinical features of dystonia?
abnormal posture is not fixed, and slow writhing movements can occur
Often improved by sensory trick
Dystonic tremor is jerky, variable in amplitude, and worsened by particular position or task
how are dystonias classified?
Age of Onset
Distribution
Etiology
what are the different age of onsets dystonia?
Early-onset (<26)
Late-onset (>26)
what are the different distribution dystonia?
Focal - single body region Segmental - contiguous body region Hemibody - Ipsilateral arm and leg Multifocal - > 2 non-contiguous body parts Generalized - entire body
What are the different aetiology dystonia?
Primary Dystonia plus syndromes Secondary Heredodengerative Paroxysmal Psychogenic
what are the features of primary dystonia?
dystonia +/- tremor are the only symptom and sign
what are the features of Dystonia-plus syndromes?
other signs may occur, but there is no secondary cause and no neurodegeneration
what are the features of secondary dystonia?
clear secondary cause is present; e.g. brain injury, drug exposure
what are the features of Heredodegenerative dystonia?
in the context of wider neurodegenerative process
what are the features of paroxysmal dystonia?
episodic attacks of dystonia, often no clinical signs between attacks
what are the clinical features of cervical dystonia?
abnormal head posture, head tremor, head pain
what are the clinical features of blepharospasm?
increased blink rate, forced eye closure, difficulty opening eyes
what are the clinical features of oromandibular dystonia?
haw clenching (bruxism), jaw in opening position, lateral jaw shift
what are the clinical features of spasmodic dystonia?
Adductor - voice breaks and strain
Abductor - breathy voice
Mixed - both
what are the clinical features of limb dystonia?
Action dystonia’s affecting writing, playing an instrument, handling tools
what are the clinical features of axial dystonia?
movements of shoulders, back, or abdomen
