Movement Disorders-
DYSKINESIAS
The basic sign of movement:
ABNORMAL
INVOLUNTARY
CLASSIFICATION
 A. OBSERVATION
 1. TREMOR
 2. DYSTONIA
 3. CHOREA
 4. BALLISM
 5. TICS
 6. MYOCLONUS
 B. ETIOLOGY
 1. Hereditary d.
 2. SECONDARY d.
 - Drug induced (neuroleptics)
 - Vascular (lacunar stroke)
 - Metabolic and Endocrine (Wilson d., thyreopathy)
 - Immunologic (lupus erythematosus)
 - Psychogenic
TREMOR
 Continual
 Rytmic movement
 patologic sign in every age
Classification
 According to:
 1. POSITION
 - rest or kinetic
 2. LOKALIZATION
 - focal (head,hands) to generalized
 3. FREQUENCY
 - slow, middle, serious
 4. AMPLITUDE
 - light, middle, serious
 According to 5. ETIOLOGY :

 Physiologic (fever, hypothermia)
 Drug induced (antidepressants, antiasthmatics, AED)
 Metabolic and Endocrine (hypoglycemia,hyperthyreoidism)
 Withdrawal syndrome (ethanol)
ESSENTIAL TREMOR
 The most frequent Dyskinesia (4%)
 The most common cause of patologic tremor
 Mostly hereditary etiology
 Only monosymptomatic disease
 Tremor is kinetic, bilateral, mostly lokalized to hands
 Positive effect of ethanol abuse
 Long time of duration
Treatment
 1. Does not take a trouble – without therapy
 2. Takes a trouble sometimes - benzodiazepines intermittent
 3. Takes a trouble most of the day – betablockers
 - barbiturates
 4. Serious disability – injections of BTX (botulinum toxin)
 - DBS (very rare)
DYSTONIA
 Slow spasm causing abnormal postures
Classification according to ETIOLOGY
 - Idiopatic
- Familiar (DYT 1……DYT 35)
DRD (doparezponsive Dystonia) – low doses of L-Dopa
- Symptomatic ( Wilson d., parkinson plus d. –CBD,PSP)
 - Drug induced (antiemetics!, antiparkinsonics,neuroleptics)
Classification according to
LOKALIZATION
 FOKAL D.
 SEGMENTAL D.
 GENERALIZED D. ( hereditary, childhood)
FOCAL Dystonia
 CERVICAL
 - torticollis (most common)
 - anterocollis, retrocollis, laterocollis
 BLEPHAROSPASM
 WRITER´S CRAMP
Treatment
 1. INJECTIONS OF BTX (focal D.)
 - chemical denervation, blocade of acetylcholintransfer
 - muscle weakness can be effective for about 3 months
 - 5% of patients develop antibodies
 2. MEDICAMENT (pills)
 - Anticholinergics ( Akineton)
 - GABA agonists (Baclofen)
 - benzodiazepines (Diazepam, Rivotril)
 3. SURGICAL (generalized D.)
 - DBS (globus palidum bilateral)
CHOREA
 Chaotic
 Nonstereotype
 Irregular
CLASSIFICATION
 A. Hereditary
 B. SECONDARY
 1. Drug induced (antiparkinsonics, AED, neuroleptics, HAK, KS, opiates)
 2. Metabolic (hepatal or uremic encefalopathy )
 3. Endocrine (chorea gravidarum)
 4. Immunologic (lupus erythematosus )
 5. Other (senile orofacial f.e. due to new dental prosthesis)
HUNTINGTON DISEASE
 - prevalence 4-10 : 100 000
 - adult form (age of onset 35 -50)
 - hereditary, AD (children – 50% risk)
 - symptom : chorea + dementia + personality changes
 - 100% mortality (survival 10 -15 years)
 - no causal treatment possibility

PATHOLOGY
 - faulty gen on the 4. chromozome and cause expansion of triplet CAG
 - less than 35 triplets : exclude HD
 - 40 and more triplets : confirm HD
 - result: production of pathologic protein Huntingtin
 Genetic testing (adult person in risk…Prague)
Clinical Symptoms
 - Dyskinesias ( CHOREA f. to g. … less dystonia)
 - Mental symptoms (depression, anxiety, aggression, ethanol abuse,
criminality)
 - Dementia (present always in late stadium)
Neuroimaging
 Brain CT or MRI - atrophy of caput ncl caudati
 Brain PET MRI - hypometabolism of ncl caudati
 Obrázek…..
Treatment
only symptomatic
 CHOREA - neuroleptics ( Tiapridal, Haloperidol,Rispen)
 DEPRESSION - SSRI
 PSYCHOSIS - neuroleptics
 DEMENTIA - no treatment
 Clinical trials (2019) gene therapy (target: inactivation of prescription
huntingtin protein)
(HEMI)BALLISM
 Rapid
 Severe
 Unilateral
 Lokalizated mostly on radical muscles of limb
 Prevalence - 1 : 500 000 (rare )
 Etiology - lession of ncl. subthalamicus Luysi mostly according to lakunar
infarkt
 Treatment - neuroleptics
 - benzodiazepines
 - AED
TICS
 Sudden
 Stereotypic
 Movement or Vocalization
CLASSIFICATION
 MOTORIC T.
 - simple brief movement (head jerks, eye blinking)
 - complex coordinated movement ( grimace)
 VOCAL T.
 - simple ( coughing)
 - complex (words, sentences)
GILLES De La TOURETTE SYNDROME
 - simple brief jerks to complex pattern of rapid coordinated movements or
vocalizations
 - onset in childhood
 - begin in the face and neck (97%)
 - relapses and remissions are common
 - association with compulsive and hyperactive behavior
 - prevalence 50 : 100 000 ( boys)
 - hereditary possibility
Treatment
 - Psychotherapy
 - Severe tics : neuroleptics
 benzodiazepines
 injection of BTX
 - Severe OCD: SSRI
MYOCLONUS
 Brief muscle jerks
 Focal
 Segmental
 Generalized
 Spontaneous
 Reflex
CLASSIFICATION
 1. CORTICAL
 - arising from the cerebral cortex
 - epileptic or nonepileptic
 2. SUBCORTICAL
 - arising from the brainstem
 - only nonepileptic
 3. SPINAL
 - arising from spinal cord
 - only nonepileptic
According to EEG + EMG results we can
differentiate between epileptic or
nonepileptic M.
 A. EPILEPTIC – progressive myoclonic epilepsy
 B. NONEPILEPTIC
 1. Physiological ( singultus)
 2. Posthypoxic (KPR)
 3. Metabolic (hepatal or uremic encephalopathy)
 4. Infectional ( JCD)
 5. Drug induced (opiats, SSRI, Lithium, Gabapentin,anticholinergics)
TREATMENT
 AED – valproic acid
 Benzodiazepines - clonazepam