1. Control of movement cerebral cortex thalamus basal ganglia cerebellum
brain stem
muscles:
movement
spinal cord
receptors

2. Neuromuscular transmission
Cl- channel
dihydropyridine rec. ryanodine rec.

3. Diseases of neuromuscular transmission
Autoimmune myasthenia gravis
acquired, antibodies against  subunit of N receptor
(immunogenic region)
Congenital forms of myasthenia gravis
heterogeneous origin:
deficit of Ach-esterase
slow-channel syndrome (ion channel of N receptor)
Lambert-Eaton syndrome
antibodies against Ca 2+ channel

4. Diseases of muscle excitability
Myotonia
myotonic muscular dystrophy, myotonia congenita, paramyotonia congenita
myotonia congenita: Cl- channel
Periodic paralyses
normo- hyper- hypokalemia
hyperkalemic: Na+ channels (noninactivating)
Malignant hyperthermia
ryanodine receptor

5. Myopathies Muscular dystrophies:
Duchenne, Becker: X linked recessive, Xp21, dystrophin
Limb girdle dystrophies: autosomal, sarcoglycans
Congenital muscular dystrophy: laminin (merosin)
Myopathies associated with
inborn errors of metabolism
(lipid, glykogen, oxidative
phosphorylation)
Acquired myopathies:
inflammatory
endocrine
toxic

6. Lower motor neuron alpha-motor neurons various etiology
neuronopathies (soma)
radiculopathies (spinal root)
neuropathies (nerve)
paresis-plegia
hypotonia of affected muscles
hyporeflexia of muscle stretch reflexes
atrophy of affected muscles
fasciculations, fibrillation potentials (EMG)

7. Upper motor neuron descending motor tracts cerebral cortex-spinal cord
various etiology
paresis-plegia
spasticity of affected muscles
hyperreflexia of muscle stretch reflexes
positive Babinski sign response
no atrophy

8. The Basal ganglia nc. caudatus putamen globus pallidus: ext., int.
substantia nigra:
pars compacta, reticulata
nc. subthalamicus
thalamus r. c. e. i.

9. g.pallidus int. / s. nigra ret.
Internal loops
cerebral cortex
Glu
GABA/Enk
Dopamin D1
g. pallidus ext.
striatum (Ach) D2
GABA
GABA/P
s. nigra comp.
Glu
Glu
nc. subtalamicus
g.pallidus int. / s. nigra ret.
GABA
Indirect:
decrease of cortical
excitability
Nigro-striatal:
decrease
Direct:
increase of cortical excitability
Nigro-striatal:
increase
thalamus
Glu
cerebral cortex

10. Hypokinetic disorders
akinesia (initiation of movements)
bradykinesia (slowness of movements)
rigidity
flexed posture
resting tremor (acral parts)
vegetative signs (increase of salivation, sweating)
Loss of influence of the nigro-striatal pathway:
overactivity in the indirect pathway (inhibitory)
relative increase of ACh over DA in the striatum
Parkinson´s disease: idiopathic neurodegenerative disease
Parkinsonlike states: vascular, viral, anoxia

11. Hyperkinetic disorders
dyskinesias: athetosis, chorea, ballism, dystonia
hypotonia
Huntington´s chorea
autosomal dominant (trinucleotid disease)
chromosome 4, huntingtin
CAG (glutamine), normal < 40, affected > 40
cholinergic and GABAergic cells : striatum
cerebral cortex (dementia)

12. Cerebellar disorders ataxia: stance, gait, balance
asynergia: coordination during multi-joint movements
dysdiadochokinezia: alternating movements
dysmetria: reaching of targets
action tremor
hypotonia
central part:
(axial and proximal limb muscles)
stance, gait, balance, asynergia
lateral part:
(distal limb muscles)
dysmetria, dysdiadochokinezia, action tremor, hypotonia

13. Spinal ataxia Vestibular ataxia Apraxia dorsal columns pathways
tactile and proprioceptive sensation
depends on the eye control
loss of conscious perception of position
Vestibular ataxia
vestibular system
depends on the head position
Apraxia
learned voluntary movement
motor associative areas
prefrontal, posterior parietal