1. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
Guillain-Barré syndrom

2. AIDP - patogenesis
Autoimune disease resulting from aberrant imunne responses against various components of periferal nerve fibers

3. AIDP - patogenesis Specific mechanisms of injury are unclear
Inflammatory lesions consists from lymfocytes, macrophages and local demyelinisation
The roots, plexuses, nerves, autonomic fibers are involved, with a predilection of roots and distal fibers of periferal nerves sometimes – axonal lesion.

4. AIDP - epidemiology Rare disease 1- 2/ 100 000
Etiology - the most often – respiratory disease precede beginning of the disease (1-3, rarely more than 6 weeks)
Viral, bacterial cause
Campylobacter jejuni - gastroenteritis

5. AIDP – clinical feature
Weakness, paresthesias, diminished or absent reflexes
Spreading of paresthesia to proximal parts of extremities
Perioral paresthesia – rare
Onset at lower extremities.

6. AIDP – clinical feature
Deep and proprioceptive sensitivity are afftected most often
Progressive phase of disease lasts from 3 to 4 weeks
Problems with breathing

7. AIDP – clinical feature Miller - Fisher syndrom
Oftalmoplegia
Areflexia
Ataxia
Relative benign disease
EMG – axonal lesion, less demyelination
CSL – increased proteins

8. AI DP - diagnostic Clinical feature CSF – increased proteins
(albumino- cytologic disociation)
Sometimes – Leu or mononuclears
Rare – normal CSL, 10% - negative
EMG – decreased velocity

9. AI DP - therapy Plazmapheresis to be effective – in the 1st week IVIg
Arteficial ventilation
Physiotherapy

10. AIDP - prognosis 75 % - without deficit – recovery from 6 to 12 month
7 – 15 % - mild residual deficit
Few % - unmovebale
5 % - death

11. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

12. CIDP Roots and proximal nerves are affected
Onset and relapse of disease can be provoke by stimuli – e.g. infection
Good response to corticoids, immunosupresants, plasmapheresis, IVIg
The age of onset from 18 year to 8 decade.

13. CIDP – clinical feature
Evolves slowly - 8 weeks to the top of disease
Weakness
Periferal neuropathy mainly of LE
Sensory signs – paresthesia, numbness,
Pain (20%), - socks and gloves distribution
Decreased tendom reflexes
Cranial nerves

14. CIDP – dif. dg. Chronic senzorimotoric neuropathy
(in diabetes, uremia, hypotyreoidizm, alcohol, makroglobulinemia)
AIDP

15. CIDP - diagnostics Clinical feature CSF – increased proteins
Nerve biopsy – inflammatory, demyelinating changes
EMG – decreased velocity

16. CIDP – therapy Corticoids
Immunosupresant – azathioprin, cyklophosfamid
Plazmapheresis
IVIg – 400 mg/kg/day 4-6 times
Physiotherapy

17. Amyotrofic lateral sclerosis - ALS
Atrophy of muscles + lost of motoneurons in anterior horns
Spasticity with pyramidal signs
Onset 40 – 65
More often – men

18. ALS Sporadic Sometimes - genetic linkage (20) Etiology – unknown –
Higher doses of glutamate, ...

19. ALS – clinical feature
Weakness mainly in distal parts, but also in proximal
Atrophy of muscles of hand
Fasciculations
Spasticity, pyramidal signs

20. ALS – clinical feature Sensitivity intact
Affected intercostal muscles – problems with breathing
Bulbar signs – dysphonia, dysphagia, Increased reflexes

21. ALS – diagnostics CSL – normal EMG – fasciculation, fibrillation,
Dif. dg. – patological process in cervical enlargment

22. ALS – therapy, prognosis
Therapy – unknown
Riluzol – aminoacids
Prognosis – bad