Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Guillain-Barré syndrom

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

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.

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

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

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

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

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

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

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

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

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.

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

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

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

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

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

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

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

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

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

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