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 featureMiller - 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