The Polyneuropathies Specific Types

ACUTE INFLAMMATORY POSTINFECTIOUS POLYNEUROPATHY (contd)

Diagnosis is based on clinical history supported by CSF and neurophysiological investigation and exclusion of acute spinal cord disease, porphyria and myasthenia gravis.

Treatment

Treatment is mainly supportive, with management of the paralysed patient and with elective ventilation for impending respiratory' failure (PaC02 >6.5 kPa and Pa02 <8kPa on oxygen).

The effectiveness of specific immunosuppressive therapy - steroids and cytotoxic drugs - is disappointing. Reports of success with plasmapheresis have led to multi-centre prospective studies. The results are encouraging, showing significant improvement in the course of the illness. A recent study comparing plasmapheresis with intravenous immunoglobulins (1VIG) - 0.4g/kg for 5 days has shown equal efficacy. These treatments should be reserved for patients who cannot walk.

Outcome

Of those progressing to respiratory failure, 20% are left severely disabled and 10% moderately disabled. In milder cases the outcome is excellent. Recurrence - 3%

Variants of Guillain-Barre Pure sensory autonomic and regional (eg polyneuritis cranialis and brachial neuritis) variants occur rarely as does the Miller Fisher syndrome consisting of ophthalmoplegia, areflexia and ataxia without significant limb weakness. Serum lgG antibodies to a specific ganglioside are characteristic.

CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY (C.I.D.P.)

Similar to Guillain Barre but with a progressive or fluctuating course over weeks or months and rarely involving cranial nerves or respiratory function.

Pathology: Segmental demyelination with remyelination (onion bulb formation) and sparse mononuclear inflammatory change.

Prevalence - 3% of all neuropathies

Incidence - 5 per million

Age of onset: mean 35 yrs (fluctuating course - younger, progressive - older) Diagnosis: Electrophysiology - conduction velocity < 70% of normal

- conduction block (outwith entrapment sites)

- prolonged distal latencies

Distinguish from - hereditary neuropathy (HSMN type 1 page 428)

- paraprotein and lymphoma associated neuropathy (page 427)

- multifocal motor neuropathy with conduction block (page 427)

- HIV neuropathy (page 496)

Treatment

MILD

MODERATE —

SEVERE

REFRACTORY

nil steroids steroids/azathioprine + plasmapheresis + cyclophosphamide or or ivig + cyclosporin a

Outcome with treatment - 30% symptom free - 45% mild disability - 25% severe disability

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