The Polyneuropathies Classification

CHRONIC cause

Develop over months, years

Malignant disease —

Carcinoma Lymphoma

Paraproteinemias -

e.g. Monoclonal gammopathies (lgG, IgA, lgM) Connective tissue —

disorders Rheumatoid arthritis

Systemic lupus erythematosis

Polyarteritis nodosa Sclerodema

Amyloid disease-

Primary, familial or secondary

Metabolic disorders

Uraemia Hypothyrodism

Diabetes Chronic functional disturbance

Sensory or sensory/motor disturbance

May predate recognition of malignancy by some years Sensory/motor disturbance

- In rheumatoid arthritis multiple mononeuropathy is common

Motor/sensory disturbance is rare

Systemic lupus erythematosis - mild motor/sensory disturbance

Polyarteritis nodosa usually produces multiple mononeuropathy Motor, sensory disturbance with autonomic involvement Also may develop 'entrapment' neuropathies

- Uraemic neuropathy is sensory/motor in type. Hypothyroidism produces mild sensory/motor disturbance.

Diabetic neuropathy takes many forms

Sensory, motor disturbance

PATHOLOGY

Axonal degeneration

Axonal or demyelinative degeneration

Occlusion of nutrient blood vessels to nerves (vasa nervorum)

Thickened nerves with amyloid deposition as well as small fibre axonal degeneration Axonal degeneration

Dcmvelination inflammatory demyelinating polyneuropathy (CIDP)

Hereditary neuropathies

Refsum's disease A phytanic acid storage disorder. Onset in first decade and slowly progressive. A severe sensorimotor neuropathy with associated cerebellar ataxia, ichthyosis, pigmentory retinal degeneration, deafness and cardiac abnormalities. Elevated serum phytanate

Hereditary motor and See page 428 sensory neuropathy (HMSN)

Schwann cell hyperplasia -hypertrophic neuropathy

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