Chronic inflammatory demyelinating Chronic inflammatory demyelinating

polyneuropathy (CIDP)polyneuropathy (CIDP) This polyneuropathy develops over weeks or months, This polyneuropathy develops over weeks or months, usually with a persistent but relapsing and remitting usually with a persistent but relapsing and remitting course. CSF protein is raised and, usually, segmental course. CSF protein is raised and, usually, segmental

demyelination is seen in peripheral nerves. CIDP demyelination is seen in peripheral nerves. CIDP responds to steroids (long term, low dose) and to i.v. responds to steroids (long term, low dose) and to i.v.

immunoglobulin (for exacerbations). immunoglobulin (for exacerbations).

Diphtheritic neuropathyDiphtheritic neuropathy Palatal weakness Palatal weakness followed by pupillary paralysis and a sensorimotor followed by pupillary paralysis and a sensorimotor neuropathy occur several weeks after the throat neuropathy occur several weeks after the throat infection.infection.

Idiopathic chronic sensorimotor neuropathyIdiopathic chronic sensorimotor neuropathy The patient complains of progressive symmetrical The patient complains of progressive symmetrical numbness and tingling in hands and feet, numbness and tingling in hands and feet, spreading proximally in glove and stocking spreading proximally in glove and stocking distribution. There is distal weakness which also distribution. There is distal weakness which also ascends. Rarely cranial nerves are affected. ascends. Rarely cranial nerves are affected. Tendon reflexes become absent. Symptoms may Tendon reflexes become absent. Symptoms may progress over many months, remain static or progress over many months, remain static or occasionally remit. Autonomic features are occasionally remit. Autonomic features are sometimes seen. sometimes seen.

Toxic, metabolic and vitamin-Toxic, metabolic and vitamin-deficiency neuropathies deficiency neuropathies

Metabolic neuropathies Diabetes mellitus Metabolic neuropathies Diabetes mellitus Several varieties of neuropathy occur in Several varieties of neuropathy occur in diabetes: diabetes:

symmetrical sensory polyneuropathy symmetrical sensory polyneuropathy acute painful neuropathy acute painful neuropathy mononeuropathy and multiple mononeuropathy and multiple

mononeuropathy: mononeuropathy: • cranial nerve lesions cranial nerve lesions • isolated peripheral nerve lesions (e.g. median) isolated peripheral nerve lesions (e.g. median)

diabetic amyotrophy diabetic amyotrophy autonomic neuropathy. autonomic neuropathy.

UraemiaUraemia Progressive sensorimotor neuropathy Progressive sensorimotor neuropathy

develops in chronic uraemia. The response develops in chronic uraemia. The response to dialysis is variable but the neuropathy to dialysis is variable but the neuropathy usually improves after renal usually improves after renal transplantation. transplantation.

Thyroid disease A mild chronic Thyroid disease A mild chronic sensorimotor neuropathy is sometimes sensorimotor neuropathy is sometimes seen in both hyperthyroidism and seen in both hyperthyroidism and hypothyroidism Myopathy also occurs in hypothyroidism Myopathy also occurs in hyperthyroidismhyperthyroidism

Refsum's disease This is a rare Refsum's disease This is a rare condition inherited as an autosomal condition inherited as an autosomal recessive. There is a sensorimotor recessive. There is a sensorimotor polyneuropathy with ataxia, retinal polyneuropathy with ataxia, retinal damage and deafness. It is due to damage and deafness. It is due to defective phytanic acid metabolismdefective phytanic acid metabolism

AlcoholAlcohol Polyneuropathy, mainly in the lower limbs, occurs with Polyneuropathy, mainly in the lower limbs, occurs with

chronic excess alcohol. Calf pain is common. Thiamine is chronic excess alcohol. Calf pain is common. Thiamine is the treatment, but the response is variable, even with the treatment, but the response is variable, even with complete abstention. Recurrence (and progression) occurs complete abstention. Recurrence (and progression) occurs with even small amounts of alcohol.with even small amounts of alcohol.

Drugs and industrial toxins Many drugs) and a wide variety Drugs and industrial toxins Many drugs) and a wide variety of industrial toxins cause polyneuropathy.of industrial toxins cause polyneuropathy.

Toxins include: Toxins include: lead poisoning - motor neuropathy lead poisoning - motor neuropathy acrylamide (plastics industry), trichlorethylene, hexane and acrylamide (plastics industry), trichlorethylene, hexane and

other fat-soluble hydrocarbons (e.g. glue-sniffing,) - other fat-soluble hydrocarbons (e.g. glue-sniffing,) - progressive sensorimotor polyneuropathy progressive sensorimotor polyneuropathy

arsenic and thallium - polyneuropathy, initially sensoryarsenic and thallium - polyneuropathy, initially sensory

Phenytoin MPhenytoin M Chloramphenicol S, MChloramphenicol S, M Metronidazole S, S/MMetronidazole S, S/M IsoniazidS, S/MIsoniazidS, S/M Dapsone MDapsone M Anti-retroviral drugs S > MAnti-retroviral drugs S > M NitrofurantoinS/MNitrofurantoinS/M VincristineS > MVincristineS > M PaclitaxelS > MPaclitaxelS > M

Vitamin-deficiency neuropathies Vitamin-deficiency neuropathies Vitamin deficiencies cause nervous Vitamin deficiencies cause nervous system diseases that are largely system diseases that are largely preventable and potentially preventable and potentially reversible if treated early - and reversible if treated early - and inexorably progressive if not. inexorably progressive if not. Deficiencies (often of multiple Deficiencies (often of multiple vitamins) occur in malnutritionvitamins) occur in malnutrition

Vitamin B12 (cobalamin) Deficiency causes damage to the spinal Vitamin B12 (cobalamin) Deficiency causes damage to the spinal cord, peripheral nerves and brain. Subacute combined cord, peripheral nerves and brain. Subacute combined degeneration of the cord (SACD). Combined spinal cord and degeneration of the cord (SACD). Combined spinal cord and peripheral nerve damage is a sequel of Addisonian pernicious peripheral nerve damage is a sequel of Addisonian pernicious anaemia and rarely other causes of vitamin B12 deficiency anaemia and rarely other causes of vitamin B12 deficiency

The patient complains initially of numbness and The patient complains initially of numbness and tingling of fingers and toes. There is combined distal tingling of fingers and toes. There is combined distal sensory loss, particularly posterior column, absent sensory loss, particularly posterior column, absent ankle jerks (neuropathy), with cord disease - ankle jerks (neuropathy), with cord disease - exaggerated knee jerks, extensor plantar responses. exaggerated knee jerks, extensor plantar responses. Optic atrophy and retinal haemorrhage may occur. In Optic atrophy and retinal haemorrhage may occur. In later stages sphincter disturbance, severe later stages sphincter disturbance, severe generalized weakness and dementia develop. generalized weakness and dementia develop. Exceptionally, dementia develops in the early stages. Exceptionally, dementia develops in the early stages. Without treatmentWithout treatment, SACD is fatal within 5 years. , SACD is fatal within 5 years. Macrocytosis with megaloblastic bone marrow are usual Macrocytosis with megaloblastic bone marrow are usual though nthough n

Charcot-Marie-Tooth diseaseCharcot-Marie-Tooth disease) Charcot-Marie-) Charcot-Marie-Tooth (CMT) disease is also called peroneal Tooth (CMT) disease is also called peroneal muscular atrophy. CMT describes the common muscular atrophy. CMT describes the common clinical phenotype - distal limb wasting and clinical phenotype - distal limb wasting and weakness that slowly progresses over many weakness that slowly progresses over many years, mostly in the legs, with variable loss of years, mostly in the legs, with variable loss of sensation and reflexes. In advanced disease, sensation and reflexes. In advanced disease, distal wasting is so marked that the legs are said distal wasting is so marked that the legs are said to resemble inverted champagne bottles. Mild to resemble inverted champagne bottles. Mild cases have pes cavus and clawing of the toes cases have pes cavus and clawing of the toes that can pass unnoticed. Multiple genetic variants that can pass unnoticed. Multiple genetic variants of the CMT phenotype are now recognized, of the CMT phenotype are now recognized,

Neuropathy in cancer Polyneuropathy is Neuropathy in cancer Polyneuropathy is seen as a non-metastatic manifestation of seen as a non-metastatic manifestation of malignancy, sometimes with anti-Hu malignancy, sometimes with anti-Hu antibodies. Polyneuropathy occurs in antibodies. Polyneuropathy occurs in myeloma and other dysproteinaemic myeloma and other dysproteinaemic states, probably owing to impaired states, probably owing to impaired perfusion of nerve trunks or to perfusion of nerve trunks or to demyelination associated with allergic demyelination associated with allergic reactions affecting peripheral nerves. reactions affecting peripheral nerves. Individual nerves are sometimes infiltrated Individual nerves are sometimes infiltrated with malignant cellswith malignant cells

Autonomic neuropathy Autonomic neuropathy Autonomic neuropathy causes postural Autonomic neuropathy causes postural

hypotension, urinary retention, impotence, hypotension, urinary retention, impotence, diarrhoea (or occasionally constipation), diarrhoea (or occasionally constipation), diminished sweating, impaired pupillary diminished sweating, impaired pupillary responses and cardiac arrhythmias. This develops responses and cardiac arrhythmias. This develops in diabetes mellitus, in amyloidosis and may in diabetes mellitus, in amyloidosis and may complicate Guillain-Barré syndrome. Many complicate Guillain-Barré syndrome. Many varieties of neuropathy cause autonomic failure varieties of neuropathy cause autonomic failure in a mild, and often subclinical, form. in a mild, and often subclinical, form. Occasionally, when there is damage to small Occasionally, when there is damage to small myelinated and non-myelinated B and C fibres, myelinated and non-myelinated B and C fibres, clinical features of the autonomic neuropathy clinical features of the autonomic neuropathy predominate, e.g. in diabetes. predominate, e.g. in diabetes.

PLEXUS AND NERVE ROOT PLEXUS AND NERVE ROOT LESIONS LESIONS

THORACIC OUTLET SYNDROMETHORACIC OUTLET SYNDROME A fibrous band or cervical rib extending from the A fibrous band or cervical rib extending from the

tip of the C7 transverse process to the first rib tip of the C7 transverse process to the first rib stretches across the lower brachial plexus (roots stretches across the lower brachial plexus (roots C8 and T1). There is forearm pain (ulnar border), C8 and T1). There is forearm pain (ulnar border), T1 sensory loss and thenar muscle wasting, T1 sensory loss and thenar muscle wasting, principally abductor pollicis brevis. Horner's principally abductor pollicis brevis. Horner's syndrome may develop. The rib or band can be syndrome may develop. The rib or band can be excised. In some patients the rib or band causes excised. In some patients the rib or band causes subclavian artery or venous occlusion. subclavian artery or venous occlusion. Neurological and vascular problems rarely occur Neurological and vascular problems rarely occur together. Thoracic outlet syndrome is also used to together. Thoracic outlet syndrome is also used to describe ill-defined upper limb symptoms - describe ill-defined upper limb symptoms - usually on poor evidence. usually on poor evidence.

Malignant infiltration Malignant infiltration

Metastatic disease of nerve roots of the Metastatic disease of nerve roots of the brachial or lumbosacral plexus causes a brachial or lumbosacral plexus causes a painful radiculopathy. A common example painful radiculopathy. A common example is an apical bronchial neoplasm is an apical bronchial neoplasm (Pancoast's tumour) causing a T1 and (Pancoast's tumour) causing a T1 and sympathetic outflow lesion. There is sympathetic outflow lesion. There is wasting of small hand muscles, pain, T1 wasting of small hand muscles, pain, T1 sensory loss with ipsilateral Horner's sensory loss with ipsilateral Horner's syndrome. This also occurs in apical TB. syndrome. This also occurs in apical TB.