Transcript Chronic inflammatory demyelinating polyneuropathy (CIDP)

Chronic inflammatory demyelinating
polyneuropathy (CIDP)
This polyneuropathy develops over weeks or months,
usually with a persistent but relapsing and remitting
course. CSF protein is raised and, usually, segmental
demyelination is seen in peripheral nerves. CIDP
responds to steroids (long term, low dose) and to i.v.
immunoglobulin (for exacerbations).
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Diphtheritic neuropathy Palatal weakness
followed by pupillary paralysis and a
sensorimotor neuropathy occur several weeks
after the throat infection.
Idiopathic chronic sensorimotor neuropathy
The patient complains of progressive symmetrical
numbness and tingling in hands and feet,
spreading proximally in glove and stocking
distribution. There is distal weakness which also
ascends. Rarely cranial nerves are affected.
Tendon reflexes become absent. Symptoms may
progress over many months, remain static or
occasionally remit. Autonomic features are
sometimes seen.
Toxic, metabolic and vitamindeficiency neuropathies
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Metabolic neuropathies Diabetes mellitus
Several varieties of neuropathy occur in
diabetes:
symmetrical sensory polyneuropathy
acute painful neuropathy
mononeuropathy and multiple
mononeuropathy:
• cranial nerve lesions
• isolated peripheral nerve lesions (e.g. median)
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diabetic amyotrophy
autonomic neuropathy.
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Uraemia
Progressive sensorimotor neuropathy
develops in chronic uraemia. The response
to dialysis is variable but the neuropathy
usually improves after renal
transplantation.
Thyroid disease A mild chronic
sensorimotor neuropathy is sometimes
seen in both hyperthyroidism and
hypothyroidism Myopathy also occurs in
hyperthyroidism
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Refsum's disease This is a rare
condition inherited as an autosomal
recessive. There is a sensorimotor
polyneuropathy with ataxia, retinal
damage and deafness. It is due to
defective phytanic acid metabolism
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Alcohol
Polyneuropathy, mainly in the lower limbs, occurs with
chronic excess alcohol. Calf pain is common. Thiamine is
the treatment, but the response is variable, even with
complete abstention. Recurrence (and progression) occurs
with even small amounts of alcohol.
Drugs and industrial toxins Many drugs) and a wide variety
of industrial toxins cause polyneuropathy.
Toxins include:
lead poisoning - motor neuropathy
acrylamide (plastics industry), trichlorethylene, hexane and
other fat-soluble hydrocarbons (e.g. glue-sniffing,) progressive sensorimotor polyneuropathy
arsenic and thallium - polyneuropathy, initially sensory
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Phenytoin M
Chloramphenicol S, M
Metronidazole S, S/M
IsoniazidS, S/M
Dapsone M
Anti-retroviral drugs S > M
NitrofurantoinS/M
VincristineS > M
PaclitaxelS > M
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Vitamin-deficiency neuropathies
Vitamin deficiencies cause nervous
system diseases that are largely
preventable and potentially
reversible if treated early - and
inexorably progressive if not.
Deficiencies (often of multiple
vitamins) occur in malnutrition
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Vitamin B12 (cobalamin) Deficiency causes damage to the spinal
cord, peripheral nerves and brain. Subacute combined
degeneration of the cord (SACD). Combined spinal cord and
peripheral nerve damage is a sequel of Addisonian pernicious
anaemia and rarely other causes of vitamin B12 deficiency
The patient complains initially of numbness and
tingling of fingers and toes. There is combined distal
sensory loss, particularly posterior column, absent
ankle jerks (neuropathy), with cord disease exaggerated knee jerks, extensor plantar responses.
Optic atrophy and retinal haemorrhage may occur. In
later stages sphincter disturbance, severe
generalized weakness and dementia develop.
Exceptionally, dementia develops in the early stages.
Without treatment, SACD is fatal within 5 years.
Macrocytosis with megaloblastic bone marrow are usual
though n
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Charcot-Marie-Tooth disease) Charcot-MarieTooth (CMT) disease is also called peroneal
muscular atrophy. CMT describes the common
clinical phenotype - distal limb wasting and
weakness that slowly progresses over many
years, mostly in the legs, with variable loss of
sensation and reflexes. In advanced disease,
distal wasting is so marked that the legs are said
to resemble inverted champagne bottles. Mild
cases have pes cavus and clawing of the toes
that can pass unnoticed. Multiple genetic variants
of the CMT phenotype are now recognized,
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Neuropathy in cancer Polyneuropathy is
seen as a non-metastatic manifestation of
malignancy, sometimes with anti-Hu
antibodies. Polyneuropathy occurs in
myeloma and other dysproteinaemic
states, probably owing to impaired
perfusion of nerve trunks or to
demyelination associated with allergic
reactions affecting peripheral nerves.
Individual nerves are sometimes infiltrated
with malignant cells
Autonomic neuropathy
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Autonomic neuropathy causes postural
hypotension, urinary retention, impotence,
diarrhoea (or occasionally constipation),
diminished sweating, impaired pupillary
responses and cardiac arrhythmias. This develops
in diabetes mellitus, in amyloidosis and may
complicate Guillain-Barré syndrome. Many
varieties of neuropathy cause autonomic failure
in a mild, and often subclinical, form.
Occasionally, when there is damage to small
myelinated and non-myelinated B and C fibres,
clinical features of the autonomic neuropathy
predominate, e.g. in diabetes.
PLEXUS AND NERVE ROOT
LESIONS
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THORACIC OUTLET SYNDROME
A fibrous band or cervical rib extending from the
tip of the C7 transverse process to the first rib
stretches across the lower brachial plexus (roots
C8 and T1). There is forearm pain (ulnar border),
T1 sensory loss and thenar muscle wasting,
principally abductor pollicis brevis. Horner's
syndrome may develop. The rib or band can be
excised. In some patients the rib or band causes
subclavian artery or venous occlusion.
Neurological and vascular problems rarely occur
together. Thoracic outlet syndrome is also used to
describe ill-defined upper limb symptoms usually on poor evidence.
Malignant infiltration
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Metastatic disease of nerve roots of the
brachial or lumbosacral plexus causes a
painful radiculopathy. A common example
is an apical bronchial neoplasm
(Pancoast's tumour) causing a T1 and
sympathetic outflow lesion. There is
wasting of small hand muscles, pain, T1
sensory loss with ipsilateral Horner's
syndrome. This also occurs in apical TB.