Zusammenfassung der Ressource
Neuropathies
- MILLER-FISHER SYNDROME
- Very rare condition considered variant of GBS
- Typical presentation is ophthalmoplegia/ophthalmoparesis
- Sensory neuropathy (especially with sensory ataxia)
- It seems to be due to anti-GQ1b antibodies
- Absent tendon reflexes and CSF dissociation
- PERIPHERAL NEUROPATHY
associated to anti-MAG antibodies
- Autoimmune condition, antibodies against
MAG protein (Myelin associated glycoprotein)
- Mostly sensory, mild motor involvement
- Electrophoresis would show a monoclonal band
of IgM proteins that represent the autoantibodies
- Presence of onion bulbs (attempt of new
Schwann cells to remyelinate the nerve fiber)
- Increase in the volume of the nerve
- Destruction of bone forming the foramina of the spinal cord
- PERIPHERAL NEUROPATHY
associated to anti-GM1 antibodies
- It casues a multifocal, pure motor, conduction block neuropathy
- In the acute setting there is a monoparesis
with NO problems involving the sensory fibers
- It mainly affects the upper limbs
- CIDP (Chronic inflammatory
demyelinating polyneuropathy)
- It can start off as GBS, but then evolves as a chronic condition
- Inflammatory autoimmune demyelinating condition
- Nerve edema
- Infiltration of lymphocytes
- Macrophage-mediated demyelination
- Development of onion-bulb formation
- On the long run there is also axonal degeneration
- It affects both spinal roots and peripheral nerves
- Mixed sensory neuropathy
- Therapy
- Generally performed with immunosuppressive
or immunomodulatory drugs
- Initially performed with steroids
- High doses generally give good control of the disease
- DISEASES associated with
PARAPROTEINEMIA and NEUROPATHIES
- MGUS
- Multiple Myeloma
- Waldenstrom macroglobulinemia
- POEMS syndrome
- Primary amyloidosis
- Peripheral neuropathy is a common
manifestation, and could be the first
- Mainly small fibers
- Abnormal pain and temperature sensation
- Also small unmyelinated fibers
of the autonomic system
- It cause sudomotor system impairment
- Cryoglobulinemia
- Lymphoma/leukemia
- Treatment has a poor effect
- Immunosuppression
- Iv-Ig infusion
- Corticosteroids
- VASCULITIC NEUROPATHIES
- Generally in the context of systemic vasculitis
- Sub-perienural edema and infiltration of
inflammatory cells in the perivascular area
- Disruption of vessels, thrombosis and
ischemic damage of the nerve
- Initial monofocal localization
progressing into a multifocal damage
- Diagnosis only made by biopsy
- NEUROPATHIES in CANCER PATIENTS
- Paraneoplastic neuropathies are
tipically sensory neuropathies
- The appearance of the sensory neuropathy
can precede the detection of cancer
- Due to many causes
- Chemotherapy
- Radiation therapy
- Nutritional aspects
- Neoplastic invasion of nerves at the periphery
- DIABETIC NEUROPATHY
- Symmetric
- Generally of mixed type (sensory-motor)
- It can involve autonomic fibers
- Acute painful neuropathy that affects distally the legs and arms
- Asymmetric
- Diabetic amyotrophy
- Mainly the lower limb girdle
- No treatment apart from metabolic control
and symptomatic treatment for pain
- Mononeuropathy, from
- Ischemic conditions
- Increased susceptibility to compression of the peripheral nerves
- Treatment is mainly focused on glycemic control and analgesia
- Multiple mononeuropathy
- HEREDITARY MOTOR AND SENSORY NEUROPATHIES
- Insidious onset and chronic progressive evolution
- Generally initially affect the distal
parts of the limbs, but the lower limbs
- Incidence is 40/100'000
- Generally the onset is in 1st decade
- Most common of these is Charcot-Marie Tooth
- Both motor and sensory nerve involvement
- Distal muscle weakness and atrophy
- Impaired sensation
- Absent or hypoactive deep tendon reflexes
- Progression is very slow
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- Only genetic studies can classify the disease
- Drug-induced neuropathies
- FOCAL NEUROPATHIES
- Entrapment neuropathies
- Inflammatory lesions
- Neoplastic infiltrations
- Traumatic injuries