Neuropathies

Neuropathies

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

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	Created by LewisLewis almost 11 years ago