Chronic inflammatory demyelinating polyneuropathy

Background

Prevalence of 1 to 2 per 100,000 adults
Acute variant is Guillain-Barre syndrome
- GBS is self limited whereas CIPD is progressive for more than two months

Symmetrical proximal and distal muscle weakness that is progressive for more than two months
Impaired sensation
Areflexia
Elevated CSF protein
EMG studies consistent with demyelination
Nerve demyelination on nerve biopsies

Can be relapsing or chronic and progressive

Neuromuscular weakness
- Upper motor neuron:
  - CVA
  - Hemorrhagic stroke
  - Multiple sclerosis
  - Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
- Lower motor neuron:
  - Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
  - Infection (Epidural abscess)
  - Infarction/ischemia
  - Trauma (Spinal Cord Syndromes)
  - Inflammation (Transverse Myelitis)
    - Acute flaccid myelitis
  - Degenerative (Spinal muscular atrophy)
  - Tumor
- Peripheral nerve disease:
  - Guillain-Barre syndrome
  - Toxins (Ciguatera)
  - Tick paralysis
  - Diabetic peripheral neuropathy
- Neuromuscular junction disease:
- Muscle disease:
  - Rhabdomyolysis
  - Dermatomyositis
  - Polymyositis
  - Alcoholic myopathy
Non-neuromuscular weakness
- Can't miss diagnoses:
  - ACS
  - Arrhythmia/Syncope
  - Severe infection/Sepsis
  - Hypoglycemia
  - Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
    - Hypokalemic periodic paralysis
    - Thyrotoxic periodic paralysis
  - Respiratory failure
- Emergent Diagnoses:
  - Symptomatic Anemia
  - Severe dehydration
  - Hypothyroidism
  - Polypharmacy
  - Malignancy
  - Aortic disease - occlusion, stenosis, dissection
- Other causes of weakness and paralysis
  - Acute intermittent porphyria (ascending weakness)