Acute Inflammatory Demyelinating Polyradiculoneuropathy Differential Diagnoses

Updated: Jul 26, 2021
  • Author: Emad R Noor, MBChB; Chief Editor: Nicholas Lorenzo, MD, CPE, MHCM, FAAPL  more...
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DDx

Diagnostic Considerations

Diagnostic criteria for Guillain–Barré syndrome (GBS) was developed by the National Institute of Neurological Disorders and Stroke (NINDS) and subsequently modified in a review paper. [24, 25, 23]  

Required features for diagnosis

See the list below:

  • Progressive bilateral arms and leg weakness (only legs may initially be involved) 

  • Absent or decreased tendon reflexes in affected limbs (at some point in clinical course)

Supportive features for diagnosis

See the list below:

  • Progressive course (days to 4 weeks) 

  • Relatively symmetric symptoms and signs

  • Fairly mild sensory symptoms and signs 

  • Cranial nerve involvement, especially bilateral facial palsy

  • Autonomic dysfunction

  • Muscular or radicular back or limb pain

  • Increased protein level in cerebrospinal fluid (CSF); normal protein levels do not rule out the diagnosis

  • Electrodiagnostic features of motor or sensorimotor neuropathy (normal electrophysiology in the early stages does not rule out the diagnosis)

Features casting doubt on diagnosis

See the list below:

  • Increased numbers of mononuclear or polymorphonuclear cells in CSF (> 50 cells/µL)

  • Marked, persistent asymmetry of weakness

  • Bladder or bowel dysfunction at onset or persistent during disease course

  • Severe respiratory dysfunction with limited limb weakness at onset

  • Sensory signs with limited weakness at onset

  • Fever at onset

  • Nadir < 24 h

  • Sharp sensory level indicating spinal cord injury

  • Hyper-reflexia or clonus

  • Extensor plantar responses

  • Abdominal pain

  • Slow progression with limited weakness without respiratory involvement

  • Continued progression for > 4 weeks after start of symptoms

  • Alteration of consciousness (except in Bickerstaff brainstem encephalitis)

Features that rule out diagnosis

See the list below:

  • History of hexacarbon abuse

  • Acute intermittent porphyria

  • Recent diphtheritic infection

  • Poliomyelitis, botulism, toxic neuropathy, functional paralysis

Comparison of clinical features of Guillain-Barré syndrome with CIDP

See the list below:

  • Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have a more slowly progressive weakness over months (at least > 2 months) and a protracted course either monophasic or relapsing, and relapses are much more common with CIDP. While a history of viral infection is often obtained with Guillain-Barré syndrome, this is rather uncommon in CIDP. Occurrence of respiratory failure is very uncommon with CIDP. Both conditions are associated with areflexia, typical CSF findings of increased protein, abnormal nerve conduction studies (patchy conduction slowing with Guillain-Barré syndrome and diffuse slowing with CIDP). While prednisone therapy on its own has no proven role in Guillain-Barré syndrome, CIDP patients are sensitive to prednisone therapy.

  • Guillain-Barré syndrome and CIDP have been associated with HIV-1 infection. They are most common in infected patients who are otherwise asymptomatic. In certain cases, Guillain-Barré syndrome may occur with seroconversion. The clinical features of Guillain-Barré syndrome and CIDP in HIV-1 infected patients are similar to patients without HIV-1 infection.

Differential Diagnoses