Giant nerves in chronic inflammatory polyradiculoneuropathy

Area of Science:

• Neurology
• Neurophysiology

Background:

• Nerve enlargement (NE) is typically multifocal in inflammatory neuropathies and generalized in inherited neuropathies.
• Massive NE is rarely reported in inflammatory conditions.

Purpose of the Study:

• To describe four cases of massive NE in inflammatory neuropathies.
• To highlight the diagnostic challenges and successful treatment of these cases.

Main Methods:

• Patients presented with progressive polyneuropathy.
• Diagnostic tools included nerve conduction studies (NCS), nerve MRI, nerve ultrasound, biopsies, PET-CT, and genetic testing.

Main Results:

• All patients exhibited demyelinating neuropathy with multifocal conduction blocks.
• Extensive NE was observed in nerve roots and nerves via MRI and ultrasound.
• Chronic inflammatory demyelinating polyneuropathy variants were diagnosed, and immunotherapy was initiated successfully.

Conclusions:

• Massive NE can occur in immune-mediated neuropathies, mimicking inherited neuropathies.
• Nerve imaging (MRI and ultrasound) is vital for diagnosis and guiding therapy.
• Differentiating inflammatory NE from inherited NE is critical for appropriate management.