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Note: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a treatable form of Guillain-Barré Syndrome (GBS) that responds to steroids.
About
- Definition: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated neuropathy characterized by progressive or relapsing weakness and sensory dysfunction.
- Onset: Typically subacute, developing over weeks, with progressive weakness and areflexia (loss of reflexes).
Aetiology
- Immune-mediated demyelination: The immune system attacks the myelin sheath of peripheral nerves, leading to demyelination and impaired nerve function.
Associations
- Infections: HIV, HBV, HCV.
- Hematologic: Monoclonal Gammopathy associated CIDP.
- Autoimmune conditions: Multiple Sclerosis (MS), Systemic Lupus Erythematosus (SLE), Diabetes.
- Other associations: Inflammatory Bowel Disease, Pregnancy.
Clinical Presentation
- Progressive weakness and sensory loss: Symptoms typically start with tingling in hands and feet and progress to difficulty rising from a chair.
- Weakness pattern: Symmetrical distal and proximal weakness, often with postural tremor.
- Cranial nerve involvement: Rare, but may include facial weakness or third cranial nerve palsy.
- Sensory loss: Distal sensory loss may be observed.
- Areflexia: Tendon reflexes are often lost, consistent with lower motor neuron (LMN) signs.
Helpful Pointers
- Upper limb onset: Symptoms may start in the arms with associated postural tremor.
- Weakness out of proportion to wasting: Notable strength loss without significant muscle wasting.
- Thickened nerves: Enlarged peripheral nerves may be palpable.
- Generalized weakness: Widespread muscle weakness is common.
Investigations
- Blood tests: Full blood count (FBC), Urea & Electrolytes (U&E), Vitamin B12, Antinuclear Antibodies (ANA), HCV, and HIV testing.
- Plasma protein electrophoresis: To detect monoclonal proteins associated with CIDP.
- CSF analysis: Elevated protein (>1g/L) with normal white cell count (WCC < 10) and glucose levels. Oligoclonal bands may be present.
- Nerve conduction studies: Show demyelination with slowed conduction and conduction block, often starting proximally.
- Nerve biopsy: May be performed in selected cases to confirm diagnosis.
Differential Diagnosis
- Acute Inflammatory Demyelinating Polyneuropathy (AIDP): The classic form of Guillain-Barré Syndrome (GBS).
- Critical illness polyneuropathy: Weakness seen in patients in intensive care unit (ICU) settings.
- Drug-induced neuropathy: For example, secondary to Amiodarone use.
- Paraproteinemia-related neuropathy: Such as in myeloma or Monoclonal Gammopathy of Undetermined Significance (MGUS).
Management
- First-line treatment: High-dose steroids (e.g., Prednisolone 60 mg once daily for 4-6 weeks), with possible addition of a steroid-sparing agent (e.g., Azathioprine or Methotrexate).
- Immunoglobulin therapy: High-dose intravenous immunoglobulins (IVIG) at 0.4g/kg/day for 5 days.
- Plasma exchange: Considered if there is no improvement with steroids or IVIG.
- Paraprotein-associated CIDP: If CIDP is associated with a paraprotein, workup for myeloma should be performed. Steroid and plasma exchange response is typically poor, but Rituximab may be effective. Collaboration with hematologists is advised.
- Rehabilitation: Ongoing physical and occupational therapy is essential for recovery over weeks to months.