Makindo Medical Notes.com

Related Subjects: |Neurological History taking |Motor Neuron Disease (MND-ALS) |Miller-Fisher syndrome |Guillain Barre Syndrome |Multifocal Motor Neuropathy with Conduction block | Multiple Sclerosis (MS) Demyelination | Transverse myelitis | Acute Disseminated Encephalomyelitis | Progressive Multifocal Leukoencephalopathy (PML) | Inclusion Body Myositis |Cervical spondylosis |Anterior Spinal Cord syndrome |Central Spinal Cord syndrome |Brown-Sequard Spinal Cord syndrome |Spinal Cord Compression |Spinal Cord Haematoma |Spinal Cord Infarction

Chronic but treatable form of GBS responsive to steroids

About

• Subacute onset over weeks of weakness with areflexia

Aetiology

• Immune mediated demyelination

Associations

• HIV, HBV, HCV
• Monoclonal Gammopathy associated CIDP
• MS, SLE, Diabetes
• Inflammatory bowel disease
• Pregnancy

Clinical

• Weakness and sensory loss comes on days/weakness
• Tingling in hands and feet and then difficulty rising from a chair
• Symmetrical distal and proximal weakness, postural tremor
• Cranial nerves rarely affected - facial weakness, IIIrd nerve
• Distal Sensory loss may be present
• Areflexia -Tendon reflexes are lost as in keeping with the LMN signs

Helpful pointers

• Upper limb onset and postural tremor
• Weakness out of proportion to wasting
• Thickened nerves
• Generalised weakness

Investigations

• FBC, U&E , B12, ANA, HCV, HIV
• Plasma protein electrophoresis
• CSF protein > 1g but the WCC < 10 and Glucose levels remain normal. Oligoclonal bands may be found.
• Nerve conduction studies show demyelination which starts proximally - there is slowed conduction and conduction block
• Nerve biopsy may be done

Differential

• AIDP (GBS)
• Critical illness polyneuropathy - weakness seen in patients in ITU settings
• Drug induced e.g. Amiodarone
• Paraproteinaemia/Myeloma/MGUS

Management

• Standard treatment is with high dose steroids e.g. Prednisolone 60 mg od for 4-6 weeks and possibly a steroid-sparing agent such as azathioprine or Methotrexate
• Alternatively High dose IV immunoglobulins 0.4g/kg/day for 5 days
• If there is no improvement then Plasma exchange may be considered and further immunosuppressive agents
• In those where CIDP is associated with a paraprotein work up for myeloma should be undertaken and response to steroids and PE is poor but Rituximab may be useful. Liaise with haematologists.
• Patients will often need ongoing rehabilitation over weeks and months