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Hereditary Neuropathy with Pressure Palsies (HNPP)

Overview

Hereditary Neuropathy with Pressure Palsies (HNPP) is a rare inherited neurological disorder, with an increased tendency for nerve compression at common compression sites like the elbow and fibular head. This can cause episodes of weakness, numbness, or tingling in certain parts of the body, most often after sitting, leaning, or resting on one position for too long.

Symptoms usually appear in teenage years or early adulthood, but they can occur at any age. They may include:

  • Weakness such as wrist drop/ foot drop/ weakness of small muscles of the hand after prolonged posturing
  • Tingling/ numbness/ pain in the affected areas
  • Atrophy of muscles
  • Loss of sensation
  • Weakness may persist for a few weeks or become permanent

HNPP is caused by a mutation in the PMP22 gene, which provides instructions for making a protective layer (myelin) around nerves. The abnormal gene makes nerves more vulnerable to pressure and injury.

Diagnosis is based on symptoms and confirmatory tests:

  • Nerve conduction studies: for site and severity of nerve compression (like elbow, wrist, knee)
  • MR neurography: imaging to look at affected nerves
  • Nerve biopsy (rarely required)
  • Genetic testing: to confirm PMP22 gene mutation

There is no cure, but symptoms can be managed with:

  • Short course of steroids (in select cases, for nerve inflammation)
  • Orthosis and assistive devices to support weak limbs like wrist drop or foot drop
  • Physiotherapy to strengthen muscles and improve mobility
  • Lifestyle modifications such as avoidance of compression at common sites prone to nerve injury

Seek medical advice if you notice:

  • Sudden weakness in the hand, foot, or arm after mild pressure
  • Repeated episodes of numbness or tingling that do not resolve quickly
  • Persistent weakness or muscle wasting

HNPP cannot be prevented since it is genetic, but nerve damage episodes can be reduced by:

  • Avoiding prolonged pressure on elbows, knees, or wrists
  • Using cushioning or padding (e.g., elbow pads, wrist rests)
  • Practicing good posture and ergonomics

For diagnosis and management of HNPP, consult a Neurologist (specialist in brain and nerve disorders).

Disclaimer:

The information in this Health Library is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare professional with any questions about a medical condition or before starting any treatment. Use of this site and its content does not establish a doctor–patient relationship. In case of a medical emergency, call your local emergency number or visit the nearest emergency facility immediately.