Pain with Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) is a nerve disorder that affects the peripheral nerves. The main effect of HNPP are pressure palsies. Pressure on the nerves can cause tingling sensations, numbness, pain, weakness, muscle atrophy, and even paralyzation of affected area. In normal individuals these symptoms disappear quickly but in sufferers of HNPP even a short period of pressure can cause the symptoms to occur. These usually subside over an extended period of time. Palsies can last from minutes, day to weeks or even months or years.However repeated incidents can cause permanent muscle weakness. HNPP evolves slowly with symptoms surfacing usually in the late teens to early twenties (range 8 to 64 years).
The symptoms vary from person to person. Some report minor problems, whilst others experience severe discomfort. In many cases the symptoms are mild enough to go unnoticed. The time period between episodes is known to vary between individuals. HNPP has not been found to alter the lifespan, although in some cases a decline in quality of life is noticed.
- Back Pain
- Pain at the sites of entrapment
- Leg/ankle foot swelling
- Muscle cramps
- Paresthesias (abnormal sensations)
- Muscle weakness
Some sufferers (10-15%) report various pains growing in severity with progression of the disease.
The nerves most commonly affected are the peroneal nerve at the fibular head (leg and feet), the ulnar nerve at the elbow (arm) and the median nerve at the wrist (palm, thumbs and fingers).
HNPP is part of the group of Hereditary Motor and Sensory Neuropathy (HMSN) and is linked to Charcot Marie Tooth disease (CMT).
- Gilbert, Brigitte (May 2004). “Hereditary Neuropathy with Liability to Pressure Palsies” (pdf). p.
- “Hereditary Neuropathy with Liability to Pressure Palsies”. April 2007.
Currently there are no effective treatments but it is a possible candidate for gene therapy. Currently simple adjustments to the individuals lifestyle are the best treatment in order to avoid excess pressure on the nerves. Avoiding repetitive injury of a nerve is important, as this makes the symptoms worse.