Hereditary Neuropathy with liability to Pressure Palsies

Also known as

  • HNPP
  • familial tomaculous neuropathy
  • Hereditary Motor and Sensory Neuropathy

Hereditary Neuropathy with liability to Pressure Palsies (HNPP) is an often painful nerve disorder that affects the peripheral nerves. The main effect of HNPP are pressure palsies.

Condition details

Hereditary neuropathy with liability to pressure palsies causes recurrent episodes of numbness, tingling, and/or loss of muscle function (palsy). An episode can last from several minutes to several months, but recovery is usually complete. Repeated incidents, however, can cause permanent muscle weakness or loss of sensation. This disorder is also associated with pain in the limbs, especially the hands.

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Photos of affected individuals

Click images to enlarge.

  • No baby imageBaby
  • No toddler imageToddler
  • No child 4-10 imageChild 4-10
  • No child 10-18 imageChild 10-18
  • No adult imageAdult

Typical age range of first manifestation

  • Does not manifest in Baby
  • Does not manifest in Toddler
  • Does not manifest in Child 4-10
  • Can manifest in Child 10-18
  • Does not manifest in Adult

Symptoms

Child 10-18
Lay terms Clinical terms
  • Numbness Unusual sensation - tingling
  • heat
  • cold
  • Pain can become intense enough to require use of opioid (narcotic) drugs Motor symptoms include loss of function. Symptoms of weakness
  • tiredness
  • heaviness
  • and gait abnormalities; and gain of function (positive) symptoms of cramps
  • tremor
  • and muscle twitch. There is also pain in the muscles cramps and there may also be autonomic dysfunction.pins and needles etc.
  • Anesthesia Paraesthesia Chronic pain Extreme fatigue. Fasciculation Myalgia
Adult
Lay terms Clinical terms
  • Numbness Unusual sensation - tingling
  • heat
  • cold
  • pins and needles etc. Inflammation of ligaments. Gain of function (positive) symptoms include tingling
  • pain
  • itching
  • crawling
  • and pins and needles. Motor symptoms include loss of function (negative) symptoms of weakness
  • tiredness
  • heaviness
  • and gait abnormalities; and gain of function (positive) symptoms of cramps
  • tremor
  • and muscle twitch. There is also pain in the muscles
  • cramps
  • etc.
  • Nervous systems malfuctions
  • Anesthesia Paraesthesia Inflammation fasciculations myalgia Autonomic dysfunctio.

Affected genes identified to date

  • PMP-22

Inheritance patterns

Is Autosomal dominant

Are carriers affected?

How many are affected?

2-5 per 100,00

Support groups and organisations

Tel
Facebook

Known experts

No experts found

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