Hereditary neuropathy with liability to pressure palsies
Last updated 21 Mar 2023
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Hereditary neuropathy with liability to pressure palsies is a disorder that affects peripheral nerves. These nerves connect the brain and spinal cord to muscles and sensory cells that detect touch, pain, and temperature. In people with this disorder, the peripheral nerves are unusually sensitive to pressure, such as the pressure that occurs when carrying heavy grocery bags, leaning on an elbow, or sitting without changing position, particularly with crossed legs. These activities would not normally cause sensation problems in people without the disorder.
Description
Hereditary neuropathy with liability to pressure palsies is a disorder that affects peripheral nerves. These nerves connect the brain and spinal cord to muscles and sensory cells that detect touch, pain, and temperature. In people with this disorder, the peripheral nerves are unusually sensitive to pressure, such as the pressure that occurs when carrying heavy grocery bags, leaning on an elbow, or sitting without changing position, particularly with crossed legs. These activities would not normally cause sensation problems in people without the disorder.
Hereditary neuropathy with liability to pressure palsies is characterised by recurrent episodes of numbness, tingling, and loss of muscle function (palsy) in the region associated with the affected nerve, usually an arm, hand, leg, or foot. 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.
A pressure palsy episode results from pressure on a single nerve, and any peripheral nerve can be affected. Although episodes often recur, they can affect different nerves. The most common problem sites involve nerves in the wrists, elbows, and knees. The fingers, shoulders, hands, feet, and scalp can also be affected. Many people with this disorder experience carpal tunnel syndrome, which occurs when a nerve in the wrist (the median nerve) is involved. Carpal tunnel syndrome is characterised by numbness, tingling, and weakness in the hand and fingers. An episode in the hand may affect fine motor activities such as writing, opening jars, and fastening buttons. An episode of nerve compression in the knee can lead to a condition called foot drop, which makes walking, climbing stairs, or driving difficult or impossible.
The symptoms of hereditary neuropathy with liability to pressure palsies usually begin during adolescence or early adulthood but may develop anytime from childhood to late adulthood. Symptoms vary in severity; many people never realize they have the disorder, while some people experience prolonged disability. Hereditary neuropathy with liability to pressure palsies does not affect life expectancy.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Compression neuropathy
Entrapment neuropathy
Familial pressure sensitive neuropathy
Hereditary motor and sensory neuropathy
Hereditary pressure sensitive neuropathy
HNPP
Inherited tendency to pressure palsies
Tomaculous neuropathy
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Peripheral neuropathy
Peripheral neuropathy means damage to one or more of your peripheral nerves. There are many different conditions that can lead to peripheral neuropathy. Inflammatory conditions Conditions including sarcoidosis and coeliac disease can also cause peripheral neuropathy. Hereditary diseases Certain diseases that you can inherit from your parents can.
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Foot drop
Foot drop, which is also sometimes called a dropped foot, is a condition in which it is difficult to lift up the front part of the foot. As a result the forefoot and toes tend to catch or drag on the floor when walking. (This is compression of the nerves in the tail of th... Diabetes with peripheral neuropathy. Stroke. Multiple sclerosis (MS)..
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Cerebral palsy
Cerebral palsy is a problem with muscle function. It is caused by brain. What is cerebral palsy. Cerebral palsy is a name for a group of life-long conditions that have problems with muscle function and control. This may be before the baby is born, during the birth or soon after birth. Most people think of cerebral palsy as causing severe problems.
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However about 1 in 10 of all premature babies will have a permanent disability such as lung disease, cerebral palsy, blindness or deafness. One study of 241 children born before 26 weeks of gestation found: 1 in 5 had severe disability (for example, cerebral palsy + not walking, low cognitive scores, blindness, profound deafness). 1 in 4 had.
Further reading and references
- Hereditary neuropathy with liability to pressure palsies; MedlinePlus, National Library of Medicine.
- Bai Y, Zhang X, Katona I, Saporta MA, Shy ME, O'Malley HA, Isom LL, Suter U, Li J. Conduction block in PMP22 deficiency. J Neurosci. 2010 Jan 13;30(2):600-8. doi: 10.1523/JNEUROSCI.4264-09.2010.
- Chance PF. Genetic evaluation of inherited motor/sensory neuropathy. Suppl Clin Neurophysiol. 2004;57:228-42. doi: 10.1016/s1567-424x(09)70360-5.
- Chance PF. Inherited focal, episodic neuropathies: hereditary neuropathy with liability to pressure palsies and hereditary neuralgic amyotrophy. Neuromolecular Med. 2006;8(1-2):159-74. doi: 10.1385/NMM:8:1:159.
- Chrestian N. Hereditary Neuropathy with Liability to Pressure Palsies. 1998 Sep 28 [updated 2020 Aug 27]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1392/
- Guo J, Wang L, Zhang Y, Wu J, Arpag S, Hu B, Imhof BA, Tian X, Carter BD, Suter U, Li J. Abnormal junctions and permeability of myelin in PMP22-deficient nerves. Ann Neurol. 2014 Feb;75(2):255-65. doi: 10.1002/ana.24086. Epub 2014 Feb 20.
- Li J, Krajewski K, Lewis RA, Shy ME. Loss-of-function phenotype of hereditary neuropathy with liability to pressure palsies. Muscle Nerve. 2004 Feb;29(2):205-10. doi: 10.1002/mus.10521.
- Potulska-Chromik A, Sinkiewicz-Darol E, Ryniewicz B, Lipowska M, Kabzinska D, Kochanski A, Kostera-Pruszczyk A. Clinical, electrophysiological, and molecular findings in early onset hereditary neuropathy with liability to pressure palsy. Muscle Nerve. 2014 Dec;50(6):914-8. doi: 10.1002/mus.24250. Epub 2014 Oct 30.
- Rosso G, Liashkovich I, Gess B, Young P, Kun A, Shahin V. Unravelling crucial biomechanical resilience of myelinated peripheral nerve fibres provided by the Schwann cell basal lamina and PMP22. Sci Rep. 2014 Dec 2;4:7286. doi: 10.1038/srep07286.
- van de Wetering RA, Gabreels-Festen AA, Timmerman V, Padberg GM, Gabreels FJ, Mariman EC. Hereditary neuropathy with liability to pressure palsies with a small deletion interrupting the PMP22 gene. Neuromuscul Disord. 2002 Oct;12(7-8):651-5. doi: 10.1016/s0960-8966(02)00025-1.
- Yilmaz U, Bird TT, Carter GT, Wang LH, Weiss MD. Pain in hereditary neuropathy with liability to pressure palsy: an association with fibromyalgia syndrome? Muscle Nerve. 2015 Mar;51(3):385-90. doi: 10.1002/mus.24331.
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