Hereditary Motor Neuropathy With Liability To Pressure Palsies (Hnpp)

(Tomaculous neuropathy)

In hereditary motor neuropathy with liability to pressure palsies (HNPP) are increasingly nervous sensitive to pressure and strain.

(See also summary of disorders of the peripheral nervous system.)

In hereditary motor neuropathy with liability to pressure palsies (HNPP) are increasingly nervous sensitive to pressure and strain. (See also overview of disorders of the peripheral nervous system.) In HNPP nerves lose their myelin sheath and lead the nerve impulses no longer normal. Inheritance is autosomal dominant in general. In 80% of cases, the cause lies in the loss of one copy of the peripheral myelin protein-22 gene (PMP22), which is located on the short arm of chromosome 17th Two copies of genes are necessary for normal functioning. The incidence of HNPP is estimated at 2-5 / 100,000. Symptoms and signs usually employ the symptoms in adolescence or young adulthood, but they can also start at any age. Peroneal paralysis with foot drop, ulnar nerve and carpal tunnel syndrome often develop. The pressure palsies can be easy or hard, and have a duration from minutes to months. In the affected areas numbness and weakness occur. After an episode, about half of the affected people recover completely, and most of the other symptoms are mild pronounced. Diagnostic Electro-diagnostic testing genetic testing HNPP should be adopted in patients with any of the following characteristics: Recurring compression Mononeuropathies Mulitple mononeuropathy unknown cause symptoms that suggest a recurrent demyelinating polyneuropathy family history of carpal tunnel syndrome Electro-diagnostic testing and genetic analysis support the diagnosis; rarely a biopsy is required. Therapy Supportive treatment Treatment of HNPP involves avoiding or modification of activities that cause symptoms. Wrist wraps and padding the elbows can reduce the pressure, prevention of recurrence of injury and give the nerves over time, the opportunity to repair the myelin structure. A surgical procedure is rarely indicated.

Health Life Media Team

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