Hereditary Neuralgic Amyotrophy – Peripheral Nerve Disorders

Also called: Neuritis, Peripheral neuritis, Peripheral neuropathy

Your peripheral nerves are the ones outside your brain and spinal cord. Like static on a telephone line, peripheral nerve disorders distort or interrupt the messages between the brain and the rest of the body.

There are more than 100 kinds of peripheral nerve disorders. They can affect one nerve or many nerves. Some are the result of other diseases, like diabetic nerve problems. Others, like Guillain-Barre syndrome, happen after a virus infection. Still others are from nerve compression, like carpal tunnel syndrome or thoracic outlet syndrome. In some cases, like complex regional pain syndrome and brachial plexus injuries, the problem begins after an injury. Some people are born with peripheral nerve disorders.

Symptoms often start gradually, and then get worse. They include

• Numbness
• Pain
• Burning or tingling
• Muscle weakness
• Sensitivity to touch

Treatment aims to treat any underlying problem, reduce pain and control symptoms.

NIH: National Institute of Neurological Disorders and Stroke

• Axillary nerve dysfunction (Medical Encyclopedia)
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• Common peroneal nerve dysfunction (Medical Encyclopedia)
• Distal median nerve dysfunction (Medical Encyclopedia)
• Femoral nerve dysfunction (Medical Encyclopedia)
• Glossopharyngeal neuralgia (Medical Encyclopedia)
• Metabolic neuropathies (Medical Encyclopedia)
• Mononeuritis multiplex (Medical Encyclopedia)
• Neuralgia (Medical Encyclopedia)
• Neuropathy secondary to drugs (Medical Encyclopedia)
• Peripheral neuropathy (Medical Encyclopedia)
• Radial nerve dysfunction (Medical Encyclopedia)
• Sensorimotor polyneuropathy (Medical Encyclopedia)
• Tibial nerve dysfunction (Medical Encyclopedia)
• Ulnar nerve dysfunction (Medical Encyclopedia)

Hereditary neuralgic amyotrophy is a disorder characterized by episodes of severe pain and muscle wasting (amyotrophy) in one or both shoulders and arms. Neuralgic pain is felt along the path of one or more nerves and often has no obvious physical cause. The network of nerves involved in hereditary neuralgic amyotrophy, called the brachial plexus, controls movement and sensation in the shoulders and arms.People with hereditary neuralgic amyotrophy usually begin experiencing attacks in their twenties, but episodes have occurred as early as the age of 1 year in some individuals. The attacks may be spontaneous or triggered by stress such as strenuous exercise, childbirth, surgery, exposure to cold, infections, immunizations, or emotional disturbance. While the frequency of the episodes tends to decrease with age, affected individuals are often left with residual problems, such as chronic pain and impaired movement, that accumulate over time.Typically an attack begins with severe pain on one or both sides of the body; right-sided involvement is most common. The pain may be difficult to control with medication and usually lasts about a month. Within a period of time ranging from a few hours to a couple of weeks, the muscles in the affected area begin to weaken and waste away (atrophy), and movement becomes difficult. Muscle wasting may cause changes in posture or in the appearance of the shoulder, back, and arm. In particular, weak shoulder muscles tend to make the shoulder blades (scapulae) “stick out” from the back, a common sign known as scapular winging. Additional features of hereditary neuralgic amyotrophy may include decreased sensation (hypoesthesia) and abnormal sensations in the skin such as numbness or tingling (paresthesias). Areas other than the shoulder and arm may also be involved.In a few affected families, individuals with hereditary neuralgic amyotrophy also have unusual physical characteristics including short stature, excess skin folds on the neck and arms, an opening in the roof of the mouth (cleft palate), a split in the soft flap of tissue that hangs from the back of the mouth (bifid uvula), and partially webbed or fused fingers or toes (partial syndactyly). They may also have distinctive facial features including eyes set close together (ocular hypotelorism), a narrow opening of the eyelids (short palpebral fissures) with a skin fold covering the inner corner of the eye (epicanthal fold), a long nasal bridge, a narrow mouth, and differences between one side of the face and the other (facial asymmetry).