About morvan disease -- neuropathy hereditary ...
What is morvan disease -- neuropathy hereditary ...?
Hereditary sensory and autonomic neuropathy type II (HSAN2) is a rare genetic disorder that usually begins in childhood by affecting the nerves that serve the lower legs and feet and the lower arms and hands. Symptoms start with inflamed fingers or toes, especially around the nails. Numbness and tingling sensations in the hands and feet may also occur. Eventually, affected individuals lose feeling (sensation) in the hands and feet. This sensory loss is due to abnormal functioning of the sensory nerves that control responses to pain and temperature and may also affect the autonomic nervous system that controls other involuntary or automatic body processes. Chronic infection of the affected areas is common and worsens as ulcers form on the fingers or the soles of the hands and feet. The loss of sensation in the hands and feet often leads to neglect of the wounds. This can become serious even leading to amputation in extreme cases if left untreated. The disorder affects many of the body's systems, is characterized by early onset (infancy or childhood) and is transmitted genetically as an autosomal recessive trait. HSAN2 occurs due to mutations in specific genes. There are a few subtypes designated A through C, each one associated with a different gene.
The hereditary sensory and autonomic neuropathies (HSAN), also known as the hereditary sensory neuropathies, include at least six similar but distinct inherited degenerative disorders of the nervous system (neurodegenerative) that frequently progress to loss of feeling, especially in the hands and feet. Some of these disorders have several subtypes based upon the specific associated genes. Some types of HSAN are related to or identical with some forms of Charcot-Marie-Tooth disease, and others are related to or identical with familial dysautonomia (Riley-Day syndrome). The classification of the HSANs is complicated, and the experts to not always agree on it. Furthermore, HSANs are classified as broadly as peripheral neuropathies or disorders or the peripheral nervous system, which encompasses all of the nerves outside of the central nervous system (i.e. brain and spinal cord).
What are the symptoms for morvan disease -- neuropathy hereditary ...?
Symptoms of hereditary neuropathy depend on the group of nerves affected. They can affect the motor, sensory, and autonomic nerves. Sometimes, they affect more than one nerve group. For instance, Charcot-Marie-Tooth (CMT) disease, one of the most common types of hereditary neuropathies, affects the motor and sensory nerves.
Hereditary neuropathies can have similar symptoms. Some of the most common symptoms include:
- Sensory symptoms: Pain, tingling, or numbness, often in the hands and feet.
- Motor symptoms: Muscle Weakness and loss of mass (muscle atrophy), often in the feet and lower legs.
- Autonomic symptoms: Impaired sweating, or low blood pressure after standing up from sitting or lying down.
- Physical deformities: High foot arches, hammer-shaped toes, or a curved spine (scoliosis).
Hereditary neuropathy symptoms can range in intensity from mild to severe. In some cases, symptoms are so mild that the disorder goes undiagnosed and untreated for a long time.
Symptoms don’t always appear at birth or during childhood. They can appear during middle age or even later in life.
What are the causes for morvan disease -- neuropathy hereditary ...?
Peripheral neuropathy is nerve damage caused by a number of different conditions. Health conditions that can cause peripheral neuropathy include:
- Autoimmune diseases. These include Sjogren's syndrome, lupus, rheumatoid arthritis, Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy and vasculitis.
- Diabetes. This is the most common cause. Among people with diabetes, more than halfwill develop some type of neuropathy.
- Infections. These include certain viral or bacterial infections, including Lyme disease, shingles, Epstein-Barr virus, hepatitis B and C, leprosy, diphtheria, and HIV.
- Inherited disorders. Disorders such as Charcot-Marie-Tooth disease are hereditary types of neuropathy.
- Tumors. Growths, cancerous (malignant) and noncancerous (benign), can develop on the nerves or press on nerves. Also, polyneuropathy can arise as a result of some cancers related to the body's immune response. These are a form of a degenerative disorder called paraneoplastic syndrome.
- Bone marrow disorders. These include an abnormal protein in the blood (monoclonal gammopathies), a form of bone cancer (myeloma), lymphoma and the rare disease amyloidosis.
- Other diseases. These include kidney disease, liver disease, connective tissue disorders and an underactive thyroid (hypothyroidism).
Other causes of neuropathies include:
- Alcoholism. Poor dietary choices made by people with alcoholism can lead to vitamin deficiencies.
- Exposure to poisons. Toxic substances include industrial chemicals and heavy metals such as lead and mercury.
- Medications. Certain medications, especially those used to treat cancer (chemotherapy), can cause peripheral neuropathy.
- Injury or pressure on the nerve. Injuries, such as from motor vehicle accidents, falls or sports injuries, can sever or damage peripheral nerves. Nerve pressure can result from having a cast or using crutches or repeating a motion such as typing many times.
- Vitamin deficiencies. B vitamins — including B-1, B-6 and B-12 — vitamin E and niacin are crucial to nerve health.
In a number of cases, no cause can be identified (idiopathic).
What are the treatments for morvan disease -- neuropathy hereditary ...?
There’s no cure for hereditary neuropathy. Instead, you’ll need ongoing treatment to manage your symptoms. Common treatments include:
- pain medication
- physical therapy
- corrective surgery
- therapeutic shoes, braces, and supports
Eating a balanced diet and getting regular exercise is also recommended.
If you have loss of sensation, your doctor might suggest certain safety measures to help you avoid hurting yourself.
What are the risk factors for morvan disease -- neuropathy hereditary ...?
Having a family member, and especially a parent, who’s been diagnosed with a hereditary neuropathy is the most significant risk factor.
Some studies suggest that health conditions, such as type 2 diabetes and obesity, may increase your risk for certain hereditary neuropathies. More research needs to be done to understand the link between health conditions and hereditary neuropathies.
Is there a cure/medications for morvan disease -- neuropathy hereditary ...?
The long-term outlook for people who have been diagnosed with hereditary neuropathy depends on the genes affected, as well as the type of neuropathy. Certain types of hereditary neuropathy progress more quickly than others.
In addition, hereditary neuropathy symptoms can be mild enough to go undiagnosed for a long time. Symptoms can also be severe and disabling.
If you have hereditary neuropathy, talk to your doctor about what to expect long term.