Peripheral Nerve System - Damage

The peripheral nerve system (PNS) are nerves in the body; face, arms, legs, and torso. These nerves send information from and to the brain to tell used to move and back to the brain about sensation felt from the muscles.

Neurofibromatosis type 2 (NF2) is best known for damage to the nerves in the skull, the cranial nerve system (CNS) as a result of tumor growth. However; 1) damage to the CNS can also damage the PNS and 2) there is no isolated region of the body tumors will grow in individuals with the NF2 condition.

Neuropathy is nerve damage. Individuals with NF2 can develop different forms of neuropathy for different reasons. There are two major forms of Neuropathy an individual with NF2 might face; 1) Peripheral Neuropathy, and 2) Radiculopathy. Both are issues of the PNS.

1. Peripheral Neuropathy (PN)

Peripheral neuropathy (PN) refers to a condition in which the nerves going from the brain and spinal cord to the legs, feet, arms, or hands are damaged. As a result, nerves no longer conduct impulses properly, either transmitting signals poorly or spontaneously activating. Depending on which nerves are damaged, the person may experience pain or numbness, a burning or tingling sensation, increased sensitivity to touch, muscle weakness, or a number of other symptoms in the extremities connected to the affected nerves.

1. Motor Nerves (Somatic Nervous System): Voluntary Movement
2. Autonomic Nerve (Autonomic Nervous System): Involuntary Function; breathing, blood pressure, sexual function, digestion
3. Sensory Nerves: Senses; temperature, pain, touch, and limb positioning
2. Radiculopathy is the term for neuropathy that affects only some part of the peripheral nervous system, resulting in the issue of the use of a part of the body on one side, example; a leg, a hand or one side of the face.

Peripheral Nerve damage has a better chance of healing more completely than Cranial Nerve damage, even if healing might take over several months of showing signs of recovery.

1. Peripheral Neuropathy

NF2 Peripheral Neuropathy is Peripheral Neuropathy - Polyneuropathy (PNP), an issue which is said to develop at some point in 66% - 70% of individuals, but as all other statistics for NF2, is debatable and could be found in a higher or lower percentage of people.

As a result of PNP, many who have NF2 are either instead diagnosed as having multiple sclerosis (MS) instead of NF2.

Misdiagnosis of NF2 PN is less likely now as neurologists are more likely to order MRIs, as a CT Scan is less likely to show there may be brain tumors for a full complete diagnosis of NF2.

1. Poor Circulation

If your body is always cold or doctors have a hard time finding veins for IV injections you may have a circulation problem. Circulation issues are not uncommon among people with NF2, it is one of the signs of Peripheral Nerve Damage.

Poor circulation might be experienced as one or a combination of the following:

Aching Feet Cold Extremities (Feet And Hands) Cramping Or Fatigue In Your Arms or Legs Dandruff Dry Skin Edema, Swelling Fatigue or Low Energy Hair Loss Infection

Irregular Heart Beats Itching Lack of Stamina Muscle Cramps and Pain Shortness of Breath Skin Break Outs Sluggish Memory Varicose Veins, Leg Ulcers Vertigo and Dizziness

The following things can make circulation worse; 1) caffeine, 2) alcohol, 3) smoking cigarettes, 4) poor diet, 5) overweight, and 6) lack of exercise.

2. Muscle Atrophy (Muscle Wasting)

Amyotrophy, Muscle Wasting and also known as Cachexia is a wasting syndrome. Muscle Wasting is Autonomic Nervous System (Organ) Dysfunction; loss of body mass that cannot be reversed nutritionally.

While this can be found in people with different health conditions, for NF2 it arises from tumor damage to the nerves in the body that stimulates the muscle which results in loss of weight, muscle atrophy, fatigue, weakness, and significant loss of appetite.

Other reasons for muscle atrophy is vertebrae damage following a slipped (herniated) disk from either tumor growth along the spinal cord or spinal cord abnormalities; scoliosis, kyphosis, or kyphoscoliosis). Unrelated to NF2 muscle atrophy is also possible as a result of; 1) multiple sclerosis (MS), 2) type 2 diabetes, and 3) axillary nerve dysfunction (AND).

How many people were diagnosed with MS or Type 2 Diabetes instead of or as well as NF2, as a result of Muscle Atrophy from NF2?
Answer: Unknown, but more than a few. If an individual with NF2 has a family with MS or Type 2 Diabetes a review of their health by a neurologist familiar with NF2 is warranted and is in part the reason genetic testing for an entire family.

3. Diagnosis and Progression of PNP

Since it is hard to find the Schwannoma on the nerves with an MRI, an Electrophysiology Nerve Test (EMG) can be done to see nerve weakness and done again as needed to monitor change in nerve compression over time.

4. Symptoms

• Somatic Nervous System - Muscles and Movement
• Difficulty breathing or swallowing
• Falling (from legs buckling or tripping over toes)
• Fasciculation - Muscle twitching or cramping, involuntary  muscle contraction and relaxation g.
• Lack of Dexterity
• Lack of muscle control
• Muscle Wasting (Muscle Atrophy) - Weakness, loss of muscle bulk and loss of dexterity. Sometimes, cramps are a sign of motor nerve involvement.
• Paralysis - Difficulty or inability to move a part of the body.
• Autonomic Nervous System - Organs
• Abdominal Bloating
• Blurred Vision
• Circulation Issues - Inability to regulate body temperature.
• Constipation
• Decreased Ability to Sweat
• Diarrhea
• Difficulty Beginning to Urinate - Urinary Hesitancy
• Dizziness - Change in Blood Pressure
• Early Satiety - Feeling full after eating a small amount.
• Feeling of incomplete bladder emptying
• Male Impotence
• Nausea or vomiting after meals
• Unintentional Weight Loss
• Urinary Incontinence

5. Treatment

Simple Treatments:

1. Vitamin B12
2. Eating organic foods, minimizing added nerve damage from pesticides.
3. Exercise: Regular Aerobic Activity or Yoga
4. Drink more Water: Water in foods and drinks can count towards daily requirement of water if food or drinks do not contain Caffeine or Alcohol.
5. Hand or Foot Massage: Never have a Back Massage if you have Spine Tumors.
6. Hot Bath or Shower: As hot water washes over skin it draws blood to the surface, stimulating circulation.

Minimal or reduced amounts of:

1. Caffeine
2. Alcohol
3. Cigarettes
4. Poor Diet
5. Weight

Treatment for Numbness, Severe Pain, or Weakness:

• Ankle Foot Orthosis (AFO Braces): Afailible in different shapes, plastic, and various metals. purchased easily off the shelf or with better fiitting braces from doctors office.
• Electrical Stimulation(e-stim)/Neurostimulation: arm, leg, foot, or spine
  stimulators are; no pain, mild, low power, stimulation
  can conflict with body implants if used to close. if a dividuals have a heart or hearing implant, they should talk to their doctor.
  spine simulater requires surgical implant
• Topical options: Biofreeze and Neuragen PN.
• Medication: neurontin (gabapentin), cymbalta (duloxetine hydrocholride), lyrica (pregabalin), or antidepressants.
• Nerve Decompression Surgery

II. Radiculopathy

Radiculopathy is damage to peripheral nerves from the spine all the way to the nerves end, as a result of tumors within the spine causing compression damaging the complete nerve.

This could be Schwannoma on nerves, or either Meningioma or Ependymoma compressing on nerves in the Spinal Cord, at the nerves root, can damage the nerve all the way to the nerve ending. Pain or weakness would be based on exact location of the tumor.

III. Damage By Location

IV. Sources

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3. Schulz, A., Kyselyova, A., Baader, S. L., Jung, M. J., Zoch, A., Mautner, V. F., ... & Morrison, H. "Neuronal merlin influences ERBB2 receptor expression on Schwann cells through neuregulin 1 type III signalling." Brain. Journal of Neurology, awt327. (2013)
   Source: http://brain.oxfordjournals.org/content/early/2013/12/05/brain.awt327.full | DOI: 10.1093/brain/awt327
4. Baumer, P., Mautner, V. F., Baumer, T., Schuhmann, M. U., Tatagiba, M., Heiland, S., ... & Pham, M. . "Accumulation of non-compressive fascicular lesions underlies NF2 polyneuropathy." Journal of Neurology, 260(1), 38-46. (2013)
   Source: http://link.springer.com/article/10.1007/s00415-012-6581-8?LI=true | DOI: 10.1007/s00415-012-6581-8
5. Sagnelli, A., Piscosquito, G., & Pareyson, D. "Inherited Neuropathies: an update." Journal of Neurology, 260(10), 2684-2690. (2013)
   Source: http://link.springer.com/article/10.1007/s00415-013-7113-x | DOI: 10.1007/s00415-013-7113-x
6. "Muscle Wasting." Health Line
   https://www.healthline.com/symptom/muscle-wasting
7. http://www.berkeleywellness.com/self-care/preventive-care/article/8-questions-about-peripheral-neuropathy
8. Dictionary. https://www.dictionary.com/browse/nervous-system
9. Leverkus, M., et al. "Multiple unilateral schwannomas: segmental neurofibromatosis type 2 or schwannomatosis?." British Journal of Dermatology 148.4 (2003): 804-809.
   https://onlinelibrary.wiley.com/doi/abs/10.1046/j.1365-2133.2003.05249.x | https://doi.org/10.1046/j.1365-2133.2003.05249.x
10. "Engineers 3D print flexible mesh for ankle and knee braces." Science Daily https://www.sciencedaily.com/releases/2019/06/190620153500.htm