Peripheral Nerve System - Damage
Last Updated: 12/28/17
The Central Nervous System (CNS), includes Cranial Nerves and the spinal Cord, these are the core nerves of the body, they
are the; primary and main nerve branches of the body. Nerves outside of the CNS are nerves of the Peripheral Nerve System (PNS).
The PNS are nerves in the; face, arms, legs, torso, but also include some nerves in the skull.
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.
- Peripheral Neuropathy (PN) is a term used to describe damage to the entire PNS. The peripheral nervous system is made up of three types of nerves:
- Motor Nerves (Somatic Nervous System): Voluntary Movement
- Autonomic Nerve (Autonomic Nervous System): Involuntary Function; breathing, blood pressure, sexual function, digestion
- Sensory Nerves: Senses; temperature, pain, touch, and limb positioning
- Radiculopathy is the term for neuropathy that affects only some part of the peripheral nerve system, resulting in issue of use of a part of the body on one side, example; a leg, a hand or one side of the face.
For an individual with NF2, while there may be more reasons than included here, either forms of Neuropathy of peripheral nerves could be the result of; 1) tumor growth
that causes brainstem or spinal cord compression, 2) tumor growth on a nerve branch off of the spinal cord to a portion of the body, or 3) even micro-lesions
(microscopic tumors not seen in MRI) on nerves.
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
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 Diabetes or Multiple Sclerosis (MS). While PNP becomes a mobility
issue later in life it can be a slow progression of body nerve damage that can start and result in mobility issues in a few years or decades.
Misdiagnosis of NF2 PNP 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
- Dry Skin
- Edema, Swelling
- Fatigue or Low Energy
- Hair Loss
- Irregular Heart Beats
- Lack of Stamina
- Muscle Cramps and Pain
- Shortness of Breath
- Skin Break Outs
- Sluggish Memory
- Varicose Veins, Leg Ulcers
- Vertigo and Dizziness
Everything should be done in moderation), but the following things can make circulation worse; Caffeine, Alcohol, Cigarettes, Poor Diet,
Overweight and / or 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
Reasons other than PN reasons for development of Muscle Atrophy is Slipped (Herniated) Disk, unrelated to NF2; Multiple Sclerosis (MS),
Type 2 Diabetes and Axillary Nerve Dysfunction (AND).
Slipped (Herniated) Disk - Any part of the spinal cord which also means individuals with spinal cord abnormalities of Scoliosis,
Kyphosis, or Kyphoscoliosis.
Axillary nerve dysfunction (AND) is a condition marked by a loss of movement or sensation in the shoulder area. It's also known as neuropathy
of the axillary nerve. Excessive stress or damage to the axillary nerve, which serves the deltoid muscles and skin of the shoulder,
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.
- 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.
- Decreased Ability to Sweat
- 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
- Vitamin B12
- Eating organic foods to avoid nerve damage from pesticides.
- Exercise: Regular Aerobic Activity or Yoga
- 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.
- Hand or Foot Massage: Never have a Back Massage if you have Spine Tumors.
- Hot Bath or Shower: As hot water washes over skin it draws blood to the surface, stimulating circulation.
Minimal or reduced amounts of:
- Poor Diet
Treatment for Numbness, Severe Pain, or Weakness:
- Electrical Stimulation (Neurostimulation): This is a no pain, mild, low power, stimulation method.
(If purchased for home use outside of a doctor's office, do not use near head or neck.)
- Topical options: Biofreeze and Neuragen PN.
- Medication: Neurontin (Gabapentin), Cymbalta (duloxetine hydrocholride), Lyrica (pregabalin), or antidepressants.
- Nerve Decompression Surgery
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.
- Widemann, B. C., Acosta, M. T., Ammoun, S., Belzberg, A. J., Bernards, A., Blakeley, J., ... & Morrison, H.
"CTF meeting 2012: Translation of the basic understanding of the biology and genetics of NF1, NF2, and Schwannomatosis toward the development of
effective therapies." American Journal of Medical Genetics Part A, 164(3), 563-578. (2014)
http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.36312/full | DOI: 10.1002/ajmg.a.36312
- Schulz, A., Baader, S. L., Niwa-Kawakita, M., Jung, M. J., Bauer, R., Garcia, C., ... & Morrison, H. "Merlin isoform
2 in Neurofibromatosis type 2-associated Polyneuropathy." Nature Neuroscience. (2013)
http://www.nature.com/neuro/journal/vaop/ncurrent/full/nn.3348.html?elq=98b2aca29f3449daab746bd5c7f90871 | DOI: 10.1038/nn.3348
- 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)
http://brain.oxfordjournals.org/content/early/2013/12/05/brain.awt327.full | DOI: 10.1093/brain/awt327
- 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)
http://link.springer.com/article/10.1007/s00415-012-6581-8?LI=true | DOI: 10.1007/s00415-012-6581-8
- Sagnelli, A., Piscosquito, G., & Pareyson, D. "Inherited Neuropathies: an update." Journal of Neurology, 260(10), 2684-2690. (2013)
http://link.springer.com/article/10.1007/s00415-013-7113-x | DOI: 10.1007/s00415-013-7113-x