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Small fiber peripheral neuropathy

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Small fiber peripheral neuropathy

Small fiber peripheral neuropathy
Classification and external resources
ICD-10 G63.3 G60.8 G62.8
MeSH D010523

Small fiber peripheral neuropathy is a type of

  • Video explaining Small Fiber Neuropathy
  • Peripheral Neuropathy Fact Sheet - NINDS
  • Painful Feet: The Small Fiber Neuropathies
  • Small Fiber Neuropathy Center Maastricht

External links

  1. ^ Overview of Small Fiber Neuropathy. Therapath Pathology.
  2. ^ Zhou, Lan (2000). Small fiber neuropathy: A burning problem Cleveland Clinic Journal of Medicine, vol.76 5.
  3. ^ Latov, Norman. Peripheral neuropathy: when the numbness, weakness, and pain wont stop. American Academy of Neurology (AAN) quality of life guides, 2007, p.8.
  4. ^ a b c Hovaguimian A, Gibbons CH (June 2011). "Diagnosis and treatment of pain in small-fiber neuropathy". Curr Pain Headache Rep 15 (3): 193–200.  
  5. ^ a b Lauria G, Hsieh ST, Johansson O, et al. (July 2010). "European Federation of Neurological Societies/Peripheral Nerve Society Guideline on the use of skin biopsy in the diagnosis of small fiber neuropathy.". Eur. J. Neurol. 17 (7): 903–12, e44–9.  
  6. ^ Lacomis D (August 2002). "Small-fiber neuropathy". Muscle Nerve 26 (2): 173–88.  
  7. ^ a b c d Lauria, G; Bakkers, M; Schmitz, C; Lombardi, R; Penza, P; Devigili, G; Smith, AG; Hsieh, ST; Mellgren, SI; Umapathi, T; Ziegler, D; Faber, CG; Merkies, IS (September 2010). "Intraepidermal nerve fiber density at the distal leg: a worldwide normative reference study.". Journal of the peripheral nervous system : JPNS 15 (3): 202–7.  
  8. ^ Polydefkis, M; et al. (2003). "New insights into diabetic polyneuropathy". JAMA 290: 1371–6.  
  9. ^ Overview of Small Fiber Neuropathy. Therapath Pathology.
  10. ^ Devigili, G; et al. (2008). "The diagnostic criteria for small fiber neuropathy; from symptoms of neuropathology". Brain 131: 1912–1925.  
  11. ^ Faber, C; et al. (2011). "Gain of function Na(V) 1.7 mutations in idiopathic small fiber neuropathy". Annals of Neurology.  
  12. ^ http://www.neurology.org/cgi/content/meeting_abstract/80/1_MeetingAbstracts/S37.005
  13. ^ http://www.ncbi.nlm.nih.gov/pubmed/16908366
  14. ^ http://news.harvard.edu/gazette/story/2013/07/nerve-damage-and-fibromyalgia/
  15. ^ http://link.springer.com/article/10.1007/s10067-014-2850-5/fulltext.html

References

See also

There is no current treatment to cure small fiber peripheral neuropathy, but Intravenous immunoglobulin (IVIG) is often used as well as plasmapheresis.

Treatment is based on the underlying cause, if any. Where the likely underlying condition is known, treatment of this condition is indicated treated to reduce progression of the disease and symptoms. For cases without those conditions, there is only symptomatic treatment.

Treatment

Recently several studies have suggested an association between autonomic small fiber neuropathy and postural orthostatic tachycardia syndrome.[12] Other notable studies have shown a link between Erythromelalgia,[13] and Fibromyalgia[14]

There are many possible causes of small fiber neuropathy. The most common cause is diabetes or glucose intolerance.[8] Other possible causes include hypothyroidism, Sjögren's syndrome, Lupus, vasculitis, sarcoidosis, nutritional deficiency, Celiac disease, Lyme disease, HIV, Fabry disease, amyloidosis and alcoholism.[9] A 2008 study reported that in approximately 40% of patients no cause could be determined after initial evaluation.[10] When no cause can be identified, the neuropathy is called idiopathic. A recent study revealed dysfunction of a particular sodium channel (Nav1.7) in a significant portion of the patient population with an idiopathic small fiber neuropathy.[11]

Causes

Intraepidermal nerve fiber density (IENFD) normative values for clinical use[7]
Females Males
Age in years 0.05 Quantile IENFD values per age span Median IENFD values per age span 0.05 Quantile IENFD values per age span Median IENFD values per age span
20-29 8.4 13.5 6.1 10.9
30-39 7.1 12.4 5.2 10.3
40-49 5.7 11.2 4.4 9.6
50-59 4.3 9.8 3.5 8.9
60-69 3.2 8.7 2.8 8.3
70-79 2.2 7.6 2.1 7.7
≥80 1.6 6.7 1.7 7.2

This skin punch biopsy measurement technique is called intraepidermal nerve fiber density (IENFD).[7] The following table describes the IENFD values in males and females.[7] Any value measured below the 0.05 Quantile IENFD values per age span, is considered a reliable positive diagnosis for Small Fiber Peripheral Neuropathy.[7]

A skin biopsy for the measurement of epidermal nerve fiber density is an increasingly common technique for the diagnosis of small fiber peripheral neuropathy.[4] Physicians can biopsy the skin with a 3-mm circular punch tool, and send the sample to a specialized laboratory for processing and analysis. Small nerve fibers are quantified by a neuropathologist to obtain a diagnostic result.[5]

Skin biopsy

Quantitative sensory testing (QST) assesses small fiber function by measuring temperature and vibratory sensation. Abnormal QST results can be attributed to dysfunction in the central nervous system. Furthermore, QST is limited by a patient’s subjective experience of pain sensation.[6] Quantitative sudomotor axon reflex testing (QSART) measures sweating response at local body sites to evaluate the small nerve fibers that innervate sweat glands. [4]

The diagnosis of small fiber neuropathy often requires ancillary testing.[4] Nerve conduction studies and electromyography are commonly used to evaluate large myelinated sensory and motor nerve fibers, but are ineffective in diagnosing small fiber neuropathies.[5]

Diagnosis

Like many polyneuropathies, the symptoms usually start in the longer nerves and progressively attack shorter nerves. This means that most often the symptoms start in the feet and progress upwards, and usually symptoms are more severe in the feet. However, patients with Fabry disease have isolated small fiber engagement, and can have a more widespread small fiber disruption.

Topographic pattern

Sensory symptoms of small fiber neuropathy are highly variable. Common complaints include paresthesias, dysesthesias, and insensitivity to pain. Paresthesias are abnormal sensations. They are often described as numbness, burning, cold, prickling, pins and needles along with other symptoms. Dysesthesias are unpleasant sensations, either spontaneous or evoked. A light breeze, the feeling of clothes, or even a soft touch can cause pain.[3] Insensitivity to pain can be particular problem. One may be bleeding or have a skin injury without even knowing it.

Symptoms

Contents

  • Symptoms 1
  • Topographic pattern 2
  • Diagnosis 3
    • Skin biopsy 3.1
  • Causes 4
  • Treatment 5
  • See also 6
  • References 7
  • External links 8

[2]). It is estimated that 15-20 million people in the United States suffer from some form of peripheral neuropathy.autonomic fibers (autonomic function) and help control somatic fibers The role of these nerves is to innervate the skin ([1]

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