Monday 15 July 2013

POSTURAL TACHYCARDIA SYNDROME (PoTS) - AUTONOMIC NERVOUS SYSTEM DYSFUNCTION

Increasingly I hear of cases where PoTS is linked to Lyme Disease and wanted to share this recent post from Joe Burrascano on Eurolyme. I am sure it will be of interest to those with PoTS, Lyme Disease and those with ME or CFS.

'Some neurological problems are from dysfunction, while others are from damage. Antibiotic treatments and supportive measures may go a long way toward improving or even totally clearing dysfunction and some damage, but in people with longstanding problems, some of the damage cannot be reversed by simply controlling the infection(s). In this group, infusions of intravenous gamma globulin (IVIG) as is given in cases of multiple sclerosis and CIDP (chronic inflammatory demyelinating polyneuropathy) has been shown to improve significantly the symptoms in this group about 80% of the time.'


ASSESSING THE CHRONIC LYME PATIENT

DIAGNOSTIC TESTING

Because of the known immune dysfunction associated with late disseminated Lyme, serodiagnosis becomes less accurate, and the diagnosis may be missed. 

CLUES THAT LYME MAY BE PRESENT: Multisystem symptoms, any musculoskeletal pain, migratory symptoms, periodicity of symptoms with intensity that waxes and wanes, unexplained neurological symptoms and potential or known tick exposure.

HOW TO TEST: While serologic testing is always recommended, because it has a low sensitivity 1 and is an indirect test (just indicates potential past exposure), a Borrelia blood culture is essential due to the highly reliable results and because it can be a direct indicator of current infection.2

ASSOCIATED IMMUNE DEFICIENCY: B, T, and NK cell dysfunction has been described in patients with Lyme.

· T-cell function- the simplest and safest diagnostic measure is to test blood to see whether antibodies still exist for illnesses the patient has either previously had or to which he/she had been vaccinated (measles, pneumococci, hepatitis, etc.).

· B-cell function- Measure total IgG levels and IgG subclasses

· NK- cells- CD 57 cell counts may be depressed 

ASSOCIATED NEUROLOGICAL COMPLICATIONS

Borrelia infections can be associated with symptoms attributable to dysfunction and/or damage to the nervous system3. The more enigmatic manifestations can include intractable pain and dysautonomia.

NEUROLOGIC PAIN: Some of the most severe and difficult to control complaints of pain are associated with neuropathy, especially when it involves the fine unmyelinated cutaneous fibers. Such involvement is often missed but can easily be diagnosed by a simple skin biopsy you can do in your office. When properly stained, the count and morphology of these fibers can be assessed. Note that abnormalities here may also be associated with autonomic neuropathy (see below).

CLUES: Hypo or hyperesthesia of the affected area, lancinating pain as opposed to dull, tingling, sense of vibration, burning, hot and/or cold sensations, abnormal skin color or temperature and localized swelling each may indicate an underlying neurologic origin for the pain. 

TESTING: Electrodiagnostic studies such as EMG and NCV are for peripheral myelinated fibers. Do a skin biopsy to assess the integrity and count of fine unmyelinated cutaneous nerve fibers. Dysfunction of these will not show up on electrodiagnostic studies.

AUTONOMIC DYSFUNCTION

CLUES: Symptoms of dysfunction of the autonomic nervous system can include dizziness, fainting, urinary problems, sexual dysfunction, delayed gastric emptying and exercise intolerance.4 

TESTING: Tilt table test, orthostatic hypotension/POTS, heart rate variability, pupillary responses, sweat tests/QSART, gastric emptying study, urodynamics/ultrasound.

TREATMENT

· Antibiotic protocols individualized to the patient as recommended by a Lyme literate practitioner

· Intravenous Immune Globulin (IVIG) for 

o replacement for deficiencies in total IgG and for significant subclass deficiencies associated with recurrent, persistent or chronic infection

o treatment for associated neurologic conditions such as demyelination, neuropathy, autonomic neuropathy and neutrally mediated chronic pain syndromes5

· Compounded topical pharmaceuticals to safely control pain

REFERENCES

1. Stricker, BMJ 2007; 335 (7628): 1008)

2. Sapi, E. et. al. Int. J. Med. Sci. 2013; 10(4):362-376. doi: 10.7150/ijms.5698

3. Alaedini, A. et. al. Journal of Neuroimmunology. Volume 159, Issue 1 , Pages 192-195, February 2005

4. Mayo Clinic

5. Goebel, A. et. al. Ann Intern Med. 2010;152:152-158

Joseph J. Burrascano Jr. M.D.

Water Mill, NY USA



An interesting presentation on Lyme Disease with a PoTS diagnosis is here on Lyme Disease Action website.

A support group for patients with PoTS is here   

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