From
Exerpts from one study:
"Brain, Vol. 125, No. 8, 1887-1895, August 2002
© 2002 Guarantors of Brain
Phenotypic variants of autoimmune peripheral nerve hyperexcitability
Ian K. Hart1, Paul Maddison2, John Newsom-Davis2, Angela Vincent2 and Kerry R. Mills3
1 Neuroimmunology Group, University Department of Neurological Science, Walton Centre, Liverpool, 2 University Department of Clinical Neurology, Institute of Molecular Medicine, Oxford, 3 Department of Neurophysiology, King’s College Hospital, London, UK
Generalized peripheral nerve hyperexcitability (PNH) usually presents as spontaneous and continuous muscle overactivity.The clinical features of the motor nerve dysfunction are diverse and include cramps, muscle twitching (fasciculations or myokymia), stiffness, pseudomyotonia (delayed muscle relaxation after contraction) and pseudotetany (spontaneous or evoked carpal or pedal spasm, or Chvostek’s sign) (Isaacs, 1961; Mertens and Zschocke, 1965; Tahmoush et al., 1991). Some patients experience paraesthesias and numbness implying sensory nerve involvement, or hyperhydrosis that might represent autonomic dysfunction or be secondary to muscle overactivity. There may also be associated CNS disorders such as mood change, sleep disorder or hallucinations(reviewed in Newsom-Davis and Mills, 1993; Serratrice and Azulay, 1994; Barber et al., 2000; Liguori et al., 2001).
Associated immune-related diseases
Autoimmune disorders occurred more frequently than would be expected by chance in both groups. Myasthenia gravis was present in 21% of group A and 12% of group B (Table 3). Manifestations of muscle overactivity appeared at the same time as myasthenia in six out of 11 patients and between 4 to 34 years after the onset of myasthenia in the other five patients
Psychiatric features
Eleven group A patients (26%) reported psychiatric symptoms—most commonly isolated mild personality change, insomnia or irritability. One patient also had visual hallucinations and one developed a delusional state. Five of them had raised serum VGKC antibody titres and the two patients with the highest serum titres in our series of 60 patients were the two group A patients with the most florid psychiatric symptoms. Eight of these 11 group A patients who underwent CSF analysis had a mildly raised total protein, but no oligoclonal bands were detected.
A similar proportion of group B patients (22%) reported mild psychiatric symptoms. One of these patients also had vivid dreams and only in this patient was the VGKC titre raised.
Although overall only 35% of patients had raised VGKC antibody titres detected by the 125I--dendrotoxin immunoprecipitation assay, this is likely to be an underestimate. We have previously discussed the relative insensitivity of this assay compared with a molecular-immunohistochemical assay that detects serum binding to frozen sections of Xenopus oocytes injected beforehand with cRNA for an individual VGKC subunit (Hart et al., 1997).
Our EMG data suggest that, in many patients, PNH was more evident distally than proximally. This is in keeping with the previous evidence from detailed peripheral nerve excitability studies of 20 of the patients reported here that spontaneous activity is often generated focally or multifocally at sites distant from the recording electrode over the trunk of the nerve (Maddison et al., 1999; Kiernan et al., 2001). Thus, our present findings together with the evidence provided by these excitatory techniques and previous histological and conventional electrophysiological findings (Isaacs, 1967; Deymeer et al., 1998; Newsom-Davis and Mills, 1993; Vincent, 2000) suggest that, in most patients, the likely locus for the generation of spontaneous discharges is at the motor nerve terminal or intramuscular arborization. This was proposed originally by Isaacs (1961). At these sites, there are VGKCs producing fast K+ currents unprotected by either the blood/nerve barrier or myelin sheath, and thus potentially more vulnerable to antibody-mediated autoimmune attack.
Thus, acquired forms are classed as autoimmune, toxic or degenerative, and hereditary forms are defined by their underlying genetic disorder. This classification has the added advantage of being flexible. It can accommodate new information about the molecular pathogenesis of the syndromes without the need for a fundamental reclassification and should facilitate the testing of new therapies. "
Personally, if I have my choice of taking advice from kevintwister or from Ian K. Hart1 Paul Maddison2, John Newsom-Davis2, Angela Vincent2 and Kerry R. Mills3 1 Neuroimmunology Group, University Department of Neurological Science, Walton Centre, Liverpool, 2 University Department of Clinical Neurology, Institute of Molecular Medicine, Oxford, 3 Department of Neurophysiology, King’s College Hospital, London, UK
I know who I would chose. I like dealing in reality. The reality is that there is a lot of scientifuc evidence beyond this one study that there is an autoimmune component to this disorder fo a number of us. I find that reassuring myself. I don't like long lists of "anxiety" symptoms with no basis in either fact or research. That helps no one with BFS/BCFS/PNH in the long run, though it might help those suffering strictly from health anxiety, of which I'm not one. There are a lot of conditions that have physical symptoms and unproven, though scientifically examined causes, like my husband's arthritis.
To each his own, but success to me means dealing with the facts so that I can be truely better, both physically and mentally, in the long term, not just an emotional short term fix.
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) in a better place. But till then, may you all find great joy in the little miracles of every day here in this world.
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