Dietary essential fatty acids, vitamin E, and Charcot‐Marie‐Tooth disease

Williams, Lowell L.; O'Dougherty, Margaret; Wright, Francis S.; Bobulski, Rosalind J.; Horrocks, Lloyd A.

doi:10.1212/wnl.36.9.1200

Cited by 16 publications

(8 citation statements)

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“…A pos sible genetic predisposition is supported by the reported association of CIDP with the HLA-A|, Bg and DW3 haplotypes [27] and the observation that certain inbred strains of rats show different genetic susceptibility to EAN [28]. Furthermore, the recently found alterations of serum fatty acids in patients with HMSN may also affect immunoregulation [29], Accordingly, the typical pheno typic expression of peroneal muscular atro phy may be altered by environmental factors triggering an autoimmune response.…”

mentioning

confidence: 96%

Response to Immunosuppressive Therapy in Patients with Hereditary Motor and Sensory Neuropathy and Associated Dysimmune Neuromuscular Disorders

1987

Eur Neurol

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We encountered 2 patients with hereditary motor and sensory neuropathy (HMSN) type I who had marked weakness developing during several months superimposed on chronic peroneal muscular atrophy. Further studies disclosed a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in one patient and CIDP associated with polymyositis in the other. Both patients responded to prednisone and azathioprine with substantial improvement. Patients with HMSN who develop rapid progression of weakness should be evaluated for superimposed, potentially treatable dysimmune neuromuscular disorders.

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confidence: 96%

Response to Immunosuppressive Therapy in Patients with Hereditary Motor and Sensory Neuropathy and Associated Dysimmune Neuromuscular Disorders

1987

Eur Neurol

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“…Although clearly not specific for EBV infection (2)(3)(4)(5)(6)20), an abnormally low ratio of serum linoleic to oleic acid appears after clinical recovery in IM. Abnormal proportions of these major FA appear to be associated with increased clinical symptoms in some post-IM patients and other chronic diseases {2, 3,20).…”

Section: Discussionmentioning

confidence: 98%

“…In addition, because our method also extracts shorter-chain FA (2,20), the significantly high proportions of 10:0-14:1 FA may represent breakdown products of longer~hain FA (10). Low total arachidonic acid, as the FA precursor of immunoregulatory metabolites, has been associated with increased amounts of prostaglandin and leukotriene formation (10,20}.…”

Section: Discussionmentioning

confidence: 99%

“…Age and sex-matched normals (20) who had no evidence of EBV infection for at least 4 yr were similarly followed. Normal college students, 10 male and 10 female, aged 18-23, with an acute illness characterized by severe pharyngitis, generalized lymphadenopathy, fever, prostration and a positive heterophile antibody test, voluntarily participated.…”

Section: Methodsmentioning

confidence: 99%

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Serum fatty acid proportions are altered during the year following acute Epstein‐Barr virus infection

Williams

Doody

Horrocks

1988

Lipids

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Because abnormal serum fatty acid (FA) proportions had been found at three months after infectious mononucleosis (IM) in a pilot study, serum total FA profiles of 20 normal college students were measured at monthly intervals for one year following an acute Epstein-Barr virus (EBV) infection. Below normal proportions of arachidonic acid and a reversal of the usual serum ratio of linoleic and oleic acids were maximal during the third month after acute IM. These FA abnormalities coincided with the symptom of increased physical malaise, despite apparent clinical recovery, common after IM. Persistence of low linoleic acid content beyond six months postinfection occurred in all seven students who showed continued clinical symptoms. Estimation of FA enzyme activities over the post-IM year suggested that FA elongation function was normal, but that FA desaturation enzyme activities were lower than normal, particularly early after EBV infection. An inability of the host to normalize the serum total linoleic/oleic acid ratio may parallel a delayed recovery from EBV infection and may offer insight into its pathogenesis.

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“…No effect of essential fatty acids was noted at 12 months; however, after a lead-in period of 3 months of vitamin E containing placebo, improvements in neurologic disability measures were seen, which were sustained for 1 year. 11 It was unclear whether this improvement reflected a sustained placebo effect, or therapeutic benefit of vitamin E. 11 A single, double-blind, placebo-controlled cross-over study of low-dose coenzyme Q10 (CoQ10) (100 mg/day) enrolled patients with several forms of muscular dystrophy, and also clinically defined CMT; benefit was reported among the three CMT subjects included. 12 …”

Section: Review Of Current Treatment Options For Cmt and Past Therapementioning

confidence: 99%

Experimental Therapeutics in Hereditary Neuropathies: The Past, the Present, and the Future

Herrmann

2008

Neurotherapeutics

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Summary:Hereditary neuropathies represent approximately 40% of undiagnosed neuropathies in a tertiary clinic setting. The Charcot-Marie-Tooth neuropathies (CMT) are the most common. Mutations in more than 40 genes have been identified to date in CMT. Approximately 50% of CMT cases are accounted for by CMT type 1A, due to a duplication within the peripheral myelin protein 22 gene (PMP22). Mutations in the gap junction beta 1 gene (GJB1), the myelin protein zero gene (MPZ), and the mitofusin 2 gene (MFN2) account for a substantial proportion of other genetically definable CMT. Some 15% of demyelinating CMT and 70% of axonal CMT await genetic clarification. Other hereditary neuropathies include the hereditary sensory and autonomic neuropathies, the familial amyloid polyneuropathies, and multisystem disorders (e.g., lipid storage diseases and inherited ataxias) that have peripheral neuropathy as a major or minor component. This review surveys the challenges of developing effective therapies for hereditary neuropathies in terms of past, present, and future experimental therapeutics in CMT.

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Dietary essential fatty acids, vitamin E, and Charcot‐Marie‐Tooth disease

Cited by 16 publications

References 0 publications

Response to Immunosuppressive Therapy in Patients with Hereditary Motor and Sensory Neuropathy and Associated Dysimmune Neuromuscular Disorders

Response to Immunosuppressive Therapy in Patients with Hereditary Motor and Sensory Neuropathy and Associated Dysimmune Neuromuscular Disorders

Serum fatty acid proportions are altered during the year following acute Epstein‐Barr virus infection

Experimental Therapeutics in Hereditary Neuropathies: The Past, the Present, and the Future

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