HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

Correspondence to:

BRIEF COMMUNICATIONS: K. Geleijns, B. A. Brouwer, B. C. Jacobs, J. J. Houwing-Duistermaat, C. M. van Duijn, and P. A. van Doorn The occurrence of Guillain-Barré syndrome within families Neurology 2004; 63: 1747-1750 [Abstract] [Full text] [PDF]

Correspondence: Submit a response to this article

Correspondence published:

Reply to Korn-Lubetzki et al

Karin Geleijns, Bart C. Jacobs, Pieter A. van Doorn   (21 January 2005)

The occurrence of Guillain-Barré syndrome within families

Isabelle Korn-Lubetzki, Israel Steiner   (21 January 2005)

Reply to Korn-Lubetzki et al 21 January 2005
Karin Geleijns, Erasmus MC, Dept. of Neurology Dr. Molewaterplein 50, room Ee2234, 3015 GE Rotterdam, The Netherlands, Bart C. Jacobs, Pieter A. van Doorn Send Correspondence to journal: Re: Reply to Korn-Lubetzki et al c.geleijns{at}erasmusmc.nl Karin Geleijns, et al.	We thank Korn-Lubetzki et al for their interest in our article. [1] They concluded that the 17p12 deletion responsible for hereditary neuropathy with liability pressure palsy (HNPP) was also present in a family in which three members had an inflammatory demyelinating poyneuropathy (IDP). Two members fulfilled the criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) and the other for acute inflammatory demyelinating polyneuropathy (AIDP). [2] This finding may indicate that CIDP and this deletion may also be present in our recently reported Dutch families in which two or more members had Guillain-Barré syndrome. [1] In this study, we excluded two families in which one or more members had a subacute or chronic IDP in response to the Editor's opinion. In one of these families, a 79-year- old grandmother developed a subacute IDP after an upper respiratory tract infection and her grandson developed a chronic IDP at age 20 with a time interval of five years. In the other family, two cousins were affected; one had an acute and the other one a subacute sensomotoric IDP without preceding events and with a time interval of 14 years. None of them reported a recurrent episode, similar to the family reported by Korn-Lubetzki et al. [2] We also know a family in which two brothers have CIDP but lack the 17p12 deletion. Nevertheless, it would be interesting to screen all our families with IDP for the 17p12 deletion to further elucidate the pathogenesis of these disorders. Screening for the 17p12 duplication or other Schwann- cell related genes like myelin protein zero (P0) or connexin 32 (CX32) that are involved in other hereditary demyelinating neuropathies would further clarify the relationship between inflammatory and hereditary polyneuropathies. [3] In the case of a positive finding within our families, it would be of further interest to screen for these mutations within our larger cohort of non-familial GBS patients. [4] References 1. Geleijns K, Brouwer BA, Jacobs BC, Houwing-Duistermaat JJ, van Duijn CM, van Doorn PA. The occurrence of Guillain-Barré syndrome within families. Neurology 2004;63:1747-1750. 2. Korn-Lubetzki I, Argov Z, Raas-Rothschild A, Wirguin I, Steiner I. Family with inflammatory demyelinating polyneuropathy and the HNPP 17p12 deletion. Am J Med Genet 2002;113:275-278. 3. Martini R, Toyka KV. Immune-mediated components of hereditary demyelinating neuropathies: lessons from animal models and patients. Lancet Neurol 2004;3:457-465. 4. Geleijns K, Jacobs BC, Van Rijs W, Tio-Gillen AP, Laman JD, van Doorn PA. Functional polymorphisms in LPS receptors CD14 and TLR4 are not associated with disease susceptibility or Campylobacter jejuni infection in Guillain-Barré patients. J Neuroimmunol 2004;150:132-138.
The occurrence of Guillain-Barré syndrome within families 21 January 2005
Isabelle Korn-Lubetzki, Shaare Zedek Medical Center PO Box 3235, Jerusalem 94342, Israel, Israel Steiner Send Correspondence to journal: Re: The occurrence of Guillain-Barré syndrome within families ikl{at}md.huji.ac.il Isabelle Korn-Lubetzki, et al.	We read with interest the report by Geleijns et al describing the occurrence of Guillain-Barré syndrome (GBS) in at least two members in each of 12 Dutch families. [1] In order to perform the study, a letter was sent to known GBS or chronic inflammatory demyelinating polyneuropathy (CIDP) patients asking them whether they had a relative with GBS or CIDP. Patients with CIDP were eventually excluded from the study. Based on the occurrence of GBS within siblings, and an earlier onset of GBS in successive generations, they suggest a role for genetic factors in the pathogenesis of GBS. They conclude that GBS is a complex genetic disorder with an outcome determined by environmental and genetic factors. We reported a father with chronic inflammatory demyelinating neuropathy (CIDP) and a daughter who developed AIDP a year later. [2] Several years later, a second daughter also developed CIDP. We then considered that the underlying genetic abnormality in all three members might be the 17p12 deletion responsible for hereditary neuropathy with liability to pressure palsy (HNPP). The mutation was discovered in this family. [3] Two of our observations may be relevant to Geleijns et al’s article. The HNPP mutation might be a predilection for the development of GBS and of CIDP. Therefore, when a genetic component is suspected in these conditions, screening for HNPP might be warranted. Including CIDP in this study might have increased the number of families with more than one member affected with an immune demyelinating neuropathy. Many clinical similarities exist between AIDP and CIDP and both may eventually share also some pathogenetic features. [4] We suggest that all the Dutch patients with familial GBS, including familial CIDP, should be screened for the HNPP deletion. Finding further cases positive for the deletion will clarify the complex relationship of inflammatory and hereditary neuropathies. [5] References 1. Geleijns K, Brouwer BA, Jacobs BC, Houwing-Duistermaat JJ, van Duijn CM, van Doorn PA. The occurrence of Guillain-Barre syndrome within families. Neurology 2004; 63: 1747-1750. 2. Korn-Lubetzki I, Steiner I, Brenner T, Brautbar C, Argov Z. Familial inflammatory demyelinating polyneuropathy: a Guillain Barre syndrome variant without autoimmune predilection. J Neurol Neurosurg Psych 1994; 57: 1008-1009. 3. Korn-Lubetzki I, Argov Z, Raas-Rothschild A, Wirguin I, Steiner I. Family with inflammatory demyelinating polyneuropathy and the HNPP 17p12 deletion. Am J Med Genet 2002; 113: 275-278. 4. Vedeler CA. Inflammatory neuropathies: update. Curr Opin Neurol 2000; 13: 305-309. 5. Martini R, Toyka KV. Immune-mediated components of hereditary demyelinating neuropathies: lessons from animal models and patients. Lancet Neurol 2004; 3: 457-465.