Light chain deposition in peripheral nerve as a cause of mononeuritis multiplex in Waldenström's macroglobulinaemia

Luigetti, Marco; Frisullo, Giovanni; Laurenti, Luca; Conte, Amelia; Madia, Francesca; Profice, Paolo; Batocchi, Anna Paola; Montano, Nicola; Tarnani, Michela; Tonali, P.; Sabatelli, Mario

doi:10.1016/j.jns.2010.01.018

Cited by 22 publications

(24 citation statements)

References 17 publications

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“…Multiple mononeuropathy was observed in 5/13 patients, with predominant involvement of the upper limbs in three (#1, 6, 7) and of cranial nerves in the other two (#2, 8). Symmetric polyneuropathy with predominant motor impairment was observed in 7/13 patients (#3, 4,5,9,10,11,13). The remaining one patient (#12) had painful neuropathy, characterized by shooting pain and burning in her lower limbs and hands, due to predominant small-fibre involvement, as previously described [16].…”

Section: Atypical Phenotypesupporting

confidence: 56%

“…We treated with glucocorticoids 10/13 patients (#2, 4,5,6,7,9,10,11,12,13), all of whom reported symptomatic improvement except for one (#12) who responded poorly, as we have previously reported [16]. Of the remaining 3/13 patients, two refused therapy (#1, 3) and one (#8) was treated with chemotherapy for an underlining WM with a good response [9].…”

Section: Atypical Phenotypementioning

confidence: 99%

“…However, the pathogenic mechanisms of IgM-related neuropathy are heterogeneous, including an autoimmune demyelinating process associated with anti-MAG (myelin associated glycoprotein) antibodies [8], endoneurial deposition of immunoglobulins or light chains [9][10][11], vasculitis [12], amyloid deposition [13] and infiltration by malignant cells [14].…”

Section: Introductionmentioning

confidence: 99%

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Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy

Luigetti

Conte

Montano

et al. 2012

Journal of the Neurological Sciences

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Section: Atypical Phenotypesupporting

confidence: 56%

Section: Atypical Phenotypementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical and pathological heterogeneity in a series of 31 patients with IgM-related neuropathy

Luigetti

Conte

Montano

et al. 2012

Journal of the Neurological Sciences

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“…Isolated ocular nerve palsies are rarely described during infections [26,27], but the negativity of all other diagnostic tests and the sudden onset of diplopia, which resolved completely in a few days, support our diagnosis. A third nerve palsy was the initial neurological sign in a patient with Waldenström's macroglobulinemia [28]. …”

Section: Discussionmentioning

confidence: 99%

A Prospective Study on 132 Cases of Ocular Palsy

et al. 2013

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Objectives: In this prospective study, we used one diagnostic protocol to establish an early diagnosis in patients with ocular palsies in absence of other neurological findings. Materials and Methods: The study was performed on a consecutive series of 132 patients who visited our Neurological Department for ptosis and/or diplopia in absence of other neurological signs, using the same diagnostic protocol. Results: An etiological diagnosis was made in 74% of cases during a mean time of 17 ± 23 months from symptom onset. Myasthenia gravis was the most common diagnosis (n = 60, 45.5%). Thirty-four cases (26%) remained undiagnosed in spite of a follow-up lasting 32 ± 33 months on average. Conclusions: Identifying the cause of an isolated ocular palsy can be difficult, and an extended follow-up time does not aid in further establishment of the diagnosis.

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“…Immunoglobulins Immunoglobulin deposits of any kind: IgG, IgA or IgM or light chains can only be proven by nerve biopsy [55,56]. These monoclonal dysglobulinemias are usually malignant (multiple myeloma, Waldenstrom's disease or non-Hodgkin's lymphoma).…”

Section: Deposits In the Interstitial Tissuementioning

confidence: 99%