Canomad presenting without ophthalmoplegia and responding to intravenous immunoglobulin

Kam, Cheryl; Balaratnam, Michelle; Purves, Alistair; Mills, Kerry; Riordan-Eva, Paul; Pollock, Steve; Bódi, István; Munro, Nancy; Bennett, David L.H.

doi:10.1002/mus.22167

Cited by 21 publications

(19 citation statements)

References 26 publications

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“…A further peculiarity of this case lies in the opportunity to substantiate the role of IgM monospecific to GD1b in peripheral nerve pathology, differently from most of previous pathologic studies where IgM reactivity was extended to other b‐series gangliosides, sulfatides, or other gangliosides lacking disialosyl epitope. Pathologic investigations on nerve tissues in CANOMAD/CANDA are still very limited.…”

Section: Discussionmentioning

confidence: 79%

“…Apart from a single case showing a few endoneurial inflammatory cells, no inflammation or macrophagic activation was detected. Intriguingly, in cases in which nerve tissues were investigated by immunocytochemistry, no immunoglobulin or complement deposition have been found …”

Section: Discussionmentioning

confidence: 99%

“…Intriguingly, the concomitant association between CLL and chronic ataxic neuropathy with antiganglioside antibodies represents a novel finding in an otherwise rare and disabling disorder. In addition, MRI detection of cauda equina and cervical roots involvement at early disease stages are unreported features in CANOMAD/CANDA, mainly due to the lack of imaging studies, except for reports describing negative findings at brain and spine MRI in two patients, and multifocal demyelinating lesions of central white matter in 2/8 brain MRI in patients lacking oligoclonal bands …”

Section: Discussionmentioning

confidence: 99%

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Myelin uncompaction and axo‐glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b

Tagliapietra

Zanusso

Ferrari

et al. 2019

J Peripheral Nervous Sys

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To describe clinical features, disease course, treatment response, and sural nerve biopsy findings in a patient with chronic sensory ataxic neuropathy, Binet stage A chronic lymphocytic leukemia, and monoclonal IgMλ paraprotein against ganglioside GD1b. During 9 months of hospitalization at two neurologic centers, the patient underwent serial neurologic examinations, neurophysiologic studies, imaging investigations, extensive laboratory work‐up, bone marrow, and sural nerve biopsies. The patient had a severe progressive sensory neuropathy accompanied by motor involvement, dysautonomia, and marked bulbar weakness with preserved ocular movements. Conduction studies were characterized by prolonged F‐wave minimal latencies, prolonged distal latencies, reduction of compound motor action potentials, and absence of sensory nerve action potentials. Sural nerve biopsy showed endoneurial edema, axonal degeneration, and regeneration, in the absence of cellular inflammation, macrophagic activation, and B‐lymphocyte infiltration; no IgM or complement deposition was detected. Myelinated fibers showed redundant/abnormally thickened myelin, myelin vacuolation, and frank intramyelinic edema with condensed axoplasm. Ultrastructural features included axo‐glial detachment, disruption of membrane integrity, and myelin uncompaction. This study shows that monospecific anti‐GD1b IgM paraprotein is associated with non‐inflammatory nerve damage. We suggest that the loss of myelin and axonal integrity reflects antibody‐induced disruption of membrane lipid rafts.

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Myelin uncompaction and axo‐glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b

Tagliapietra

Zanusso

Ferrari

et al. 2019

J Peripheral Nervous Sys

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“…Data to guide treatment in these patients are limited 221222223224. In case series, intravenous immunoglobulins have been used with some success,119 whereas rituximab was the most effective treatment in one small cohort of patients, halting disease in eight of nine patients 117…”

Section: Current Disease Specific Treatmentsmentioning

confidence: 99%

Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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Sensory polyneuropathies, which are caused by dysfunction of peripheral sensory nerve fibers, are a heterogeneous group of disorders that range from the common diabetic neuropathy to the rare sensory neuronopathies. The presenting symptoms, acuity, time course, severity, and subsequent morbidity vary and depend on the type of fiber that is affected and the underlying cause. Damage to small thinly myelinated and unmyelinated nerve fibers results in neuropathic pain, whereas damage to large myelinated sensory afferents results in proprioceptive deficits and ataxia. The causes of these disorders are diverse and include metabolic, toxic, infectious, inflammatory, autoimmune, and genetic conditions. Idiopathic sensory polyneuropathies are common although they should be considered a diagnosis of exclusion. The diagnostic evaluation involves electrophysiologic testing including nerve conduction studies, histopathologic analysis of nerve tissue, serum studies, and sometimes autonomic testing and cerebrospinal fluid analysis. The treatment of these diseases depends on the underlying cause and may include immunotherapy, mitigation of risk factors, symptomatic treatment, and gene therapy, such as the recently developed RNA interference and antisense oligonucleotide therapies for transthyretin familial amyloid polyneuropathy. Many of these disorders have no directed treatment, in which case management remains symptomatic and supportive. More research is needed into the underlying pathophysiology of nerve damage in these polyneuropathies to guide advances in treatment.

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“…Similar to POEMS syndrome, there is a male predominance and age of onset is typically in the 6th decade [48]. The neuropathy is often severe, but tends to be more slowly progressive than in POEMS syndrome, and predominantly sensory, often with a disabling sensory ataxia, and either demyelinating or axonal features on electrodiagnostic testing [48, 50]. …”

Section: Patient Evaluation and Investigationsmentioning

confidence: 99%

POEMS syndrome: clinical update

Brown

Ginsberg

2018

J Neurol

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.

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Canomad presenting without ophthalmoplegia and responding to intravenous immunoglobulin

Cited by 21 publications

References 26 publications

Myelin uncompaction and axo‐glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b

Myelin uncompaction and axo‐glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b

Diagnosis and management of sensory polyneuropathy

POEMS syndrome: clinical update

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