Chronic ataxic neuropathy mimicking dorsal midbrain syndrome

Arbogast, S D; Khanna, Sangeeta; Koontz, None Daniel; Tomsak, Robert L.; Katirji, Bashar; Leigh, R. John

doi:10.1136/jnnp.2007.120444

Cited by 17 publications

(11 citation statements)

References 4 publications

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“…Accurate diagnosis of immune‐mediated neuropathies is important as several forms are potentially treatable. Our findings confirm previous studies reporting that IgM anti‐GD1b antibodies are more often present in chronic immune‐mediated neuropathies presenting with sensory ataxic neuropathy and chronic ataxic neuropathy, ophthalmoplegia, monoclonal IgM protein, cold agglutinins, and disialosyl antibodies (CANOMAD), while IgG anti‐GD1b antibodies are more often found in patients with an acute immune‐mediated neuropathy . As IgG anti‐GD1b antibodies were not found in the chronic neuropathies or AID control group, this antibody may be considered as a useful diagnostic biomarker for acute immune‐mediated neuropathies.…”

Section: Discussionsupporting

confidence: 90%

“…more often found in patients with an acute immune-mediated neuropathy. 12,27 As IgG anti-GD1b antibodies were not found in the chronic neuropathies or AID control group, this antibody may be considered as a useful diagnostic biomarker for acute immune-mediated neuropathies.…”

Section: Gbs Patient Characteristics Related To the Presence Of Sermentioning

confidence: 99%

“…9,11 Serum anti-GD1b antibodies have been reported in patients with acute or chronic forms of ataxic neuropathy. [12][13][14] In a subgroup of patients, anti-GD1b antibodies may cross-react to similar glycan structure of either GM1 (terminal Gal-GalNAc moiety) or GQ1b (disialosyl moiety). 15 Comparative studies on the anti-GD1b antibody frequencies in immune-mediated neuropathies and controls are limited, and the diagnostic and prognostic value of these antibodies is undefined.…”

Section: Introductionmentioning

confidence: 99%

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Clinical relevance of serum antibodies to GD1b in immune‐mediated neuropathies

Taams

Notermans

Fokkink

et al. 2018

J Peripheral Nervous Sys

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Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60). All samples were tested by enzyme-linked immunosorbent assay according to the Inflammatory Neuropathy Cause and Treatment protocol. IgM anti-GD1b antibodies were found in GBS (N = 4; 2.4%), MGUS (N = 3; 3.0%), and PNS patients (N = 1; 9.1%). IgG anti-GD1b antibodies were found in GBS (N = 20; 12.1%) and MF-GBS (N = 4; 14.3%) patients, but not in the AID and HC group. In the combined group of MF-GBS and GBS patients ((MF-)GBS), 14/36 (38.9%) patients with IgG anti-GD1b antibodies also had IgG anti-GM1 antibodies, and IgG anti-GD1b and IgG anti-GQ1b antibodies were found in 3/29 (10.3%) patients. Patients with (MF-)GBS and anti-GD1b without anti-GM1 antibodies did not differ regarding sensory disturbances or disease severity but recovered faster regarding the ability to walk independently compared with patients without anti-GD1b antibodies (P = 0.031) and with patients with both anti-GD1b and anti-GM1 antibodies (P = 0.034). In conclusion, testing for anti-GD1b antibodies may identify a specific group of immune-mediated neuropathies and (MF-)GBS patients with only anti-GD1b antibodies tend to recover faster.

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Section: Discussionsupporting

confidence: 90%

Section: Gbs Patient Characteristics Related To the Presence Of Sermentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Clinical relevance of serum antibodies to GD1b in immune‐mediated neuropathies

Taams

Notermans

Fokkink

et al. 2018

J Peripheral Nervous Sys

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“…Pathologic investigations on nerve tissues in CANOMAD/CANDA are still very limited. Neuropathologic findings described in previous reports include moderate to severe loss of large fibers, regeneration clusters, or early onion bulb formation . Apart from a single case showing a few endoneurial inflammatory cells, no inflammation or macrophagic activation was detected.…”

Section: Discussionmentioning

confidence: 61%

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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“…There is also significant overlap with the Miller Fisher Syndrome (MFS) [ 3 , 4 ]. The ideal treatment for CANOMAD remains unknown [ 1 , 5 , 6 ]. We describe a patient with CANOMAD who responded well to intravenous immunoglobulins (IVIG) and has been maintained on this treatment, without further exacerbations for over 12 months, providing support for the use of this immunomodulatory therapy for CANOMAD.…”

Section: Introductionmentioning

confidence: 99%

Facial Involuntary Movements and Respiratory Failure in CANOMAD, Responsive to IVIG Therapy

Johnson

Malkan

Shaffi

2015

Case Reports in Medicine

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CANOMAD is a rare chronic neuropathy, characterized by chronic sensory ataxia and intermittent brain stem symptoms due to antidisialosyl antibodies. The disorder results in significant morbidity but is poorly understood and often misdiagnosed. We describe a unique case of CANOMAD, associated with involuntary movements of the face; patient reported exacerbations with citrus and chocolate and respiratory muscle weakness. Our patient was initially misdiagnosed with Miller Fisher Syndrome, highlighting the need for vigilance should neurological symptoms recur in patients initially diagnosed with a Guillain Barre variant. Moreover, the optimal treatment is unknown. This patient responded remarkably to intravenous immunoglobulin and has been maintained on this treatment, without further exacerbations.

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Chronic ataxic neuropathy mimicking dorsal midbrain syndrome

Cited by 17 publications

References 4 publications

Clinical relevance of serum antibodies to GD1b in immune‐mediated neuropathies

Clinical relevance of serum antibodies to GD1b in immune‐mediated neuropathies

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

Facial Involuntary Movements and Respiratory Failure in CANOMAD, Responsive to IVIG Therapy

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