Identifying a therapeutic window in acute and subacute inflammatory sensory neuronopathies

Antoine, Jean‐Christophe; Robert-Varvat, Florence; Maisonobe, Thierry; Créange, Alain; Franques, Jérôme; Mathis, Stéphane; Delmont, Émilien; Küntzer, Thierry; Lefaucheur, Jean‐Pascal; Pouget, Jean; Desnuelle, Claude; Echaniz‐Laguna, Andoni; Rotolo, Francesco; Camdessanché, Jean‐Philippe

doi:10.1016/j.jns.2015.12.044

Cited by 35 publications

(23 citation statements)

References 24 publications

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“…A case series of serial nerve conduction studies in patients with sensory neuronopathy suggests that sensory abnormalities plateau after 7-10 months from symptom onset. On the basis of the rate of decline of sensory response amplitudes, treatment should be started within the first eight months if possible 232. Beyond this window, the inflammatory reaction probably dampens and treatment becomes unsuccessful.…”

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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Section: Current Disease Specific Treatmentsmentioning

confidence: 99%

Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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“…Such diagnostic delay has become even more relevant for SN, as Antoine et al 12 recently published an electrodiagnostic-based study that argued in favor of an eight-month window from the disease onset as the ideal period for starting therapy to achieve SN stabilization. Moreover, even retrospective data pointing to a modest treatment response in Sjögren-related SN 13 , one should not generalize these findings to all SN-related diseases or even to the idiopathic ones that may benefit from an early diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Misdiagnosis and diagnostic delay in non-paraneoplastic sensory neuronopathies

Martinez

Ribeiro

Lima

et al. 2019

Arq. Neuro-Psiquiatr.

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Sensory neuronopathy (SN) is characterized by primary dorsal root ganglia damage and typically manifests as sensory ataxia with multifocal non-length-dependent sensory deficits 1,2 . First described in 1948 by Dr. Derek Denny-Brown 3 , the full phenotypic spectrum and the identification of all SN-related disorders are still pursued 4 . In a way rarely seen ABSTRACT Sensory neuronopathies (SN) are a group of peripheral nerve disorders characterized by multifocal non-length-dependent sensory deficits and sensory ataxia. Its recognition is essential not only for proper management but also to guide the etiological investigation. The uncommon SN clinical picture and its rarity set the conditions for the misdiagnosis and the diagnostic delay, especially in non-paraneoplastic SN. Therefore, our objectives were to characterize the diagnostic odyssey for non-paraneoplastic SN patients, as well as to identify possible associated factors. Methods: We consecutively enrolled 48 non-paraneoplastic SN patients followed in a tertiary neuromuscular clinic at the University of Campinas (Brazil). All patients were instructed to retrieve their previous medical records, and we collected the data regarding demographics, disease onset, previous incorrect diagnoses made and the recommended treatments. Results: There were 34 women, with a mean age at the diagnosis of 45.9 ± 12.2 years, and 28/48 (58%) of the patients were idiopathic. Negative sensory symptoms were the heralding symptoms in 25/48 (52%); these were asymmetric in 36/48 (75%) and followed a chronic course in 35/48 (73%). On average, it took 5.4 ± 5.3 years for SN to be diagnosed; patients had an average of 3.4 ± 1.5 incorrect diagnoses. A disease onset before the age of 40 was associated to shorter diagnosis delay (3.7 ± 3.4 vs. 7.8 ± 6.7 years, p = 0.01). Conclusions: These results suggest that diagnostic delay and misdiagnosis are frequent in non-paraneoplastic SN patients. As in other rare conditions, increased awareness in all the healthcare system levels is paramount to ensure accurate diagnosis and to improve care of these patients.

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“…The need for such rating instruments is especially important because the paper by Antoine and coworkers describing a therapeutic window beyond which there is a modest neurophysiological recovery in SN patients . Therefore, measuring the disease severity is paramount for the design of clinical trials.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3]7,8 The need for such rating instruments is especially important because the paper by Antoine and coworkers describing a therapeutic window beyond which there is a modest neurophysiological recovery in SN patients. 9 Therefore, measuring the disease severity is paramount for the design of clinical trials. There are several instruments devoted to rating symptoms in conditions that may resemble SN, however, their items have heavier weights for cerebellar ataxia signs, [10][11][12] length-dependent sensory deficits 13 or motor symptoms.…”

Section: Introductionmentioning

confidence: 99%

Sensory ataxia rating scale: Development and validation of a functional scale for patients with sensory neuronopathies

Martínez

Martins

et al. 2019

J Peripheral Nervous Sys

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Sensory neuronopathies (SN) result from dorsal root ganglia damage and manifest with a combination of sensory deficits and proprioceptive ataxia. Characterization of the natural history and development of therapeutic trials are hampered by the lack of clinical scales that capture the whole spectrum of SN‐related manifestations. We propose and validate a rating instrument for SN. Three experienced neuromuscular specialists developed items to rate SN. The resultant instrument was later validated by the assessment of the intra‐class correlation coefficient, for inter‐rater validity in 48 SN patients, and later in a smaller subset of 16 patients to assess its intra‐rater validity. Standardized Crombach's alpha and Oblimin rotation analysis were performed to verify internal consistency and items' relationship, respectively. Evaluation of Sensory Ataxia Rating Scale (SEARS)'s external validity was performed by comparison to: scale for the assessment and rating of ataxia (SARA), Beck balance scale (BBS), and INCAT sensory sum score (ISS). A 10‐item scale with an intra‐class correlation coefficient >0.95 for intra‐ and inter‐rating measurements with a good internal consistency (standardized Cronbach's alpha of 0.83) were observed. There was a normal distribution of the scores without a floor or ceiling effect. A moderate to good correlation between SEARS and SARA, BBS, and ISS was observed. SEARS is a reliable, easy‐to‐perform and consistent instrument to rate SN. Larger cohorts and multicenter studies are needed to validate its usefulness towards possible treatment trials.

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Identifying a therapeutic window in acute and subacute inflammatory sensory neuronopathies

Cited by 35 publications

References 24 publications

Diagnosis and management of sensory polyneuropathy

Diagnosis and management of sensory polyneuropathy

Misdiagnosis and diagnostic delay in non-paraneoplastic sensory neuronopathies

Sensory ataxia rating scale: Development and validation of a functional scale for patients with sensory neuronopathies

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