Definition and diagnosis of small fiber neuropathy: consensus from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology

Gondim, Francisco de Assis Aquino; Barreira, Amilton Antunes; Claudino, Rinaldo; Cruz, Márcia Waddington; Cunha, Francisco Marcos Bezerra da; Freitas, Marcos R. G. de; França, Marcondes Cavalcante; Gonçalves, Marcus Vinícius Magno; Marques, Wilson; Nascimento, Osvaldo J. M.; Oliveira, Acary Souza Bullé; Pereira, Raquel Campos; Pupe, Camila; Rotta, Francisco Tellechea; Schestatsky, Pedro

doi:10.1590/0004-282x20180015

Cited by 17 publications

(18 citation statements)

References 63 publications

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“…Depending on the specific cause, that is whether it is a degenerative neurological disease or a non‐neurological chronic disorder, neurogenic OH can be further divided into primary and secondary, respectively. In secondary neurogenic OH, diseases like diabetes, chronic kidney disease (possibly even in the absence of diabetes or other OH risk factors), autoimmune diseases, endocrine disturbances, increased alcohol intake, and paraneoplastic syndromes can cause OH due to impairment of autonomic nerves . The main causes of all types of OH are shown in Figure .…”

Section: Pathophysiologymentioning

confidence: 99%

Orthostatic hypotension: From pathophysiology to clinical applications and therapeutic considerations

Magkas

Tsioufis

Thomopoulos

et al. 2019

J of Clinical Hypertension

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Orthostatic hypotension (OH), that is blood pressure fall when standing from the supine to the erect position, is a common cardiovascular disorder, highly prevalent in elderly and frail individuals and in patients with multiple comorbidities. Orthostatic hypotension is considered a manifestation of dysfunction of the autonomic nervous system, caused or facilitated by several neurological or non-neurological diseases and conditions, while its clinical significance is increasingly recognized as a cause of impairment of quality of life and potentially of worse outcomes. Indeed, OH has been extensively studied and numerous prospective cohort studies support its association with adverse events, including coronary artery disease, heart failure, stroke, cognitive dysfunction, and, most importantly, mortality rates. Specific pharmacological and non-pharmacological interventions have been established for the treatment of OH. However, randomized data evaluating the impact of therapeutic interventions on morbidity and mortality outcomes are lacking. Thus, despite that OH seems to have important prognostic implications indicated by several reported associations with adverse events, it remains unclear whether OH treatment could improve prognosis. In the present review, we discuss the clinical applications associated with ΟΗ by outlining the current perspectives on ΟΗ definition, diagnosis, pathophysiology, prognostic role, and treatment.

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Section: Pathophysiologymentioning

confidence: 99%

Orthostatic hypotension: From pathophysiology to clinical applications and therapeutic considerations

Magkas

Tsioufis

Thomopoulos

et al. 2019

J of Clinical Hypertension

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“…Laboratory autonomic testing (including quantitative sudomotor axonal sensory testing [QSART]) and CCM are also not widely available, making their routine incorporation as part of a diagnostic criteria challenging and explaining in part why autonomic testing was used either as a mandatory or optional criterion in 11% of the studies. Only one review article recommended the use of CCM as a diagnostic criterion for SFN 29 …”

Section: Discussionmentioning

confidence: 99%

Diagnostic criteria for idiopathic small fiber neuropathy: A systematic review

et al. 2020

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Idiopathic small fiber neuropathy (iSFN) lacks broadly accepted diagnostic criteria, which hinders its timely diagnosis and treatment. A systematic literature review was performed to assess the published screening and diagnostic criteria for iSFN, excluding studies where SFN was of well‐established etiology. Eighty‐four clinical studies and seven guideline/review publications were included in this systematic review. Substantial heterogeneity existed in iSFN diagnostic criteria. The most common set of criteria to diagnose iSFN [presence of any symptoms of iSFN, absence of large fiber involvement, and reduced intraepidermal nerve fiber density (IENFD)] was used in only 14% of studies. Mandatory individual criteria to confirm iSFN included any sensory symptoms (60% of studies), pain (19% of studies), small fiber signs (20% of studies), absence of large fiber signs (62% of studies), reduced IENFD (38% of studies), and autonomic symptoms (1% of studies). This review highlights a clear need for standardized, evidence‐based guidelines for diagnosing iSFN.

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“…As Gondim et al 2 correctly point out in their manuscript, the use of relatively sophisticated electrodiagnostic techniques has led us to uncover a larger number of patients with SFN. Electrodiagnostic testing has an important task in the characterization of the nerve lesion causing SFN.…”

Section: In This Issuementioning

confidence: 99%

Challenges in the diagnosis and treatment of small fiber neuropathies

Valls‐Solé

2018

Arq. Neuro-Psiquiatr.

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S mall fiber neuropathies (SFN) are challenging at the level of the diagnosis, treatment and follow-up. Patients usually complain of severe pain, mostly in their feet, greatly affecting their quality of life, while the examiner finds no, or very few, clinical signs of peripheral neuropathy. Conventional electrodiagnostic testing does not give much additional information and, in idiopathic cases, routine blood tests do not show any abnormality. What to do in these cases? Symptomatic treatment is also often disappointing. Antiepileptic drugs, serotonin reuptake inhibitors and tricyclic antidepressants are the first line of treatment 1 . They may improve 30% in the rating of pain scores in about 30% of the patients, which is not much different from the effect of placebo. Being a little more aggressive with pharmacological treatment leads to opioids, which may produce side effects, and invasive treatments such as neural blockade, spinal cord stimulation, intrathecal medication, and neurosurgical interventions, which are elected by some patients impelled by the severity of their symptoms.On this topic, as in many others, a correct diagnosis is more than half the medical action to be taken. A thorough explanation to the patient about the nature of the problem, the origin of his/her symptoms, the benefits and side effects of the available medication and the reassurance of close follow-up are the most logical steps to complete the medical act. However, symptoms may continue in spite of all the care. It is in this situation that we need guidelines and consensus papers, like that by Gondim et al., in this issue 2 . Experience from other experts in the field becomes very handy when we, and our patients, need reassurance in the diagnosis. In fact, the syndromic diagnosis of SFN is not a difficult one to reach on the basis of clinical assessment. It is more difficult to have laboratory support of the diagnosis, as conventional electrodiagnostic methods are insufficient and more sophisticated equipment is not accessible to all 3 . It is even more difficult to reach an etiological diagnosis. The percentage of unknown causes of SFN remains at about 30% of the patients, in spite of advancement in the tools used for the diagnosis 4 . In those with known causes, impaired glucose metabolism, chronic inflammatory demyelinating polyneuropathy, and monoclonal gammopathies are the disorders most frequently observed. The importance of a complete blood test for an etiological diagnosis has been stressed by Lang et al.5 . These authors determined the prevalence of each abnormal blood test result among a battery of 21 tests. The most prevalent abnormalities were a high erythrocyte sedimentation rate and antinuclear antibodies, each present in 28% of 231 patients with skin biopsy confirmation of SFN. Apart from that, a number of other antibodies with elevated titers were found, which led the authors to suggest an association between SFN and dysimmunity.As Gondim et al.2 correctly point out in their manuscript, the use of relatively sophisti...

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Definition and diagnosis of small fiber neuropathy: consensus from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology

Cited by 17 publications

References 63 publications

Orthostatic hypotension: From pathophysiology to clinical applications and therapeutic considerations

Orthostatic hypotension: From pathophysiology to clinical applications and therapeutic considerations

Diagnostic criteria for idiopathic small fiber neuropathy: A systematic review

Challenges in the diagnosis and treatment of small fiber neuropathies

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