Adult CSF total protein upper reference limits should be age-partitioned and significantly higher than 0.45 g/L: a systematic review

Breiner, Ari; Moher, David; Brooks, John; Cheng, Wei; Hegen, Harald; Deisenhammer, Florian; McCudden, Christopher R.; Bourque, Pierre R.

doi:10.1007/s00415-018-09174-z

Cited by 47 publications

(48 citation statements)

References 33 publications

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“…Several studies have shown that the widely used cutoff of 0.45 g/L is erroneously low [34,35]. Implementation of CSF values of 0.50 g/L for patients aged B 50 years and 0.60 g/L for those aged [ 50 years have been proposed as a better standard CSF reference value [36]. Adoption of these higher age-dependent reference values in a population of CIDP patients was shown to improve CIDP diagnostic specificity, without a meaningful compromise in diagnostic sensitivity [37].…”

Section: Laboratory Pitfallsmentioning

confidence: 99%

The Misdiagnosis of CIDP: A Review

Allen

2020

Neurol Ther

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There is a growing realization that many patients are incorrectly diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), with at least half of patients that carry a diagnosis of CIDP in the USA possibly having a different explanation for their neuropathy or having no neuropathy at all. Many misdiagnosed patients go on to receive costly and potentially harmful treatments for a disease that they do not have, while at the same time missing an opportunity to treat their true ailment. The cost of misdiagnosis on patients and society is not trivial. Many factors contribute to misdiagnosis. Particular points of vulnerability include the evaluation of ''atypical'' CIDP, interpretation of equivocal nerve conduction studies, over-reliance on elevations in cerebrospinal fluid protein concentration in indeterminate ranges, and placing excessive diagnostic weight on subjective changes following the initiation of immunotherapy. In addition to heighted awareness of the challenges, adherence to CIDP diagnostic guidelines, utilization of objective metrics to document clinical change, and referrals to CIDP centers of excellence are strategies that may improve diagnostic accuracy.

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Section: Laboratory Pitfallsmentioning

confidence: 99%

The Misdiagnosis of CIDP: A Review

Allen

2020

Neurol Ther

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“…The adoption of 45 mg/dl as the URL was based on the publication in 1938 of a study that sampled a reference population of younger patients. Moreover, recent laboratory studies and a systematic review of the literature support implementation of CSF URL values of 50 mg/dl for patients aged ≤50 years and 60 mg/dl for those aged >50 years . Because CIDP misdiagnosis is frequent, CSF protein elevation contributes to CIDP (mis)diagnosis, and the CSF protein URL is undefined in CIDP, we sought to determine how different CSF‐TP URLs impact diagnostic sensitivity and specificity in patients in whom CIDP is correctly and incorrectly diagnosed.…”

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confidence: 99%

Updated cerebrospinal fluid total protein reference values improve chronic inflammatory demyelinating polyneuropathy diagnosis

2019

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Introduction Recent literature has concluded that cerebrospinal fluid total protein (CSF‐TP) upper reference limits (URL) should be higher than 45 mg/dl and stratified by age. Methods Data‐driven URLs were applied to the analysis of a cohort of patients with correctly and incorrectly diagnosed chronic inflammatory demyelinating polyneuropathy (CIDP). Descriptive statistics were calculated, and exploratory analyses were used to test the impact of different CSF‐TP URLs on sensitivity and specificity of CIDP diagnosis. Results The adoption of higher and age‐dependent CSF‐TP URLs reduced the sensitivity of CSF analysis slightly (from 95% to 84%‐86%); however, the overall CIDP detection rate was unchanged. Twelve of 36 (33%) false‐positive diagnoses occurred with CSF‐TP elevation as the sole supportive criteria. By applying updated CSF‐TP URLs, the specificity of CSF analysis increased from 39% to 57%‐64%. Discussion Implementation of data‐driven CSF‐TP URLs improves CIDP diagnostic specificity without compromising sensitivity, thereby lessening CIDP misdiagnosis. Muscle Nerve 60: 180–183, 2019

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“…Отмечено, что наибольший процент пациентов с ошибочно установленным диагнозом ХВДП имели небольшое отклонение содержания белка от референсных значенийв среднем 53 мг/дл при верхней границе нормы 45 мг/дл, тогда как такое отклонение у больных с подтвержденным ХВДП было значительнымв среднем 105 мг/дл [16]. Помимо прочего, следует учитывать вероятность повышения данных значений до 0,6 г/л у здоровых людей старше 60 лет [17].…”

Section: классификация и краткое описание форм хронической воспалителunclassified

Clinical heterogeneity of chronic inflammatory demyelinating polyneuropathy: diagnostic challenges

2020

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Сhronic inflammatory demyelinating polyneuropathy (CIDP) is the most prevalent acquired dysimmune neuropathy with clinical picture of symmetric motor and sensory disturbances. Since the first description of CIDP, many atypical variants have been described, which may reach up to 50% of cases. Diagnosis of atypical CIDP may be challenging due to different clinical presentation and treatment response. Current researches improve our knowledge about dysimmune neuropathies and highlight the importance of its classification. Nowadays CIDP is considered as a spectrum of disorders rather than a separate disease entity. Up to date, more than 15 diagnostic criteria have been proposed reflecting the complexity of СIDP diagnosis. Many polyneuropathies may mimic CIDP, therefore CIDP is frequently a diagnosis of exclusion. The key diagnostic instrument is electroneuromyography; however, the issues related to results misinterpretation and some technical aspects are the most important in CIDP misdiagnosis. Supportive instrumental and laboratory methods have variable sensitivity and specificity, making challenging CIDP diagnosis, especially its atypical forms. The importance of an early and accurate diagnosis of CIDP is supported by an effective pathogenic treatment, which affects the patient's prognosis and level of disability.

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Adult CSF total protein upper reference limits should be age-partitioned and significantly higher than 0.45 g/L: a systematic review

Cited by 47 publications

References 33 publications

The Misdiagnosis of CIDP: A Review

The Misdiagnosis of CIDP: A Review

Updated cerebrospinal fluid total protein reference values improve chronic inflammatory demyelinating polyneuropathy diagnosis

Clinical heterogeneity of chronic inflammatory demyelinating polyneuropathy: diagnostic challenges

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