Intrathecal immunoglobulin synthesis in patients with symptomatic epilepsy and epilepsy of unknown etiology (‘cryptogenic’)

Fauser, Susanne; Soellner, Christian; Bien, Christian G.; Tumani, Hayrettin

doi:10.1111/ene.13348

Cited by 8 publications

(5 citation statements)

References 9 publications

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“…An immune mediated mechanism has been suggested in some epilepsy types [21]. While Kowski et al reported a high incidence of intrathecal immunoglobulin synthesis in patients with so far classified cryptogenic epilepsy (34.1%), Fauser and colleagues detected intrathecal immunoglobulin synthesis only in 8% patients with epilepsy of unknown origin, and in 5% of patients with first seizures of unknown cause, and concluded that the findings were too infrequent and not indicating a leading humoral pathomechanism [22,23].…”

Section: Discussionmentioning

confidence: 99%

Evidence of Oligoclonal Bands Does Not Exclude Non-Inflammatory Neurological Diseases

et al. 2020

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Cerebrospinal fluid analysis is an essential part of the diagnostic workup in various neurological disorders. Evidence of an intrathecal immunoglobulin synthesis, as demonstrated by Reiber’s diagram or the more sensitive oligoclonal bands (OCB), are typical for neuroinflammatory diseases, and normally not expected in non-inflammatory neurological diseases. Therefore, patients with non-inflammatory neurological diseases are often used in control groups in studies investigating autoimmune diseases of the central nervous system. However, data about the frequency of intrathecal immunoglobulin synthesis in non-inflammatory neurological disease are scarce. The cerebrospinal fluid (CSF) records of a total of 3622 patients were screened and 2114 patients included with presumably non-inflammatory neurological diseases like dementia, idiopathic peripheral neuropathy, motoneuron disease, stroke, and epileptic seizures. Evidence of an intrathecal immunoglobulin synthesis can be found with low frequency also in non-inflammatory neurological diseases. A much higher rate of patients showed intrathecal immunoglobulin synthesis as demonstrated by OCB than by Reiber’s diagram. In patients with disorders of the peripheral nervous system the frequency of OCB was much lower than in patients presenting with central nervous system manifestations. Evidence of an intrathecal immunoglobulin synthesis should not automatically lead to exclusion of non-inflammatory neurological diseases but should rather prompt the way to investigate for the origin of the intrathecal immunoglobulin synthesis.

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Section: Discussionmentioning

confidence: 99%

Evidence of Oligoclonal Bands Does Not Exclude Non-Inflammatory Neurological Diseases

et al. 2020

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“…One reason for the difference in Ig detection may be the unselective indication for lumbar puncture in the above‐mentioned study. Another reason may be the difference in laboratory methods, since OCB analysis was performed via silver staining in the above‐mentioned study, as suggested by Fauser et al in a different context …”

Section: Discussionmentioning

confidence: 96%

Intrathecal inflammation in young stroke

et al. 2019

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Stroke is a major cause of disability in the population and the second most common cause of death worldwide. 1 Approximately 10%-14% of stroke patients are younger than 45 years. 2 Stroke etiology in these patients remains unknown in 11% 2 up to 20%. 3 Young stroke patients have a high prevalence of vascular risk factors like hypertension, diabetes mellitus, dyslipidemia, or smoking. 2 Rare causes with potential therapeutic consequences require extensive diagnostic work-up to prevent recurrent stroke. Coagulopathies, genetic, and metabolic diseases as well as autoimmune or infectious vasculopathies have to be considered. 4,5 Acute neuroborreliosis, neurosyphilis, or vasculopathy after bacterial meningitis 6-8 require cerebrospinal fluid (CSF) analysis to obtain the diagnosis. In CSF, elevated cell count, blood-CSF barrier dysfunction, quantitative intrathecal synthesis of immunoglobulin (Ig), as well as the presence of Objectives: Correct identification of inflammatory etiologies of stroke is of outmost importance as they require treatment of the underlying disease. Aim of this study was to determine the prevalence of inflammatory changes in cerebrospinal fluid (CSF) observed in young cryptogenic stroke patients. Materials and Methods:Of 6476 records of patients diagnosed with ischemic stroke, 278 had confirmed ischemia in brain imaging and received lumbar puncture. A total of 122 were classified as young stroke (≤55 years), and 156 were classified as older stroke patients; lumbar puncture in this cohort was indicated due to atypical clinical presentation.Results: An infectious etiology was detected in 2.5% of young stroke patients (n = 3: vasculitis due to opportunistic infection, vasculitis due to neuroborreliosis, secondary vasospasm after viral meningitis) and in 1.9% (n = 3) in the older stroke cohort (vasculitis due to neurotuberculosis, septic embolic ischemia, vasculitis post-haemophilus influenza meningoencephalitis). Isolated vasculitis was evident in one patient of the older stroke cohort (0.6%). Non-specific alterations in CSF included increased cell count in 10% in young and in 9.3% in the older stroke cohort. Intrathecal Ig synthesis was present in 3.4% of the younger and in 4% of the older stroke cohort. Conclusions:The prevalence of an infectious etiology in young stroke is modest but slightly higher in comparison with older stroke patients. As brain imaging is not always sufficient for suspecting vasculitis, we recommend implementation of lumbar puncture in young cryptogenic stroke patients. If an infectious disease is present in ischemic stroke, it is of high therapeutic relevance. K E Y W O R D Scerebrospinal fluid analysis, infection, inflammation, young stroke

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“…Consistent with our findings of 5%, Fauser et al . found intrathecal IgG synthesis in 4–8% of their patients not related to seizure etiology (cryptogenic, symptomatic, idiopathic) using immunoblot for OCB detection. In contrast, Kowski et al .…”

Section: Discussionmentioning

confidence: 99%

“…patients with infectious encephalitis) in the above‐mentioned study. Methodical differences between the studies also have to be considered (silver staining versus more specific immunoblot) .…”

Section: Discussionmentioning

confidence: 99%

Cerebrospinal fluid changes following epileptic seizures unrelated to inflammation

Süße

Saathoff

Hannich

et al. 2019

Euro J of Neurology

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Background and purpose Analyzing cerebrospinal fluid (CSF) is crucial in the diagnostic workup of epileptic seizures to rule out autoimmunity or infections as the underlying cause. Therefore, the description of post‐ictal changes in CSF is essential to differentiate between negligible and etiopathologically relevant changes in the CSF profile. Methods A retrospective analysis of 247 patients newly diagnosed with epileptic seizures and CSF analysis during diagnostic workup was conducted. Patients with possible or definitive autoimmune or infectious encephalitis were excluded. CSF results were evaluated for associations with seizure types, seizure etiology and electroencephalography (EEG) findings. Results An increased cell count (>4/μL) was found in 4% (n = 10), increased lactate concentration (>2.5 mmol/L) in 28% (n = 70), increased total protein (>500 mg/L) in 51% (n = 125) and a dysfunction of the blood–brain barrier in 29% (n = 71) of patients. Intrathecal immunoglobulin G production was observed in 5% (n = 12) of patients. Higher lactate concentrations were found in seizures with motor onset (P = 0.02) compared with those with non‐motor onset. Patients with generalized slow activity on EEG had significantly higher lactate values (P = 0.01) and albumin quotient (P = 0.05) than those with normal EEG. Conclusions Compared with mild pleocytosis and immunoglobulin synthesis, elevated lactate and total protein concentrations as well as blood–brain barrier dysfunction are frequently found following epileptic seizures. Our data suggest that seizure semiology might impact CSF profiles. The highest lactate concentrations were found following motor‐onset seizures. Our findings may help clinicians to avoid over‐interpretation of minor CSF changes; however, the exclusion of alternative causes should always be carefully considered, taking into account further clinical features.

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Intrathecal immunoglobulin synthesis in patients with symptomatic epilepsy and epilepsy of unknown etiology (‘cryptogenic’)

Cited by 8 publications

References 9 publications

Evidence of Oligoclonal Bands Does Not Exclude Non-Inflammatory Neurological Diseases

Evidence of Oligoclonal Bands Does Not Exclude Non-Inflammatory Neurological Diseases

Intrathecal inflammation in young stroke

Cerebrospinal fluid changes following epileptic seizures unrelated to inflammation

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