A variety of antineuronal antibodies have been detected in the cerebrospinal fluid (CSF) of patients with neurological diseases. This raises the question of whether these antibodies are disease-specific or merely an epiphenomenon of inflammatory processes in the brain.
The registry was established with the following objectives: \[1\] Are antineuronal antibodies much more common than previously thought in various neurological disorders for which the etiology has not yet been elucidated? \[2\] Can further correlations, such as those between HSV infection and NMDA receptor autoimmunity, be identified? \[3\] Are these antibodies mainly non-specific epiphenomena or are they crucial for the pathogenesis? \[4\] What is the clinical course of patients with antineuronal antibodies and their response to therapy? These questions will be addressed in a broad immunohistological screening of a large number of CSF samples and a clinical database of patients with neurological disorders.
Differential diagnosis: suspected neuroimmunological disease in which a lumbar puncture is indicated for further diagnosis and treatment decision
Individuals with unclear clinical diagnosis where additional CSF is to be collected for isolation of B cells and production of monoclonal antibodies. The clinical condition of the patients and his/her compliance have to allow an extra 2-3 ml of CSF to be collected.
Age: all age groups
Gender: patients of both sexes will be included
None
Patients with neurological disease in which a lumbar puncture is indicated for further diagnosis and treatment decision
Cohort
Prospective
Not listed
Screening for a panel of anti-neuronal antibodies via commercial assays
The investigators use this commercial test battery to identify whether the CSF of the patients contains known anti-neuronal antibodies. The tests will be perfromed by Euroimmune (Lübeck, Germany). If positive, the result will be the kind of the antibody and its titer.
Screening for unknown anti-neuronal antibodies via immunofluorescence staining on fresh mouse brain (tissue-based assay, TBA)
The tissue-based assay is a non-specific screening method for anti-neuronal antibodies. The investigators stain freshly cryosectioned mouse brain with patient cerebrospinal fluid at various dilutions and counterstain the sample with fluorescently labelled anti-human IgG antibodies. The sample is then imaged using fluorescence microscopy. The recorded measure will be the presence and quality of a characteristic staining pattern (cell population stained by the patient CSF) or its absence.
Determination of the molecular weight of the antibody target
If the tissue based assay (TBA) is positive for a cell surface signal, the investigators will use Western blot to determine the molecular weight after staining mouse brain homogenates with patient CSF and secondary anti human anti-IgG antibodies
CSF cell count
Counting and differentiating the cells found in the patient's CSF. Cell number given as "cell number per microliter". The cell type will ge given as "mononuclear cells" or "polymorph-nuclear cells"
Basic clinical data
The investigators will record age, height, weight, and ask about the presence of fever, weight loss, and positive findings on the internal and neurological clinical examination.
Duration from symptom onset to diagnosis
The investigators will record the time delay between the occurrence of the first symptoms and the establishmen of the diagnosis. The results will be recorded as "number of days"
Assessment of the severity of ataxia
The severity of the ataxia will be quantified using the Scale for the Assessment and Rating of Ataxia (SARA). The SARA score ranges between 0 (no ataxia) and 40 (most severe ataxia).
Assessment for the severity of psychiatric symptoms
The severity of psychiatric symptoms, especially depression, will be quantified with the Patient Health Questionnaire-9 (PHQ-9). The PHQ-9 is a mulitpurpose instrument for screening, diagnosing, monitoring, and measuring the severity of depression. Interpretation of results: minimal symptoms (5-9), minor depression (10-14), major depression (15-19), severe major depression (20-27)
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Charite University, Berlin, Germany
Lead sponsor
University of Ulm
Collaborator
