PMC10396436_01
(A) Slice of mouse subfornical organ (SFO) tissue with control serum of a seronegative healthy control subject showing no immunological reaction in comparison to a seropositive control and to the patient's serum showing antibody binding to SFO as visible by immunostaining. (B) 7T-MRI showing a small T2-hyperintensity (~2 mm) in left hypothalamus close to third ventricle in the circumventricular region (left and upper right image, red arrow) 1 year after diagnosis with adipsic hypernatremia. One and a half years later the enhancement is not visible any more (lower right image). It is suspected that the hyperintensity corresponds to a lesion caused by autoantibodies, which reconstituted later on. (C) Serum copeptin (vasopressin marker peptide) in relation to serum osmolality. The patient's copeptin values only show minimal rise in relation to increasing serum osmolality. The expected range of copeptin values in relation to serum osmolality is marked in yellow. (D) Depicted is elevated serum prolactin beginning with the patient's first visit to the hospital. The physiological range of prolactin is marked in yellow. (E) Levels of serum sodium beginning with the patient's first visit to the hospital. The physiological range of sodium is marked in yellow. Timepoints of plasmapheresis are marked as black vertical lines.
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F1
Right after the first plasmapheresis cycle, it was possible to lower the dosage of desmopressin to 2 x 60 mug/day and Ben maintained normal serum sodium levels (Figure 1E).
Timeline of Ben's most important medical and life events, starting with his initial visit to the pediatric university hospital. In red the years following this first visit are marked. The P's indicate timepoints of plasmapheresis. Plasmapheresis led to a replicable short-term improvement (sodium levels and water homeostasis improved directly after the first plasmapheresis; psychotic, cognitive and catatonic symptoms decreased about 3-4 months after every plasmapheresis). However, relapse/worsening of neurocognitive symptoms occurred on the long term (about 6-9 months after every plasmapheresis).
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F3
PMC10396436_02
(A) Slice of mouse subfornical organ (SFO) tissue with control serum of a seronegative healthy control subject showing no immunological reaction in comparison to a seropositive control and to the patient's serum showing antibody binding to SFO as visible by immunostaining. (B) 7T-MRI showing a small T2-hyperintensity (~2 mm) in left hypothalamus close to third ventricle in the circumventricular region (left and upper right image, red arrow) 1 year after diagnosis with adipsic hypernatremia. One and a half years later the enhancement is not visible any more (lower right image). It is suspected that the hyperintensity corresponds to a lesion caused by autoantibodies, which reconstituted later on. (C) Serum copeptin (vasopressin marker peptide) in relation to serum osmolality. The patient's copeptin values only show minimal rise in relation to increasing serum osmolality. The expected range of copeptin values in relation to serum osmolality is marked in yellow. (D) Depicted is elevated serum prolactin beginning with the patient's first visit to the hospital. The physiological range of prolactin is marked in yellow. (E) Levels of serum sodium beginning with the patient's first visit to the hospital. The physiological range of sodium is marked in yellow. Timepoints of plasmapheresis are marked as black vertical lines.
fpsyt-14-1206226-g0001.jpg
PMC10396436_02_fpsyt-14-1206226-g0001.jpg
F1
Right after the first plasmapheresis cycle, it was possible to lower the dosage of desmopressin to 2 x 60 mug/day and Ben maintained normal serum sodium levels (Figure 1E).
PMC10396436_03
Results of volume analysis of left and right basal ganglia (caudate, pallidum, putamen) of Ben (blue dots, four contrast-enhanced MRIs have been conducted over a span of 4 years) in relation to a sample of patients with multiple sclerosis (gray dots, no healthy sample available from contrast-enhanced MRI). The black line displays the fitted mean of the multiple sclerosis patients as reference. The quantitative analysis of basal ganglia volume was performed with DL + DiReCT which allows to extract brain morphometry from contrast-enhanced T1-weighted MRI.
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F4
Structural analysis of contrast-enhanced MRIs showed a substantial longitudinal decrease of gray matter volume in bilateral basal ganglia (Figure 4).