PMC8481775_01
A summary of the prevalence of different symptoms. (A) The prevalence of initial symptoms; (B) The prevalence of all the symptoms.
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The median age of onset was 22 (ranging from 10 to 70) years old. The most common initial symptoms (Figure 1A) were polydipsia and polyuria (19/25, 76.0%) and amenorrhea (9/20, 45%, Patient No.5 had had menopause before onset). With the progression of the disease, new manifestations showed up subsequently (Figure 1B).
The T1-weighted postcontrast MR images of two typical cases. (A) Sagittal view of patient No. 7; (B) Coronal view of patient No. 7; (C) Sagittal view of patient No. 10; (D) Coronal view of patient No. 10.
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T1-weighted postcontrast coronal and sagittal views of MR images of two typical cases (patients No. 7 and 10) were shown in Figure 2. The MRI features mainly demonstrated hypointense or isointense T1WI and hyperintense T2WI for the hypothalamic lesions.
The correlations between complex hypothalamic symptoms and the lesion size (A), the third ventricle floor (TVF) identification (B) and third ventricle involvement (C). *P < 0.05, **P < 0.01.
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TVF identification, and third ventricle involvement were analyzed. The absence of any of such symptoms was only seen in cases with lesion sizes less than 1cm (see the blue bars in Figure 3A). TVFs showed no manifestations of such complex hypothalamic symptoms (Figure 3B). Those with more notable involvement of the TVFs were more likely to have symptoms of affective disorders and hyperphagia (P=0.042 for both). Generally, patients with these complex symptoms had higher degrees of the third ventricle involvement (Figure 3C), despite the statistic insignificance.
Histopathology and immunohistochemistry manifestations of Langerhans cell histiocytosis (LCH) in patient No. 10. (A) H&E staining reflected marked histiocyte proliferation (H&E, x200 original magnification); Immunohistochemistry indicated clusters of various immunophenotypical markers [(B): CD68+, (C): CD1a+, (D): leukocyte common antigen (LCA)+, (E): CD138+, x200 original magnification].
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Figures 4, 5 show the histopathologic findings of patients No. 10 and 17, which were diagnosed with LCH and hypothalamitis separately.
Histopathology and immunohistochemistry manifestations of hypothalamitis in patient No. 17. (A) Notable proliferation of lymphocyte, plasma cells and histiocyte was found in H&E staining (H&E, x200 original magnification); Immunohistochemical analysis revealed scattered CD68 (B), CD138 (C) and CD3 (D) cells, with IgG deposition (E). CD20 (F) and CD1a (G) cells were absent.
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Figures 4, 5 show the histopathologic findings of patients No. 10 and 17, which were diagnosed with LCH and hypothalamitis separately.
Pituitary dysfunctions and metabolic disorders. (A) Description of hypopituitarism of all the cases; (B) BMIs of the evaluated cases (n = 20); (C) Blood lipid levels of the evaluated cases (n = 16); (D) Serum uric acid levels of the evaluated cases (n = 20). CAI, central adrenal insufficiency; CHT, central hypothyroidism; CHG, central hypogonadism; HPL, hyperprolactinemia; CDI, central diabetes insipidus; N/A, not available.
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In the evaluation of pituitary functions, central hypogonadism (21/22, 95.5%) and central diabetes insipidus (19/21, 90.5%) were found the two most commonly affected axes (Figure 6A). Pre-operation/treatment data of body mass index (BMI) were available in 20 cases (Figure 6B). Patient No. 5 even had a TG level as high as 25.0mmol/L (Figure 6C). Hyperuricemia was found in 10 of the 15 females and 2 of the 5 males, with serum uric acid levels of 0.460 +- 0.156mmol/L and 0.342 +- 0.120mmol/L separately (Figure 6D).