Successful Diagnosis of Hypothalamitis Using Stereotactic Biopsy and Treatment

Zhang, Shuo; Ye, Hongying; Zhang, Zhaoyun; Lü, Bin; Yang, Yehong; He, Min; Wu, Hanfeng; Zhou, Lei; Wang, Yin; Pan, Li; Li, Yiming; Hu, Renming

doi:10.1097/md.0000000000000447

Cited by 13 publications

(18 citation statements)

References 12 publications

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“…This pattern is no longer in accordance with the typical pattern of "ACTH>TSH>LH/FSH>IGF-1" which, based on previous studies, has diagnostic value. 12 While two patients, each from single-case reports, presented with panhypopituitarism at the onset of illness, 4,6 we suggest that the typical pattern of hormone deficiency may not be present in all the subtypes and populations. This speculation should be verified in larger cohorts.…”

Section: Discussionmentioning

confidence: 79%

“…Biopsy specimens showed inflammatory cell infiltrates, which were mainly composed of lymphocytes, plasma cells, and histiocytes. [5][6][7] Remarkable improvements in MRI and patients' symptoms were observed after administration of steroid and immunosuppressive therapy with AZP. The hypothalamus is a direct upward extension of the neurohypophysis and infundibulum, and it is closely associated with the pituitary gland in both histology and function.…”

Section: Discussionmentioning

confidence: 99%

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Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center

et al. 2019

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Objective Autoimmune hypothalamitis (AHT) is a rare inflammatory disorder that involves the hypothalamus. It remains unclear whether autoimmune hypophysitis (AH) and AHT represent different diseases or different aspects of the same disease. Thus, further investigation of AHT is required. Methods A retrospective review of medical and pathological records of AHT patients from the Chinese PLA General Hospital were examined from January 1, 2005 to May 1, 2017. Clinical data, treatments, and outcomes were investigated. Results Five female patients were identified (median age, 42.6 years). Symptoms included central diabetes insipidus, hypopituitarism, hyperprolactinemia, headache, and hypothalamic syndrome. The following hormonal deficits were noted: follicle-stimulating hormone, luteinizing hormone, adrenocorticotropic hormone, thyroid stimulating hormone, and growth hormone. One patient underwent high-dose methylprednisolone pulse treatment (HDMPT) and azathioprine plus intensity modulated radiation therapy (IMRT), and two patients underwent HDMPT and two rounds of replacement therapy. During follow-up, one patient died because of non-compliance with therapy and the others were in remission or they recovered. Conclusions AHT had similar MRI results, pathology, and treatment compared with AH. Thus, it may be a subtype of AH, and AHT patients may also show hypothalamic syndrome.

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center

et al. 2019

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“…Histopathology is considered the gold standard for the diagnosis of LHT [ 3 , 6 ]. Although our patient’s biopsy revealed infiltrates of lymphocytes, plasma cells, and histiocytes suggestive of an inflammatory process, the diagnosis of LHT could not be confirmed until autopsy.…”

Section: Discussionmentioning

confidence: 99%

“…Although multiple surgical approaches for biopsy have been reported in the literature (including stereotactic, trans-sphenoidal, and intraventricular approach) [ 6 , 17 ], careful consideration must be taken to lessen the risk of complications like edema, hematoma, syndrome of inappropriate antidiuretic hormone, or DI due to the hypothalamus’ vasculature and location of its nuclei. It remains questionable as to whether the severe swelling of the hypothalamus in our patient’s case originated secondarily to the progression of the disease or to biopsy-related trauma.…”

Section: Discussionmentioning

confidence: 99%

Anti–Ma-1 and Anti–Ma-2 Antibodies in Isolated Fatal Hypothalamitis

Bustos

Berger

Hannoush

et al. 2018

Journal of the Endocrine Society

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Lymphocytic hypothalamitis (LHT) is a rare disease characterized by pituitary dysfunction, autonomic instability, metabolic disturbances, and neuropsychiatric disorders. We report the case of a 30-year-old man found to have LHT that progressed despite treatment with methylprednisolone and intravenous immunoglobulin (IVIG). A literature review was conducted to identify prior studies pertaining to LHT. Our patient presented with several weeks of fatigue, cold intolerance, weight loss, confusion, and headache. Laboratory tests were consistent with panhypopituitarism. Brain magnetic resonance imaging revealed an infiltrative enhancing mass in the area of the hypothalamus, and stereotactic biopsy findings showed reactive inflammatory changes. A course of hormone replacement (levothyroxine), methylprednisolone, and IVIG was initiated. The patient’s course was complicated by a fatal tonsillar herniation. Autopsy revealed LHT. The diagnosis and management of autoimmune LHT are challenging. The entity should be considered in the setting of panhypopituitarism with a hypothalamic mass. Differentiating paraneoplastic and nonparaneoplastic hypothalamitis should be established for management and prognosis. Definitive treatment remains unclear; treatment with corticosteroids should be attempted, followed by consideration of other immunosuppressive agents, such as rituximab. If a paraneoplastic syndrome is suspected, management should also be directed toward the primary tumor.

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“…However, histological diagnosis is not practical because of the highly invasive nature of biopsy and the deep location of the hypothalamus. Several reported cases of diagnostic stereotactic biopsy have revealed inflammatory infiltrates mainly constituting lymphocytic inflammation [ 11 – 13 ]. Based on these findings of a common histological pattern, we suggest that highly invasive biopsy has lower priority than imaging.…”

Section: Discussionmentioning

confidence: 99%

Paraneoplastic limbic encephalitis with associated hypothalamitis mimicking a hyperdense hypothalamic tumor: a case report

Bataduwaarachchi

Tissera

2016

BMC Med Imaging

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BackgroundParaneoplastic limbic encephalitis is an uncommon association of common malignancies such as small cell lung carcinoma, testicular teratoma, and breast carcinoma. The nonspecific nature of the clinical presentation, lack of freely available diagnostic markers, and requirement for advanced imaging techniques pose a great challenge in the diagnosis of this disease in resource-poor settings.Care presentationA 64-year-old previously healthy Sri Lankan man was admitted to the general medical unit with subacute memory impairment regarding recent events that had occurred during the previous 3 weeks. Initial noncontrast computed tomography of the brain revealed a hyperdensity in the hypothalamic region surrounded by hypodensities extending toward the bilateral temporal lobes; these findings were consistent with a possible hypothalamic tumor with perilesional edema. The patient later developed cranial diabetes insipidus, which was further suggestive of hypothalamic disease. Interestingly, gadolinium-enhanced magnetic resonance imaging of the brain showed no such lesions; instead, it showed prominent T2-weighted signals in the inner mesial region, characteristic of encephalitis. The possibility of tuberculosis and viral encephalitis was excluded based on cerebrospinal fluid analysis results. Limbic encephalitis with predominant hypothalamitis was suspected based on the radiological pattern. Subsequent screening for underlying malignancy revealed a mass lesion in the right hilum on chest radiographs. Histological examination of the lesion showed small cell lung cancer of the “oat cell” variety.ConclusionWe suggest that the initial appearance of a hyperdensity in the hypothalamus region on noncontrast computed tomography is probably due to hyperemia caused by hypothalamitis. If hypothalamitis is predominant in a patient with paraneoplastic limbic encephalitis, magnetic resonance imaging will help to differentiate it from a hypothalamic secondary deposit. Limbic encephalitis should be considered in a patient with computed tomographic evidence of a central hyperdensity surrounded by bitemporal hypodensities. This pattern of identification will be useful for early diagnosis in resource-poor settings.

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Successful Diagnosis of Hypothalamitis Using Stereotactic Biopsy and Treatment

Cited by 13 publications

References 12 publications

Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center

Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center

Anti–Ma-1 and Anti–Ma-2 Antibodies in Isolated Fatal Hypothalamitis

Paraneoplastic limbic encephalitis with associated hypothalamitis mimicking a hyperdense hypothalamic tumor: a case report

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