scholarly journals Two Cases of Pituitary Stalk Interruption Syndrome in Syrian Children

2020 ◽  
Vol 2020 ◽  
pp. 1-5 ◽  
Author(s):  
Ibrahim Alali ◽  
Reem Saad ◽  
Younes Kabalan
Keyword(s):  
Magnetic Resonance Imaging ◽  
Anterior Pituitary ◽  
Growth Failure ◽  
Pituitary Stalk ◽  
Delayed Puberty ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Resonance Imaging ◽  
Exact Etiology ◽  
Magnetic Resonance Imaging Mri

Pituitary stalk interruption syndrome (PSIS) is an extremely rare cause of growth failure and delayed puberty. It can be diagnosed by magnetic resonance imaging (MRI) of the hypothalamus and pituitary gland, showing an ectopic or absent posterior pituitary, an absent or interrupted pituitary stalk, or small anterior pituitary, in combination with growth hormone or other pituitary hormone deficiencies. The exact etiology of PSIS is unknown. In this article, we describe two cases of PSIS in Syria which are, as far as we know, the first published cases.

scholarly journals Pituitary stalk transection syndrome

2020 ◽  
Vol 7 (12) ◽  
pp. 2397
Author(s):  
Gayathri Sajeevan ◽  
Sajitha Nair ◽  
Devika Geetha ◽  
Nisha Bhavani ◽  
C. Jayakumar ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Resonance Imaging ◽  
Current Report

Growth hormone deficiency is one of the most common endocrinological causes for short stature. It can either be idiopathic or associated with organic causes like tumors or following surgery. One of the rare causes for growth hormone deficiency in children is pituitary stalk transection syndrome. It can be diagnosed by magnetic resonance imaging of the hypothalamus and pituitary gland which shows an ectopic or absent posterior pituitary, an absent or interrupted pituitary stalk, or small anterior pituitary in combination with growth hormone or other pituitary hormone deficiencies. Current report presents a child with pituitary stalk transection syndrome who was brought for evaluation of hypoglycemic seizures.

Suckling-Induced Rise in Prolactin: Mediation by Prolactin-Releasing Factor From Posterior Pituitary

Physiology ◽  
1988 ◽  
Vol 3 (4) ◽  
pp. 172-175
Author(s):  
N Ben-Jonathan ◽  
JF Hyde ◽  
I Murai
Keyword(s):  
Anterior Pituitary ◽  
Pituitary Stalk ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Small Peptide ◽  
Anterior Pituitary Hormone

The suckling-induced rise in prolactin, an anterior pituitary hormone that is essential for the maintenance of lactation, is mediated by the posterior pituitary. The posterior pituitary contains prolactin-releasing factor (PRF), a small peptide that is distinct from known prolactin secretagogues. The hypothalamus, the site of all known releasing hormones, has only little PRF activity. Pituitary stalk secretion abolishes the PRF activity in the posterior pituitary, indicating that it originates in the hypothalamus, perhaps as a biologically inactive precursor. The chemical identity of PRF is unknown but it appears to play an important role during lactation and might also be involved in the pathology of hyperprolactinea.

The relation between pituitary magnetic resonance imaging findings and GH, TSH, PRL dynamics in patients with idiopathic GH deficiency

1991 ◽  
Vol 125 (4) ◽  
pp. 342-347 ◽  
Author(s):  
Kunihiko Hanew ◽  
Atsushi Utsumi ◽  
Akira Sugawara ◽  
Yasuyuki Shimizu ◽  
Satoru Tazawa ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Anterior Pituitary ◽  
Gh Deficiency ◽  
Resonance Imaging ◽  
Mri Findings ◽  
Short Children ◽  
Basal Plasma ◽  
Magnetic Resonance Imaging Mri ◽  
Plasma Gh

Abstract. The relation between pituitary magnetic resonance imaging (MRI) findings and anterior pituitary function was studied in 36 patients with classic idiopathic GH deficiency. These patients were divided into three groups based on MRI findings which were compared with those of 14 normal short children; i.e. normal stalk (N=6), narrowed stalk (N=20), and transected stalk (N=10). The transected and narrowed stalk groups showed significantly delayed TSH responses to TRH compared with the normal stalk group and with the normal short children. Further, the mean maximal TSH increment in the narrowed and transected stalk group was slightly greater than that in normal short children. In contrast, there were no differences in basal plasma GH and PRL levels and their responses to GHRH and TRH among the three groups. When the patients were divided into normal anterior pituitary and atrophic pituitary groups regardless of stalk changes or when they were divided into groups of stalk changes (narrowing and transection) with and without pituitary atrophy, no differences in GH, TSH and PRL dynamics between the groups were observed. These results indicate that pituitary thyrotrope functions, but not somatotrope and lactotrope functions, in patients with idiopathic GH deficiency are more closely correlated to stalk changes than to anterior pituitary changes observed on MRI.

Pituitary Stalk Interruption Syndrome: Presentation of a Rare Case

2018 ◽  
Vol 17 (05) ◽  
pp. 176-179
Author(s):  
Burcin Agridag Ucpinar ◽  
Ahmet Ucar ◽  
Evrim Ozmen
Keyword(s):  
Mental Retardation ◽  
Incidence Rate ◽  
Anterior Pituitary ◽  
Hormone Replacement ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Magnetic Resonance Imaging Scan ◽  
Pituitary Hormone ◽  
Posterior Pituitary ◽  
Mild Mental Retardation

AbstractPituitary stalk interruption syndrome is a congenital anomaly characterized by interrupted or thin pituitary stalk, hypoplastic or absent anterior pituitary, and an absent or ectopic posterior pituitary gland. The exact incidence rate of this syndrome is not known. However, the estimated incidence rate is 0.5/1,000,000 births. In this case report, we wanted to present a case of interrupted pituitary stalk syndrome, which presented with seizures and thyroid hormone deficiency. A 5-year-old female patient was admitted to our emergency department with vomiting, fever, and seizures with new onset. She had a twin who was ex-utero intrapartum in taken history. She was diagnosed with hypothyroidism and started on levothyroxine. Her height was in 25 to 50th percentile and her weight was in 10 to 25th percentile. She had mild mental retardation. On contrast-enhanced cranial magnetic resonance imaging scan, the pituitary stalk was absent, posterior pituitary was ectopic, and anterior pituitary was hypoplastic. The patient was diagnosed with interrupted pituitary stalk syndrome. After the symptoms were relieved, patient started on carbamazepine for epileptic seizures and hormone replacement therapy with levothyroxine and hydrocortisone. She was routinely followed up after the proper diagnosis. Leuprolide (gonadotropin-releasing hormone) and Norditropin (biosynthetic growth hormone) were added to medical therapy. Her height and weight were in 25th percentile after the long-term follow-up of approximately 10 years. On neurological examination, situation of mild mental retardation persisted. Pituitary stalk interruption syndrome is a very rare entity. However, radiologists should keep this syndrome in mind for patients who present with hypoglycemia, seizures, jaundice, cryptorchidism, and hypothyroidism in neonatal period and growth retardation with pituitary hormone deficiencies in childhood.

scholarly journals A triad of cluster-like headaches with delayed development of a macroscopic prolactinoma: A case report

2020 ◽  
Vol 3 ◽  
pp. 251581632091103
Author(s):  
Stephanie A Ihezie ◽  
Manisha Chandalia ◽  
Mark J Burish
Keyword(s):  
Magnetic Resonance Imaging ◽  
Case Report ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Pituitary Hormone ◽  
Supporting Evidence ◽  
Resonance Imaging ◽  
Headache Onset ◽  
Magnetic Resonance Imaging Mri ◽  
Pituitary Lesion

Pituitary hormone testing is recommended in refractory cluster headache (CH), but supporting evidence is limited. We present a patient with cluster-like headaches and a negative brain magnetic resonance imaging (MRI) 1 year after headache onset. He failed multiple medication trials. Three years after headache onset, additional workup showed abnormal pituitary labs including hyperprolactinemia and a brain MRI with a 15 × 15 × 14 mm3 enhancing pituitary lesion. With cabergoline 0.25 mg twice weekly, the patient has been headache-free for over 2 years. This case supports the recommendations for pituitary testing in refractory CH, even if imaging is initially negative for a pituitary tumor.

scholarly journals Clinical features and endoscopic findings of granular cell tumor of the sellar region: A case report and review of the literature

2020 ◽  
Vol 11 ◽  
pp. 101
Author(s):  
Akari Kusakawa ◽  
Akihiro Inoue ◽  
Yawara Nakamura ◽  
Naoya Nishida ◽  
Mana Fukushima ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Anterior Pituitary ◽  
Granular Cell Tumor ◽  
Granular Cell ◽  
Cell Tumor ◽  
Accurate Diagnosis ◽  
Sellar Region ◽  
Posterior Pituitary ◽  
Resonance Imaging

Background: Granular cell tumor (GCT) of the sellar region is a rare tumor of the sellar and suprasellar regions that originate from the neurohypophysis. This tumor is very difficult to differentiate from other pituitary neoplasms, such as pituitary adenoma, pituicytoma, and spindle cell oncocytoma. We report a rare case of GCT arising from the posterior pituitary of the sellar region and suggest a useful indicator for accurate diagnosis and pitfalls for surgical procedures. Case Description: A 42-year-old woman was admitted to our hospital with bitemporal hemianopsia. Neuroimaging showed a large pituitary tumor in the sellar and suprasellar regions with a hypointense part on T2-weighted magnetic resonance imaging, and the enhanced anterior pituitary gland was displaced anteriorly. Laboratory findings showed mild hyperprolactinemia. Subtotal resection of the tumor was achieved using an endoscopic endonasal transsphenoidal approach. Histological findings showed round or polygonal cells with abundant granular eosinophilic cytoplasm staining strongly for thyroid transcription factor 1. The tumor was, therefore, diagnosed as a GCT of the sellar region, belonging to tumors of the posterior pituitary. After surgery, visual impairment and anterior pituitary function were improved. Follow-up neuroimaging after 1 year showed no signs of recurrence. Conclusion: GCT of the sellar region is difficult to diagnose on routine neuroimaging. Therefore, accurate diagnosis requires careful identification of clinical signs, magnetic resonance imaging including hypointensity on T2-weighted imaging, and analysis of combined morphological and immunohistochemical studies.

scholarly journals Lymphocytic Panhypophysitis: Its Clinical Features in Japanese Cases

2011 ◽  
Vol 2 ◽  
pp. JCM.S6254 ◽  
Author(s):  
Yoshiharu Wada ◽  
Yoshiyuki Hamamoto ◽  
Yoshio Nakamura ◽  
Sachiko Honjo ◽  
Yukiko Kawasaki ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Anterior Pituitary ◽  
Lymphocytic Infiltration ◽  
Pituitary Hormone ◽  
General Malaise ◽  
Resonance Imaging ◽  
Appetite Loss ◽  
Anterior Pituitary Hormone ◽  
Female Predominance ◽  
Lymphocytic Adenohypophysitis

Lymphocytic hypophysitis is divided into three forms according to the involved tissues, lymphocytic adenohypophysitis, lymphocytic infundibulo-neurohypophysitis, and lymphocytic panhypophysitis (LPH). The term LPH was first proposed by us in 1995, although its entity and pathogenesis still remain controversial. Here we report five cases of LPH, who visited our clinics during 1994 to 2009. All cases were female of 20 to 77 years of age, and one case was associated with pregnancy. They presented with polyuria (n = 4), headache (n = 3), general malaise, polydipsia (n = 2), blunted vision, diplopia, amenorrhea or appetite loss (n = 1). Magnetic resonance imaging showed the pituitary swelling, the thickened stalk, the loss of the T1 hyperintense neurohypophysis (n = 4), or the atrophic pituitary (n = 1). Endocrinological examinations revealed deficiencies of TSH, ADH in all cases, GH, ACTH in three cases, LH, PRL in two cases, and FSH in one case, respectively. The severity of ADH deficiency varied among the cases. Anti-pituitary antibody was not detected in the cases examined. The biopsy of the pituitary lesions was performed except for one case, all of which revealed the diffuse lymphocytic infiltration. These results suggest that LPH is characterized by the female predominance, the atypical patterns of anterior pituitary hormone deficiencies and the variable degrees of diabetes insipidus in Japanese.

scholarly journals Long-term follow-up evaluation of magnetic resonance imaging in the prognosis of permanent GH deficiency

2000 ◽  
pp. 493-496 ◽  
Author(s):  
M Bozzola ◽  
F Mengarda ◽  
P Sartirana ◽  
L Tato ◽  
JL Chaussain
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Prognostic Marker ◽  
Gh Deficiency ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Resonance Imaging ◽  
Long Term Follow Up ◽  
Magnetic Resonance Imaging Mri

OBJECTIVE: In patients with GH deficiency (GHD), magnetic resonance imaging (MRI) has revealed morphological abnormalities such as pituitary hypoplasia, pituitary stalk agenesis (PSA) and ectopia of the posterior pituitary (PPE). The MRI anomalies have been more frequently reported in patients with multiple pituitary hormone deficiency (MPHD) than in subjects with isolated GH deficiency (IGHD). The aim of this work was to define which MRI anatomical abnormalities of the hypothalamo-pituitary area can be considered as a prognostic marker of permanent GHD. DESIGN: To investigate the relationship between the neuroradiological images and endocrine findings, we clinically re-evaluated 93 out of the 121 GHD patients with IGHD and MPHD previously studied. RESULTS: No additional hormone deficiencies were observed in 55 out of 60 patients initially classified as having IGHD with a normal (15 cases) or reduced (40 cases) pituitary gland size, without other MRI abnormalities. The remaining five children, who had initially shown an apparently IGHD in spite of PSA and PPE, developed a MPHD over time. In 33 MPHD patients with (25 cases) or without (8 cases) MRI abnormalities, the associated hormone deficiencies were confirmed during follow-up. CONCLUSIONS: The IGHD patients showing PSA and PPE inevitably develop additional hormone deficiencies, while IGHD subjects having no MRI abnormalities maintain IGHD. Moreover, the anatomical abnormalities of the hypothalamo-pituitary area can be considered as a prognostic marker of permanent GHD.

scholarly journals SAT-067 Maternal Transmission of Pituitary Stalk Interruption Syndrome(PSIS)

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Snigdha Reddy Likki ◽  
Holley F Allen ◽  
Chelsea Gordner
Keyword(s):  
Pituitary Gland ◽  
Adrenal Insufficiency ◽  
Anterior Pituitary ◽  
Hormone Replacement ◽  
Growth Failure ◽  
Pituitary Stalk ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Z Score ◽  
Central Hypothyroidism

Abstract BACKGROUND: Pituitary stalk interruption syndrome (PSIS) is a rare entity characterized by thin or absent pituitary stalk, hypoplastic/aplastic anterior pituitary and ectopic posterior pituitary (EPP) on magnetic resonance imaging (MRI). PSIS can be associated with variable degrees of pituitary insufficiency 1. Most cases of combined pituitary hormone deficiency are sporadic, however in familial cases, there can be AD or AR inheritance with more than 30 genes identified in association with combined pituitary hormone deficiency (CPHD). We describe how diagnosis of 2 children with PSIS led to the discovery of the condition in their mother. Clinical Case: Child 1 presented at age 3yrs with growth failure in 2003 with ht z score -4.24 SD. Subsequent work up revealed low IGF-1 (< 25 ng /mL) and MRI showed EPP, small anterior pituitary gland and absent pituitary stalk. No GH stim test was performed. He was started on GH supplementation and later was diagnosed with central hypothyroidism, central adrenal insufficiency and hypogonadotropic hypogonadism and is doing well on multiple hormone replacement at age 19 yrs. Child 2, a half-brother to child 1 (same mother), presented at age 1yr with growth failure in 2017 with ht z score -2.06. GH stimulation test with glucagon was abnormal and resulted in a very low GH response (peak GH 0.52 ng/mL). MRI showed EPP with small anterior pituitary gland and interruption of the stalk. Later he was found to have central hypothyroidism and mild central adrenal insufficiency. He is receiving standard hormone replacement at 3 yrs of age. Mother of above 2 patients presented 6 mos postpartum in 2017 after her 7th and last pregnancy with fatigue and amenorrhea. Laboratory evaluation revealed central hypothyroidism (FT4 0.76 ng/dL) and she was prescribed levothyroxine followed by resumption of her menses. She was unable to breastfeed her children due to lack of supply. There were no concerns for DI, amenorrhea or infertility. She was referred to Endocrinology in 2019 for persistent fatigue with a question of GH deficiency. IGF-1 level was normal 114 ng/mL(z score -0.39) and GH stimulation test (clonidine + glucagon) was abnormal with peak GH 1.85 ng/ml. MRI showed EPP with hypoplastic pituitary stalk. Genetic testing was done for CPHD Sequencing Panel at Prevention Genetics which includes GL12, HESX1, LHX3, LHX4, OTX2, POU1F1, PROP1F1, PROP1, SOX2, SOX3 genes and results were negative. She has 4 other children (21, 12, 11, 10yrs) who are currently being investigated for hormone deficiencies. One child died at 3 months of age due to SIDS. Conclusion: We present 3 family members with PSIS. This family highlights the variable clinical phenotype of PSIS and importance of careful family history when evaluating children with congenital pituitary abnormalities and supports the need for more extensive gene panels for evaluation of CPHD. Reference:. Acta Endocrinologica, 2017. 13(1):96–105

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